BACKGROUND Hepatic epithelioid hemangioendothelioma(HEHE)is a rare malignant vascular liver tumor diagnosed by histopathological evaluation.Standardized treatment is challenging because of its rarity;hepatectomy is pr...BACKGROUND Hepatic epithelioid hemangioendothelioma(HEHE)is a rare malignant vascular liver tumor diagnosed by histopathological evaluation.Standardized treatment is challenging because of its rarity;hepatectomy is preferred for solitary lesions and multiple transplantations.There is no consensus on the optimal treatment for HEHE;however,surgical excision is often considered effective.This report presents a case of initially suspected cholangiocarcinoma or renal cell carcinoma(RCC)metastasis,which was later confirmed as HEHE,with no recurrence during follow-up.CASE SUMMARY A 52-year-old man with a history of left nephrectomy for RCC presented with an incidentally detected liver mass and nonspecific abdominal discomfort.Imaging revealed a 3-cm centripetal enhancing lesion in the right hepatic dome with indeterminate malignant potential.The patient underwent a laparoscopic right anterior sectionectomy and remained recurrence-free without complications during the 3-year follow-up period.CONCLUSION Managing HEHE is challenging.Accurate diagnosis and surgical options,such as resection or transplantation,are essential with tailored multidisciplinary followup.The authors have read the CARE Checklist(2016)and the manuscript was prepared and revised according to the CARE Checklist(2016).展开更多
BACKGROUND Hepatic perivascular epithelioid cell tumor(PEComa)is an extremely rare neo-plasm that is often difficult to diagnose due to its nonspecific clinical and radio-logical features.This case emphasizes the impo...BACKGROUND Hepatic perivascular epithelioid cell tumor(PEComa)is an extremely rare neo-plasm that is often difficult to diagnose due to its nonspecific clinical and radio-logical features.This case emphasizes the importance of imaging and histopatho-logical examination in distinguishing PEComa from other liver tumors,and the necessity of early intervention.CASE SUMMARY A 36-year-old woman presented with a liver mass discovered during a routine physical examination.Initially diagnosed as focal nodular hyperplasia based on contrast-enhanced computed tomography,she declined surgical intervention.Two years later,re-examination revealed an approximately 60%increase in the size of the mass and suggested the possibility of hepatocellular carcinoma.She subsequently underwent surgical resection.Postoperative histopathological and immunohistochemical analysis revealed positivity for HMB-45 and Melan-A,confirming the diagnosis of hepatic PEComa.A 13-month follow-up revealed no recurrence or metastasis.CONCLUSION Hepatic PEComa requires a combination of radiological and immunohistoche-mical evaluation for accurate diagnosis.This case highlights the importance of early surgical intervention and regular follow-up to monitor for recurrence or metastasis.展开更多
BACKGROUND Perivascular epithelioid cell tumor(PEComa)of the colorectum is exceedingly rare,with only a few published reports.It presents with a wide spectrum of biological behavior,ranging from benign to malignant.Th...BACKGROUND Perivascular epithelioid cell tumor(PEComa)of the colorectum is exceedingly rare,with only a few published reports.It presents with a wide spectrum of biological behavior,ranging from benign to malignant.The prognosis for malignant PEComa is poor and there is little consensus on its treatment.AIM To fully characterize PEComa and standardize its diagnosis and treatment.METHODS Patients with colorectal malignant PEComa were identified from the First Affiliated Hospital,Zhejiang University School of Medicine and People's Hospital of Anji.Cases with controversial pathology and cases lost to follow-up were excluded,leaving seven remaining cases that formed the basis of the study.We collected relevant clinicopathological,therapeutic and followup details.Disease stage and progression were assessed by contrast-enhanced computed tomography at baseline and at 3-month intervals.RESULTS The mean age was 43 years,with a range of 5 years to 73 years.The average body mass index was 21.8±3.0 kg/m^(2),and 71.4%of cases occurred in the colon.The main symptoms of colorectal PEComas were abdominal mass and hematochezia.The most common microscopic finding of malignant behavior was infiltrative growth.Immunohistochemical analysis found that 6/7 cases were positive for HMB45,5/7 were positive for melan-A,and 3/5 were positive for MiTF.The watch-and-wait approach to treatment was a risky option.Radical resection was preferable to systemic treatment.The median progression-free survival exceeded 38 months,longer than previously reported.CONCLUSION Radical or extended resection is the key to prolonged survival of malignant PEComa.More meaningful studies are urgently needed to establish the standardized diagnosis and treatment.展开更多
BACKGROUND Epithelioid angiosarcoma(EA)is an aggressive,malignant endothelial-cell tumor of vascular or lymphatic origin.EA often arises from deep soft tissues such as pleura,breast,bone and gastrointestinal tract.It ...BACKGROUND Epithelioid angiosarcoma(EA)is an aggressive,malignant endothelial-cell tumor of vascular or lymphatic origin.EA often arises from deep soft tissues such as pleura,breast,bone and gastrointestinal tract.It usually affects patients aged 60-70 years and is associated with high recurrence and metastasis rates with surgical resection as the primary treatment of choice.Overall survivals are generally poor,ranging from 6 to 16 months.More than 50%of patients died of disease within 2 to 3 years of diagnosis.CASE SUMMARY We present a rare case of EA of the cervical spine causing a C6 pathological fracture complicated by severe kyphosis.The patient received C4-7 posterior laminectomy and C2/3/4/7/T1 transpedicular screw fixation,followed by anterior C5-6 corpectomy with allograft bone fusion and cervical plate fixation.Postoperative radiotherapy was administered without delay.However,the patient died of rapidly progressive acute respiratory distress syndrome 3 weeks after the second surgery.CONCLUSION EA with spinal involvement is extremely rare.Early detection and cord decompression may prevent neurological deterioration and preserve better quality of life.展开更多
Perivascular epithelioid cell tumors(PEComas)are a diverse group of mesenchymal neoplasms.While they have been described throughout the genitourinary system,PEComas are quite rare within the bladder.We present the cas...Perivascular epithelioid cell tumors(PEComas)are a diverse group of mesenchymal neoplasms.While they have been described throughout the genitourinary system,PEComas are quite rare within the bladder.We present the case of a 37-year-old male who presented in clot retention and was found to have a bladder PEComa.Staging images seemingly demonstrated solid tumor confinement to the bladder and pelvis.Intraoperative pathology revealed peritoneal metastasis.The patient underwent a pelvic mass excision and partial cystectomy.The patient had plans for adjuvant chemotherapy,but later returned to the hospital and passed away from acute hypoxic respiratory failure.展开更多
To investigate the histological,immunohistochemical,and molecular characteristics of sclerosing epithelioid fibrosarcoma(SEF),a rare intracranial lesion,we analyzed the clinical,pathological,and molecular features of ...To investigate the histological,immunohistochemical,and molecular characteristics of sclerosing epithelioid fibrosarcoma(SEF),a rare intracranial lesion,we analyzed the clinical,pathological,and molecular features of a group of cases involving both intracranial(case 1)and extracranial(cases 2,3,and 4)soft tissue tumors.These tumors were located in the bilateral parietal sinuses(one of four cases;25%),thoracic vertebrae(1 of 4 cases;25%),mediastinum(1 of 4 cases;25%),and parathyroid soft tissue(1 of 4 cases;25%).Microscopically,tumor cells were observed in both sparse and dense areas,arranged in sheets,cords,and a fusiform braided pattern,all within a dense sclerotic matrix.All four patients exhibited strong and diffuse positivity for MUC4.Cases 1 and 2 harbored an EWSR1–CREB3L1 fusion;case 3 had a CPSF6–ITPR2 fusion;and case 4 exhibited multiple fusion genes,including FUS–CREB3L2,KDM5A–ERC1,AKAP8L–BRD4,and ATF2–CHN1.Methylation analysis indicated that all cases clustered within the SEF spectrum.Copy number variation analysis revealed deletions in chromosomal arms 11p and 22q across all cases.The morphologies of intracranial and extracranial SEF were found to be similar.Although MUC4 serves as a reliable marker for diagnosing SEF,its molecular findings remain nonspecific.Methylation clustering and aneuploidy score assessment should be considered as additional tools to assist in diagnosis and prognostic monitoring.展开更多
AIM: To improve the clinical diagnosis and recognition of hepatic epithelioid angiomyolipoma(HEAML).METHODS: Four cases of primary HEAML were confirmed based on the pathology archive system in our hospital from Januar...AIM: To improve the clinical diagnosis and recognition of hepatic epithelioid angiomyolipoma(HEAML).METHODS: Four cases of primary HEAML were confirmed based on the pathology archive system in our hospital from January 2009 to November 2015. The general state, clinical symptoms, imaging manifestations, histological results and immunohistochemistry of these patients were retrospectively reviewed and analyzed. Studies of HEAML published in the last 15 years were collected from Pub Med and MEDLINE to summarize the clinical symptoms, imaging characteristics, pathological features and management of HEAML.RESULTS: Four cases of primary HEAML were retrieved from our archives. These included three female patients and one male patient, with a mean age of 41.8 ± 11.5 years(ranging from 31 to 56 years). The meantumor size was 7.3 ± 5.5 cm(ranging from 3.0 to 15 cm). In the contrast-enhanced imaging, the tumor was obviously enhanced in the arterial phase, but enhanced continuously or exhibited a slow-density masse during the venous and delayed phases. Histologically, the tumors mainly consisted of epithelioid cells that comprised approximately 95% of the total neoplastic mass. Although no metastases occurred in our patients, pathological studies revealed necrosis, mitotic figures and liver invasion in two patients, which indicates aggressive behavior. Immunohistochemical staining revealed that human melanoma black 45(HMB-45) and Melan-A were positive in 4 cases. We only identified 81 cases with primary HEAML, including our present patients, from 26 articles available from Pub Med and MEDLINE. The majority of the papers were published as case reports. Only 5(5/75, 6%) cases were associated with tuberous sclerosis complex(TSC). More than half(35/66) were discovered incidentally upon physical examination. Approximately 65%(22/34) of the patients were misdiagnosed with HCC or other tumors before surgery. Approximately 10%(8/81) of the patients with HEAML had recurrence or metastasis after surgery, which was a very high and alarming rate.CONCLUSION: HEAML is a very rare primary hepatic tumor that is often misdiagnosed before surgery. Patients should be followed closely after surgery because of its malignant potential.展开更多
A 58-year-old man presented with the chief complaint of abdominal bloating and was incidentally found to have a liver tumor.As diagnostic imaging studies could not rule out malignancy,the patient underwent partial res...A 58-year-old man presented with the chief complaint of abdominal bloating and was incidentally found to have a liver tumor.As diagnostic imaging studies could not rule out malignancy,the patient underwent partial resection of segment 3 of the liver.The lesion pathologically showed eosinophilic proliferation,in addition to immunohistochemical positivity for human melanoma black 45 and Melan-A,thereby leading to the diagnosis of a hepatic perivascular epithelioid cell tumor(PEComa).A PEComa arising from the liver is relatively rare.Moreover,the name ‘PEComa' has not yet been widely recognized,and the same disease entity has been called epithelioid angiomyolipoma(EAML),further diminishing the recognition of PEComa.In addition,PEComa imaging findings mimic those of malignant liver tumors,and clinically,this tumor tends to enlarge.Therefore,a PEComa is difficult to diagnose.We conducted a systematic review of PEComa and EAML cases and discuss the results,including findings useful for differentiating perivascular epithelioid cell tumors from malignant liver tumors.展开更多
BACKGROUND: Hepatic epithelioid hemangioendothelioma (EHE) is a rare, low-grade malignant vascular tumor. Although its unusual imaging and pathologic findings are being recognized with increasing frequency, diagnosis ...BACKGROUND: Hepatic epithelioid hemangioendothelioma (EHE) is a rare, low-grade malignant vascular tumor. Although its unusual imaging and pathologic findings are being recognized with increasing frequency, diagnosis is still difficult. This study aimed to analyze the CT and MRI features of hepatic EHE with a pathological study in order to improve the diagnostic accuracy and knowledge of this disease in daily practice. METHODS: Nine patients with hepatic EHE confirmed pathologically underwent plain and dynamic contrast-enhanced multi-detector row CT examination. Of these patients, four underwent additional MRI (plain Ti-weighted imaging (T1WI), T2-weighted imaging (T2WI), and dynamic contrast-enhanced scanning) and one had selective hepatic arteriography. The imaging findings were reviewed retrospectively together with the pathological results. RESULTS: A total of 79 lesions, ranging from 3.0 to 44.6 mm in maximum diameter, with an average of 16.8 +/- 7.1 mm, were found in various segments of the liver. Thirty of the 79 lesions grew adjacent to the hepatic capsule. In the 4 patients receiving MRI, 39 lesions were found with low signal intensity on unenhanced T1WI and intermediate to high signal intensity on T2WI. The 'capsular retraction' sign was found in all the 4 patients. Nine of the 39 lesions showed the 'halo' sign after contrast enhancement on MRI. Of the 79 lesions (hypodense nodules) in the 9 patients shown by unenhanced plain CT, 26 were confluent. Calcification was found in 2 patients and the 'capsular retraction' sign in 7. Thirty-eight of the 79 lesions demonstrated the 'halo' sign after contrast enhancement on CT, and this sign was more clearly demonstrated in the portal venous phase. In one patient, selective hepatic arteriography showed patchy stain in the peripheral liver parenchyma with small vessels around them. Histology in all patients revealed proliferation of abnormal fibrous tissue and vessel-like structures scattered with irregular epithelioid cells having a signet ring-like structure. Immunohistochemically, all patients were positive for CD34, 4 were positive for CD31, and 3 were positive for factor VIII-related antigen. CONCLUSIONS: Hepatic EHE may manifest as solitary or diffuse nodular lesions with a predilection for peripheral subcapsular growth and nodular confluence, together with the 'halo' and 'capsular retraction' signs. These imaging findings can help to improve the diagnostic accuracy of this rare hepatic tumor. (Hepatobiliary Pancreat Dis Int 2010; 9: 154-158)展开更多
AIM: To explore the characteristics of multi-slice computed tomography (CT) manifestations of hepatic epithelioid angiomyolipoma (HEA), improve the rate of accurate diagnosis, and reduce the misdiagnostic rate.
Epithelioid hemangioendothelioma(EH)is a rare tumor arising from the vascular endothelial cells of soft tissue or visceral organs.The most common visceral site is the liver,where it is often involved in a multifocal m...Epithelioid hemangioendothelioma(EH)is a rare tumor arising from the vascular endothelial cells of soft tissue or visceral organs.The most common visceral site is the liver,where it is often involved in a multifocal man-ner known as hepatic EH(HEH).Surgical resection with curative intent represents the gold standard therapy.When surgery is not feasible,or in cases of metastatic disease,no standard medical treatment is currently indicated.In small series,drugs with anti-angiogenic activity(such as bevacizumab,sorafenib,thalidomide,and lenalidomide)have been proposed with promising results.We describe a 73-year-old man with multifocal non-resectable HEH treated with lenalidomide.Disease status was evaluated by abdominal ultrasound and magnetic resonance every four months.The patient was treated for a total of 39 mo with prolonged disease stabilization and,at the time of writing,is still under treatment with a good tolerance profile.During a short period of treatment discontinuation,the disease showed slight progression that immediately resolved after the reintroduction of lenalidomide.Lenalidomide may represent a valid treatment option for HEH due to its anti-angiogenic and antineoplastic activities.This preliminary result merits further study in a large series.展开更多
Background: Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor of vascular origin with an unknown etiology, a low incidence, and a variable natural course. We evaluated the management and prognosis of HEH ...Background: Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor of vascular origin with an unknown etiology, a low incidence, and a variable natural course. We evaluated the management and prognosis of HEH from the Surveillance, Epidemiology and End Results (SEER) program and changes in treatment modalities of HEH over 30 years. Methods: From 1973 to 2014 in the SEER database, we selected patients diagnosed with HEH. We analyzed the clinical characteristics, patterns of management, and clinical outcomes of patients with HEH. Results: We identi ed 79 patients with HEH (median age: 54.0 years;male to female ratio: 1:2.6). The initial extent of disease was local in 22 (27.8%) patients, regional metastasis in 22 (27.8%), distant metas-tasis in 31 (39.2%) and unknown in 4 (5.1%). The median size of primary tumor was 3.85 cm (interquartile range, 2.50 7.93 cm). Among 74 patients with available management data, the most common manage-ment was no treatment (29/74, 39.2%), followed by chemotherapy only (22/74, 29.7%), liver resection-based (13/74, 17.6%), and transplantation-based therapy (6/74, 8.1%). The 5-year cancer-speci c survival rate was 57.8%. Patients who underwent surgical treatment had signi cantly higher survival than those who underwent non-surgical treatment (5-year survival;88% vs. 49%, P=0.019). Multivariate analysis revealed that surgical therapy was the only independent prognostic factor for survival (hazard ratio: 0.20, P=0.040). Conclusions: Resection or liver transplantation is worth considering for treatment of patients with HEH.展开更多
AIM： To retrospectively evaluate the computed tomography （CT）/magnetic resonance imaging （MRI） imaging features of epithelioid angiomyolipoma of the liver （Epi-HAML）, with pathology as a reference. METHODS： Th...AIM： To retrospectively evaluate the computed tomography （CT）/magnetic resonance imaging （MRI） imaging features of epithelioid angiomyolipoma of the liver （Epi-HAML）, with pathology as a reference. METHODS： The CT/MRI findings （number, diameter, lobar location, and appearance of lesions） in a series of 10 patients with 12 pathologically proven epithelioid angiomyolipomas of the liver were retrospectively analyzed. The imaging features, including attenuation/ signal intensity characteristics, presence of fat, hypervascular, outer rim, and vessels within lesion, were evaluated and compared with that of non-Epi- HAML in 11 patients （13 lesions）. The Fisher exact test was used to compare difference in probability of imaging features between the two types. RESULTS： For 21 patients, CT images of 15 patients and MR images of six patients were available. No patient underwent two examinations. For the 15 patients with a CT scan, all HAML lesions in the two groups （10 Epi-HAML and seven non-Epi-HAML） manifested as hypoattenuation. For the six patients with MRI, all lesions （two Epi-HAML and six non-Epi- HAML） were hypointense on TlWI （fat suppression） and hyperintense on T2WI. There were 10 non-Epi-HAML, but only two Epi-HAML lesions showed the presence of fat, which significantly different between the two types （P = 0.005）. On the dynamic contrast enhancement （DCE） imaging, eight Epi-HAML, and 13 non-Epi lesions manifested as hypervascular. Punctate or curved vessels were displayed in 10 Epi-HAML as well as in nine non- Epi lesions and outer rim enhancement could be found with eight Epi-HAML as well as six non-Epi lesions. CONCLUSION： Little or no presence of adipose tissue was found to be an imaging feature of Epi- HAML, compared with the non-Epi type. In addition, hypervascularity with opacification of central punctiform or filiform vessels on DCE would be a characteristic enhancement pattern for Epi-HAML.展开更多
BACKGROUND Hepatic epithelioid angiomyolipoma(HEAML) is a rare liver disease and is easily misdiagnosed. Enhanced recognition of HEAML is beneficial to the differential diagnosis of rare liver diseases.CASE SUMMARY We...BACKGROUND Hepatic epithelioid angiomyolipoma(HEAML) is a rare liver disease and is easily misdiagnosed. Enhanced recognition of HEAML is beneficial to the differential diagnosis of rare liver diseases.CASE SUMMARY We presented two cases of HEAML in Changzheng Hospital, Naval Medical University, and then collected and analyzed all reports about HEAML recorded in PubMed, MEDLINE, China Science Periodical Database, and VIP database from January 2000 to March 2018. A total of 409 cases of HEAML in 97 reports were collected, with a ratio of men to women of 1:4.84 and an age range from 12 years to 80 years(median 44 years). Among the patients with clinical symptoms mentioned, 61.93%(205/331) were asymptomatic, 34.74%(115/331) showed upper or right upper quadrant abdomen discomfort, while a few of them showed abdominal mass, gastrointestinal symptoms, low fever, or weight loss. The misdiagnosis rate of HEAML was as high as 40.34%(165/409) due to its nonspecific imaging findings. Most of the tumors were solitary and round in morphology, with clear boundaries. Ultrasound scan indicated low echo with internal nonuniformity and rich blood supply in most cases. Computer tomography/magnetic resonance imaging enhanced scan showed varied characteristics. The ratio of fast wash-in and fast wash-out, fast wash-in and slow wash-out, and delayed enhancement was roughly 4:5:1. A definite diagnosis of HEAML depended on the pathological findings of the epithelioid cells in lesions and the expression of human melanoma black 45, smooth muscle actin,melanoma antigen, and actin by immunohistochemical staining. HEAML had a relatively low malignant rate of 3.91%. However, surgical resection was the main treatment for HEAML, due to the difficulty diagnosing before operation.CONCLUSION HEAML is a rare and easily misdiagnosed disease, and it should be diagnosed carefully, taking into account clinical course, imaging, pathological,and immunohistochemical findings.展开更多
AIM： To investigate and review the contrast-enhanced multiple-phase computed tomography （CEMP CT） and magnetic resonance imaging （MRI） findings in patients with pathologically confirmed hepatic epithelioid hemang...AIM： To investigate and review the contrast-enhanced multiple-phase computed tomography （CEMP CT） and magnetic resonance imaging （MRI） findings in patients with pathologically confirmed hepatic epithelioid hemangioendothelioma （HEHE）. METHODS： Findings from imaging examinations in 8 patients （5 women and 3 men） with pathologically confirmed HEHE were retrospectively reviewed （CT images obtained from 7 patients and MR images obtained from 6 patients）. The age of presentation varied from 27 years to 60 years （average age 39.8 years）. RESULTS： There were two types of HEHE： multifocal type （n = 7） and diffuse type （n = 1）. Tn the multifocal-type cases, there were 74 lesions on CT and 28 lesions on MRI with 7 lesions found with diffusion weighted imaging; 18 （24.3%） of 74 lesions on plain CT and 26 （92.9%） of 28 lesions on pre-contrast MRI showed the target sign. On CEMP CT, 28 （37.8%） of 74 lesions appeared with the target sign and a progressive-enhancement rim and 9 （12.2%） of 74 lesions displayed progressive enhancement, maintaining a state of persistent enhancement. On CEMP MRI, 27 （96.4%） of 28 lesions appeared with the target sign with a progressive-enhancement rim and 28 （100%） of 28 lesions displayed progressive-enhancement, maintaining a state of persistent enhancement. In the diffuse-type cases, an enlarged liver was observed with a large nodule appearing with persistent enhancement on CEMP CT and MRI. CONCLUSION： The most important imaging features of HEHE are the target sign and/or progressive en- hancement with persistent enhancement on CEMP CT and MRI. MRI is advantageous over CT in displaying these imaging features.展开更多
With an estimated incidence of only 1-2 cases in every 1 million people,hepatic epithelioid hemangioendothelioma(HEHE)is a rare vascular endothelial cell tumor occurring in the liver and consisting of epithelioid and ...With an estimated incidence of only 1-2 cases in every 1 million people,hepatic epithelioid hemangioendothelioma(HEHE)is a rare vascular endothelial cell tumor occurring in the liver and consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix.HEHE is characterized as a low-to-moderate grade malignant tumor and is classified into three types:solitary,multiple,and diffuse.Both the etiology and characteristic clinical manifestations of HEHE are unclear.However,HEHE has a characteristic appearance on imaging including ultrasound,magnetic resonance imaging,and positron emission tomography/computerized tomography.Still,its diagnosis depends mainly on pathological findings,with immunohistochemical detection of endothelial markers cluster of differentiation 31(CD31),CD34,CD10,vimentin,and factor VIII antigen as the basis of diagnosis.Hepatectomy and/or liver transplantation are the first choice for treatment,but various chemotherapeutic drugs are reportedly effective,providing a promising treatment option.In this review,we summarize the literature related to the diagnosis and treatment of HEHE,which provides future perspectives for the clinical management of HEHE.展开更多
Hepatic epithelioid hemangioendothelioma(HEHE) is a rare category of vascular tumor with uncertain malignant potential. It commonly presents nonspecific and variable clinical manifestations, ranging from asymptomatic ...Hepatic epithelioid hemangioendothelioma(HEHE) is a rare category of vascular tumor with uncertain malignant potential. It commonly presents nonspecific and variable clinical manifestations, ranging from asymptomatic to hepatic failure. In addition, laboratory measurements and imaging features also lack specificity in the diagnosis of HEHE. The aim of the present study is to highlight the dilemma and challenges in the preoperative diagnosis of HEHE, and to enhance awareness of the range of hepatobiliary surgery available in patients with multiple hepatic nodular lesions on imaging. In these patients, HEHE should at least be considered in the differential diagnosis.展开更多
BACKGROUND Perivascular epithelioid cell tumor(PEComa)is an uncommon tumor of mesenchymal origin.Cases of PEComa in the liver are extremely rare.AIM To analyze the clinicopathological features and treatment of hepatic...BACKGROUND Perivascular epithelioid cell tumor(PEComa)is an uncommon tumor of mesenchymal origin.Cases of PEComa in the liver are extremely rare.AIM To analyze the clinicopathological features and treatment of hepatic PEComa and to evaluate the prognosis after different treatments.METHODS Clinical and pathological data of 26 patients with hepatic PEComa were collected.All cases were analyzed by immunohistochemistry and clinical follow-up.RESULTS This study included 17 females and 9 males,with a median age of 50 years.Lesions were located in the left hepatic lobe in 13 cases,in the right lobe in 11,and in the caudate lobe in 2.The median tumor diameter was 6.5 cm.Light microscopy revealed that the tumor cells were mainly composed of epithelioid cells.The cytoplasm contained heterogeneous eosinophilic granules.There were thick-walled blood vessels,around which tumor cells were radially arranged.Immunohistochemical analysis of pigment-derived and myogenic markers in PEComas revealed that 25 cases were HMB45(+),23 were Melan-A(+),and 22 SMA(+).TFE3 and Desmin were negative in all cases.All the fluorescence in situ hybridization samples were negative for TFE3 gene break-apart probe.Tumor tissues were collected by extended hepatic lobe resection or simple hepatic tumor resection as the main treatments.Median follow-up was 62.5 mo.None of the patients had metastasis or recurrence,and there were no deaths due to the disease.CONCLUSION Hepatic PEComa highly expresses melanin and smooth muscle markers,and generally exhibits an inert biological behavior.The prognosis after extended hepatic lobe resection and simple hepatic tumor resection is semblable.展开更多
BACKGROUND The perivascular epithelioid cell tumour(PEComa)family of tumours mainly includes renal and hepatic angiomyolipomas,pulmonary lymphangioleiomyomatosis and clear cell“sugar”tumour of the lung.Several uncom...BACKGROUND The perivascular epithelioid cell tumour(PEComa)family of tumours mainly includes renal and hepatic angiomyolipomas,pulmonary lymphangioleiomyomatosis and clear cell“sugar”tumour of the lung.Several uncommon tumours with similar morphological and immunophenotypical characteristics arising at a variety of sites(abdominal cavity,digestive tract,retroperitoneum,skin,soft tissue and bones)are also included in the PEComa family and are referred to as PEComas not otherwise specified.CASE SUMMARY We present a 37-year-old female patient who underwent resection of an 8.5 cm×8 cm×4 cm retroperitoneal tumour,which eventually was diagnosed as PEComa of uncertain biological behaviour.Three years after the operation,the patient remains without any evidence of recurrence.A search was performed in the Medline and EMBASE databases for articles published between 1996 and 2018,and we identified 31 articles related to retroperitoneal and perinephric PEComas.We focused on sex,age,maximum dimension,histological and immunohistochemical characteristics of the tumour,follow-up and long-term outcome.Thirty-four retroperitoneal(including the present one)and ten perinephric PEComas were identified,carrying a malignant potential rate of 44%and 60%,respectively.Nearly half of the potentially malignant PEComas presented with or developed metastases during the course of the disease.CONCLUSION Retroperitoneal PEComas are not as indolent as they are supposed to be.Radical surgical resection constitutes the treatment of choice for localized disease,while mammalian target of the rapamycin(mTOR)inhibitors constitute the most promising therapy for disseminated disease.The role of mTOR inhibitors as adjuvant or neoadjuvant therapies needs to be evaluated in the future.展开更多
Perivascular epithelioid cell tumor （PEComa） is a rare, soft tissue tumor that can occur in various locations. The present report included three patients （one male and two females; age range, 25-51 years） with hep...Perivascular epithelioid cell tumor （PEComa） is a rare, soft tissue tumor that can occur in various locations. The present report included three patients （one male and two females; age range, 25-51 years） with hepatic PEComas. The collected data included the clinical manifestations, diagnosis, management, treatment, and prognosis. Since it is difficult to diagnose hepatic PEComas by imaging, the patients were diagnosed by tumor tissue examination such as immunohistochemistry, which was positive for HMB-45, Melan-A, and SMA on all slides. The tumor was composed of diverse tissues including smooth muscle, adipose tissue, and thick-walled blood vessels. During the follow-up period, one of the tumors was malignant （double-positive for CD34 and Ki-67） and recurred 3 months after surgery. In addition, malignant hepatic PECo- mas were reviewed in the literature, indicating that the majority of hepatic PEComas are benign, but few hepatic PEComas exhibit malignant behaviors in older female patients （〉50 years of age） with abdominal discomfort and pain, larger tumor size （〉10 cm）, or positive staining for CD34 and Ki-67. In conclusion, there is no effective method to diagnose PEComas. Currently, the diagnosis of PEComas depends on immunohistochemical staining. Tumor resection and close follow-up are the principal methods for the management of PEComas.展开更多
文摘BACKGROUND Hepatic epithelioid hemangioendothelioma(HEHE)is a rare malignant vascular liver tumor diagnosed by histopathological evaluation.Standardized treatment is challenging because of its rarity;hepatectomy is preferred for solitary lesions and multiple transplantations.There is no consensus on the optimal treatment for HEHE;however,surgical excision is often considered effective.This report presents a case of initially suspected cholangiocarcinoma or renal cell carcinoma(RCC)metastasis,which was later confirmed as HEHE,with no recurrence during follow-up.CASE SUMMARY A 52-year-old man with a history of left nephrectomy for RCC presented with an incidentally detected liver mass and nonspecific abdominal discomfort.Imaging revealed a 3-cm centripetal enhancing lesion in the right hepatic dome with indeterminate malignant potential.The patient underwent a laparoscopic right anterior sectionectomy and remained recurrence-free without complications during the 3-year follow-up period.CONCLUSION Managing HEHE is challenging.Accurate diagnosis and surgical options,such as resection or transplantation,are essential with tailored multidisciplinary followup.The authors have read the CARE Checklist(2016)and the manuscript was prepared and revised according to the CARE Checklist(2016).
基金Supported by National Natural Science Foundation of China,No.U22A2039.
文摘BACKGROUND Hepatic perivascular epithelioid cell tumor(PEComa)is an extremely rare neo-plasm that is often difficult to diagnose due to its nonspecific clinical and radio-logical features.This case emphasizes the importance of imaging and histopatho-logical examination in distinguishing PEComa from other liver tumors,and the necessity of early intervention.CASE SUMMARY A 36-year-old woman presented with a liver mass discovered during a routine physical examination.Initially diagnosed as focal nodular hyperplasia based on contrast-enhanced computed tomography,she declined surgical intervention.Two years later,re-examination revealed an approximately 60%increase in the size of the mass and suggested the possibility of hepatocellular carcinoma.She subsequently underwent surgical resection.Postoperative histopathological and immunohistochemical analysis revealed positivity for HMB-45 and Melan-A,confirming the diagnosis of hepatic PEComa.A 13-month follow-up revealed no recurrence or metastasis.CONCLUSION Hepatic PEComa requires a combination of radiological and immunohistoche-mical evaluation for accurate diagnosis.This case highlights the importance of early surgical intervention and regular follow-up to monitor for recurrence or metastasis.
文摘BACKGROUND Perivascular epithelioid cell tumor(PEComa)of the colorectum is exceedingly rare,with only a few published reports.It presents with a wide spectrum of biological behavior,ranging from benign to malignant.The prognosis for malignant PEComa is poor and there is little consensus on its treatment.AIM To fully characterize PEComa and standardize its diagnosis and treatment.METHODS Patients with colorectal malignant PEComa were identified from the First Affiliated Hospital,Zhejiang University School of Medicine and People's Hospital of Anji.Cases with controversial pathology and cases lost to follow-up were excluded,leaving seven remaining cases that formed the basis of the study.We collected relevant clinicopathological,therapeutic and followup details.Disease stage and progression were assessed by contrast-enhanced computed tomography at baseline and at 3-month intervals.RESULTS The mean age was 43 years,with a range of 5 years to 73 years.The average body mass index was 21.8±3.0 kg/m^(2),and 71.4%of cases occurred in the colon.The main symptoms of colorectal PEComas were abdominal mass and hematochezia.The most common microscopic finding of malignant behavior was infiltrative growth.Immunohistochemical analysis found that 6/7 cases were positive for HMB45,5/7 were positive for melan-A,and 3/5 were positive for MiTF.The watch-and-wait approach to treatment was a risky option.Radical resection was preferable to systemic treatment.The median progression-free survival exceeded 38 months,longer than previously reported.CONCLUSION Radical or extended resection is the key to prolonged survival of malignant PEComa.More meaningful studies are urgently needed to establish the standardized diagnosis and treatment.
文摘BACKGROUND Epithelioid angiosarcoma(EA)is an aggressive,malignant endothelial-cell tumor of vascular or lymphatic origin.EA often arises from deep soft tissues such as pleura,breast,bone and gastrointestinal tract.It usually affects patients aged 60-70 years and is associated with high recurrence and metastasis rates with surgical resection as the primary treatment of choice.Overall survivals are generally poor,ranging from 6 to 16 months.More than 50%of patients died of disease within 2 to 3 years of diagnosis.CASE SUMMARY We present a rare case of EA of the cervical spine causing a C6 pathological fracture complicated by severe kyphosis.The patient received C4-7 posterior laminectomy and C2/3/4/7/T1 transpedicular screw fixation,followed by anterior C5-6 corpectomy with allograft bone fusion and cervical plate fixation.Postoperative radiotherapy was administered without delay.However,the patient died of rapidly progressive acute respiratory distress syndrome 3 weeks after the second surgery.CONCLUSION EA with spinal involvement is extremely rare.Early detection and cord decompression may prevent neurological deterioration and preserve better quality of life.
文摘Perivascular epithelioid cell tumors(PEComas)are a diverse group of mesenchymal neoplasms.While they have been described throughout the genitourinary system,PEComas are quite rare within the bladder.We present the case of a 37-year-old male who presented in clot retention and was found to have a bladder PEComa.Staging images seemingly demonstrated solid tumor confinement to the bladder and pelvis.Intraoperative pathology revealed peritoneal metastasis.The patient underwent a pelvic mass excision and partial cystectomy.The patient had plans for adjuvant chemotherapy,but later returned to the hospital and passed away from acute hypoxic respiratory failure.
基金supported by grants from the National Natural Science Foundation of China(No.82273082)Chongqing Technical Innovation and Application Development Key Project(No.cstc2021jscx-cylhX0005)。
文摘To investigate the histological,immunohistochemical,and molecular characteristics of sclerosing epithelioid fibrosarcoma(SEF),a rare intracranial lesion,we analyzed the clinical,pathological,and molecular features of a group of cases involving both intracranial(case 1)and extracranial(cases 2,3,and 4)soft tissue tumors.These tumors were located in the bilateral parietal sinuses(one of four cases;25%),thoracic vertebrae(1 of 4 cases;25%),mediastinum(1 of 4 cases;25%),and parathyroid soft tissue(1 of 4 cases;25%).Microscopically,tumor cells were observed in both sparse and dense areas,arranged in sheets,cords,and a fusiform braided pattern,all within a dense sclerotic matrix.All four patients exhibited strong and diffuse positivity for MUC4.Cases 1 and 2 harbored an EWSR1–CREB3L1 fusion;case 3 had a CPSF6–ITPR2 fusion;and case 4 exhibited multiple fusion genes,including FUS–CREB3L2,KDM5A–ERC1,AKAP8L–BRD4,and ATF2–CHN1.Methylation analysis indicated that all cases clustered within the SEF spectrum.Copy number variation analysis revealed deletions in chromosomal arms 11p and 22q across all cases.The morphologies of intracranial and extracranial SEF were found to be similar.Although MUC4 serves as a reliable marker for diagnosing SEF,its molecular findings remain nonspecific.Methylation clustering and aneuploidy score assessment should be considered as additional tools to assist in diagnosis and prognostic monitoring.
文摘AIM: To improve the clinical diagnosis and recognition of hepatic epithelioid angiomyolipoma(HEAML).METHODS: Four cases of primary HEAML were confirmed based on the pathology archive system in our hospital from January 2009 to November 2015. The general state, clinical symptoms, imaging manifestations, histological results and immunohistochemistry of these patients were retrospectively reviewed and analyzed. Studies of HEAML published in the last 15 years were collected from Pub Med and MEDLINE to summarize the clinical symptoms, imaging characteristics, pathological features and management of HEAML.RESULTS: Four cases of primary HEAML were retrieved from our archives. These included three female patients and one male patient, with a mean age of 41.8 ± 11.5 years(ranging from 31 to 56 years). The meantumor size was 7.3 ± 5.5 cm(ranging from 3.0 to 15 cm). In the contrast-enhanced imaging, the tumor was obviously enhanced in the arterial phase, but enhanced continuously or exhibited a slow-density masse during the venous and delayed phases. Histologically, the tumors mainly consisted of epithelioid cells that comprised approximately 95% of the total neoplastic mass. Although no metastases occurred in our patients, pathological studies revealed necrosis, mitotic figures and liver invasion in two patients, which indicates aggressive behavior. Immunohistochemical staining revealed that human melanoma black 45(HMB-45) and Melan-A were positive in 4 cases. We only identified 81 cases with primary HEAML, including our present patients, from 26 articles available from Pub Med and MEDLINE. The majority of the papers were published as case reports. Only 5(5/75, 6%) cases were associated with tuberous sclerosis complex(TSC). More than half(35/66) were discovered incidentally upon physical examination. Approximately 65%(22/34) of the patients were misdiagnosed with HCC or other tumors before surgery. Approximately 10%(8/81) of the patients with HEAML had recurrence or metastasis after surgery, which was a very high and alarming rate.CONCLUSION: HEAML is a very rare primary hepatic tumor that is often misdiagnosed before surgery. Patients should be followed closely after surgery because of its malignant potential.
文摘A 58-year-old man presented with the chief complaint of abdominal bloating and was incidentally found to have a liver tumor.As diagnostic imaging studies could not rule out malignancy,the patient underwent partial resection of segment 3 of the liver.The lesion pathologically showed eosinophilic proliferation,in addition to immunohistochemical positivity for human melanoma black 45 and Melan-A,thereby leading to the diagnosis of a hepatic perivascular epithelioid cell tumor(PEComa).A PEComa arising from the liver is relatively rare.Moreover,the name ‘PEComa' has not yet been widely recognized,and the same disease entity has been called epithelioid angiomyolipoma(EAML),further diminishing the recognition of PEComa.In addition,PEComa imaging findings mimic those of malignant liver tumors,and clinically,this tumor tends to enlarge.Therefore,a PEComa is difficult to diagnose.We conducted a systematic review of PEComa and EAML cases and discuss the results,including findings useful for differentiating perivascular epithelioid cell tumors from malignant liver tumors.
文摘BACKGROUND: Hepatic epithelioid hemangioendothelioma (EHE) is a rare, low-grade malignant vascular tumor. Although its unusual imaging and pathologic findings are being recognized with increasing frequency, diagnosis is still difficult. This study aimed to analyze the CT and MRI features of hepatic EHE with a pathological study in order to improve the diagnostic accuracy and knowledge of this disease in daily practice. METHODS: Nine patients with hepatic EHE confirmed pathologically underwent plain and dynamic contrast-enhanced multi-detector row CT examination. Of these patients, four underwent additional MRI (plain Ti-weighted imaging (T1WI), T2-weighted imaging (T2WI), and dynamic contrast-enhanced scanning) and one had selective hepatic arteriography. The imaging findings were reviewed retrospectively together with the pathological results. RESULTS: A total of 79 lesions, ranging from 3.0 to 44.6 mm in maximum diameter, with an average of 16.8 +/- 7.1 mm, were found in various segments of the liver. Thirty of the 79 lesions grew adjacent to the hepatic capsule. In the 4 patients receiving MRI, 39 lesions were found with low signal intensity on unenhanced T1WI and intermediate to high signal intensity on T2WI. The 'capsular retraction' sign was found in all the 4 patients. Nine of the 39 lesions showed the 'halo' sign after contrast enhancement on MRI. Of the 79 lesions (hypodense nodules) in the 9 patients shown by unenhanced plain CT, 26 were confluent. Calcification was found in 2 patients and the 'capsular retraction' sign in 7. Thirty-eight of the 79 lesions demonstrated the 'halo' sign after contrast enhancement on CT, and this sign was more clearly demonstrated in the portal venous phase. In one patient, selective hepatic arteriography showed patchy stain in the peripheral liver parenchyma with small vessels around them. Histology in all patients revealed proliferation of abnormal fibrous tissue and vessel-like structures scattered with irregular epithelioid cells having a signet ring-like structure. Immunohistochemically, all patients were positive for CD34, 4 were positive for CD31, and 3 were positive for factor VIII-related antigen. CONCLUSIONS: Hepatic EHE may manifest as solitary or diffuse nodular lesions with a predilection for peripheral subcapsular growth and nodular confluence, together with the 'halo' and 'capsular retraction' signs. These imaging findings can help to improve the diagnostic accuracy of this rare hepatic tumor. (Hepatobiliary Pancreat Dis Int 2010; 9: 154-158)
基金Supported by Jilin Provincial Science and Technology Department,No.201015158 and No.20110922Jilin Provincial Administration of Traditional Chinese Medicine,No.2011-JS20
文摘AIM: To explore the characteristics of multi-slice computed tomography (CT) manifestations of hepatic epithelioid angiomyolipoma (HEA), improve the rate of accurate diagnosis, and reduce the misdiagnostic rate.
文摘Epithelioid hemangioendothelioma(EH)is a rare tumor arising from the vascular endothelial cells of soft tissue or visceral organs.The most common visceral site is the liver,where it is often involved in a multifocal man-ner known as hepatic EH(HEH).Surgical resection with curative intent represents the gold standard therapy.When surgery is not feasible,or in cases of metastatic disease,no standard medical treatment is currently indicated.In small series,drugs with anti-angiogenic activity(such as bevacizumab,sorafenib,thalidomide,and lenalidomide)have been proposed with promising results.We describe a 73-year-old man with multifocal non-resectable HEH treated with lenalidomide.Disease status was evaluated by abdominal ultrasound and magnetic resonance every four months.The patient was treated for a total of 39 mo with prolonged disease stabilization and,at the time of writing,is still under treatment with a good tolerance profile.During a short period of treatment discontinuation,the disease showed slight progression that immediately resolved after the reintroduction of lenalidomide.Lenalidomide may represent a valid treatment option for HEH due to its anti-angiogenic and antineoplastic activities.This preliminary result merits further study in a large series.
基金supported by the Bio&Medical Tech-nology Development Program of the National Research Foun-dation(NRF)funded by the Korean government(MSIT)(NRF-2018M3A9E8023861)by a grant from the Korean Health R&D Project.Ministry of Health Welfare,Korea(HI18C0531)
文摘Background: Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor of vascular origin with an unknown etiology, a low incidence, and a variable natural course. We evaluated the management and prognosis of HEH from the Surveillance, Epidemiology and End Results (SEER) program and changes in treatment modalities of HEH over 30 years. Methods: From 1973 to 2014 in the SEER database, we selected patients diagnosed with HEH. We analyzed the clinical characteristics, patterns of management, and clinical outcomes of patients with HEH. Results: We identi ed 79 patients with HEH (median age: 54.0 years;male to female ratio: 1:2.6). The initial extent of disease was local in 22 (27.8%) patients, regional metastasis in 22 (27.8%), distant metas-tasis in 31 (39.2%) and unknown in 4 (5.1%). The median size of primary tumor was 3.85 cm (interquartile range, 2.50 7.93 cm). Among 74 patients with available management data, the most common manage-ment was no treatment (29/74, 39.2%), followed by chemotherapy only (22/74, 29.7%), liver resection-based (13/74, 17.6%), and transplantation-based therapy (6/74, 8.1%). The 5-year cancer-speci c survival rate was 57.8%. Patients who underwent surgical treatment had signi cantly higher survival than those who underwent non-surgical treatment (5-year survival;88% vs. 49%, P=0.019). Multivariate analysis revealed that surgical therapy was the only independent prognostic factor for survival (hazard ratio: 0.20, P=0.040). Conclusions: Resection or liver transplantation is worth considering for treatment of patients with HEH.
文摘AIM： To retrospectively evaluate the computed tomography （CT）/magnetic resonance imaging （MRI） imaging features of epithelioid angiomyolipoma of the liver （Epi-HAML）, with pathology as a reference. METHODS： The CT/MRI findings （number, diameter, lobar location, and appearance of lesions） in a series of 10 patients with 12 pathologically proven epithelioid angiomyolipomas of the liver were retrospectively analyzed. The imaging features, including attenuation/ signal intensity characteristics, presence of fat, hypervascular, outer rim, and vessels within lesion, were evaluated and compared with that of non-Epi- HAML in 11 patients （13 lesions）. The Fisher exact test was used to compare difference in probability of imaging features between the two types. RESULTS： For 21 patients, CT images of 15 patients and MR images of six patients were available. No patient underwent two examinations. For the 15 patients with a CT scan, all HAML lesions in the two groups （10 Epi-HAML and seven non-Epi-HAML） manifested as hypoattenuation. For the six patients with MRI, all lesions （two Epi-HAML and six non-Epi- HAML） were hypointense on TlWI （fat suppression） and hyperintense on T2WI. There were 10 non-Epi-HAML, but only two Epi-HAML lesions showed the presence of fat, which significantly different between the two types （P = 0.005）. On the dynamic contrast enhancement （DCE） imaging, eight Epi-HAML, and 13 non-Epi lesions manifested as hypervascular. Punctate or curved vessels were displayed in 10 Epi-HAML as well as in nine non- Epi lesions and outer rim enhancement could be found with eight Epi-HAML as well as six non-Epi lesions. CONCLUSION： Little or no presence of adipose tissue was found to be an imaging feature of Epi- HAML, compared with the non-Epi type. In addition, hypervascularity with opacification of central punctiform or filiform vessels on DCE would be a characteristic enhancement pattern for Epi-HAML.
基金Supported by the National Science Foundation of China under Grant numbers,No.81702923Outstanding Postgraduate Seedling Cultivation Fund of Naval Medical University
文摘BACKGROUND Hepatic epithelioid angiomyolipoma(HEAML) is a rare liver disease and is easily misdiagnosed. Enhanced recognition of HEAML is beneficial to the differential diagnosis of rare liver diseases.CASE SUMMARY We presented two cases of HEAML in Changzheng Hospital, Naval Medical University, and then collected and analyzed all reports about HEAML recorded in PubMed, MEDLINE, China Science Periodical Database, and VIP database from January 2000 to March 2018. A total of 409 cases of HEAML in 97 reports were collected, with a ratio of men to women of 1:4.84 and an age range from 12 years to 80 years(median 44 years). Among the patients with clinical symptoms mentioned, 61.93%(205/331) were asymptomatic, 34.74%(115/331) showed upper or right upper quadrant abdomen discomfort, while a few of them showed abdominal mass, gastrointestinal symptoms, low fever, or weight loss. The misdiagnosis rate of HEAML was as high as 40.34%(165/409) due to its nonspecific imaging findings. Most of the tumors were solitary and round in morphology, with clear boundaries. Ultrasound scan indicated low echo with internal nonuniformity and rich blood supply in most cases. Computer tomography/magnetic resonance imaging enhanced scan showed varied characteristics. The ratio of fast wash-in and fast wash-out, fast wash-in and slow wash-out, and delayed enhancement was roughly 4:5:1. A definite diagnosis of HEAML depended on the pathological findings of the epithelioid cells in lesions and the expression of human melanoma black 45, smooth muscle actin,melanoma antigen, and actin by immunohistochemical staining. HEAML had a relatively low malignant rate of 3.91%. However, surgical resection was the main treatment for HEAML, due to the difficulty diagnosing before operation.CONCLUSION HEAML is a rare and easily misdiagnosed disease, and it should be diagnosed carefully, taking into account clinical course, imaging, pathological,and immunohistochemical findings.
文摘AIM： To investigate and review the contrast-enhanced multiple-phase computed tomography （CEMP CT） and magnetic resonance imaging （MRI） findings in patients with pathologically confirmed hepatic epithelioid hemangioendothelioma （HEHE）. METHODS： Findings from imaging examinations in 8 patients （5 women and 3 men） with pathologically confirmed HEHE were retrospectively reviewed （CT images obtained from 7 patients and MR images obtained from 6 patients）. The age of presentation varied from 27 years to 60 years （average age 39.8 years）. RESULTS： There were two types of HEHE： multifocal type （n = 7） and diffuse type （n = 1）. Tn the multifocal-type cases, there were 74 lesions on CT and 28 lesions on MRI with 7 lesions found with diffusion weighted imaging; 18 （24.3%） of 74 lesions on plain CT and 26 （92.9%） of 28 lesions on pre-contrast MRI showed the target sign. On CEMP CT, 28 （37.8%） of 74 lesions appeared with the target sign and a progressive-enhancement rim and 9 （12.2%） of 74 lesions displayed progressive enhancement, maintaining a state of persistent enhancement. On CEMP MRI, 27 （96.4%） of 28 lesions appeared with the target sign with a progressive-enhancement rim and 28 （100%） of 28 lesions displayed progressive-enhancement, maintaining a state of persistent enhancement. In the diffuse-type cases, an enlarged liver was observed with a large nodule appearing with persistent enhancement on CEMP CT and MRI. CONCLUSION： The most important imaging features of HEHE are the target sign and/or progressive en- hancement with persistent enhancement on CEMP CT and MRI. MRI is advantageous over CT in displaying these imaging features.
基金Supported by the Projects of Department of Science and Technology of Jilin Province,No.20180622004JC.
文摘With an estimated incidence of only 1-2 cases in every 1 million people,hepatic epithelioid hemangioendothelioma(HEHE)is a rare vascular endothelial cell tumor occurring in the liver and consisting of epithelioid and histiocyte-like vascular endothelial cells in mucus or a fibrotic matrix.HEHE is characterized as a low-to-moderate grade malignant tumor and is classified into three types:solitary,multiple,and diffuse.Both the etiology and characteristic clinical manifestations of HEHE are unclear.However,HEHE has a characteristic appearance on imaging including ultrasound,magnetic resonance imaging,and positron emission tomography/computerized tomography.Still,its diagnosis depends mainly on pathological findings,with immunohistochemical detection of endothelial markers cluster of differentiation 31(CD31),CD34,CD10,vimentin,and factor VIII antigen as the basis of diagnosis.Hepatectomy and/or liver transplantation are the first choice for treatment,but various chemotherapeutic drugs are reportedly effective,providing a promising treatment option.In this review,we summarize the literature related to the diagnosis and treatment of HEHE,which provides future perspectives for the clinical management of HEHE.
基金Supported by National Nature Science of China,No.30801111Science and Technology Support Project of Sichuan Province,No.2014SZ0002-10
文摘Hepatic epithelioid hemangioendothelioma(HEHE) is a rare category of vascular tumor with uncertain malignant potential. It commonly presents nonspecific and variable clinical manifestations, ranging from asymptomatic to hepatic failure. In addition, laboratory measurements and imaging features also lack specificity in the diagnosis of HEHE. The aim of the present study is to highlight the dilemma and challenges in the preoperative diagnosis of HEHE, and to enhance awareness of the range of hepatobiliary surgery available in patients with multiple hepatic nodular lesions on imaging. In these patients, HEHE should at least be considered in the differential diagnosis.
基金Supported by the Anhui Provincial Natural Science Foundation,No.1908085MH275Bengbu Medical College Key projects of Natural Science Foundation,No.BYKF1710Bengbu City-Bengbu Medical College Joint Science and Technology Project,No.BYLK201812.
文摘BACKGROUND Perivascular epithelioid cell tumor(PEComa)is an uncommon tumor of mesenchymal origin.Cases of PEComa in the liver are extremely rare.AIM To analyze the clinicopathological features and treatment of hepatic PEComa and to evaluate the prognosis after different treatments.METHODS Clinical and pathological data of 26 patients with hepatic PEComa were collected.All cases were analyzed by immunohistochemistry and clinical follow-up.RESULTS This study included 17 females and 9 males,with a median age of 50 years.Lesions were located in the left hepatic lobe in 13 cases,in the right lobe in 11,and in the caudate lobe in 2.The median tumor diameter was 6.5 cm.Light microscopy revealed that the tumor cells were mainly composed of epithelioid cells.The cytoplasm contained heterogeneous eosinophilic granules.There were thick-walled blood vessels,around which tumor cells were radially arranged.Immunohistochemical analysis of pigment-derived and myogenic markers in PEComas revealed that 25 cases were HMB45(+),23 were Melan-A(+),and 22 SMA(+).TFE3 and Desmin were negative in all cases.All the fluorescence in situ hybridization samples were negative for TFE3 gene break-apart probe.Tumor tissues were collected by extended hepatic lobe resection or simple hepatic tumor resection as the main treatments.Median follow-up was 62.5 mo.None of the patients had metastasis or recurrence,and there were no deaths due to the disease.CONCLUSION Hepatic PEComa highly expresses melanin and smooth muscle markers,and generally exhibits an inert biological behavior.The prognosis after extended hepatic lobe resection and simple hepatic tumor resection is semblable.
文摘BACKGROUND The perivascular epithelioid cell tumour(PEComa)family of tumours mainly includes renal and hepatic angiomyolipomas,pulmonary lymphangioleiomyomatosis and clear cell“sugar”tumour of the lung.Several uncommon tumours with similar morphological and immunophenotypical characteristics arising at a variety of sites(abdominal cavity,digestive tract,retroperitoneum,skin,soft tissue and bones)are also included in the PEComa family and are referred to as PEComas not otherwise specified.CASE SUMMARY We present a 37-year-old female patient who underwent resection of an 8.5 cm×8 cm×4 cm retroperitoneal tumour,which eventually was diagnosed as PEComa of uncertain biological behaviour.Three years after the operation,the patient remains without any evidence of recurrence.A search was performed in the Medline and EMBASE databases for articles published between 1996 and 2018,and we identified 31 articles related to retroperitoneal and perinephric PEComas.We focused on sex,age,maximum dimension,histological and immunohistochemical characteristics of the tumour,follow-up and long-term outcome.Thirty-four retroperitoneal(including the present one)and ten perinephric PEComas were identified,carrying a malignant potential rate of 44%and 60%,respectively.Nearly half of the potentially malignant PEComas presented with or developed metastases during the course of the disease.CONCLUSION Retroperitoneal PEComas are not as indolent as they are supposed to be.Radical surgical resection constitutes the treatment of choice for localized disease,while mammalian target of the rapamycin(mTOR)inhibitors constitute the most promising therapy for disseminated disease.The role of mTOR inhibitors as adjuvant or neoadjuvant therapies needs to be evaluated in the future.
基金supported by grants from the Foundation of Jiangsu Collaborative Innovation Center of Biomedical Functional Materials,Basic Research Program-Youth Fund Project of Jiangsu Province(BK20140092)the National Natural Science Foundation of China(81400650,81273261 and 81270583)
文摘Perivascular epithelioid cell tumor （PEComa） is a rare, soft tissue tumor that can occur in various locations. The present report included three patients （one male and two females; age range, 25-51 years） with hepatic PEComas. The collected data included the clinical manifestations, diagnosis, management, treatment, and prognosis. Since it is difficult to diagnose hepatic PEComas by imaging, the patients were diagnosed by tumor tissue examination such as immunohistochemistry, which was positive for HMB-45, Melan-A, and SMA on all slides. The tumor was composed of diverse tissues including smooth muscle, adipose tissue, and thick-walled blood vessels. During the follow-up period, one of the tumors was malignant （double-positive for CD34 and Ki-67） and recurred 3 months after surgery. In addition, malignant hepatic PECo- mas were reviewed in the literature, indicating that the majority of hepatic PEComas are benign, but few hepatic PEComas exhibit malignant behaviors in older female patients （〉50 years of age） with abdominal discomfort and pain, larger tumor size （〉10 cm）, or positive staining for CD34 and Ki-67. In conclusion, there is no effective method to diagnose PEComas. Currently, the diagnosis of PEComas depends on immunohistochemical staining. Tumor resection and close follow-up are the principal methods for the management of PEComas.
