Splenic histiocytic sarcoma(SHS)is a rare,aggressive hematological malignancy with unclear progression and management.Our case illustrates the progression and pathophysiological processes of SHS and provides key data ...Splenic histiocytic sarcoma(SHS)is a rare,aggressive hematological malignancy with unclear progression and management.Our case illustrates the progression and pathophysiological processes of SHS and provides key data for the diagnosis,treatment and management of SHS.A 60-year-old female with incidentally detected splenic mass(6.0 cm×5.7 cm)underwent splenectomy,confirmed as SHS in 2020.Post-op imatinib therapy was given.In 2022,hepatic metastases(2.4 cm×2.9 cm)with pancytopenia led to supportive care.Lesions enlarged to 4.3 cm×2.7 cm,leading to multi-organ failure and death at 33 months.The case was categorized into three distinct stages based on the pathophysiology of SHS:Early-stage splenic tumor growth,mid-stage liver metastasis with hematological abnormalities,and late-stage tumor infiltration leading to multiorgan failure.For SHS,this case highlights the pivotal role of early intervention and the value of personalized treatment strategies.展开更多
BACKGROUND Primary renal synovial sarcoma(PRSS)is extremely rare in clinical practice,and most cases are associated with SYT-SSX gene fusion.The PRSS with specific MDM2 gene amplification has not been reported so far....BACKGROUND Primary renal synovial sarcoma(PRSS)is extremely rare in clinical practice,and most cases are associated with SYT-SSX gene fusion.The PRSS with specific MDM2 gene amplification has not been reported so far.Therefore,there is no practical experience regarding the clinical,pathological features and diagnosis and treatment plans for patients of this type.This article reports a case of PRSS with specific MDM2 gene amplification.CASE SUMMARY The patient was preoperatively diagnosed with a malignant tumor of the left kidney(with a high probability of clear cell carcinoma).During the operation,a radical left nephrectomy was performed.The postoperative pathological examination report confirmed synovial sarcoma,and the gene test suggested PRSS with specific MDM2 gene amplification.Forty-eight days after the operation,the patient presented with"abdominal distension and diarrhea"and was found to have a huge metastatic tumor in the original left renal area.The patient died clinically 17 hours after admission due to"multiple organ failure".CONCLUSION PRSS with MDM2 gene amplification has a poorer prognosis,a higher degree of malignancy,and a faster progression,and clinicians need to be highly vigilant.展开更多
Adamantinoma-like Ewing sarcoma(ALES)is a rare variant of Ewing sarcoma that predominantly occurs in the head and neck region,characterized by epithelial differentiation and overlapping morphology with other small rou...Adamantinoma-like Ewing sarcoma(ALES)is a rare variant of Ewing sarcoma that predominantly occurs in the head and neck region,characterized by epithelial differentiation and overlapping morphology with other small round cell tumors.We present three cases of head and neck ALES(two in the parotid gland and one in the nasal cavity)in patients aged 23-42 years.Histologically,the tumors exhibited a spectrum of features,including small round blue cells and basaloid squamous morphologies.Immunohistochemically,the tumor cells variably expressed CKpan,p63,CD99,NKX2.2,FLI1,CD56,and Syn.The Ki-67 proliferation index ranged widely from 15%to 90%.Notably,one case initially misdiagnosed as squamous cell carcinoma harbored a canonical EWSR1::FLI1 fusion.Crucially,we report the first case of ALES had a negative EWSR1 rearrangement but a somatic NF1 mutation,expanding the molecular spectrum of this entity.Our findings underscore the diagnostic challenge of ALES and highlight the necessity of comprehensive molecular profiling,including next-generation sequencing,for accurate diagnosis and the identification of potential therapeutic targets,particularly in genetically atypical cases.展开更多
Lipid nanoparticles have become attractive for its prominent properties recent years. In this paper, in vivo anti-tumor efficacy of nanostructured lipid carrier of dihydroartemisinin （DHA-NLC） were evaluated in sarc...Lipid nanoparticles have become attractive for its prominent properties recent years. In this paper, in vivo anti-tumor efficacy of nanostructured lipid carrier of dihydroartemisinin （DHA-NLC） were evaluated in sarcoma 180-bearing mice model through intraperitoneal （i.p.） administration. In vivo biodistribution was also investigated in Kunming mice bearing S180. Results demonstrated that the intraperitoneally injected DHA-NLC could significantly inhibit tumor growth at the dose levels of 20, 40 and 80 mg/kg, and their inhibition rates were 71.24%, 79.20% and 85.74%, respectively. The biodistribution of DHA after intraperitoneal injection of DHA-NLC in S180-bearing mice is remarkably different from the DHA solution. Therefore, DHA encapsulated in NLC does demonstrate superior anticancer effect to DHA suspension on S 180-bearing mice at the same dose and displayed a dose-dependent antitumor efficacy.展开更多
Objective: Soft tissue sarcomas(STSs) are rare malignancies deriving from mesenchyme.In this study, we reported the epidemiology of STS in China using population-based cancer registry data.Methods: In 2017, qualified ...Objective: Soft tissue sarcomas(STSs) are rare malignancies deriving from mesenchyme.In this study, we reported the epidemiology of STS in China using population-based cancer registry data.Methods: In 2017, qualified data from 339 cancer registries were included in the national database.All STS cases were retrieved based on the morphological and topographical codes of International Classification of Diseases for Oncology, and were categorized into different histological subtypes and primary sites accordingly.Nationwide new STS cases were estimated using incidence rate of STS and the national population, and were reported for gastrointestinal stromal tumor(GIST) and STSs other than GIST separately by sex and region.Distribution of histological subtypes and primary sites of STS were calculated, as well as primary sites of GIST.Results: Approximately 39,900 new STS cases occurred nationwide in China in 2014, accounting for 1.05% of overall cancer incidence.The crude incidence rate was 2.91/100,000 and generally increased with age.An overall female predilection was found.GIST was the most common histological subtype, followed by nerve sheath tumor and malignant peripheral nerve sheath tumor,leiomyosarcoma, liposarcoma, and fibrosarcoma.About 67.5% of GIST occurred in stomach while 1.4% were recorded outside the gastrointestinal tract.Connective, subcutaneous and other soft tissues were the most common primary site, of which extremities were the major subsite.Conclusions: The burden of STS is not serious in China relatively.However, due to their histological and topographical complexity, STSs should not be unnoticed, and more basic and clinical studies should focus on STSs.展开更多
AIM: To evaluate the undifferentiated embryonal sarcoma of liver (UESL) in adults in order to improve its diagnosis and treatment. METHODS: Four primary and one recurrent cases of UESL were clinicopathologically evalu...AIM: To evaluate the undifferentiated embryonal sarcoma of liver (UESL) in adults in order to improve its diagnosis and treatment. METHODS: Four primary and one recurrent cases of UESL were clinicopathologically evaluated and immunohistochemically investigated with a panel of antibodies using the EnVision+ system. Relevant literature about UESL in adults was reviewed. RESULTS: Three males and one female were enrolled in this study. Their chief complaints were abdominal pain, weight loss, or fever. Laboratory tests, imaging and pathological features of UESL in adults were similar to those in children. Immunohistochemistry showed evidence of widely divergent differentiation into mesenchymal and epithelial phenotypes. The survival time of patients who underwent complete tumor resection followed by adjuvant transcatheter arterial chemoembolization (TACE) was significantly longer than that of those who underwent surgical treatment alone.CONCLUSION: UESL in adults may undergo pluripotential differentiation and its diagnosis should be made based on its morphological and immunohistochemical features. Complete tumor resection after adjuvant TACE may improve the survival time of such patients.展开更多
BACKGROUND:Undifferentiated embryonal sarcoma (UES) of the liver is a rare,highly malignant neoplasm with a poor prognosis occurring almost exclusively in late childhood (6-10 years of age).Only a few cases have been ...BACKGROUND:Undifferentiated embryonal sarcoma (UES) of the liver is a rare,highly malignant neoplasm with a poor prognosis occurring almost exclusively in late childhood (6-10 years of age).Only a few cases have been reported in adults accounting for less than 1% of all primary liver neoplasms.METHODS:A 47-year-old woman presented with a palpable mass in the left upper abdomen.Magnetic resonance imaging revealed a 12×10 cm cystic mass with hemorrhage in the left lateral segment of the liver.The initial impression was a hemorrhagic cystic tumor of the liver.The patient underwent a left lateral sectionectomy of the liver.Histopathology and immunohistochemistry helped make a diagnosis of UES.RESULTS:The patient recovered uneventfully and received systemic chemotherapy.Radiologic examination for follow-up revealed a metastatic lesion in the lumbar spine (L5).She was subjected to radiotherapy at the lumbar spine.She survived 48 months.CONCLUSION:Although hepatic cyst as UES of the liver is difficult to diagnose because of its rarity in adults and lack of specific findings,it should be considered in a differential diagnosis.展开更多
BACKGROUND:A malignant fibrous histiocytoma,recently referred to as an undifferentiated pleomorphic sarcoma (UPS),is very rare in the liver,and only 34 cases of primary hepatic malignant fibrous histiocytoma have been...BACKGROUND:A malignant fibrous histiocytoma,recently referred to as an undifferentiated pleomorphic sarcoma (UPS),is very rare in the liver,and only 34 cases of primary hepatic malignant fibrous histiocytoma have been reported in the English literature. METHODS:We report a rare case of a hepatic UPS presenting as a unilocular cystic lesion with respect to histopathologic features,the newly revised diagnostic criteria,and the differential diagnosis. RESULTS:A 60-year-old man presented for evaluation of epigastric pain of 7 months duration.Abdominal computed tomography revealed a hypodense,unilocular cystic mass in the right lobe of the liver,measuring 14.0×11.1 cm.A right lobectomy was performed.Grossly,the cut surface showed a single,well-circumscribed unilocular cystic tumor mass containing dark red-brown necrotic debris and blood clots, which occupied most of the mass.Microscopically,the tumor consisted of haphazardly arranged mononuclear pleomorphic tumor cells,admixed with abundant osteoclast- like multinucleated giant cells.Immunohistochemically,the tumor cells expressed vimentin only.The histopathologic and immunohistochemical findings were compatible with a UPS.The patient is alive and well 41 months after surgery without recurrence. CONCLUSIONS:Clinically,most of the hepatic UPSs are solid masses.Only two cases have presented as multilocular cystic masses.A primary hepatic UPS presenting as a unilocular cyst has never been reported.A UPS should be included in the differential diagnosis of unilocular cystic lesions in the liver.展开更多
Pulmonary artery sarcoma(PAS)is a rare and lethal neoplasm that is usually diagnosed during surgery or autopsy.Early diagnosis and radical surgical resection offer the only chance for survival.However,making a preoper...Pulmonary artery sarcoma(PAS)is a rare and lethal neoplasm that is usually diagnosed during surgery or autopsy.Early diagnosis and radical surgical resection offer the only chance for survival.However,making a preoperative histopathological diagnosis is quite difficult.We encountered a 57-year-old woman presenting a PAS that mimicked a pulmonary thromboembolism.After confirming a definitive diagnosis using a catheter suction biopsy,we successfully performed a right pneumonectomy via a median sternotomy without cardiopulmonary bypass.Eighteen months after surgery,no recurrence was observed.展开更多
BACKGROUND Cardiac tumors are rare and complex entities.Surgery represents the cornerstone of therapy,while the role of adjuvant treatment remains unclear and,in case of relapse or metastatic disease,the prognosis is ...BACKGROUND Cardiac tumors are rare and complex entities.Surgery represents the cornerstone of therapy,while the role of adjuvant treatment remains unclear and,in case of relapse or metastatic disease,the prognosis is very poor.Lack of prospective,randomized clinical trials hinders the generation of high level evidence for the optimal diagnostic workup and multimodal treatment of cardiac sarcomas.Herein,we describe the multidisciplinary clinical management and molecular characterization of a rare case of cardiac myxofibrosarcoma in an elderly woman.CASE SUMMARY A 73-year-old woman presented signs and symptoms of acute left-sided heart failure.Imaging examination revealed a large,left atrial mass.With suspicion of a myxoma,she underwent surgery,and symptoms were promptly relieved.Histology showed a cardiac myxofibrosarcoma,a rare histotype of cardiac sarcoma.Eight months later,disease unfortunately relapsed,and after a multidisciplinary discussion,a chemotherapy with doxorubicin and then gemcitabine was started,achieving partial radiologic and complete metabolic response,which was maintained up to 2 years and is still present.This report is focused on the entire clinical path of our patient from diagnosis to follow-up,through surgery and strategies adopted at relapse.Moreover,due to their rarity,very little is known about the molecular landscape of myxofibrosarcomas.Thus,we also performed and described preliminary genome analysis of the tumor tissue to get further insight on mechanisms involved in tumor growth,and to possibly unveil new clinically actionable targets.CONCLUSION We report a case of cardiac myxofibrosarcoma that achieved a very good prognosis due to an integrated surgical,cardiac and oncologic treatment strategy.展开更多
Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age...Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age of incidence. Rhabdomyosarcoma(RMS) is diagnosed more frequently in younger children, whereas adult-type non-RMS soft tissue sarcoma is predominately observed in adolescents. The latter group comprises a variety of rare tumors for which diagnosis can be difficult and typically requires special studies, including immunohistochemistry and molecular genetic analysis. Current management for the majority of pediatric sarcomas is based on the data from large multi-institutional trials, which has led to great improvements in outcomes over recent decades. Although surgery remains the mainstay of treatment, the curative aim cannot be achieved without adjuvant treatment. Pre-treatment staging and risk classification are of prime importance in selecting an effective treatment protocol. Tumor resectability, the response to induction chemotherapy, and radiation generally determine the risk-group, and these factors are functions of tumor site, size and biology. Surgery provides the best choice of local control of small resectable tumors in a favorable site. Radiation therapy is added when surgery leaves residual disease or there is evidence of regional spread. Chemotherapy aims to reduce the risk of relapse and improve overall survival. In addition, upfront chemotherapy reduces the aggressiveness of the required surgery and helps preserve organ function in a number of cases. Long-term survival in low-risk sarcomas is feasible, and the intensity of treatment can be reduced. In high-risk sarcoma, current research is allowing more effective disease control.展开更多
BACKGROUND Metastatic low-grade endometrial stromal sarcoma(LG-ESS) with sex cord-like and smooth muscle-like differentiation is rare. This article reports such a case with multiple recurrences and with extensive pelv...BACKGROUND Metastatic low-grade endometrial stromal sarcoma(LG-ESS) with sex cord-like and smooth muscle-like differentiation is rare. This article reports such a case with multiple recurrences and with extensive pelvic and abdominal metastasis.CASE SUMMARY A 47-year-old female patient was diagnosed with multiple cystic masses in the pelvic cavity by magnetic resonance imaging examination. Based on the postoperative pathological and immunohistochemical analyses of the surgical specimen, she was diagnosed with a metastatic low-grade endometrial stromal sarcoma with sex cord and smooth muscle differentiation.CONCLUSION LG-ESS is a low-grade malignant tumor with a high recurrence rate and metastasis probability. It is easily misdiagnosed initially. It is essential to distinguish LG-ESS with sex cord-like differentiation from uterine tumour resembling ovarian sex cord tumour.展开更多
Granulocytic sarcoma is an uncommon and localized extramedullary tumor composed of immature granu-locytic cells.It may present in association with acute myeloid leukaemia,myelodysplastic syndrome and chronic myelogeno...Granulocytic sarcoma is an uncommon and localized extramedullary tumor composed of immature granu-locytic cells.It may present in association with acute myeloid leukaemia,myelodysplastic syndrome and chronic myelogenous leukaemia.Granulocytic sarcoma may occur in any anatomical site but involvement of the gastrointestinal tract is rare,especially in the rectum.We report on the case of a 17 year old female who presented with rectal bleeding,abdominal pain and weight loss one mo prior to admission.Recto-sigmoidoscopy revealed a rectal polypoid and ulcerated mass.The histological examination of the mass showed granulocytic sarcoma.Bone marrow examination was compatible with acute promyelocytic leukaemia(FABtype M3).This case report is a reminder of this peculiar sign of tumoral syndrome in acute myeloid leukaemia.We also discuss diagnostic methods and analyze the disease course.展开更多
BACKGROUND Primary intimal sarcoma of the pulmonary artery is a rare malignant tumor originating from the pulmonary artery,which has a low incidence rate and is easily misdiagnosed as pulmonary embolism.There is no st...BACKGROUND Primary intimal sarcoma of the pulmonary artery is a rare malignant tumor originating from the pulmonary artery,which has a low incidence rate and is easily misdiagnosed as pulmonary embolism.There is no standard protocol for the treatment of primary intimal sarcoma of the pulmonary artery.CASE SUMMARY This study reports a patient with primary intimal sarcoma of the pulmonary artery who was admitted to our hospital in 2017.The clinical characteristics,diagnosis,treatment and outcome of the patient were retrospectively analyzed.The patient was a Chinese Han male aged 44 years.He had three consecutive episodes of syncope,and was thus admitted to a local hospital.Computed tomography pulmonary angiography showed multiple lesions with abnormal densities in the pulmonary trunk,left pulmonary artery,mediastinum and pericardium,which were consistent with recurrence after tumor resection.He underwent surgery,and was pathologically diagnosed with intimal sarcoma of the pulmonary artery.He relapsed 3 mo after surgery,and apatinib was administered.His condition was stable after 4 mo,with tolerable and controllable adverse reactions.He subsequently died 19 mo after surgery.CONCLUSION Primary intimal sarcoma of the pulmonary artery has no specific clinical or imaging manifestations.The diagnosis of this disease depends on histopathology and immunohistochemistry,and has a poor clinical prognosis.Surgical treatment is currently a favorable option for primary intimal sarcoma of the pulmonary artery,and targeted therapy may provide new insights for the development of effective treatment methods.展开更多
基金Supported by the Program of General Hospital of Western Theater,No.2021-XZYG-C33.
文摘Splenic histiocytic sarcoma(SHS)is a rare,aggressive hematological malignancy with unclear progression and management.Our case illustrates the progression and pathophysiological processes of SHS and provides key data for the diagnosis,treatment and management of SHS.A 60-year-old female with incidentally detected splenic mass(6.0 cm×5.7 cm)underwent splenectomy,confirmed as SHS in 2020.Post-op imatinib therapy was given.In 2022,hepatic metastases(2.4 cm×2.9 cm)with pancytopenia led to supportive care.Lesions enlarged to 4.3 cm×2.7 cm,leading to multi-organ failure and death at 33 months.The case was categorized into three distinct stages based on the pathophysiology of SHS:Early-stage splenic tumor growth,mid-stage liver metastasis with hematological abnormalities,and late-stage tumor infiltration leading to multiorgan failure.For SHS,this case highlights the pivotal role of early intervention and the value of personalized treatment strategies.
基金Supported by The Joint Special Project of Universities in Yunnan Province in 2022,No.2022-ES-530101-207The Hospital-level Scientific Research Project of The Fourth Affiliated Hospital of Dali University,No.2024YJ14.
文摘BACKGROUND Primary renal synovial sarcoma(PRSS)is extremely rare in clinical practice,and most cases are associated with SYT-SSX gene fusion.The PRSS with specific MDM2 gene amplification has not been reported so far.Therefore,there is no practical experience regarding the clinical,pathological features and diagnosis and treatment plans for patients of this type.This article reports a case of PRSS with specific MDM2 gene amplification.CASE SUMMARY The patient was preoperatively diagnosed with a malignant tumor of the left kidney(with a high probability of clear cell carcinoma).During the operation,a radical left nephrectomy was performed.The postoperative pathological examination report confirmed synovial sarcoma,and the gene test suggested PRSS with specific MDM2 gene amplification.Forty-eight days after the operation,the patient presented with"abdominal distension and diarrhea"and was found to have a huge metastatic tumor in the original left renal area.The patient died clinically 17 hours after admission due to"multiple organ failure".CONCLUSION PRSS with MDM2 gene amplification has a poorer prognosis,a higher degree of malignancy,and a faster progression,and clinicians need to be highly vigilant.
文摘Adamantinoma-like Ewing sarcoma(ALES)is a rare variant of Ewing sarcoma that predominantly occurs in the head and neck region,characterized by epithelial differentiation and overlapping morphology with other small round cell tumors.We present three cases of head and neck ALES(two in the parotid gland and one in the nasal cavity)in patients aged 23-42 years.Histologically,the tumors exhibited a spectrum of features,including small round blue cells and basaloid squamous morphologies.Immunohistochemically,the tumor cells variably expressed CKpan,p63,CD99,NKX2.2,FLI1,CD56,and Syn.The Ki-67 proliferation index ranged widely from 15%to 90%.Notably,one case initially misdiagnosed as squamous cell carcinoma harbored a canonical EWSR1::FLI1 fusion.Crucially,we report the first case of ALES had a negative EWSR1 rearrangement but a somatic NF1 mutation,expanding the molecular spectrum of this entity.Our findings underscore the diagnostic challenge of ALES and highlight the necessity of comprehensive molecular profiling,including next-generation sequencing,for accurate diagnosis and the identification of potential therapeutic targets,particularly in genetically atypical cases.
基金Fundamental Research Funds of Lanzhou University for the Central Universities (Grant No. lzujbky-2012-85)the Lanzhou Science and Technology Bureau (Grant No. 2012-2-80)
文摘Lipid nanoparticles have become attractive for its prominent properties recent years. In this paper, in vivo anti-tumor efficacy of nanostructured lipid carrier of dihydroartemisinin （DHA-NLC） were evaluated in sarcoma 180-bearing mice model through intraperitoneal （i.p.） administration. In vivo biodistribution was also investigated in Kunming mice bearing S180. Results demonstrated that the intraperitoneally injected DHA-NLC could significantly inhibit tumor growth at the dose levels of 20, 40 and 80 mg/kg, and their inhibition rates were 71.24%, 79.20% and 85.74%, respectively. The biodistribution of DHA after intraperitoneal injection of DHA-NLC in S180-bearing mice is remarkably different from the DHA solution. Therefore, DHA encapsulated in NLC does demonstrate superior anticancer effect to DHA suspension on S 180-bearing mice at the same dose and displayed a dose-dependent antitumor efficacy.
基金supported by CAMS Innovation Fund for Medical Sciences (CIFMS) (Grant No.2016-12M-2-004)Ministry of Science and Technology (Grant No.2014FY121100)
文摘Objective: Soft tissue sarcomas(STSs) are rare malignancies deriving from mesenchyme.In this study, we reported the epidemiology of STS in China using population-based cancer registry data.Methods: In 2017, qualified data from 339 cancer registries were included in the national database.All STS cases were retrieved based on the morphological and topographical codes of International Classification of Diseases for Oncology, and were categorized into different histological subtypes and primary sites accordingly.Nationwide new STS cases were estimated using incidence rate of STS and the national population, and were reported for gastrointestinal stromal tumor(GIST) and STSs other than GIST separately by sex and region.Distribution of histological subtypes and primary sites of STS were calculated, as well as primary sites of GIST.Results: Approximately 39,900 new STS cases occurred nationwide in China in 2014, accounting for 1.05% of overall cancer incidence.The crude incidence rate was 2.91/100,000 and generally increased with age.An overall female predilection was found.GIST was the most common histological subtype, followed by nerve sheath tumor and malignant peripheral nerve sheath tumor,leiomyosarcoma, liposarcoma, and fibrosarcoma.About 67.5% of GIST occurred in stomach while 1.4% were recorded outside the gastrointestinal tract.Connective, subcutaneous and other soft tissues were the most common primary site, of which extremities were the major subsite.Conclusions: The burden of STS is not serious in China relatively.However, due to their histological and topographical complexity, STSs should not be unnoticed, and more basic and clinical studies should focus on STSs.
文摘AIM: To evaluate the undifferentiated embryonal sarcoma of liver (UESL) in adults in order to improve its diagnosis and treatment. METHODS: Four primary and one recurrent cases of UESL were clinicopathologically evaluated and immunohistochemically investigated with a panel of antibodies using the EnVision+ system. Relevant literature about UESL in adults was reviewed. RESULTS: Three males and one female were enrolled in this study. Their chief complaints were abdominal pain, weight loss, or fever. Laboratory tests, imaging and pathological features of UESL in adults were similar to those in children. Immunohistochemistry showed evidence of widely divergent differentiation into mesenchymal and epithelial phenotypes. The survival time of patients who underwent complete tumor resection followed by adjuvant transcatheter arterial chemoembolization (TACE) was significantly longer than that of those who underwent surgical treatment alone.CONCLUSION: UESL in adults may undergo pluripotential differentiation and its diagnosis should be made based on its morphological and immunohistochemical features. Complete tumor resection after adjuvant TACE may improve the survival time of such patients.
文摘BACKGROUND:Undifferentiated embryonal sarcoma (UES) of the liver is a rare,highly malignant neoplasm with a poor prognosis occurring almost exclusively in late childhood (6-10 years of age).Only a few cases have been reported in adults accounting for less than 1% of all primary liver neoplasms.METHODS:A 47-year-old woman presented with a palpable mass in the left upper abdomen.Magnetic resonance imaging revealed a 12×10 cm cystic mass with hemorrhage in the left lateral segment of the liver.The initial impression was a hemorrhagic cystic tumor of the liver.The patient underwent a left lateral sectionectomy of the liver.Histopathology and immunohistochemistry helped make a diagnosis of UES.RESULTS:The patient recovered uneventfully and received systemic chemotherapy.Radiologic examination for follow-up revealed a metastatic lesion in the lumbar spine (L5).She was subjected to radiotherapy at the lumbar spine.She survived 48 months.CONCLUSION:Although hepatic cyst as UES of the liver is difficult to diagnose because of its rarity in adults and lack of specific findings,it should be considered in a differential diagnosis.
文摘BACKGROUND:A malignant fibrous histiocytoma,recently referred to as an undifferentiated pleomorphic sarcoma (UPS),is very rare in the liver,and only 34 cases of primary hepatic malignant fibrous histiocytoma have been reported in the English literature. METHODS:We report a rare case of a hepatic UPS presenting as a unilocular cystic lesion with respect to histopathologic features,the newly revised diagnostic criteria,and the differential diagnosis. RESULTS:A 60-year-old man presented for evaluation of epigastric pain of 7 months duration.Abdominal computed tomography revealed a hypodense,unilocular cystic mass in the right lobe of the liver,measuring 14.0×11.1 cm.A right lobectomy was performed.Grossly,the cut surface showed a single,well-circumscribed unilocular cystic tumor mass containing dark red-brown necrotic debris and blood clots, which occupied most of the mass.Microscopically,the tumor consisted of haphazardly arranged mononuclear pleomorphic tumor cells,admixed with abundant osteoclast- like multinucleated giant cells.Immunohistochemically,the tumor cells expressed vimentin only.The histopathologic and immunohistochemical findings were compatible with a UPS.The patient is alive and well 41 months after surgery without recurrence. CONCLUSIONS:Clinically,most of the hepatic UPSs are solid masses.Only two cases have presented as multilocular cystic masses.A primary hepatic UPS presenting as a unilocular cyst has never been reported.A UPS should be included in the differential diagnosis of unilocular cystic lesions in the liver.
文摘Pulmonary artery sarcoma(PAS)is a rare and lethal neoplasm that is usually diagnosed during surgery or autopsy.Early diagnosis and radical surgical resection offer the only chance for survival.However,making a preoperative histopathological diagnosis is quite difficult.We encountered a 57-year-old woman presenting a PAS that mimicked a pulmonary thromboembolism.After confirming a definitive diagnosis using a catheter suction biopsy,we successfully performed a right pneumonectomy via a median sternotomy without cardiopulmonary bypass.Eighteen months after surgery,no recurrence was observed.
文摘BACKGROUND Cardiac tumors are rare and complex entities.Surgery represents the cornerstone of therapy,while the role of adjuvant treatment remains unclear and,in case of relapse or metastatic disease,the prognosis is very poor.Lack of prospective,randomized clinical trials hinders the generation of high level evidence for the optimal diagnostic workup and multimodal treatment of cardiac sarcomas.Herein,we describe the multidisciplinary clinical management and molecular characterization of a rare case of cardiac myxofibrosarcoma in an elderly woman.CASE SUMMARY A 73-year-old woman presented signs and symptoms of acute left-sided heart failure.Imaging examination revealed a large,left atrial mass.With suspicion of a myxoma,she underwent surgery,and symptoms were promptly relieved.Histology showed a cardiac myxofibrosarcoma,a rare histotype of cardiac sarcoma.Eight months later,disease unfortunately relapsed,and after a multidisciplinary discussion,a chemotherapy with doxorubicin and then gemcitabine was started,achieving partial radiologic and complete metabolic response,which was maintained up to 2 years and is still present.This report is focused on the entire clinical path of our patient from diagnosis to follow-up,through surgery and strategies adopted at relapse.Moreover,due to their rarity,very little is known about the molecular landscape of myxofibrosarcomas.Thus,we also performed and described preliminary genome analysis of the tumor tissue to get further insight on mechanisms involved in tumor growth,and to possibly unveil new clinically actionable targets.CONCLUSION We report a case of cardiac myxofibrosarcoma that achieved a very good prognosis due to an integrated surgical,cardiac and oncologic treatment strategy.
文摘Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age of incidence. Rhabdomyosarcoma(RMS) is diagnosed more frequently in younger children, whereas adult-type non-RMS soft tissue sarcoma is predominately observed in adolescents. The latter group comprises a variety of rare tumors for which diagnosis can be difficult and typically requires special studies, including immunohistochemistry and molecular genetic analysis. Current management for the majority of pediatric sarcomas is based on the data from large multi-institutional trials, which has led to great improvements in outcomes over recent decades. Although surgery remains the mainstay of treatment, the curative aim cannot be achieved without adjuvant treatment. Pre-treatment staging and risk classification are of prime importance in selecting an effective treatment protocol. Tumor resectability, the response to induction chemotherapy, and radiation generally determine the risk-group, and these factors are functions of tumor site, size and biology. Surgery provides the best choice of local control of small resectable tumors in a favorable site. Radiation therapy is added when surgery leaves residual disease or there is evidence of regional spread. Chemotherapy aims to reduce the risk of relapse and improve overall survival. In addition, upfront chemotherapy reduces the aggressiveness of the required surgery and helps preserve organ function in a number of cases. Long-term survival in low-risk sarcomas is feasible, and the intensity of treatment can be reduced. In high-risk sarcoma, current research is allowing more effective disease control.
文摘BACKGROUND Metastatic low-grade endometrial stromal sarcoma(LG-ESS) with sex cord-like and smooth muscle-like differentiation is rare. This article reports such a case with multiple recurrences and with extensive pelvic and abdominal metastasis.CASE SUMMARY A 47-year-old female patient was diagnosed with multiple cystic masses in the pelvic cavity by magnetic resonance imaging examination. Based on the postoperative pathological and immunohistochemical analyses of the surgical specimen, she was diagnosed with a metastatic low-grade endometrial stromal sarcoma with sex cord and smooth muscle differentiation.CONCLUSION LG-ESS is a low-grade malignant tumor with a high recurrence rate and metastasis probability. It is easily misdiagnosed initially. It is essential to distinguish LG-ESS with sex cord-like differentiation from uterine tumour resembling ovarian sex cord tumour.
文摘Granulocytic sarcoma is an uncommon and localized extramedullary tumor composed of immature granu-locytic cells.It may present in association with acute myeloid leukaemia,myelodysplastic syndrome and chronic myelogenous leukaemia.Granulocytic sarcoma may occur in any anatomical site but involvement of the gastrointestinal tract is rare,especially in the rectum.We report on the case of a 17 year old female who presented with rectal bleeding,abdominal pain and weight loss one mo prior to admission.Recto-sigmoidoscopy revealed a rectal polypoid and ulcerated mass.The histological examination of the mass showed granulocytic sarcoma.Bone marrow examination was compatible with acute promyelocytic leukaemia(FABtype M3).This case report is a reminder of this peculiar sign of tumoral syndrome in acute myeloid leukaemia.We also discuss diagnostic methods and analyze the disease course.
文摘BACKGROUND Primary intimal sarcoma of the pulmonary artery is a rare malignant tumor originating from the pulmonary artery,which has a low incidence rate and is easily misdiagnosed as pulmonary embolism.There is no standard protocol for the treatment of primary intimal sarcoma of the pulmonary artery.CASE SUMMARY This study reports a patient with primary intimal sarcoma of the pulmonary artery who was admitted to our hospital in 2017.The clinical characteristics,diagnosis,treatment and outcome of the patient were retrospectively analyzed.The patient was a Chinese Han male aged 44 years.He had three consecutive episodes of syncope,and was thus admitted to a local hospital.Computed tomography pulmonary angiography showed multiple lesions with abnormal densities in the pulmonary trunk,left pulmonary artery,mediastinum and pericardium,which were consistent with recurrence after tumor resection.He underwent surgery,and was pathologically diagnosed with intimal sarcoma of the pulmonary artery.He relapsed 3 mo after surgery,and apatinib was administered.His condition was stable after 4 mo,with tolerable and controllable adverse reactions.He subsequently died 19 mo after surgery.CONCLUSION Primary intimal sarcoma of the pulmonary artery has no specific clinical or imaging manifestations.The diagnosis of this disease depends on histopathology and immunohistochemistry,and has a poor clinical prognosis.Surgical treatment is currently a favorable option for primary intimal sarcoma of the pulmonary artery,and targeted therapy may provide new insights for the development of effective treatment methods.
