BACKGROUND Primary splenic lesions are rare and often detected incidentally through imaging,biopsy,or autopsy,typically without distinct clinical symptoms.Although imaging can help differentiate benign from malignant ...BACKGROUND Primary splenic lesions are rare and often detected incidentally through imaging,biopsy,or autopsy,typically without distinct clinical symptoms.Although imaging can help differentiate benign from malignant lesions,splenic hamartomas,and angiosarcomas may exhibit overlapping features,making diagnosis challenging.This report presents a case of splenic hamartoma suspected to be an angiosarcoma based on preoperative imaging.Splenic hamartomas that mimic angiosarcomas are exceedingly rare.CASE SUMMARY A 33-year-old male presented to the Department of Emergency with frank red blood hematemesis and a 1-week history of epigastric pain.On arrival,he was alert and hemodynamically stable.Contrast-enhanced abdominal computed tomography revealed splenomegaly with significant engorgement of the portal and splenic veins,along with a diffuse nodular splenic lesion measuring 8.2 cm×6.2 cm.Following esophageal varix ligation,abdominal magnetic resonance imaging demonstrated iso-to high-signal intensity within the splenic mass and multiple hypervascular lesions in the right hepatic lobe,raising suspicion for splenic an-giosarcoma with hepatic metastases.18F-fluorodeoxyglucose positron emission tomography-computed tomography showed diffusely mild increased metabolic activity in the spleen.The patient subsequently underwent splenectomy and liver biopsy.Histopathological examination revealed chronic inflammation in the liver,and the splenic lesion was confirmed to be a splenic hamartoma.The patient successfully returned to work and remains in good health.CONCLUSION This rare case of splenic hamartoma mimicking angiosarcoma highlights the importance of differential diagnosis in managing splenic tumors.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
AIM: To investigate the expression of ERG, CD34, CD31 (PECAM-1, platelet/endothelial cell adhesion molecule 1) and factor VIII-related antigen (FVIIIRAg) in the diagnosis of hepatic angiosarcoma patients.
Primary splenic angiosarcoma(PSA) is the most unusual type of malignancy with early multifocal metastasis through hematogenous spread. PSA is generally believed to originate from splenic sinusoidal vascular endotheliu...Primary splenic angiosarcoma(PSA) is the most unusual type of malignancy with early multifocal metastasis through hematogenous spread. PSA is generally believed to originate from splenic sinusoidal vascular endothelium with a high rate of metastasis and to have a poor prognosis. Its etiology and pathogenetic mechanisms have not yet been clearly described. Thus far, only approximately 200 cases have been reported. PSA has variable symptomatology with the potential to present with life-threatening complications. The diagnosis of PSA is challenging; and often late. PSA should be considered in the differential diagnosis of patients with splenomegaly and anemia of unknown etiology. Surgical treatment with splenectomy is considered the only curative intervention for potential long-term disease-free survival. Early diagnosis and treatment are very important. It is important that clinical doctors improve the understanding of PSA. Herein, we report one rare case of PSA with hepatic metastases, along with a review of the current literature.展开更多
Background： Hepatic angiosarcoma is a rare malignant vascular tumor presenting unique treatment challenges.The aim of the present study was to determine the treatment and prognosis of this entity.Data sources： A sys...Background： Hepatic angiosarcoma is a rare malignant vascular tumor presenting unique treatment challenges.The aim of the present study was to determine the treatment and prognosis of this entity.Data sources： A systematic literature search was conducted using PubMed, Embase and Chinese BiomedicalLiterature database, to identify articles published from January 1980 to July 2017. Search termswere “hepatic angiosarcoma” and “liver angiosarcoma”. Additional articles were retrieved through manualsearch of bibliographies of the relevant articles. Pooled individual data concerning the prognosis followingvarious therapeutic modalities were analyzed.展开更多
Primary splenic angiosarcoma(PSA) is an unusual and highly malignant vascular tumour with a high rate of metastatic. Moreover, the research on prognosis of the disease is poor. The epidemiology, etiology, clinical dia...Primary splenic angiosarcoma(PSA) is an unusual and highly malignant vascular tumour with a high rate of metastatic. Moreover, the research on prognosis of the disease is poor. The epidemiology, etiology, clinical diagnosis and treatment of the disease remain challenging, because case reports of the disease are few in number. In accordance with other malignant tumors, PSA is very aggressive, and the majority of patients in which this disease is found are at an advanced stage. Almost all patients die within 12 mo of diagnosis irrespective of treatment. We report here a woman who had complained of upper bellyache and anorexia for 10 d. Magnetic resonance imaging showed enlargement of the spleen with multiple heterogeneous masses in the lower pole of the spleen. A hand-assisted laparoscopic splenectomy was performed which allowed histopathologic diagnosis. The patient was diagnosed with PSA and liver metastasis, and succumbed to the disease 35 d after surgery. The literature was finished combined with the clinical features, diagnosis and management of PSA.展开更多
Bevacizumab, an angiogenesis inhibitor, is a recombined humanized monoclonal antibody against vascular endothelial growth factor and a promising therapeutic option for angiosarcoma management. This is a ease report an...Bevacizumab, an angiogenesis inhibitor, is a recombined humanized monoclonal antibody against vascular endothelial growth factor and a promising therapeutic option for angiosarcoma management. This is a ease report and review of the literature using bevacizumab and combination chemotherapy for angiosarcoma. The understanding of the effectiveness of combined therapy of bevacizumab and chemotherapy agents is still limited. The benefits of bevacizumab treatment for angiosarcoma will need to be weighed against the risks of venous thromboembolism in this population.展开更多
Primary malignant tumors of the small intestine are rare, comprising less than 2% of all gastrointestinal tumors. An 85-year-old woman was admitted with fever of 40  °C and marked a...Primary malignant tumors of the small intestine are rare, comprising less than 2% of all gastrointestinal tumors. An 85-year-old woman was admitted with fever of 40  °C and marked abdominal distension. Her medical history was unremarkable, but blood examination showed elevated inflammatory markers. Abdominal computed tomography showed a giant tumor with central necrosis, extending from the epigastrium to the pelvic cavity. Giant gastrointestinal stromal tumor of the small intestine communicating with the gastrointestinal tract or with superimposed infection was suspected. Because no improvement occurred in response to antibiotics, surgery was performed. Laparotomy revealed giant hemorrhagic tumor adherent to the small intestine and occupying the peritoneal cavity. The giant tumor was a solid tumor weighing 3490 g, measuring 24 cm × 17.5 cm × 18 cm and showing marked necrosis. Histologically, the tumor comprised spindle-shaped cells with anaplastic large nuclei. Immunohistochemical studies showed tumor cells positive for vimentin, CD31, and factor VIII-related antigen, but negative for c-kit and CD34. Angiosarcoma was diagnosed. Although no postoperative complications occurred, the patient experienced enlargement of multiple metastatic tumors in the abdominal cavity and died 42 d postoperatively. The prognosis of small intestinal angiosarcoma is very poor, even after volume-reducing palliative surgery.展开更多
Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulate...Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulated coagulopathy that can be seen in hepatic angiosarcoma fulfills the clinical diagnostic criteria of disseminated intravascular coagulation. However, the mechanism that governs this coagulopathy has been poorly understood. This case report provides histological evidence of the consumption of coagulation factors along with trapped platelets occurring within the tumor, which is the foundation for the concept of Kasabach-Merritt syndrome(KMS). KMS is characterized by thrombocytopenia and hyperconsumption of coagulation factors within a vascular tumor. However, KMS associated with angiosarcoma has not been well recognized. This case report describes, for the first time, the histological evidence of KMS that occurred in an extremely rare mesenchymal malignant tumor of the liver.展开更多
Primary hepatic angiosarcoma is the most common malignant mesenchymal tumor of the liver. It has a poor prognosis and various appearances on magnetic resonance(MR) images. We report a case of hepatic angiosarcoma with...Primary hepatic angiosarcoma is the most common malignant mesenchymal tumor of the liver. It has a poor prognosis and various appearances on magnetic resonance(MR) images. We report a case of hepatic angiosarcoma with a characteristic appearance on gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid(Gd-EOB-DTPA)-enhanced MR imaging in the hepatobiliary phase. A 72-year-old man was admitted with a complaint of abdominal pain. Gd-EOBDTPA-enhanced MR imaging revealed a liver tumor that showed slight hyperintensity in the hepatobiliary phase. These findings suggested Gd-EOB-DTPA uptake in the tumor. An autopsy revealed the solid proliferation and sinusoidal spreading of hepatic angiosarcoma cells. Immunohistochemistry indicated that the tumor was negative for OATP1B3. Gd-EOB-DTPA uptake in the liver tumor in the hepatobiliary phase suggested sinusoidal tumor invasion with residual normal hepatocytes.展开更多
Angiosarcoma is a rare primary malignant neoplasm of the liver with a poor prognosis. Here, we report a case of a patient with a ruptured hepatic angiosarcoma which was treated by emergency catheter-directed embolizat...Angiosarcoma is a rare primary malignant neoplasm of the liver with a poor prognosis. Here, we report a case of a patient with a ruptured hepatic angiosarcoma which was treated by emergency catheter-directed embolization, followed by leflc-sided hemihepatectomy.展开更多
We report a case of an 84-year-old male patient with primary small intestinal angiosarcoma.The patient initially presented with anemia and melena.Consecutive endoscopy revealed no signs of upper or lower active gastro...We report a case of an 84-year-old male patient with primary small intestinal angiosarcoma.The patient initially presented with anemia and melena.Consecutive endoscopy revealed no signs of upper or lower active gastrointestinal bleeding.The patient had been diagnosed 3 years previously with an aortic dilation,which was treated with a stent.Computed tomography suggested an aorto-intestinal fistula as the cause of the intestinal bleeding,leading to operative stent explantation and aortic replacement.However,an aorto-intestinal fistula was not found,and the intestinal bleeding did not arrest postoperatively.The constant need for blood transfusions made an exploratory laparotomy imperative,which showed multiple bleeding sites,predominately in the jejunal wall.A distal loop jejunostomy was conducted to contain the small intestinal bleeding and a segmental resection for histological evaluation was performed.The histological analysis revealed a lessdifferentiated tumor with characteristic CD31,cytokeratin,and vimentin expression,which led to the diagnosis of small intestinal angiosarcoma.Consequently,the infiltrated part of the jejunum was successfully resected in a subsequent operation,and adjuvant chemotherapy with paclitaxel was planned.Angiosarcoma of the small intestine is an extremely rare malignant neoplasm that presents with bleeding and high mortality.Early diagnosis and treatment are essential to improve outcome.A small intestinal angiosarcoma is a challenging diagnosis to make because of its rarity,nonspecific symptoms of altered intestinal function,nonspecific abdominal pain,severe melena,and acute abdominal signs.Therefore,a quick clinical and histological diagnosis and decisive measures including surgery and adjuvant chemotherapy should be the aim.展开更多
Angiosarcoma is an uncommon malignancy, which spread out from the endothelial cells of vessels. Scalp angiosarcoma with cervical lymph node metastasis is particularly rare. This article describes a rare case of angios...Angiosarcoma is an uncommon malignancy, which spread out from the endothelial cells of vessels. Scalp angiosarcoma with cervical lymph node metastasis is particularly rare. This article describes a rare case of angiosarcoma of the scalp, presenting as neck inflammation. Imaging procedures such as computed tomography （CT）, magnetic resonance image （MRI） and ultrasonography （US） were not sufficient to diagnose this case. A needle biopsy provided an effective and accurate diagnosis of cervical lymph node metastasis. Additional observation and physical examination was required to diagnose the origin of the primary cancerous lesion. Once the angiosarcoma diagnosis was confirmed histologically, sequential weekly and monthly docetaxel （DTX） treatment was effective in preventing reoccurrence. Nonetheless, the optimization of angiosarcoma treatment remains a future goal. Although patients generally describe pain and swelling at the primary lesion site, this patient complained only of painful neck inflammation, without any indication of pain or swelling of the scalp. A revised diagnostic protocol should note that cervical lymph node metastasis of unknown primary origin may result from angiosarcoma of the scalp.展开更多
Splenic tumors are rare. Differentiation of the tumors before operation is of great value regarding the outcome. A case of a 32-year-old man with a splenic inflammatory pseudotumor (IPT) mimicking splenic angiosarcoma...Splenic tumors are rare. Differentiation of the tumors before operation is of great value regarding the outcome. A case of a 32-year-old man with a splenic inflammatory pseudotumor (IPT) mimicking splenic angiosarcoma is described. The tumor was highly suspected of being splenic angiosarcoma based on radiological findings preoperatively. However, after splenectomy, histopathological examinations revealed splenic IPT. Splenic IPT and angiosarcoma are rare and often pose diagnostic difficulties because the clinical and radiological fi ndings are obscure. Due to large differences in prognosis, we briefl y reviewed the clinical, radiological, and pathological features of both of the tumors.展开更多
BACKGROUND Primary hepatic angiosarcoma(PHA)is a rare malignancy with a poor prognosis.It is difficult to diagnose PHA because of the lack of specific symptoms or tumour markers,and it rapidly progresses and has a hig...BACKGROUND Primary hepatic angiosarcoma(PHA)is a rare malignancy with a poor prognosis.It is difficult to diagnose PHA because of the lack of specific symptoms or tumour markers,and it rapidly progresses and has a high mortality.To our knowledge,PHA has not been reported to mimic hepatic sinusoidal obstruction syndrome.Herein,we present a case of PHA manifesting as hepatic sinusoidal obstruction syndrome,diagnosed using transjugular liver biopsy,that resulted in the death of the patient.CASE SUMMARY A 71-year-old man was admitted with the primary complaint of abdominal distension,decreased appetite,fatigue in the previous month,and loss of 10 kg of weight in the past 2 years.Both the liver and spleen were enlarged,and the liver had a medium-hard texture on percussion.Laboratory examinations were performed,and abdominal plain computed tomography(CT)and contrastenhanced CT showed hepatomegaly and splenomegaly,as well as diffuse lowdensity shadows distributed in the liver and spleen.Contrast-enhanced CT revealed diffuse,hypodense,nodular or flake shadows in the liver and heterogeneous enhancement in the spleen.A transjugular liver biopsy was performed.Based on the pathology results,the patient was diagnosed with hepatic sinusoidal obstruction syndrome secondary to PHA.The patient’s status further deteriorated and he developed serious hepatic failure.The patient was discharged,and died 3 d later.CONCLUSION PHA is rare and has a poor prognosis;however,transjugular liver biopsy can be safely performed to aid in diagnosis.展开更多
Background: Primary pulmonary angiosarcoma is extremely rare and the prognosis is very poor. We report a combination therapy of pneumonectomy and intrapleural hypotonic hyperthermic chemotherapy (IPHHC). Case report: ...Background: Primary pulmonary angiosarcoma is extremely rare and the prognosis is very poor. We report a combination therapy of pneumonectomy and intrapleural hypotonic hyperthermic chemotherapy (IPHHC). Case report: A 48-year-old male with exertional dyspnea was found to have a left massive pleural effusion. Bronchoscopic examination displayed endobronchial stenosis of the left lower bronchus B8,9,10?and diagnosed with pulmonary angiosarcoma. Chest computed-tomographic scanning revealed a 5-cm mass in the left inferior lobe, which invaded the left upperbronchus. Intrapleural dissemination and malignant pleural effusion were also suspected (cT3N1M1a,?c-stage IV). As a palliative initial therapy, we performed a pneumonectomy. On the 7th?postoperative day, under general anesthesia, we performed video-assisted IPHHC (43°C, 60 min, 200 mg/m2?of CDDP). After the IPHHC, there was no major adverse event (more than Grade 3) during the postoperative course. Histological examination of the resected specimen revealed a highly-cellular growth of atypical spindle cells with a storiform pattern. By immunohistochemical testing, the tumor cells stained positive for markers including CD31 and the factor VIII related antigen, and the diagnosis of pulmonary angiosarcoma was made. No adjuvant chemoradiotherapy was given, and the postoperative clinical course was uneventful. Although there had been a recurrence in the chest wall, the patient eventually died twenty-one months later. Conclusion: As a new therapeutic option, we performed IPHHC after the pneumonectomy for an advanced pulmonary angiosarcoma with malignant pleural effusion. Although the patient had a recurrence in the chest wall, he had a reasonable postoperative outcome, that is, he returned to a work, retained a good quality of life and had a longer survival in spite of the poor prognosis of the pulmonary angiosarcoma.展开更多
BACKGROUND Primary splenic angiosarcoma(PSA)is an extremely rare and aggressive mesenchymal malignancy with high metastatic potential and a poor prognosis.There are no established treatment guidelines for PSA,even for...BACKGROUND Primary splenic angiosarcoma(PSA)is an extremely rare and aggressive mesenchymal malignancy with high metastatic potential and a poor prognosis.There are no established treatment guidelines for PSA,even for adjuvant therapy.This rare case may provide a reliable therapeutic regime for a better prognosis.CASE SUMMARY A 49-year-old female who complained of right-upper quadrant abdominal pain was diagnosed as having PSA with splenic rupture and liver metastasis.After splenectomy and liver tumor resection,she received sorafenib and camrelizumab therapy.After 15 mo of follow-up,she is in good condition,without recurrence or any identified metastasis.CONCLUSION Immunotherapy combined with targeted therapy could be a potential option for the adjuvant therapy of PSA.展开更多
BACKGROUND Primary hepatic angiosarcoma(PHA)is a rare malignant tumor of the vascular endothelium.Clinical manifestations and laboratory and imaging examinations often lack specificity for PHA.We report a case of PHA,...BACKGROUND Primary hepatic angiosarcoma(PHA)is a rare malignant tumor of the vascular endothelium.Clinical manifestations and laboratory and imaging examinations often lack specificity for PHA.We report a case of PHA,and describe the ultrasound characteristics and characteristic changes in laboratory values associated with PHA.CASE SUMMARY A 75-year-old woman presented with right upper quadrant abdominal pain for half a month.Magnetic resonance imaging(MRI)at a local hospital revealed multiple liver space-occupying lesions,and she was admitted to our hospital for further diagnosis.Contrast-enhanced ultrasound(CEUS)revealed multiple slightly hyperechoic nodules in the liver,which were suspected to be of malignant vascular origin.Contrast-enhanced computed tomography revealed multiple lowdensity nodules in the liver,considered to be metastatic hematopoietic malignancies.Contrast-enhanced MRI showed that the multiple liver nodules shared features with infectious lesions.Laboratory examination revealed normal alpha-fetoprotein levels,slightly increased other liver enzymes,decreased platelets,and significantly increased D-dimer levels.Liver biopsy and histopathology confirmed the presence of PHA.CONCLUSION CEUS can provide valuable clues for the diagnosis of PHA and greatly improve the success rate of puncture biopsy.展开更多
BACKGROUND Angiosarcoma is a highly malignant soft-tissue sarcoma derived from vascular endothelial cells that mainly occurs in the skin and subcutaneous tissues.Smallintestinal angiosarcomas are rare,and the prognosi...BACKGROUND Angiosarcoma is a highly malignant soft-tissue sarcoma derived from vascular endothelial cells that mainly occurs in the skin and subcutaneous tissues.Smallintestinal angiosarcomas are rare,and the prognosis is poor.CASE SUMMARY We reported a case of primary multifocal ileal angiosarcoma and analyze previously reported cases to improve our understanding of small intestinal angiosarcoma.Small intestinal angiosarcoma is more common in elderly and male patients.Gastrointestinal bleeding,anemia,abdominal pain,weakness,and weight loss were the common symptoms.CD31,CD34,factor VIII-related antigen,ETS-related gene,friend leukemia integration 1,and von Willebrand factor are valuable immunohistochemical markers for the diagnosis of small-intestinal angiosarcoma.Small-intestinal angiosarcoma most commonly occurs in the jejunum,followed by the ileum and duodenum.Radiation and toxicant exposure are risk factors for angiosarcoma.After a definite diagnosis,the mean and median survival time was 8 mo and 3 mo,respectively.Kaplan-Meier survival analysis showed that age,infiltration depth,chemotherapy,and the number of small intestinal segments invaded by tumor lesions were prognostic factors for small intestinal angiosarcoma.Multivariate Cox regression analysis showed that chemotherapy and surgery significantly improved patient prognosis.CONCLUSION Angiosarcoma should be considered for unexplained melena and abdominal pain,especially in older men and patients with a history of radiation exposure.Prompt treatment,including surgery and adjuvant chemotherapy,is essential to prolonging patient survival.展开更多
Angiosarcoma is a rare tumor that account for less than 1% of all sarcomas. Although hepatic angiosarcoma usually presents with unspecific symptoms, it rapidly progresses and has a high mortality. We report a rare cas...Angiosarcoma is a rare tumor that account for less than 1% of all sarcomas. Although hepatic angiosarcoma usually presents with unspecific symptoms, it rapidly progresses and has a high mortality. We report a rare case of primary hepatic angiosarcoma manifested as recurrent hemoperitoneum.展开更多
基金Supported by Clinical Research Grant from Pusan National University Hospital in 2024.
文摘BACKGROUND Primary splenic lesions are rare and often detected incidentally through imaging,biopsy,or autopsy,typically without distinct clinical symptoms.Although imaging can help differentiate benign from malignant lesions,splenic hamartomas,and angiosarcomas may exhibit overlapping features,making diagnosis challenging.This report presents a case of splenic hamartoma suspected to be an angiosarcoma based on preoperative imaging.Splenic hamartomas that mimic angiosarcomas are exceedingly rare.CASE SUMMARY A 33-year-old male presented to the Department of Emergency with frank red blood hematemesis and a 1-week history of epigastric pain.On arrival,he was alert and hemodynamically stable.Contrast-enhanced abdominal computed tomography revealed splenomegaly with significant engorgement of the portal and splenic veins,along with a diffuse nodular splenic lesion measuring 8.2 cm×6.2 cm.Following esophageal varix ligation,abdominal magnetic resonance imaging demonstrated iso-to high-signal intensity within the splenic mass and multiple hypervascular lesions in the right hepatic lobe,raising suspicion for splenic an-giosarcoma with hepatic metastases.18F-fluorodeoxyglucose positron emission tomography-computed tomography showed diffusely mild increased metabolic activity in the spleen.The patient subsequently underwent splenectomy and liver biopsy.Histopathological examination revealed chronic inflammation in the liver,and the splenic lesion was confirmed to be a splenic hamartoma.The patient successfully returned to work and remains in good health.CONCLUSION This rare case of splenic hamartoma mimicking angiosarcoma highlights the importance of differential diagnosis in managing splenic tumors.
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AIM: To investigate the expression of ERG, CD34, CD31 (PECAM-1, platelet/endothelial cell adhesion molecule 1) and factor VIII-related antigen (FVIIIRAg) in the diagnosis of hepatic angiosarcoma patients.
基金Department of Gastroenterology,the First Affiliated Hospital of Zhejiang Chinese Medical University,Hangzhou,Zhejiang Province,China
文摘Primary splenic angiosarcoma(PSA) is the most unusual type of malignancy with early multifocal metastasis through hematogenous spread. PSA is generally believed to originate from splenic sinusoidal vascular endothelium with a high rate of metastasis and to have a poor prognosis. Its etiology and pathogenetic mechanisms have not yet been clearly described. Thus far, only approximately 200 cases have been reported. PSA has variable symptomatology with the potential to present with life-threatening complications. The diagnosis of PSA is challenging; and often late. PSA should be considered in the differential diagnosis of patients with splenomegaly and anemia of unknown etiology. Surgical treatment with splenectomy is considered the only curative intervention for potential long-term disease-free survival. Early diagnosis and treatment are very important. It is important that clinical doctors improve the understanding of PSA. Herein, we report one rare case of PSA with hepatic metastases, along with a review of the current literature.
文摘Background： Hepatic angiosarcoma is a rare malignant vascular tumor presenting unique treatment challenges.The aim of the present study was to determine the treatment and prognosis of this entity.Data sources： A systematic literature search was conducted using PubMed, Embase and Chinese BiomedicalLiterature database, to identify articles published from January 1980 to July 2017. Search termswere “hepatic angiosarcoma” and “liver angiosarcoma”. Additional articles were retrieved through manualsearch of bibliographies of the relevant articles. Pooled individual data concerning the prognosis followingvarious therapeutic modalities were analyzed.
基金Supported by The First Affiliated Hospital of Nanchang University,Jiangxi Province,China
文摘Primary splenic angiosarcoma(PSA) is an unusual and highly malignant vascular tumour with a high rate of metastatic. Moreover, the research on prognosis of the disease is poor. The epidemiology, etiology, clinical diagnosis and treatment of the disease remain challenging, because case reports of the disease are few in number. In accordance with other malignant tumors, PSA is very aggressive, and the majority of patients in which this disease is found are at an advanced stage. Almost all patients die within 12 mo of diagnosis irrespective of treatment. We report here a woman who had complained of upper bellyache and anorexia for 10 d. Magnetic resonance imaging showed enlargement of the spleen with multiple heterogeneous masses in the lower pole of the spleen. A hand-assisted laparoscopic splenectomy was performed which allowed histopathologic diagnosis. The patient was diagnosed with PSA and liver metastasis, and succumbed to the disease 35 d after surgery. The literature was finished combined with the clinical features, diagnosis and management of PSA.
基金supported by the Wu Jieping Medical Foundation Grant (No. 320.6750.11074)
文摘Bevacizumab, an angiogenesis inhibitor, is a recombined humanized monoclonal antibody against vascular endothelial growth factor and a promising therapeutic option for angiosarcoma management. This is a ease report and review of the literature using bevacizumab and combination chemotherapy for angiosarcoma. The understanding of the effectiveness of combined therapy of bevacizumab and chemotherapy agents is still limited. The benefits of bevacizumab treatment for angiosarcoma will need to be weighed against the risks of venous thromboembolism in this population.
文摘Primary malignant tumors of the small intestine are rare, comprising less than 2% of all gastrointestinal tumors. An 85-year-old woman was admitted with fever of 40  °C and marked abdominal distension. Her medical history was unremarkable, but blood examination showed elevated inflammatory markers. Abdominal computed tomography showed a giant tumor with central necrosis, extending from the epigastrium to the pelvic cavity. Giant gastrointestinal stromal tumor of the small intestine communicating with the gastrointestinal tract or with superimposed infection was suspected. Because no improvement occurred in response to antibiotics, surgery was performed. Laparotomy revealed giant hemorrhagic tumor adherent to the small intestine and occupying the peritoneal cavity. The giant tumor was a solid tumor weighing 3490 g, measuring 24 cm × 17.5 cm × 18 cm and showing marked necrosis. Histologically, the tumor comprised spindle-shaped cells with anaplastic large nuclei. Immunohistochemical studies showed tumor cells positive for vimentin, CD31, and factor VIII-related antigen, but negative for c-kit and CD34. Angiosarcoma was diagnosed. Although no postoperative complications occurred, the patient experienced enlargement of multiple metastatic tumors in the abdominal cavity and died 42 d postoperatively. The prognosis of small intestinal angiosarcoma is very poor, even after volume-reducing palliative surgery.
文摘Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulated coagulopathy that can be seen in hepatic angiosarcoma fulfills the clinical diagnostic criteria of disseminated intravascular coagulation. However, the mechanism that governs this coagulopathy has been poorly understood. This case report provides histological evidence of the consumption of coagulation factors along with trapped platelets occurring within the tumor, which is the foundation for the concept of Kasabach-Merritt syndrome(KMS). KMS is characterized by thrombocytopenia and hyperconsumption of coagulation factors within a vascular tumor. However, KMS associated with angiosarcoma has not been well recognized. This case report describes, for the first time, the histological evidence of KMS that occurred in an extremely rare mesenchymal malignant tumor of the liver.
文摘Primary hepatic angiosarcoma is the most common malignant mesenchymal tumor of the liver. It has a poor prognosis and various appearances on magnetic resonance(MR) images. We report a case of hepatic angiosarcoma with a characteristic appearance on gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid(Gd-EOB-DTPA)-enhanced MR imaging in the hepatobiliary phase. A 72-year-old man was admitted with a complaint of abdominal pain. Gd-EOBDTPA-enhanced MR imaging revealed a liver tumor that showed slight hyperintensity in the hepatobiliary phase. These findings suggested Gd-EOB-DTPA uptake in the tumor. An autopsy revealed the solid proliferation and sinusoidal spreading of hepatic angiosarcoma cells. Immunohistochemistry indicated that the tumor was negative for OATP1B3. Gd-EOB-DTPA uptake in the liver tumor in the hepatobiliary phase suggested sinusoidal tumor invasion with residual normal hepatocytes.
文摘Angiosarcoma is a rare primary malignant neoplasm of the liver with a poor prognosis. Here, we report a case of a patient with a ruptured hepatic angiosarcoma which was treated by emergency catheter-directed embolization, followed by leflc-sided hemihepatectomy.
文摘We report a case of an 84-year-old male patient with primary small intestinal angiosarcoma.The patient initially presented with anemia and melena.Consecutive endoscopy revealed no signs of upper or lower active gastrointestinal bleeding.The patient had been diagnosed 3 years previously with an aortic dilation,which was treated with a stent.Computed tomography suggested an aorto-intestinal fistula as the cause of the intestinal bleeding,leading to operative stent explantation and aortic replacement.However,an aorto-intestinal fistula was not found,and the intestinal bleeding did not arrest postoperatively.The constant need for blood transfusions made an exploratory laparotomy imperative,which showed multiple bleeding sites,predominately in the jejunal wall.A distal loop jejunostomy was conducted to contain the small intestinal bleeding and a segmental resection for histological evaluation was performed.The histological analysis revealed a lessdifferentiated tumor with characteristic CD31,cytokeratin,and vimentin expression,which led to the diagnosis of small intestinal angiosarcoma.Consequently,the infiltrated part of the jejunum was successfully resected in a subsequent operation,and adjuvant chemotherapy with paclitaxel was planned.Angiosarcoma of the small intestine is an extremely rare malignant neoplasm that presents with bleeding and high mortality.Early diagnosis and treatment are essential to improve outcome.A small intestinal angiosarcoma is a challenging diagnosis to make because of its rarity,nonspecific symptoms of altered intestinal function,nonspecific abdominal pain,severe melena,and acute abdominal signs.Therefore,a quick clinical and histological diagnosis and decisive measures including surgery and adjuvant chemotherapy should be the aim.
文摘Angiosarcoma is an uncommon malignancy, which spread out from the endothelial cells of vessels. Scalp angiosarcoma with cervical lymph node metastasis is particularly rare. This article describes a rare case of angiosarcoma of the scalp, presenting as neck inflammation. Imaging procedures such as computed tomography （CT）, magnetic resonance image （MRI） and ultrasonography （US） were not sufficient to diagnose this case. A needle biopsy provided an effective and accurate diagnosis of cervical lymph node metastasis. Additional observation and physical examination was required to diagnose the origin of the primary cancerous lesion. Once the angiosarcoma diagnosis was confirmed histologically, sequential weekly and monthly docetaxel （DTX） treatment was effective in preventing reoccurrence. Nonetheless, the optimization of angiosarcoma treatment remains a future goal. Although patients generally describe pain and swelling at the primary lesion site, this patient complained only of painful neck inflammation, without any indication of pain or swelling of the scalp. A revised diagnostic protocol should note that cervical lymph node metastasis of unknown primary origin may result from angiosarcoma of the scalp.
文摘Splenic tumors are rare. Differentiation of the tumors before operation is of great value regarding the outcome. A case of a 32-year-old man with a splenic inflammatory pseudotumor (IPT) mimicking splenic angiosarcoma is described. The tumor was highly suspected of being splenic angiosarcoma based on radiological findings preoperatively. However, after splenectomy, histopathological examinations revealed splenic IPT. Splenic IPT and angiosarcoma are rare and often pose diagnostic difficulties because the clinical and radiological fi ndings are obscure. Due to large differences in prognosis, we briefl y reviewed the clinical, radiological, and pathological features of both of the tumors.
基金Supported by Tianjin Science and Technology Plan Project,No.19ZXDBSY00030Beijing iGandan Foundation,No.RGGJJ-2021-014.
文摘BACKGROUND Primary hepatic angiosarcoma(PHA)is a rare malignancy with a poor prognosis.It is difficult to diagnose PHA because of the lack of specific symptoms or tumour markers,and it rapidly progresses and has a high mortality.To our knowledge,PHA has not been reported to mimic hepatic sinusoidal obstruction syndrome.Herein,we present a case of PHA manifesting as hepatic sinusoidal obstruction syndrome,diagnosed using transjugular liver biopsy,that resulted in the death of the patient.CASE SUMMARY A 71-year-old man was admitted with the primary complaint of abdominal distension,decreased appetite,fatigue in the previous month,and loss of 10 kg of weight in the past 2 years.Both the liver and spleen were enlarged,and the liver had a medium-hard texture on percussion.Laboratory examinations were performed,and abdominal plain computed tomography(CT)and contrastenhanced CT showed hepatomegaly and splenomegaly,as well as diffuse lowdensity shadows distributed in the liver and spleen.Contrast-enhanced CT revealed diffuse,hypodense,nodular or flake shadows in the liver and heterogeneous enhancement in the spleen.A transjugular liver biopsy was performed.Based on the pathology results,the patient was diagnosed with hepatic sinusoidal obstruction syndrome secondary to PHA.The patient’s status further deteriorated and he developed serious hepatic failure.The patient was discharged,and died 3 d later.CONCLUSION PHA is rare and has a poor prognosis;however,transjugular liver biopsy can be safely performed to aid in diagnosis.
文摘Background: Primary pulmonary angiosarcoma is extremely rare and the prognosis is very poor. We report a combination therapy of pneumonectomy and intrapleural hypotonic hyperthermic chemotherapy (IPHHC). Case report: A 48-year-old male with exertional dyspnea was found to have a left massive pleural effusion. Bronchoscopic examination displayed endobronchial stenosis of the left lower bronchus B8,9,10?and diagnosed with pulmonary angiosarcoma. Chest computed-tomographic scanning revealed a 5-cm mass in the left inferior lobe, which invaded the left upperbronchus. Intrapleural dissemination and malignant pleural effusion were also suspected (cT3N1M1a,?c-stage IV). As a palliative initial therapy, we performed a pneumonectomy. On the 7th?postoperative day, under general anesthesia, we performed video-assisted IPHHC (43°C, 60 min, 200 mg/m2?of CDDP). After the IPHHC, there was no major adverse event (more than Grade 3) during the postoperative course. Histological examination of the resected specimen revealed a highly-cellular growth of atypical spindle cells with a storiform pattern. By immunohistochemical testing, the tumor cells stained positive for markers including CD31 and the factor VIII related antigen, and the diagnosis of pulmonary angiosarcoma was made. No adjuvant chemoradiotherapy was given, and the postoperative clinical course was uneventful. Although there had been a recurrence in the chest wall, the patient eventually died twenty-one months later. Conclusion: As a new therapeutic option, we performed IPHHC after the pneumonectomy for an advanced pulmonary angiosarcoma with malignant pleural effusion. Although the patient had a recurrence in the chest wall, he had a reasonable postoperative outcome, that is, he returned to a work, retained a good quality of life and had a longer survival in spite of the poor prognosis of the pulmonary angiosarcoma.
基金Supported by the Natural Science Foundation of Jiangxi Province (No. 20192BAB215012 and No. 20212BAB206027)the Health Commission of Jiangxi Province (No. 20203206)
文摘BACKGROUND Primary splenic angiosarcoma(PSA)is an extremely rare and aggressive mesenchymal malignancy with high metastatic potential and a poor prognosis.There are no established treatment guidelines for PSA,even for adjuvant therapy.This rare case may provide a reliable therapeutic regime for a better prognosis.CASE SUMMARY A 49-year-old female who complained of right-upper quadrant abdominal pain was diagnosed as having PSA with splenic rupture and liver metastasis.After splenectomy and liver tumor resection,she received sorafenib and camrelizumab therapy.After 15 mo of follow-up,she is in good condition,without recurrence or any identified metastasis.CONCLUSION Immunotherapy combined with targeted therapy could be a potential option for the adjuvant therapy of PSA.
文摘BACKGROUND Primary hepatic angiosarcoma(PHA)is a rare malignant tumor of the vascular endothelium.Clinical manifestations and laboratory and imaging examinations often lack specificity for PHA.We report a case of PHA,and describe the ultrasound characteristics and characteristic changes in laboratory values associated with PHA.CASE SUMMARY A 75-year-old woman presented with right upper quadrant abdominal pain for half a month.Magnetic resonance imaging(MRI)at a local hospital revealed multiple liver space-occupying lesions,and she was admitted to our hospital for further diagnosis.Contrast-enhanced ultrasound(CEUS)revealed multiple slightly hyperechoic nodules in the liver,which were suspected to be of malignant vascular origin.Contrast-enhanced computed tomography revealed multiple lowdensity nodules in the liver,considered to be metastatic hematopoietic malignancies.Contrast-enhanced MRI showed that the multiple liver nodules shared features with infectious lesions.Laboratory examination revealed normal alpha-fetoprotein levels,slightly increased other liver enzymes,decreased platelets,and significantly increased D-dimer levels.Liver biopsy and histopathology confirmed the presence of PHA.CONCLUSION CEUS can provide valuable clues for the diagnosis of PHA and greatly improve the success rate of puncture biopsy.
文摘BACKGROUND Angiosarcoma is a highly malignant soft-tissue sarcoma derived from vascular endothelial cells that mainly occurs in the skin and subcutaneous tissues.Smallintestinal angiosarcomas are rare,and the prognosis is poor.CASE SUMMARY We reported a case of primary multifocal ileal angiosarcoma and analyze previously reported cases to improve our understanding of small intestinal angiosarcoma.Small intestinal angiosarcoma is more common in elderly and male patients.Gastrointestinal bleeding,anemia,abdominal pain,weakness,and weight loss were the common symptoms.CD31,CD34,factor VIII-related antigen,ETS-related gene,friend leukemia integration 1,and von Willebrand factor are valuable immunohistochemical markers for the diagnosis of small-intestinal angiosarcoma.Small-intestinal angiosarcoma most commonly occurs in the jejunum,followed by the ileum and duodenum.Radiation and toxicant exposure are risk factors for angiosarcoma.After a definite diagnosis,the mean and median survival time was 8 mo and 3 mo,respectively.Kaplan-Meier survival analysis showed that age,infiltration depth,chemotherapy,and the number of small intestinal segments invaded by tumor lesions were prognostic factors for small intestinal angiosarcoma.Multivariate Cox regression analysis showed that chemotherapy and surgery significantly improved patient prognosis.CONCLUSION Angiosarcoma should be considered for unexplained melena and abdominal pain,especially in older men and patients with a history of radiation exposure.Prompt treatment,including surgery and adjuvant chemotherapy,is essential to prolonging patient survival.
文摘Angiosarcoma is a rare tumor that account for less than 1% of all sarcomas. Although hepatic angiosarcoma usually presents with unspecific symptoms, it rapidly progresses and has a high mortality. We report a rare case of primary hepatic angiosarcoma manifested as recurrent hemoperitoneum.
