Angiosarcoma of the gallbladder: case report and review of the literature.

A 62-year-old white woman with an unremarkable past medical history presented with acute cholecystitis. A cholecystectomy was performed, revealing an acute hemorrhagic and chronic cholecystitis associated with cholelithiasis. Two months after the operation, the patient developed a massive hemoperitoneum and died by hypo-volemic shock. At autopsy, an angiosarcoma measuring 5 cm in diameter was found in the liver, at the site of the gallbladder fossa. There were multiple hepatic, splenic, ovarian and peritoneal metastases and a massive hemoperitoneum consisting of 8 L of blood and blood clots. Review of the tissue sections from the patient's gallbladder confirmed the presence of an acute hemorrhagic and chronic cholecystitis and also revealed residual foci of an angiosarcoma. A review of eight previously reported cases of gallbladder angiosarcoma is also presented.     

Primary liposarcoma of gallbladder diagnosed by preoperative imagings： A case report and review of literature

A 49-year-old Japanese woman was referred to our department because of high fever and a huge abdominal mass. Computed tomography (CT) and magnetic resonance (MR) imagings revealed a tumor, about 30 cm in diameter, occupied the right hepatic lobe and the peritoneal cavity. Abdominal angiography showed that the tumor was fed mainly by the cystic artery. We pre-operatively diagnosed angiosarcoma of the gallbladder and performed tumor resection with cholecystectomy because the tumor was almost casplated, however the posterior wall of the gallbladder attached to the tumor firmly. Histologically, the tumor was composed of spindle cells including lipoblasts with cellular pleomorphism, which were also detected in the muscular layer of the gallbladder. We finally diagnosed pleomorphic liposarco-ma of the gallbladder. At 10 mo and 29 mo after the first operation, she underwent two more operations because of recurrence. Now she has a good quality of life 3 years and 6 mo after the first operation.     

Gallbladder Sarcoma: A Clinicopathological Study of Seven Cases from the UK and Austria with Emphasis on Morphological Subtypes

Background Primary sarcoma of the gallbladder (PGBS) is rare, with only 40 cases reported in the literature. Most of these have been diagnosed as leiomyosarcoma. We aimed to evaluate the histological features of a case series of this rare tumor and correlate these with clinical features. Design Cases recorded as “gallbladder sarcoma” from different institutes were reviewed and the clinicopathological features of these cases were recorded. Only primary gallbladder wall mesenchymal tumors were included. Epithelial tumors, mixed tumors (carcinosarcoma or sarcomatoid carcinoma), and tumors extending into the gallbladder from the abdomen or sarcoma with other known primaries were specifically excluded. Result PGBS occurred in one male and six females with a median age of 70 (range 64–82) years. Patients presented with acute or chronic cholecystitis, abdominal pain, weight loss, and pruritis. They were generally found to have elevated alkaline phosphatase and bilirubin, and leukocytosis. Tumors ranged from 1.1 to 4 cm with a median size of 3 cm. Most PGBS arose in the body but one arose in the fundus. All tumors were associated with ulcerated mucosa. Based on morphological and immunohistochemical features of the PGBS, there were three myxofibrosarcomas (malignant fibrous histiocytoma, MFH, storiform pleomorphic), one leiomyosarcoma (LMS), one angiosarcoma (AS), and two liposarcomas (LS). All patients received cholecystectomy and three received adjuvant chemotherapy. Follow-up revealed that six patients died of the disease 6 weeks to 2 years after diagnosis and one died of unrelated causes. Conclusion PGBS are rare and mainly occur in the gallbladder body in middle-aged females. They generally present with acute cholecystitis and have a very poor prognosis. A variety of sarcoma types are found with MFH being the predominant variant. E. A. Husain and R. J. Prescott contributed equally to this paper.     

Primary pulmonary angiosarcoma

Pulmonary angiosarcoma is an uncommon vascular and usually secondary tumor. Only a few primary cases of pulmonary angiosarcoma have been described. We report a case of primary pulmonary angiosarcoma in an adult man who presented with hemoptysis. Chest x-ray film and chest CT showed a right para-cardiac opacity associated with diffuse alveolar consolidation of the right basal lobe. Right inferior lobectomy has been performed. Pulmonary angiosarcoma was diagnosed on histological and immuno-histochemical studies of the operative specimen. The primitive character was retained after ruling out all other tumor localizations. The clinical outcome was rapidly fatal. This observation is added to the other rare cases published of primitive pulmonary angiosarcoma. It confirms the poor prognosis and the extremely hemorrhagic nature of this tumor. Isolated necrotic parenchymatous mass was an original radiological pattern observed in this patient.     

Angiosarcomas of the bilateral breast and heart: which one is the primary site?

A 29-year-old pregnant woman with recurrent pericardial effusion and a cardiac tumor, diagnosed as an angiosarcoma, was treated with surgical resection of the tumor followed by radiotherapy. Immediately after completion of radiotherapy, she developed bilateral breast masses, which were also confirmed as angiosarcomas. We thought this might be the first case of bilateral angiosarcoma of the breast metastasizing to heart mimicking a primary cardiac angiosarcoma, although we could not conclude with certainty that angiosarcoma of the heart was not the primary site.     

Massive bleeding after fine needle aspiration of liver angiosarcoma

Open or closed biopsy of liver angiosarcoma is a life-threatening procedure. A case of massive bleeding after fine needle aspiration of the liver is reported in a patient having an angiosarcoma of the liver and spleen. Fine needle aspiration seems a hazardous procedure in this disease.     

Small-bowel angiosarcoma after pelvic irradiation: a report of two cases

Angiosarcoma is a rare tumor in the small bowel. Several predisposing factors have been suggested, including external irradiation for another malignancy. Only six cases of small-bowel angiosarcoma in patients previously treated with pelvic irradiation for gynecological malignancies have been reported hitherto. We present here two patients in whom a diagnosis of small-bowel angiosarcoma was made 10 years or more after intrapelvic irradiation. These cases demonstrate poor prognosis in this disease. Although postirradiation angiosarcoma is a rare condition, its possibility should be kept in mind when operating on previously irradiated patients. Accepted: 3 November 1999     

Spontaneous ruptured primary hepatic angiosarcoma coincident with Schistosoma Japonica liver fibrosis

Aim: We report herein a case of spontaneous ruptured primary hepatic angiosarcoma coincident with Schistosoma Japonica liver fibrosis and review the correlative literature. Methods: The resected specimen was examined by histopathological and immunohistochemical evaluation. Results: The final diagnosis was spontaneous ruptured primary hepatic angiosarcoma coincident with Schistosoma Japonica liver fibrosis Conclusion: Considering the nature of primary hepatic angiosarcoma, in particular the ruptured hepatic angiosarcoma, it is obviously desirable to avoid any unnecessary delay or definitive surgical treatment. It is presumed that angiosarcoma in the liver has a possible association with S. japonicum and the deposition of ovae in liver.     

Epithelioid angiosarcoma of the small bowel

BACKGROUND: Angiosarcoma is a rare soft-tissue neoplasm that occurs most often in the skin and the subcutaneous tissues but very rarely in the GI tract. DESIGN: We report a case of multifocal epithelioid angiosarcoma involving skin and bowel and review the 18 previously reported cases of epithelioid intestinal angiosarcoma. MAIN OUTCOME MEASUREMENTS: Our patient presented with weakness from anemia. Two small blue-black nodules were present on the skin. Fecal occult blood tests were positive, but gastroscopy and colonoscopy showed normal results. Enteroscopy revealed small ulcerated nodules in the distal duodenum and the proximal jejunum. Biopsy specimens of skin and duodenal nodules showed epithelioid angiosarcoma. RESULTS: The intestinal nodules were treated with argon plasma coagulation, but the patient died 6 weeks after diagnosis. CONCLUSIONS: The survival rate of intestinal angiosarcoma is poor, and most cases progress rapidly, with a 2-month median survival after diagnosis. Because of the infiltrative and the multifocal nature of this malignancy, complete surgical excision is often not possible. Further studies are needed to establish the role of adjuvant radiation or chemotherapy in the treatment of angiosarcoma.     

Successful treatment of primary pulmonary angiosarcoma

Angiosarcoma in the lung is an uncommon disorder and is usually attributable to metastasis from a primary site. Primary pulmonary angiosarcoma is extremely rare, and the prognosis of affected individuals is dismal, with most patients dying within months of presentation. Indeed, there have been no reported instances of successful treatment of this condition. We now report the case of a patient with primary pulmonary angiosarcoma who responded to a combination of radiotherapy and immunotherapy with recombinant interleukin-2. The patient remains well without signs of recurrence 1 year after initial presentation. This combination therapy may be a promising strategy to prolong the survival of patients with primary pulmonary angiosarcoma.     

Right coronary artery-right atrium fistula in primary angiosarcoma of the heart.

We report a case of angiosarcoma of the heart, manifested as a continuous murmur. Right coronary arteriography disclosed a paracardiac mass with fistulas from the coronary vessel to the right atrium. Histologic study revealed this to be an angiosarcoma with sinusoidal pattern. To our knowledge, this is the first case of this kind of cardiac tumor presenting as a fistula from a coronary artery to the right atrium.     

Hepatic angiosarcoma associated with short-term arsenic ingestion

In a 45 year old man, hepatic angiosarcoma developed 33 years after medicinal arsenic ingestion of only six months' duration. In addition, the patient had a history of basal cell carcinomas without having had excessive sunlight exposure. Factor VIII staining of the tumor supports an endothelial cell origin of the tumor. The development of skin cancers and hepatic angiosarcoma in this patient suggests that there may be no safe threshold of arsenic exposure. A careful history concerning even short-term arsenic exposure in all patients with hepatic angiosarcoma may uncover more examples of this association.     

A case of hepatic angiosarcoma surviving for more than 16 months after hepatic resection

Hepatic angiosarcoma is a non-epithelial malignant tumor and a rare primary neoplasm accounting for only a 0.1% of primary liver malignant tumors. Hepatic angiosarcoma progresses rapidly; therefore, most cases are discovered at an advanced stage, and less than 20% of the patients have received surgery. The lack of specific symptoms and radiological findings leads to the delay of diagnosis resulting in the poor prognosis. To the best of our knowledge, only three patients have been reported to survive for more than one year after hepatic resection for angiosarcoma. We herein report a patient with hepatic angiosarcoma, 4 cm in size, who underwent hepatic resection after confirming the diagnosis as angiosarcoma by the intraoperative frozen section examination. The patient is still alive for more than 16 months postoperatively without any signs of recurrence.     

Diffuse liver angiosarcoma and cerebral cavernous angiomas in a young patient

A case of a thirty-nine year old woman with cerebral cavernous angiomas who developed anaemia and thrombocytopenia secondary to diffuse liver angiosarcoma is reported. This unique association of liver angiosarcoma and cerebral cavernous angiomas may suggest that this tumour may potentially develop from benign vascular lesions. Hematologic abnormalities in angiosarcomas are moreover reviewed based on recent literature search.     

Pseudomesotheliomatous angiosarcoma of the chest wall and pleura

Angiosarcoma is a rare soft tissue sarcoma that usually occurs in deep soft tissues, breast, spleen, liver, and bone. Primary thoracic parietal localization of angiosarcoma is rare and prognosis is poor. In this report, we present the CT and PET features of a patient with pseudomesotheliomatous angiosarcoma of the chest wall and pleura, which, to the best of our knowledge, have not previously been described.     

The clinical features of hepatic angiosarcoma: a report of four cases and a review of the English literature.

Four cases of hepatic angiosarcoma are reported with a review of 99 other cases in the English literature. Angiosarcoma of the liver is associated with chronic exposure to thorotrast, vinyl chloride, arsenicals, radium and possibly copper and with chronic idiopathic hemochromatosis. Although 40% of patients have hepatic fibrosis or cirrhosis at autopsy, the nature of the association between chronic liver disease and hepatic angiosarcoma is unknown. The clinical presentation of hepatic angiosarcoma is nonspecific with abdominal pain, weakness and weight loss common complaints and with hepatomegaly, ascites and jaundice common findings. Liver function tests are usually abnormal but there is no one liver function test or set of tests specific for the tumor. The occurrence of thrombocytopenia and disseminated intravascular coagulation is characteristic of hepatic angiosarcoma and may be related to local consumption of clotting factors and formed blood elements in the tumor. Catastrophic intraabdominal bleeding is also characteristic and occurs in one-fourth of all cases. This complication is likely related to the high incidence of clotting abnormalities and the vascular nature of the neoplasm. Selective hepatic arteriogram and open liver biopsy are the foundations of diagnostic evaluation. Percutaneous liver biopsy should be avoided. Failure to appreciate the possibility of hepatic angiosarcoma in the proper clinical setting, leading to blind percutaneous biopsy, may result in failure to make the diagnosis at the cost of significant morbidity and mortality. Survival of patients with hepatic angiosarcoma is brief; only 3% live longer than 2 years. Treatment of the tumor to date is empirical. There are probably a few patients who might benefit from radical surgery with curative intent. For all others chemotherapy is indicated. Adriamycin is active against hepatic angiosarcoma, but optimal dose and mode of administration require further investigation. Further study is also required to delineate the cause of hepatic angiosarcoma in the 60% of cases without definite epidemiologic association.     

Multicentric Epithelioid Angiosarcoma of the Bone

ABSTRACT: Epithelioid angiosarcoma of the bone represents a challenging diagnosis by bone marrow biopsy. We present a case of a multicentric high grade angiosarcoma of the bone with epithelioid features. On the basis of the clinical presentation, the radiological findings, and the appearance of loosely clustered tumor cells detected in the initial bone marrow biopsy, the main differential diagnoses considered were a poorly differentiated non-secretory multiple myeloma and metastatic carcinoma. Subsequent morphologic, immunohistochemical and electron microscopic examination of tissue samples clarified the nature of the tumor as epithelioid angiosarcoma. We discuss potential pitfalls in clinical and morphological diagnosis. The strong reactivity of the tumor cells with the nonspecific but ubiquitous mesenchymal marker vimentin in similar cases should direct early attention to the rare malignant bone tumor, epithelioid angiosarcoma, with subsequent confirmation of this diagnosis with specific immunohistochemical endothelial cell markers and/or electron microscopy.     

Angiosarcoma of the lung

Angiosarcoma is a rare malignant vascular tumor. Pulmonary involvement is usually attributable to metastasis from other primary sites, primary pulmonary angiosarcoma therefore being quite uncommon. We report a case of angiosarcoma with pulmonary involvement, probably primary to the lung, which had gone untreated for more than two years. We describe this rare neoplasm and its growth, as well as the extensive local invasion and hematogenous metastasis at presentation. We also discuss its poor prognosis.     

Primary Angiosarcoma of the Pulmonary Trunk Mimicking Pulmonary Thromboembolism

We present a case of primary angiosarcoma of the pulmonary trunk that was initially misdiagnosed as a subacute massive pulmonary thromboembolism in a 26‐year‐old woman. This is an extremely rare disease that is usually indistinguishable from acute or chronic thromboembolic disease of the pulmonary arteries because the clinical and radiologic findings of pulmonary artery angiosarcoma are similar to those of pulmonary thromboembolism. Although the incidence of pulmonary artery angiosarcoma is very low, our case demonstrates that this disease entity should be included in the differential diagnosis of pulmonary thromboembolism, especially in patients who do not respond to anticoagulant therapy or present with no identifiable source of thromboembolic events. (ECHOCARDIOGRAPHY 2010;27:E23‐E26)