Splenic Angiosarcoma

Abstract: Background and Objectives Primary splenic angiosarcoma is a very rare and aggressive neoplasm with a high metastatic rate and dismal prognosis. Since only a few cases have been adequately reported in the medical literature, we report here a further six cases. Methods The records of all cases of primary splenic angiosarcoma treated at Chang Gung Memorial Hospital from April 1991 to July 2004 were retrospectively reviewed. Results Of the six cases identified (three men and three women; range, 7–69 years; median, 44 years), four presented with palpable abdominal masses or left upper quadrant abdominal pain. Other systemic symptoms, such as bleeding gums, fatigue, fever, body weight loss, and gastrointestinal bleeding were noted. An abnormal hematogram was found in five patients, with all of these five showing anemia, and three thrombocytopenia. Splenomegaly was present in all patients. Hemoperitoneum due to splenic rupture was noted in one patient. Three patients had distant metastasis to the liver (n = 2), bone (n = 1), bone marrow (n = 1), and small bowel (n = 1) at diagnosis. Liver (n = 3), bone (n = 1), and bone marrow (n = 1) metastases were found in four patients after initial therapy. Five of the six cases underwent a splenectomy, one underwent partial hepatectomy, and one received chemotherapy. The respective 1-, 3- and 5-year survival rates were 60%, 40%, and 40%, and the median survival time was 36 months. One patient was disease-free 162 months after splenectomy. Conclusions The clinical presentations of splenic angiosarcoma were similar to those of previous reports apart from the higher rate of splenomegaly observed in this study. In contrast to reported pediatric cases, our patient achieved long-term disease-free survival after splenectomy alone. J. Surg. Oncol. 2005;92:312–316. © 2005 Wiley-Liss, Inc.

Abstract: OBJECTIVE. Splenic lesions are commonly encountered and are often incidental in nature. Benign splenic vascular neoplasms include hemangioma, hamartoma, lymphangioma, extra-medullary hematopoiesis (EMH), and sclerosing angiomatoid nodular transformation (SANT). Uncommonly encountered entities of the spleen include focal EMH, focal myeloma, angiomyolipoma, and SANT. Primary splenic angiosarcoma is the most common malignant nonhematolymphoid malignancy of the spleen. Lymphoma, myeloma, and metastases are the other malignant entities involving the spleen. The clinical presentation, key imaging findings, and associations of benign, neoplastic, and malignant diseases that can involve the spleen will be discussed. CONCLUSION. Radiologists can use multimodality imaging to diagnose entities involving the spleen by recognizing key imaging features and considering patient characteristics. However, biopsy may be warranted for definitive diagnosis when imaging findings are nonspecific.

Abstract: Maffucci's syndrome is classically defined as the association of multiple enchondromas and hemangiomas. Spindle-cell hemangioendothelioma (SCH), a recently described vascular tumor of purported low malignant potential, has both cavernous hemangioma and Kaposi-like features. We report six patients with Maffucci's syndrome in whom all vascular lesions were SCH. The enchondromas involved the small and long tubular bones of the extremities in all of these patients; flat bones were also involved in three patients. The SCH usually arose in the extremities, distal to the knees and elbows. Five of the six patients had multiple and separate nodules of SCH, and in four patients there was recurrent or persistent SCH within 6 months to 4 years after initial removal. One patient also had a vascular tumor in the spleen mainly with features of a low-grade angiosarcoma with separate SCH-like foci. None of the SCH have metastasized within a follow-up period averaging 20 years. Five patients are alive 14 to 31 years after presentation. One patient died from metastatic dedifferentiated chondrosarcoma. The patient with the low-grade splenic angiosarcoma is alive approximately 2 years after diagnosis. Reappraisal of the older literature suggests that some of the vascular tumors occurring in Maffucci's syndrome, previously diagnosed as hemangiomas, may in fact be SCH. The apparent association between Maffucci's syndrome and SCH, the presence of SCH in other congenital syndromes, and the young patient age and multicentric distribution of SCH unassociated with Maffucci's syndrome raise the possibility that SCH may be a manifestation of a congenital mesodermal disorder with a genetic background related to Maffucci's syndrome. Although the behavior of SCH appears to be one of a locally recurrent or persistent multicentric lesion that does not metastasize, the association of SCH-like foci in a low-grade angiosarcoma of the spleen raises the possibility that SCH may rarely be associated with a higher grade lesion. Therefore, SCH, at least in the setting of Maffucci's syndrome, should be carefully monitored.