Article

Ruptured Littoral Cell Angiosarcoma Causing Hemoperitoneum

Division of Clinical Oncology, Sumitomo-Besshi Hospital Cancer Center, Japan.
Internal Medicine (Impact Factor: 0.9). 01/2012; 51(3):337-8. DOI: 10.2169/internalmedicine.51.6663
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Available from: Hiromichi Yamane
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    ABSTRACT: Littoral cell tumors (LCT) are rare primary splenic neoplasms, unique for their morphologic and immunolabeling features resembling the endothelial littoral cells lining the sinusoids of the red pulp. They include the more common and typically benign littoral cell angioma, as well as the less common, potentially malignant, littoral cell hemangioendothelioma (LCHE) and the aggressive littoral cell angiosarcoma (LCAS). The most common presentation of these neoplasms is splenomegaly, and diagnosis is made histologically following biopsy or resection. To better understand these tumors, a comprehensive, international literature search was performed. Patient and tumor data, including presenting symptoms, comorbid cancers, immunosuppressive states, splenic mass and tumor size were analyzed. Massive splenomegaly (≥1500 g) following splenic resection, which correlates with a splenic length of 20 cm preoperatively, was found to be significantly associated with the presence of malignancy in the LCT (P<0.05).
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    ABSTRACT: Primary splenic lesions are rare entities among which littoral cell angioma (LCA) is a recently described, uncommon vascular lesion that is unique to the spleen. It has heretofore been described primarily in pathologic series and has been found mostly to behave as a benign entity. A few reports of malignant variants have been reported. We present a case report of a solitary LCA discovered after splenectomy for an incidentally discovered splenic lesion, along with a literature review.
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