Primary splenic angiosarcoma (PSA) is a rare, fatal neoplasm originating from sinusoidal vascular endothelial cells, and usually metastasizes and almost always has a poor prognosis. Surgical excision is the main treatment of this highly malignant disease.

We reported a special case of a 68-year-old female who had a 6-month history of scalp masses.

The patient was found to have 2 skull masses on computed tomography (CT). Laboratory findings revealed erythropenia and thrombocytopenia. Enhanced abdomen magnetic resonance imaging (MRI) showed multiple masses in liver and spleen. The pathological result of the skull masses was revealed to be metastatic angiosarcoma.

The patient underwent surgical excision of skull masses, and no subsequent radiotherapy or chemotherapy was done.

The patient died due to dyscrasia at August 12, 2015, with a survival of nearly 1 month.

We highlight the importance for clinicians to be aware of this rare neoplasm, and to consider it in the differential diagnosis when encountering a skull mass. Early confirmation and treatment may improve the prognosis.

Littoral cell angioma is a unique splenic tumor that is generally considered to be benign. We present a case of a low-grade littoral cell splenic tumor that metastasized to the liver and retroperitoneum 4 years after splenectomy. Although the splenic lesion showed the typical morphology of a littoral cell angioma, it also contained areas with unusual solid nests of cytologically bland, plump cells with clear cytoplasm. The liver was diffusely infiltrated exclusively by cells with similar clear cell features. Both splenic and liver lesions demonstrated identical immunophenotypes, typical of littoral cell angioma, expressing CD31, CD68, CD21, and CD163, although negative for CD8 and CD34. A single prior description of a littoral cell hemangioendothelioma showed nuclear atypia and necrosis, and this is the first case report of a splenic littoral cell hemangioendothelioma with a completely bland histologic appearance. This case suggests that the presence of solid areas of clear cells in a littoral cell angioma may be a marker of low-grade malignant potential in these tumors.

Primary vascular neoplasms of the spleen constitute the majority of nonhematolymphoid splenic tumors. The benign primary vascular tumors include hemangioma, hamartoma, and lymphangioma, whereas those of variable or uncertain biologic behavior include littoral cell angioma, hemangioendothelioma, and hemangiopericytoma. The primary malignant vascular neoplasm of the spleen is angiosarcoma. Peliosis is a rare lesion of unknown cause that is usually found incidentally in asymptomatic patients but may be associated with hematologic or metastatic disease. Although these vascular neoplasms of the spleen are uncommon, their importance lies in that they must be differentiated from the more common neoplastic disorders of the spleen, such as lymphoma and metastasis. The most common echogenic solid or complex cystic mass in an asymptomatic patient is splenic hemangioma. However, the imaging appearance of splenic hemangiomas may be complex, and differentiation of these lesions from malignant disease may not be possible. The diagnosis of splenic hamartoma may be suggested when findings of increased blood flow on color Doppler images are seen in association with a homogeneous solid echogenic mass. A large subcapsular solitary cystic abnormality discovered incidentally in a child in association with internal septations and tiny mural nodules favors the diagnosis of lymphangioma. Any invasion of the surrounding splenic parenchyma by a splenic lesion should indicate a more aggressive or malignant process. Evaluation of a focal splenic abnormality identified on sonograms should be followed up with computed tomography or magnetic resonance imaging with and without contrast material enhancement. Splenectomy may be required for definitive evaluation of a splenic mass with atypical features.

Littoral cell angioma (LCA) is a rare benign vascular tumor of the spleen with characteristic histomorphologic features. Only a few descriptions of the radiologic appearance of this tumor have been published, and those descriptions are variable. We report a case of LCA in a 37-year-old man with psoriasis and nonspecific symptoms of weakness, pain and fatigue, normocytic anemia, and thrombocytopenia. The results of abdominal sonography and contrast-enhanced CT correlated: the 2 modalities revealed hepatosplenomegaly and multiple round splenic lesions of similar appearance and size (on sonograms, ill-defined echogenic lesions up to 3.2 cm without acoustic enhancement; on CT scans, hypodense, nonenhancing lesions up to 3.5 cm). Because making a differential diagnosis was difficult and our presumptive diagnosis was hemangioma or lymphoma, splenectomy was performed. Postoperative pathologic examinations confirmed a final diagnosis of LCA. The patient's recovery was uneventful. LCA should be considered when making a differential diagnosis of splenic lesions, and sonography may be more helpful than CT in reaching a diagnosis of LCA.

Primary angiosarcoma of the spleen is a rare neoplasm that has not been well characterized. We describe the clinical, morphologic, and immunophenotypic findings of 28 cases of primary splenic angiosarcoma, including one case that shares features of lymphangioma/lymphangiosarcoma. The patients included 16 men and 12 women, aged 29 to 85 years, with a mean of 59 years and median of 63 years. The majority of patients (75%) complained of abdominal pain, and 25% presented with splenic rupture. The most common physical finding was splenomegaly (71%). Seventeen of 21 patients were reported to have anemia. Macroscopic examination showed splenomegaly in 85% cases. Sectioning revealed discrete lesions in 88% of cases, ranging from well-circumscribed firm nodules to poorly delineated foci of necrosis and hemorrhage associated with cystic spaces. Microscopically, the tumors were heterogenous; however, all cases demonstrated at least a focal vasoformative component lined by atypical endothelial cells. Solid sarcomatous, papillary, and epithelioid growth patterns were observed. The solid sarcomatous component resembled fibrosarcoma in two cases and malignant fibroushistiocytoma in one case. Hemorrhage, necrosis, hemosiderin, extramedullary hematopoiesis, and intracytoplasmic hyaline globules were frequently identified. A panel of immunohistochemical studies revealed that the majority of tumors were immunoreactive for at least two markers of vascular differentiation (CD34, FVIIIRAg, VEGFR3, and CD31) and at least one marker of histiocytic differentiation (CD68 and/or lysozyme). Metastases developed in 100% of patients during the course of their disease. Twenty-six patients died of disease despite aggressive therapy, whereas only two patients are alive at last follow-up, one with disease at 8 years and the other without disease at 10 years. In conclusion, primary splenic angiosarcoma is an extremely aggressive neoplasm that is almost universally fatal. The majority of splenic angiosarcomas coexpress histiocytic and endothelial markers by immunohistochemical analysis, which suggest that some tumors may originate from splenic lining cells.

A 49 years old man in good physical condition suffering from abdominal pain in the right hypochondrium was hospitalized and CT scan revealed an hemoperitoneum, an enlargement of the liver and a rupture of an heterogeneous spleen. The patient was operated on and spleen removed. Pathological examination of the spleen concluded to a splenic cavernous hemangioma. In the postoperative course, an increase of liver nodules occurred within two weeks. A localized splenic angiosarcoma was recognized by reexamination of the specimen. The patient treated by chemotherapy was still alive after 5 months. Prognosis of splenic angiosarcoma is very poor; there is no curative treatment.