According to our knowledge, this is the second case of multicentric splenic hemangioendothelioma and is the first case presenting together with cholecystolithiasis, anemia and mild indirect hyperbilirubinemia[3].

Histopathological findings
On macroscopic examination, the spleen was measured to be 15x9x4.5 cm in diameter with a normal configuration. Two distinct non-encapsulated nodules 2 cm away from each other with the diameters of 20x20x10 mm and 9x7x4 mm were detected beneath the capsule on the examination of splenic cross sections. The rest of the spleen was normal. Microscopically, the tumor was composed of vascular channels lined by plump or spindle endothelial cells and solid tumor cells in between these channels simulating hemangiopericytoma (Figure 1A, B). On high power examination, some cells of the solid areas showed cytoplasmic vacuolization or spaces partially containing erythrocytes. There were few atypical cells both among the space lining cells and in the solid areas (Figure 2A, B). However, despite this atypical appearance of the cells, the number of mitotic figures was low. Thin reticulin fibers surrounding individual and group of cells examined on reticulin staining. The tumors revealed an immunohistochemical profile for factor VIII-RA (Figure 3) and CD34 consistent with the immunophenotype of endothelial origin (Figure 4).

Fig 1: (A,B). (A): Tumor composed of vascular channels lined by plump or spindle endothelial cells and solid tumor cells in between these channels simulating hemangiopericytoma (H&E;, x40), (B): On high power examination, some cells of the solid areas showed cytoplasmic vacuolization or cytoplasmic spaces some containing erythrocytes (H&E;, x100)

Fig 2: (A,B). There were few atypical cells both among the space lining cells and in the solid areas (A): H&E;, x200, (B): H&E;, x400

Fig 3: The strong positivity with FVIII-RA revealing endothelial origin of the cells in the solid areas

Fig 4: The strong positivity with CD34 revealing endothelial origin of the cells in the solid areas

The previous cases of splenic hemangioendothelioma discussed in the literature have similar histopathological and indolent clinical characteristics similar to the forms described at other sites[6-8]. Commonly, accompanying clinic situation to the splenic hemangioendothelioma is chronic anemia[9]. Functional hyposplenism, spontaneous and traumatic ruptures have been described as single cases[10-12]. The other cases reported in literature were asymptomatic and diagnosed incidentally during abdominal imaging for other reasons. However, our patient presented with right upper quadrant pain due to multiple stones in the gall bladder. A mild anemia and indirect hyperbilirubinemia probably related to splenic hyper function were detected.

Although spindle cell hemangioendotheliomas are often multicentric when confined to a single anatomic region, the tumors described in the spleen up to date were not multicentric except a case reported by Heese et al[3]. Hemangioendotheliomas are well-separated lesions from healthy parenchyma. In a previously reported case, a primary lesion and multiple intra splenic metastases were described[13]. However, in our case, two distinct tumor masses that do not have continuity with each other were detected. According to our knowledge, this is the second multicentric hemangioendothelioma located in the spleen[3]. Additionally, this is the first case of hemangioendothelioma presenting with indirect hyperbilirubinemia and anemia both of which recovered after splenectomy.

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