A 58-year-old woman was seen at the gastroenterology department for persistent pain in the left upper quadrant. Physical examination revealed a flat abdomen, with mild tenderness to deep palpation in the left hypochondrium. Abdominal magnetic resonance imaging revealed hepatic hemangiomas and a well-defined solid nodular formation, partially exophytic at the splenic border, with low signal intensity on T1 and T2-weighted images and progressive contrast enhancement, measuring 6.3 × 5.7 cm (Fig. 1).

Figure 1.

Solid exophytic nodular formation on the upper border of the spleen, measuring 6.3 × 5.7 cm.

The patient underwent a scheduled laparoscopic splenectomy (Figs. 2 and 3), with an adequate and uneventful postoperative course. Histopathological examination identified a smooth, firm, brownish-white nodular lesion. Immunohistochemical staining demonstrated neoplastic cell positivity for AML, CD31, ERG, KI-67, and factor VIII, establishing the diagnosis of splenic hemangioendothelioma (Fig. 4). Splenic hemangioendothelioma is an entity that may represent a vascular lesion with morphological and clinical properties intermediate between those of hemangioma and angiosarcoma. Its malignant potential is best described as borderline or intermediate.1 The differential diagnosis includes a broad spectrum of autoimmune granulomatous diseases, infections, and vascular malignancies.2 There is a slight female predominance in incidence and prevalence, and incidence peaks between the fourth and fifth decades of life.3 Patients report nonspecific symptoms, such as upper quadrant or epigastric pain, weight loss, weakness, fever, and fatigue.4 Treatments include surgery, systemic/regional chemotherapy, and/or radiotherapy.

Figure 2.

Splenectomy specimen showing nodule in the superior pole of the spleen.

Figure 3.

Surgical specimen showing a splenic hemangioendothelioma lesion.

Figure 4.

Histopathological image characterized by rounded endothelial cells arranged in cords or nests with diffuse cytoplasmic vacuoles.