A case of cord capillary hemangioma of the spleen: A recently proven true neoplasm

Abstract

Cord capillary hemangioma (CCH) of the spleen is an extremely rare lesion. Once classified under splenic hamartoma, CCH is now differentiated from hamartoma, as its clonality has recently been proven. Herein, we present the case of an incidentally found CCH, measuring 6 × 5.5 × 5 cm, in a 45-year-old man. He underwent splenectomy and has been recurrence-free for 8 years. Macroscopically, the cut surface of the mass showed a central stellate scar with peripheral interspersed reddish areas of variable sizes and intervening fibrous bands. Microscopically, the mass was well demarcated from the splenic tissue. The macroscopic reddish areas were found to correspond to nodules of various sizes, which were predominantly composed of CD34-expressing capillaries without the presence of CD8-expressing sinuses. The relative proportion of these two types of vessels distinguishes CCH from sclerosing angiomatoid nodular transformation (SANT), as SANT shows the apparent presence of CD8-expressing sinuses. In longstanding cases of SANT, sinuses might become ambiguous and the differences between CCH and SANT might become subtle. Nonetheless, immunohistochemistry should be performed when these conditions are suspected, since accurate distinction is usually achieved as a result.

Incidentally discovered asymptomatic splenic hamartoma misdiagnosed as an aneurysm: A case report

“…SH should be differentiated from other vascular tumors of the spleen including hemangioma, lymphangioma, hemangioendothelioma, and angiosarcoma. Solid mass-forming lesions of the spleen such as lymphoma, metastatic disease, inflammatory pseudotumor and sarcoidosis are also included in the differential diagnosis[ 2 , 7 , 9 , 14 - 16 ]. Although imaging findings of SH are nonspecific, there are useful clues, such as on CT, SH appears as an isodense or hypodense solid mass, and heterogeneous contrast enhancement relative to adjacent normal spleen parenchyma was present in the arterial phase, venous phase or delayed phase[ 17 ].…”

Splenic cord capillary hemangioma with non-islet cell tumor hypoglycemia: a case report

“…This situation might have arisen because immunohistochemical studies are needed to precisely classify the types of splenic hamartoma. In 2015, Tajima et al [1] mentioned that, when strictly defined, there were eight cases of splenic cord capillary hemangioma that were reported before them. After the report, there was only one report about splenic cord capillary hemangioma.…”

“…Splenic cord capillary hemangioma is a rare benign vascular lesion. Once classified under splenic hamartoma, cord capillary hemangioma is now differentiated from hamartoma, as its clonality has recently been proven [1]. And it is also a rare lesion, with an incidence of 0.024–0.13% in tumors of the spleen [2].…”