Giant cavernous hemangioma coexistent with diffuse hepatic hemangiomatosis presenting as portal vein thrombosis and hepatic lobar atrophy

Abstract

A combination of giant hepatic hemangioma and diffuse hemangiomatosis is extremely rare in adults. Even when they are large, hemangiomas are soft and rarely compress adjacent structures. A 78-year-old man presented with abdominal pain and distension. Ultrasonography, computed tomography, and magnetic resonance imaging demonstrated a large expansile mass replacing the medial segment and caudate lobe with diffusely scattered nodules in the entire liver. The large hilar mass contained a central nonenhancing area and had a mass effect, leading to left portal vein occlusion. The image findings also revealed two unprecedented findings: left lateral segmental atrophy of the liver and recent portomesenteric vein thrombosis. The hepatic lesions were confirmed with hemangiomas by ultrasonography-guided biopsy. We diagnosed intrahepatic portal vein obstruction caused by a mass effect of giant hepatic hemangioma coexistent with diffuse hemangiomatosis, resulting in hepatic segmental atrophy and extrahepatic portal vein thrombosis.

Adult diffuse hepatic hemangiomatosis

“…3 Notably, DHH is associated with giant cavernous hemangioma (GCH), a cavernous hemangioma >5 cm in size, in more than half of the cases. 2,13,14 DHH usually affects infants and children. A female preponderance is noted among the adults.…”

“…A PubMed search till May, 2022 along with the literature review by He et al 6 showed only 19 reported cases of adult DHH depicted in the English literature. 3,4,[6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] Approximately half of the DHH cases can be seen in association with GCH. 2 The etiopathogenesis of DHH is uncertain, although it has been diagnosed in association with hereditary disorders, such as skeletal hemangiomatosis and Osler-Weber-Rendu disease, and drugs like oral contraceptives (OC) and metoclopramide.…”

A case of diffuse hepatic hemangiomatosis coexistent with giant hemangioma: case report and literature review

“…Additional reports of hemangiomatosis associated with a giant hemangioma have been described after that one [4 , [11] , [12] , [13] , [14] , [15] , [16] .…”

Vascular tumors of the liver: A brief review

“…Diffuse haemangiomas in liver (haemangiomatosis) might be associated with some syndromes such as Osler-Weber-Rendu disease [13] and medications such as estrogen [14] and metoclopramide [15] with a female predilection. These may present with abdominal pain, distension, weight loss, fever, night sweats, and jaundice.…”