Angiosarcoma variant of Kasabach–Merritt syndrome

Abstract

Kasabach-Merritt syndrome is characterized by the occurrence of disseminated intravascular coagulation (DIC) usually caused by benign angiomatous tumours. Here we report the case of a 70-year-old man in whom DIC revealed a locally advanced hepatic tumour. Although DIC resolved with heparin, antithrombin III, fresh frozen plasma and corticosteroids, the patient died from haemoperitoneum following a fall, 3 months after the initial observation. Histopathological examination by autopsy allowed the diagnosis of hepatic angiosarcoma. The physiopathogenic mechanisms and treatment options are discussed.

Consumptive Coagulopathy in Angiosarcoma: A Recurrent Phenomenon?

“…Specific to angiosarcoma, the occurrence of consumptive coagulopathy has been documented in several case reports. Two reports described bulky de novo breast angiosarcomas [8, 11], two others reported nonbulky scalp angiosarcomas in elderly Asian males [9, 10], and one case described a bulky hepatic angiosarcoma [7]. The two patients with breast disease and one with scalp disease had concomitant hemorrhage or thrombosis.…”

“…There are sporadic case reports describing the coexistence of a consumptive coagulopathy with angiosarcoma [7–11]. These have been characterized as variants of Kasabach-Merritt Phenomenon (KMP), the eponymous syndrome first described in 1941 in an infant with a giant capillary hemangioma, and henceforth sometimes loosely defined as a thrombocytopenic consumptive coagulopathy associated with an enlarging vascular neoplasm or malformation.…”

Kasabach–Merritt phenomenon in an adult man with a tufted angioma and cirrhosis responding to radiation, bevacizumab, and prednisone

“…There are rare reports of “KMP” associated with other vascular tumors, including angiosarcomas and hemangiopericytomas . Experts, however, argue that consumptive coagulopathies in such cases are mislabeled and that their pathogenesis differs from KMP .…”

Angiosarcoma of the Retroperitoneum: Report on a Patient Treated with Sunitinib

“…Although sunitinib suppresses bone marrow function, and a single case report of sunitinib-induced thrombotic thrombocytopenia purpura has appeared [12], it is difficult to conclude that the reported hematologic abnormalities were caused by sunitinib alone, because such problems have been reported in only very few GIST or renal cell carcinoma patients enrolled in large phase III clinical studies. The occurrence of severe anemia, and thrombocytopenia with laboratory features of MAHA, might be in part attributable to KMP, a syndrome associated with vascular tumors, which shows features similar to those of MAHA and consumptive coagulopathy [12–14]. …”