Primary Hepatic Leiomyosarcoma: a Case Report and Review of the Literature

Abstract

A B ST R AC T Background/Aim: Primary hepatic leiomyosarcoma is an extremely rare type of liver sarcoma with relatively poor prognosis, with about 50 cases having been reported in the literature. Potential origins of this tumor in the liver are the smooth muscle cells in the round ligament, intrahepatic blood vessels and intrahepatic bile ducts. There is no apparent sex predilection and there is a wide age range. The clinical presentation is not specific and the diagnosis depends on the expression of markers such as smooth muscle actin, desmin and vimentin by tumor cells. Patients and Methods: Herein, we present a case of a bulky primary hepatic leiomyosarcoma in a 68-year-old female patient. The patient underwent resection of the exophytic mass en block with the hepatic segments III and IVB. Conclusion: Surgical resection is the most effective among the treatment options. K E Y WO R D S hepatic; liver; leiomyosarcoma, primary

Case of primary hepatic leiomyosarcoma successfully treated with laparoscopic right hepatectomy

“…Review of the literature reveals only about 76 cases of primary hepatic leiomyosarcoma reported 3–63. As in our case, an important prerequisite to its diagnosis is to exclude the possibility of an extrahepatic source of metastasis, with primary tumours being typically identified in the gastrointestinal tract, uterus or retroperitoneum 64–67…”

Large surgically resected leiomyosarcoma of the liver: a case report

“…PHL usually arises from intrahepatic vascular structures, bile ducts, or ligamentum teres [ 7 – 9 ]. The underlying pathogenetic mechanisms of the disease have not yet been identified; however, it is reportedly associated with AIDS, Epstein-Barr virus, Hodgkin’s lymphoma, immunosuppression after organ transplantation, and HCV-related liver cirrhosis [ 4 , 7 , 10 , 11 ].…”

“…Clinical manifestations and laboratory and imaging examinations have limited value for diagnosis and differential diagnosis. Some patients may present with a wide spectrum of symptoms, including abdominal pain, abdominal mass, weight loss, nausea, vomiting, anorexia, abdominal distension, jaundice, and, rarely, acute intra-abdominal bleeding secondary to tumor rupture [ 4 , 7 ]. PHL is often asymptomatic until it increases significantly in size, resulting in nonspecific symptoms, or is unexpectedly found during physical examination [ 7 , 8 ].…”

“…Tumor size at diagnosis varies greatly, with the largest being 30 cm in diameter. The mean and median diameters for all reported PHLs are 10.3 cm and 9.1 cm, respectively [ 4 ]. In this case, the tumor was detected as a large mass, 15 cm in diameter, by detailed examination for a feeling of epigastric compression.…”

“…Ultrasonography generally shows a hypoechoic mass or a mass with heterogeneous echogenicity [ 4 ]. CT findings of PHL generally describe enlarged, heterogeneous, hypodense masses with peripheral enhancement and evidence of central necrosis [ 4 , 7 , 8 , 12 , 13 ], whereas MRI usually displays homogeneous or heterogeneous hypointensity tumors on T1-weighted images and hyperintensity on T2-weighted images [ 4 , 7 ]. Ferrozzi et al [ 12 ] reported that hemorrhagic necrosis is likely to occur inside the tumor when the tumor grows, and the tumor may show cystic degeneration after necrosis.…”