Imaging features of primary hepatic leiomyosarcoma: A case report and review of literature

Abstract

Abstract. Primary hepatic leiomyosarcoma (PHL) is an extremely rare tumour. This tumour is difficult to diagnose by imaging examinations due to its rarity, and non-specific conventional imaging manifestations and clinical presentation. The present study reports the case of a 42-year-old male with PHL that was confirmed by histopathological and immunohistochemical examinations. Multimodal imaging examinations, including ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography-CT and digital subtraction angiography, were performed. The imaging manifestations were analysed and the associated literature was reviewed. The results found that no characteristic imaging appearance was present on ultrasound or plain CT scan. However, on unenhanced MRI, the tumours presented with a heterogeneous low signal density on T1-weighted imaging (WI) and a high signal density on T2WI and diffusion-WI. On gadopentetate dimeglumine enhanced MRI, the lesions were not enhanced during the arterial and portal venous phases; by contrast, these lesions were evidently enhanced during the 5-min delayed phase. Therefore, the delayed imaging of enhanced MRI is likely to be used to differentiate PHL from other hepatic tumours. IntroductionPrimary hepatic leiomyosarcoma (PHL) is an exceedingly rare tumour. In total, <50 patients with PHL were reported in the English literature up until 2011. Among these patients, only five cases were involved in radiological studies (1-5). PHL may develop from the smooth muscle cells of intrahepatic vascular structures, bile ducts or ligamentum teres (1,3). It is difficult to obtain an early diagnosis due to the rarity, and non-specific conventional imaging manifestations and clinical presentation (6). Imaging examinations play a significant role in detecting and differentiating the hepatic masses (7). The present study reports the case of a middle-aged male with PHL. The patient was subjected to multimodal imaging examinations, including ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography-CT (PET-CT) and digital subtraction angiography (DSA). The imaging manifestations were analysed and the associated literature was reviewed. Case reportIn July 2013, a 42-year-old male presented to the First Affiliated Hospital of Shandong University (Jinan, China) with abdominal pain, marasmus and weakness that had persisted for two months. A physical examination revealed a notable hepatomegaly extending ~3 cm below the right costal margin. The patient had no medical history of previous liver diseases or alcohol abuse. Laboratory analysis showed normal liver and kidney functions. Other laboratory tests, including red blood cell, white blood cell and platelet counts, and hepatitis B surface antigen, hepatitis C virus antibody, α-fetoprotein, carbohydrate antigen 19-9 and carcinoembryonic antigen analysis, were normal.Abdominal ultrasonography (ClearVue 350; Philips Medical Systems, Best, the Netherlands) revealed two well-defined hetero...

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

“…It is a malignant tumor that arises from smooth muscle cells (4, 8, 10-13, 15, 17, 19, 21, 22, 26, 28, 29, 31-33, 36-38, 40). Potential origins of this tumor in the liver are the smooth muscle cells in the round ligament (29,31,37,38), the intrahepatic blood vessels and the intrahepatic bile ducts (15,17,22,26,29,31,32,37,38,40). There is no apparent sex predilection, since there have been reported 23 (46%) cases of male patients ( 25,27,28,31,36,[38][39][40].…”

“…Concerning the clinical image of primary hepatic leiomyosarcomas, they are often asymptomatic (21,25,30) or their symptoms are non-specific (4, 8, 10-12, 14, 16, 17, 19, 21, 23, 27-29, 31, 32, 34, 37, 38, 40). Abdominal pain or discomfort (4, 8, 11, 12, 14, 16, 17, 19, 22, 23, 27-29, 34, 37, 38), a palpable mass (4,19,22,28,29,38), fever (17,23,28,31,32,40), jaundice (39), anorexia (10,12,27,28,32), nausea or vomiting (31,34) and weight loss (4,8,10,12,14,16,19,28,32,37) are among the reported manifestations. A case with acute bleeding has also been reported (24).…”

“…A case with acute bleeding has also been reported (24). There is usually a single mass (4, 8, 11-13, 16, 18-25, 27-36, 38-40), although there are cases with two (25,37), three (26) or even multiple tumors (15). The size of the tumor at diagnosis varies greatly (4, 8, 11, 12, 15, 16, 18-23, 25-35, 37-40) with the smallest tumor being 0.6 cm in diameter (35) and the largest one being 30 cm in diameter (11).…”

“…There is a number of data about radiological and histological findings of primary hepatic leiomyosarcoma in the literature. Ultrasonography usually shows a hypoechoic mass (15,17,19,29,30,33,37) or a mass with heterogeneous echogenicity (17,22,27,30,31,37). Computed tomography usually reveals a hypodense (12, 17, 19, 21, 23, 27-33, 37, 39) and often heterogeneous mass (17,21,23,31,34) with inhomogeneous and often peripheral enhancement after administration of intravenous contrast (15,17,19,23,(27)(28)(29)(30)(31)(32)(33)40), which may show regions of cystic degeneration (27,28,31,32).…”

“…Computed tomography usually reveals a hypodense (12, 17, 19, 21, 23, 27-33, 37, 39) and often heterogeneous mass (17,21,23,31,34) with inhomogeneous and often peripheral enhancement after administration of intravenous contrast (15,17,19,23,(27)(28)(29)(30)(31)(32)(33)40), which may show regions of cystic degeneration (27,28,31,32). Magnetic resonance imaging displays on the other hand a usually heterogeneous mass that is hypointense in T1-weighted images and hyperintense in T2-weighted images (16,19,21,37,39,40). Histological examination of primary hepatic leiomyosarcomas shows spindle-shaped cells with fascicular arrangement (4, 8, 10-12, 15-17, 19, 21, 22, 26-34, 36-38, 40).…”