Primary Hepatic Leiomyosarcoma in Adults: Analysis of Prognostic Factors

Ming, Chi; Dudek, Arkadiusz Z.; Wind, Kenneth P.

doi:10.1159/000337416

Cited by 17 publications

(29 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…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).…”

Section: Introductionmentioning

confidence: 99%

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

Han

Cheng

et al. 2015

Oncology Letters

View full text Add to dashboard Cite

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...

show abstract

Section: Introductionmentioning

confidence: 99%

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

Han

Cheng

et al. 2015

Oncology Letters

View full text Add to dashboard Cite

show abstract

“…Surgical resection is recommended for curative treatment, while diagnosis is challenging and often delayed until reaching a large size, resulting in extremely poor prognosis [ 7 ]. Chi et al [ 6 ] reported a median overall survival of 19 months with 1-, 2-, and 5-year survival rates of 61.2%, 41.1%, and 14.5%, respectively. They concluded that complete surgical resection with clear margin was necessary to improve survival of patients.…”

Section: Discussionmentioning

confidence: 99%

“…Primary hepatic leiomyosarcoma (PHL) is an extremely rare tumor, accounting for 0.2–2% of all primary hepatic malignancies [ 1 – 6 ]. PHL usually arises from intrahepatic vascular structures, bile ducts, or ligamentum teres [ 7 – 9 ]; however, the underlying pathogenetic mechanisms have not yet been identified.…”

Section: Introductionmentioning

confidence: 99%

Large surgically resected leiomyosarcoma of the liver: a case report

et al. 2020

View full text Add to dashboard Cite

Background Primary hepatic leiomyosarcoma (PHL) is an extremely rare type of tumor. We herein report a case of a large surgically resected leiomyosarcoma of the liver. Case presentation A 69-year-old man with a feeling of epigastric compression was referred for examination of an abdominal mass. He had no history of liver disease or alcohol abuse. Liver function tests indicated Child-Pugh class A. Tumor markers were negative. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a relatively well-contrasted 12 × 11 × 8 cm tumor with well-defined boundary replacing the lateral segment of the liver alongside multiple intrahepatic metastases. Several nodules up to 12 mm were found in both lungs, suggestive of metastasis. SUVmax of the liver mass and lung tumor in positron emission tomography were 10.4 and 1.5, respectively. Hepatocellular carcinoma was primarily suspected. Lateral segmentectomy of the liver was performed to confirm diagnosis and prevent tumor rupture. Macroscopically, the lateral segment of the liver had been replaced by a lobular or multinodular tumor with a maximum diameter of 15 cm. In pathological findings, the tumor consisted of bundle-like proliferation of complicated banding spindle-like cells with clear cytoplasm, accompanied by storiform pattern and compressed blood vessels. Nuclear fission images were observed in 8/10 HPF. Partial necrosis was present, with associated venous invasion and intrahepatic metastasis. Immunohistochemical staining for tumor cells revealed desmin, α-smooth muscle actin (αSMA), and h-caldesmon were all positive, informing a final diagnosis of PHL. The postoperative course was uneventful, and he was discharged on the 12th postoperative day. Conclusions PHL is a rare malignant disease with relatively poor prognosis. To confirm a diagnosis of PHL, immunohistochemical analysis as well as histopathological findings is important. The preferred treatment is surgical resection, sometimes in combination with adjuvant chemotherapy and radiotherapy. Further studies are needed to elucidate and better understand this uncommon clinical entity.

show abstract

“…Commonest primary liver sarcomas are angiosarcoma (36 %) followed by leiomyosarcoma (12 %), fibrosarcoma (7 %), and other sarcomas (44 %) [5]. Mean age of diagnosis is about 58 years [6]. There is no gender predisposition.…”

Section: Discussionmentioning

confidence: 99%