Clinical characteristics of the primary hepatic malignant fibrous histiocytoma in China: case report and review of the literature

Dianbo Yao,Chaoliu Dai

doi:10.1186/1477-7819-10-2

Abstract

BackgroundA malignant fibrous histiocytoma is a soft tissue tumor that most commonly occurs in the extremities, but rarely involves the liver. The clinical characteristics and therapeutic experiences of primary hepatic malignant fibrous histiocytoma are still limited.MethodsTwo cases of primary hepatic malignant fibrous histiocytoma were analyzed retrospectively, and all the literature concerning primary hepatic malignant fibrous histiocytoma was analyzed.ResultsIn China, a total of 76 cases had been reported, among which 50 were men, with a male to female ratio of 1.9:1. Mean age of the patients was 51.0 years old, and more than 85 percent were older than 40 years. 82.9 percent (63/76) of hepatic MFH were solitary lesions, with tumor size ranging from 2.5 to 23.5 cm (average 10.3 cm). Major clinical presentation (78.4%) was abdominal pain or discomfort, accompanied with some other non-specific symptoms such as malaise, anorexia, weight loss, jaundice and fever, and small cases (14.9%) were asymptomatic. Computed tomography and ultrasound usually revealed the location of lesions. The rate of pre-operative misdiagnosis was extremely high, and 14.9 percent of patients were even misdiagnosed as a benign liver cyst, liver abscess or hematoma. Integrated resection was performed among the most cases (49/68), among which only a few ones (12 cases) were introduced to have no recurrence or metastasis or be still alive with no detail information provided, while among the cases with palliative operation or only a biopsy, the cases that were followed-up all died.ConclusionsHepatic malignant fibrous histiocytoma is a rare malignant mesenchymal tumor. The variable features of clinical presentations and images make the diagnosis difficult. Though the prognosis of primary hepatic malignant fibrous histiocytoma was rather poor, integrated resection might provide a few cases a good opportunity for surviving, suggesting that surgery might be an effective treatment.

Highlights

A malignant fibrous histiocytoma is a soft tissue tumor that most commonly occurs in the extremities, but rarely involves the liver
Levels of alpha-fetoprotein (AFP), carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA 19-9) in serum were all within normal limits
The tumor cells showed positive for vimentin and CD68, while stains for CK, CK8/18, CD35, CD21, Table 1 Clinical Features of 45 Cases of Primary Hepatic Malignant Fibrous Histiocytoma in the English literature

Summary

Introduction

A malignant fibrous histiocytoma is a soft tissue tumor that most commonly occurs in the extremities, but rarely involves the liver. The clinical characteristics and therapeutic experiences of primary hepatic malignant fibrous histiocytoma are still limited. Since its initial description in 1964 by O’Brien and Stout [1], malignant fibrous histiocytoma (MFH) has been regarded as one of the most common soft tissue sarcomas of late adult life[2]. MFH primarily occurs in the extremities and less commonly involves the retroperitoneal spaces, abdominal cavity, or other sites [3]. The primary hepatic MFH is extremely rare, and there is still an incomplete understanding of the clinicopathology of

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