Abdominal bleeding due to ruptured primary hepatic malignant fibrous histiocytoma

Abstract

Introduction: Malignant fibrous histiocytoma (MFH) is a soft tissue sarcoma with ill-defined characteristics, common in late adult life and usually occurring in the extremities, though rarely occurs in the liver. Advanced histological grade and large tumor size constitute unfavorable prognostic agents. The case of a 22-year-old female is presented who was diagnosed with MFH. The patient warranted a surgical management which was extensive hepatectomy. Beside to the scarcity of the tumor, we regard the case unique as there are no previously reported cases of ruptured hepatic MHF tumors in the literature, presenting with intra-abdominal bleeding. Case Report: A 22-year-old female was admitted to hospital due to symptoms of intra-abdominal bleeding, with a palpable large mass in the right upper of the abdomen and general malaise. After the patient's stabilization, she underwent CT and MRI imaging which revealed advanced hepatomegaly and a 25cm vascular liver tumor occupying the liver segments IV, V, and VIII. Intra-arterial angiography was performed with embolization of the feeding vessels of the tumor which originated from the right hepatic artery and the right phrenic artery. The biochemical markers of liver function were slightly elevated or normal, HBsAg and HBcAb positive, HCVAb negative and the following tumor markers, CEA, AFP, and CA19-9 were found to be negative. Though signs of malignancy were present in the radiology examination, such as heterogeneous multinodular mass badly delineated from surrounding liver parenchyma, heterogeneous vascular enhancement, and necrotic areas, radiology failed to identify the type of tumor and led to an initial diagnosis as hepatocellular carcinoma. The liver remnant (segments I, II, III) was found to be 1.132.69 ml or 93.16% of the estimated standard liver volume. The immunohistochemistry of the tumor obtained upon right extended hepatectomy revealed a diagnosis of MFH. Conclusion: Hepatic malignant fibrous histiocytoma is a rare mesenchymal tumor with varying features and clinical presentation that makes the diagnosis difficult. The prognosis on MFH is poor due to its insidious appearance, aggressive behavior and frequent occurrence of distant metastases. Surgical resection of the tumor may provide the only chance of survival.