Primary Malignant Fibrous Histiocytoma: A Rare Case

Abstract

Malignant fibrous histiocytoma (MFH) of the small intestine is an extremely rare condition. It occurs most commonly in the extremities and the trunk. We report a case of a 67-year-old woman who admitted with fever, myalgia, and altered status. After thorough investigation, a tumor of the jejunum was found. The patient underwent complete surgical removal of the tumor. A diagnosis of MFN (undifferentiated high-grade pleomorphic sarcoma) was made. The patient received adjuvant chemotherapy with Gemcitabine. Two years after the operation, the patient died due to recurrence of the disease. MFH of the small intestine is an extremely rare neoplasm with an aggressive biological behaviour. In this paper, pathogenesis, natural history, and treatment are reviewed.

Abdominal bleeding due to ruptured primary hepatic malignant fibrous histiocytoma

“…According to the WHO classification, the terms myxofibrosarcoma and angiomatoid fibrous histiocytoma are used instead of myxoid MFH and angiomatoid MFH respectively. Now the myxofibrosarcoma is included in the myofibroblastic list and the angiomatoid fibrous histiocytoma in the tumors of uncertain differentiation [4,7,9,[11][12][13][14][15]. It has been suggested that the tissue of origin of an MFH is the primitive undifferentiated mesenchymatic cells which have the capability of multidirectional differentiation [4,7,18,17].…”

“…It is a relatively uncommon entity in the posterior retroperitoneum, the abdominal cavity, or other sites such as the skin and the head. Primary MFH of the liver is rare accounting for less than 1% of primary hepatic malignancies according to Liver Cancer Study Group of Japan and since the first report in the literature in 1985, a limited number of cases have been published [1][2][3][4][5][6][7][8][9][10][11][12][13]. The clinical presentation of primary hepatic MFH miscellaneous and nonspecific varies as there are asymptomatic cases while others present with symptoms which are common in a variety of diseases [8,13].…”

“…The clinical-pathology of primary hepatic MFH is far to be well understood. Histologically, MFH is composed of a heterogeneous group of tumors with the most commonly found form, the pleomorphic variant with spindle cells in a storiform pattern [4,7,9,[11][12][13][14][15]. Our patient was managed by extended hepatectomy.…”

Primary malignant fibrous histiocytoma of the breast: report of one case

“…According to the main morphological characteristics of tumor cells, MFH can be grouped into five types 1,5. First is the pleomorphic type, where the most prominent morphological features include the fact that the tumor cells are pleomorphic and have a spoke wheel-like structure, showing spindle cells arranged in spoke wheel shapes.…”

“…The mass does not usually cause pain unless it is compressing a nearby nerve. Symptoms such as weight loss and fatigue are not typical, but can present in patients if the disease has advanced 3–5. The literature has reported that primary breast MFH has an average size of 6 cm and a variable clinical presentation.…”