Primary Malignant Fibrous Histiocytoma of the Small Intestine Presenting as an Intussusception: Report of a Case

Abstract

Malignant fibrous histiocytoma occurs most commonly in the extremities and trunk, but rarely in the visceral organs. Malignant fibrous histiocytoma of the small intestine is an extremely rare condition. To our knowledge, only ten cases of primary tumors involving the small intestine have been described up to now. Among them only one case has been reported to lead to intussusception. This case report documents the appearance of a storiform-pleomorphic type primary malignant fibrous histiocytoma of the ileum, which led to intussusception, in a 58-year-old man with a 3-month history of dyspepsia, weight loss, general fatigue, and nonspecific abdominal pain. The patient was well with no sign of disease at 8 months after surgery. The clinical and pathologic characteristics of malignant fibrous histiocytoma of the small intestine as found in the literature are also reviewed.

Primary malignant fibrous histiocytoma of the abdominal cavity: CT findings and pathological correlation

“…The occurrence of MFH in the alimentary tract is less well documented in the English literature. In our MEDLINE search we could only yield 11 cases of primary ileum MFH including ours [1,[27][28][29] . Compared to MFHs in other 154 April 28, 2012|Volume 4|Issue 4| WJR|www.wjgnet.com parts of the body, small intestinal MFHs are relatively smaller in size.…”

Primary and metastatic high-grade pleomorphic sarcoma/malignant fibrous histiocytoma of the gastrointestinal tract: an approach to the differential diagnosis in a series of five cases with emphasis on myofibroblastic differentiation

“…(+) Rare isolated tumour cells positive, + 10-25% tumour cells positive, ++ 25-50% tumour cells positive, +++ >50% tumour cells positive, ND not done metastases [44] and intussusception [22]. In a previous review [41], 24% of patients with a mean follow-up of 15 months died of disease 2-48 months post-operatively (mean, 15).…”

“…Previously, reported primary MFH of the GI tract involved the small and large bowel more frequently and only rarely the stomach [22,33,41,42,44]. As the majority of them have preceded the era of sub-classifying pleomorphic STS, it is likely that a considerable fraction of previous reports might have represented pleomorphic variants of other specific sarcoma types.…”

“…Primary and metastatic MFH is rare in the gastrointestinal (GI) tract with ≈50 primary [33,41,42,44] and five metastatic cases reported from different parts of the GI tract, usually as single case reports [1,6,9,22,33,[39][40][41][42]44]. In this study, we describe and compare the clinicopathological and immunohistochemical features of two primary gastric MFH and three metastatic MFH involving the small and large bowel and discuss their broad differential diagnosis with a reference to myofibroblastic differentiation.…”

Primary Intra-abdominal Malignant Fibrous Histiocytoma: A Highly Aggressive Tumor

“…Visceral MFH is a very rare occurrence. It has been reported to arise in spleen, liver, small intestine, colon, mesentery gallbladder, urachus, kidney, and pancreas [2,10].…”