Malignant fibrous histiocytoma metastases to the small intestine and colon presenting as an intussusception

A case of malignant fibrous histiocytoma metastases to the small intestine and colon presenting as an intussusception is described. Although malignant fibrous histiocytoma is the most common soft tissue sarcoma in late adult life, GI involvement has rarely been reported. The review of both our case and eight cases in the English-language literature suggests that GI involvement from malignant fibrous histiocytoma occurs most frequently in the small intestine (six of nine) and that two major clinical manifestations of GI involvement are GI bleeding (five of nine) from ulcerated tumors and intussusception (two of nine) led by polypoid tumors.     

Primary malignant fibrous histiocytoma of the small bowel: a report of an additional case in duodenum

AIMS: We report herein an additional case of primary malignant fibrous histiocytoma (MFH) in the duodenum and provide a review of the existing literature. METHODS AND RESULTS: A 61-yr-old Chinese man was admitted to our hospital with symptoms of melena, anorexia, and weight loss. An abdominal computed tomography (CT) and gastrointestinal barium meal examination demonstrated a tumor of the duodenum suggestive of primary malignancy. The tumor was successfully treated by pancreaticoduodenectomy. It was histopathologically and immunohistochemically diagnosed to be a storiform-type primary MFH of the duodenum. There have been a total of 40 cases of primary malignant fibrous histiocytoma of the small bowel documented in the literature including our Chinese cases. CONCLUSION: Primary malignant fibrous histiocytoma of the small bowel, especially in the duodenum is extremely rare. The final diagnosis is made only after pathological and immunopathological examination of the tumor. The malignant potential of such tumors is high. The prognosis may be mainly dependent on the invasion and metastasis of tumor, while tumor size is irrelevant. The treatment should be surgery if possible. Early surgical intervention may be the best form of management that may offer the patient good result.     

Metastatic cardiac malignant fibrous histiocytoma presenting as right ventricular outflow tract obstruction.

An unusual case of metastatic disease of the right ventricle is described in a 74-year-old woman who had presented with a malignant fibrous histiocytoma of the right thigh previously treated with surgery and radiotherapy. Two years later she presented with signs and symptoms of tricuspid regurgitation and right ventricular outflow tract obstruction. The diagnosis of right ventricular tumor was made by echocardiographic and angiographic studies and confirmed on postmortem examination.     

Malignant fibrous histiocytoma of peritoneum presenting as intestinal obstruction.

Malignant fibrous histiocytoma (MFH) is a high-grade soft-tissue sarcoma of fibroblast-cell origin with a propensity for metastasis and recurrence. Primary MFH of the peritoneum is rare. We report a 60-year-old man with MFH of the peritoneum presenting with obstructive symptoms. Complete surgical excision of the tumor was done, and he is well six months later.     

Primary malignant fibrous histiocytoma of the heart

A 72-year-old male diagnosed with heart failure and dyspnea was found to have a lesion in his right atrium. Histological examination of the tumor revealed it to be a malignant fibrous histiocytoma. The patient's management and subsequent follow-up are presented.     

Primary malignant fibrous histiocytoma of the lung

BACKGROUND: Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma in adults. Its appearance as a primary lung tumor is extremely rare. The cell origin of MFH remains controversial. The treatment of choice for MFH is surgical resection, while the role of chemo- and radiotherapy remains unclear. METHODS: A retrospective analysis of 5 patients operated on for primary MFH in the Department of Thoracic Surgery of the Medical University in Gdansk between 1990 and 2000 was performed. RESULTS: Out of approximately 2000 patients operated on for primary malignant lung tumors, five (0.25 %) had MFH. The mean age of the 4 men and 1 woman was 62 years. In all cases radical resection was performed without adjuvant chemo- or radiotherapy. Four patients died within 2 - 7 months after the operation, three of them from distant metastases. The follow-up of one patient is not available. One patient is alive 11 years after the operation. CONCLUSION: Although surgical resection of MFH is the treatment of choice in MFH, the results are unsatisfactory.     

Primary inflammatory malignant fibrous histiocytoma of the colon

A unique case of inflammatory malignant fibrous histiocytoma of the large bowel is presented. This lesion occurred in the colon of an elderly man suffering from weakness, anemia, anorexia, and weight loss. A right hemicolectomy was performed, and six months later, on follow-up, he was found to be well. The literature on visceral involvement by malignant fibrohistiocytic tumors is reviewed.     

Left paraduodenal hernia presenting as recurrent small bowel obstruction

Internal herniation of the small bowel is a relatively rare cause of intestinal obstruction. Left paraduodenal hernia resulting from abnormal rotation of the midgut during embryonic development is the most common form of congenital internal hernia. We report our experience in the diagnosis and management of a young male with left paraduodenal hernia presenting as recurrent intestinal obstruction. Correct preoperative diagnosis of left paraduodenal hernia had been difficult due to non-specific clinical presentations, but the advent of modern imaging technology makes early and correct diagnosis possible. Due to the risk of obstruction and strangulation, surgical treatment is indicated; however, timely intervention increases the likelihood of a favorable outcome.     

原发性肝恶性纤维组织细胞瘤3例分析

原发性肝恶性纤维组织细胞瘤（MFH）是罕见的肿瘤,查阅我院1954年-2005年的病例,仅发现1例原发性肝MFH肺转移,2例原发性肝MFH.3例均为病理诊断,病例资料详实,结合有关文献,报道如下.     

Primary amyloidosis presenting as small bowel encapsulation.

Amyloidosis is a pathological process which encompasses a spectrum of diseases that result from extracellular deposition of pathological fibrillar proteins. Clinical presentations vary depending on the organs involved. There is no documented case of amyloidosis presenting as small bowel encapsulation. A previously healthy 62-year-old man developed a small bowel obstruction in 1997. At surgery, a peculiar membrane encasing his entire small bowel was discovered. This appeared to have no vascularity and was removed without difficulty, exposing a grossly normal bowel. Histopathology revealed thick bands of collagen overlying the peritoneal surface, which was congo red positive and showed apple green birefringence. The findings were consistent with encapsulating peritonitis due to amyloidosis. There was no history or symptoms of any chronic inflammatory condition and he became symptom-free postoperatively. An abdominal fat pad biopsy failed to demonstrate amyloidosis. Endoscopic duodenal biopsies revealed classical primary amyloidosis. Quantitative immunoglobulins, lactate dehydrogenase, C3, C4 and beta-2 microglobulin were normal. Protein electrophoresis identified monoclonal paraprotein, immunoglobulin G lambda 3.7 g/L. Bone marrow biopsy and aspirate revealed only a mild plasmacytosis (5% to 10%). Echocardiogram and skeletal survey were normal. He had mild proteinuria. Complete blood count, C-reactive protein, calcium, albumin and total protein were normal. No specific therapy was instituted. In January of 1998 the patient remained asymptomatic with no gastrointestinal, cardiovascular or constitutional symptoms. He had developed nephrotic range proteinuria (3.95 g/24 h), microalbuminuria, hypoalbuminemia and a renal biopsy consistent with renal amyloidosis. In 1999 there was an increase in the monoclonal paraprotein (6.2 g/L). The remaining investigations were normal except for an echocardiogram which showed left ventricular hypertrophy but a normal ejection fraction and no diastolic dysfunction. He went on to have high-dose chemotherapy and an autologous stem cell transplant in September, 2000. He has subsequently developed renal insufficiency. To our knowledge this is the first reported case of primary amyloidosis presenting as small bowel obstruction from encapsulating peritonitis.     

Persistent omphalomesenteric duct causing small bowel obstruction in an adult

An extremely rare case of persistent omphalomesenteric duct causing small bowel obstruction is presented. A 20-year-old female patient without medical history presented with colicky abdominal pain, vomiting, absence of passage of gas and feces, and abdominal distension of 24 h duration. Physical examination and blood tests were normal. Abdominal X-ray showed small bowel obstruction. Computed tomography of the abdomen demonstrated dilated small bowel and a band originating from the umbilicus and continuing between the small bowel loops; an omphalomesenteric duct remnant was suspected. In exploratory laparotomy, persistent omphalomesenteric duct causing small bowel obstruction was identified and resected. The patient had an uneventful recovery and was discharged on the 5^th postoperative day. Although persistent omphalomesenteric duct is an extremely infrequent cause of small bowel obstruction in adult patients, it should be taken into consideration in patients without any previous surgical history.     

Primary malignant fibrous histiocytoma of the lung: a case report

Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma in adults. However, primary MFH of the lung is rare, with only a few cases reported in the literature. Here, we report the case of an 86-year-old male who was admitted to our hospital with the chief complaint of exertional dyspnea and poor appetite. Chest roentgenography revealed a 9 x 15 cm, pleural-based opacity in the left lower lobe. Chest computerized tomography disclosed a well-defined mass with heterogeneous density in the left lower lung field. The diagnosis of MFH was confirmed by thoracoscopic lung biopsy and pathologic examination. Supportive care was given because of extreme old age and poor performance status (the patient's Karnofsky performance status was 30). The patient died from respiratory failure 2 months later.     

Pulmonary malignant fibrous histiocytoma (inflammatory type) showing intracavitary fungus ball-like shadow]

We report a rare case of primary malignant fibrous histiocytoma of the lung showing intracavitary fungus ball-like shadows. Fiberoptic bronchoscopy revealed no visible tumor, but adenocarcinoma cells were detected in samples of lavage fluid from the cavitary lesion. Staging procedures (T 2 N 0 M 0) confirmed that there were no metastatic lesions. A complete resection of the left lower lobe was performed. The tumor showed polypoid growth that obstructed a small peripheral bronchus, and formed a cavitary lesion. It was histologically diagnosed as an inflammatory type of malignant fibrous histiocytoma, and consisted of atypical histiocyte-like cells, neutrophils, lymphocytes, foamy cells, and fibroblast-like cells in a storiform pattern. The patient has been in complete remission for 3 years after surgery.     

Primary malignant fibrous histiocytoma of the aorta associated with aortic dissection.

We report a unique case of primary aortic malignant fibrous histiocytoma presenting clinically as aortic dissection. To our knowledge, this occurrence is the first ever reported. The magnetic resonance imaging technique may provide superiority in the differential diagnosis between tumor and hematoma of aortic dissection. Aortic tumor, although rare, should be included among the causes of aortic dissection.     

Polyarteritis nodosa as a presenting feature of angiomatoid fibrous histiocytoma

SIR, A 13-yr-old Caucasian boy presented with an 8-month historyof night sweats, intermittent fever, occasional cough, lethargy,myalgia and headaches. The initial examination was unremarkable,and investigations showed haemoglobin of 8.1 g/dl, white bloodcell count of 13.9 x 10⁹/l, platelet count of 899 x10⁹/l, erythrocytesedimentation rate (ESR) of 133 mm/h, C-reactive protein (CRP)of 3 mg/dl and creatine kinase of less than 20 units/l (referencerange 0–120 U/l). Over the next 3 months he developedabdominal