An autopsy report of malignant fibrous histiocytoma of the left atrium and a review of the pertinent literature

A 30-year-old male complaining of fever was admitted to hospital and a diagnosis of a malignant, fibrous histiocytoma (MFH) was established after a biopsy examination. Antitumor chemotherapy and Co60 irradiation was initiated. The patient, however, suddenly died of cardiac arrest 9 months after admission. The autopsy revealed a polypoid, yellow-white tumor (5 cm in diameter) arising from the antero-lateral wall of the left atrium and occupying the chamber. Twenty-two cases of MFH (21 previous reports and 1 current case) arising from the heart are reviewed.     

Malignant fibrous histiocytoma of the heart

An autopsied case of malignant fibrous histiocytoma (MFH) in the left atrium of the heart of a 29-year-old Japanese woman was reported light and electron microscopically, and immunohistochemically. Metastasis was found in the adrenal, jejunum, and cervical regions. This is the fourth case of MFH of the heart in the literature. The tumor consisted of undifferentiated mesenchymal cells and histiocytoid cells, including giant cells and xanthomatous cells. Dense patches were commonly detected in all tumor cells. On frozen sections, histiocytoid cells formed EA and EAC rosettes, while fibroblastic cells formed EA rosettes only. Difference between them was also recognized in activity or amount of marker enzymes of histiocytes. These analyses suggested that MFH is a mesenchymal cell tumor with binary differentiation into histiocytoid cells and fibroblastic cells.     

Primary malignant fibrous histiocytoma of the jejunum metastatic to the spine

We report a case of malignant fibrous histiocytoma (MFH) metastatic to the spine. A 41-yr-old male was admitted to our hospital for radiation treatment of MFH of the spine. He began to show signs suggestive of partial small bowel obstruction. Computed tomography demonstated jejuno-jejunal intussusception. The patient was taken to the operating room, where the diagnosis was confirmed. Partial jejunal resection was performed. The lead point of the intussusception was histologically diagnosed to be a high-grade malignant fibrous histiocytoma. We believe that the spinal lesion was the metastatic lesion and that metastasis occurred via the vessels of Adamciewicz. To our knowledge this is the first case thus reported.     

Retroperitoneal malignant fibrous histiocytoma--report of a case

A 52-year-old man with a three-month history of left epigastralgia and body weight loss was referred to us for a possible abdominal tumor in March 1984. Retroperitoneal malignant tumor was suspected by ERCP, angiography, US, and CT. At laparotomy, a child's-head-sized retroperitoneal tumor, which weighed 1,800 g, was resected. Pathological examination revealed the diagnosis of retroperitoneal malignant fibrous histiocytoma (MFH). He died because of recurrence in the retroperitoneum six months after the operation. We selected 26 Japanese cases of retroperitoneal MFH and discussed their clinical findings and prognosis.     

脑部恶性纤维组织细胞瘤1例报道

Malignant fibrous histiocytoma (MFH) firstly described as "malignant fibrous xanthoma" by O'Brien and Stout in 1964, is the most common soft tissue sarcoma of late adult life. Uncertain histogenesis and numerous subtypes make MFH a rather controversial entity. MFH only rare arises from structures of the head and neck. When it does, it most often originates in facial structures, particularly the maxilla. This report details a case of a patient with malignant fibrous histiocytoma presenting clinically as a right-sided large indurated frontoparietal mass, three months after head trauma and eight years after radiation therapy for brain lymphoma located in the right frontal and parietal lobes. Radical excision was a surgical challenge because of the extensiveness of the lesion.     

Cardiac Sarcomas: An Update

Primary cardiac sarcomas are rare and represent 20% of all primary cardiac tumors. Symptoms depend on the chambers and the cardiac structures involved. Transthoracic echocardiography is commonly used to identify a cardiac mass. The diagnosis of cardiac sarcoma requires adequate sampling and the careful use of ancillary diagnostic techniques. In the most recent histologic classification, angiosarcoma is the most common malignant tumor of the heart with recognizable differentiation. Undifferentiated sarcomas account for one-third of all cardiac sarcomas and have been incorporated in the malignant fibrous histiocytoma/pleomorphic sarcoma subgroup. Elective cardiac sarcoma therapy includes complete surgical excision when possible, followed by radio and chemotherapeutic regimen, the latter preferably containing anthracyclines, ifosfamide, or taxanes. Prognosis of cardiac sarcomas is very poor, with mean survival ranging from 9.6 to 16.5 months. A less-aggressive course seems related to the left atrium location, a low histologic grading with scarce cellular pleomorphism and low-mitotic activity, absence of necrosis, myxoid tumor appearance, and absence of metastasis at diagnosis.     

Prolonged remission of metastatic malignant fibrous histiocytoma induced by combination chemotherapy

A patient with metastatic malignant fibrous histiocytoma (MFH) achieved a complete remission with polychemotherapy lasting 42+ months. In view of this long-term survival and two similar prior reports of long-term survival after aggressive treatment, the goal of therapy for metastatic MFH must be reconsidered.     

A case of malignant fibrous histiocytoma after breast conserving therapy for breast cancer

A 45-year-old woman with malignant fibrous histiocytoma (MFH) of the breast following breast conserving therapy (BCT) is described. She noticed a lump in her left breast 52 months after BCT for breast cancer. The lump was excised and nodular fasciitis was initially diagnosed. However, the tumor recurred locally 4 times in the next 18 months. MFH was finally diagnosed. This case is considered to be radiation-induced sarcoma. The risk of radiation-induced sarcoma after BCT seems to be very low, however careful follow-up is necessary.     

Malignant fibrous histiocytoma coexistent with mucinous cystadenoma of the pancreas.

A patient with malignant fibrous histiocytoma (MFH) coexistent with mucinous cystadenoma of the pancreas is reported. Primary MFH of the pancreas is rare, with only six patients reported in the world medical literature. A patient with MFH coexistent with a pancreatic mucinous epithelial tumor has not been reported previously, although two patients with pseudosarcomatous tumor associated with mucinous cystadenocarcinoma of the pancreas have been reported. Mural nodules of similar histologic appearance have been reported in ovarian mucinous tumors. The authors believe this to be the first report of the occurrence of MFH in mucinous cystadenoma of the pancreas.     

恶性纤维组织细胞瘤起源和分类及其治疗的研究进展

目的:总结关于恶性纤维组织细胞瘤(MFH)研究的历史、现状及面临的问题.方法:应用PubMed及CNKI期刊全文数据库检索系统,以"恶性纤维组织细胞瘤、组织学起源、分类和预后"为关键词,检索1990-2010年的相关文献,共检索到英文文献2 520篇和中文文献986篇.纳入标准:1)MFH的组织来源;2)MFH的分类;...     

Primary malignant fibrous histiocytoma of spleen with spontaneous rupture: a case report and literature review

Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma that mainly occurs in the lower and upper extremities, trunk and retroperitoneum. However, primary MFH of the spleen is especially rare. Only 11 cases of splenic MFH have been reported in the English literature. In this report, we describe a 35-year-old man who was found to have a large accumulation of free fluid in the abdominal cavity and a tumor mass 6 cm in diameter with rupture within the spleen by the abdominal ultrasonography. A splenectomy was performed and the histological diagnosis was malignant fibrous histiocytoma. The patient died 7 months after the operation as a result of generalized metastasis. Compared with the 11 previously documented patients of splenic MFH, our patient is the youngest and the first case with spontaneous rupture, which makes our case exceedingly rare. A literature review of primary MFH of spleen is also provided.     

Cardiac metastases in malignant fibrous histiocytoma. A case report

Malignant fibrous histiocytoma metastasizing to the left ventricle is an uncommon form of cardiac malignancy. This report describes a rare case of left ventricular metastases from a malignant fibrous histiocytoma of the posterior compartment of the right thigh, recurring five years after treatment with surgery, hyperthermic perfusion of the limb and radiotherapy. As the patient presented symptoms of cardiac tamponade, open heart surgery was performed through a median sternotomy; however, the tumor was not resectable and only a biopsy was performed. A partial response was obtained with standard and high-dose chemotherapy with peripheral blood progenitor cell transplantation. The response continued to improve with immunotherapy. The patient returned to normal physical activity. He died four years later due to a ventricular arrhythmia.     

A case of interleukin-6-producing malignant fibrous histiocytoma originating in the heart.

Primary cardiac malignant fibrous histiocytoma is extremely rare and its pathophysiological characteristics remain largely unknown. We treated a female patient with persistent fever and disseminated intravascular coagulation. Since ultrasonic echocardiography revealed the presence of a cardiac tumor and her serum interleukin-6 level was elevated, we speculated she had a cardiac myxoma. Histological examination of the surgically resected specimen, however, revealed that the tumor was malignant fibrous histiocytoma. Although her disseminated intravascular coagulation and heart failure were transiently improved after operation, local recurrence and systemic metastasis occurred and she died 7 weeks after operation. Using the autopsied specimen, we examined whether the malignant fibrous histiocytoma constitutively synthesized interleukin-6. The interleukin-6 content in the tumor was high, consistent with interleukin-6 production by the tumor. This was confirmed by immunohistochemical analysis. To our knowledge, this is the first report demonstrating interleukin-6 production by a cardiac malignant fibrous histiocytoma.     

Soft Tissue Paraspinal Inflammatory Malignant Fibrous Histiocytoma Presenting as a Lumbar Abscess

The rarest subtype of malignant fibrous histiocytoma (MFH) is the inflammatory type, which due to its peculiar clinical presentation may mimic an infectious process such as an abscess. The rarity of this disease and the unique features of its presentation may mislead the surgeon with consequent catastrophic results for the patient. In this study, a case report of a 65-year-old female patient with a soft-tissue inflammatory MFH presenting as a lumbar abscess and a review of the current literature are presented.Key words: Malignant fibrous histiocytoma, inflammatory type; Soft-tissue sarcoma     

Postradiation malignant fibrous histiocytoma and osteosarcoma of a patient with high telomerase activities

BACKGROUND: An extremely rare case of postradiation malignant fibrous histiocytoma (MFH) and osteosarcoma (OS) secondary to radiation therapy for leukemia-related osteolytic lesions is presented. In addition, the telomere biology of these tumors was investigated. CASE REPORT: A 14-year-old boy was diagnosed with acute lymphocytic leukemia. The right tibia was irradiated at a total dose of 60 Gy, and the left tibia was irradiated at a total dose of 40 Gy. The left tibia developed MFH and the right tibia developed OS. RESULTS: Telomere reduction (MFH 70.2, OS 70.0%) and high telomerase activities (MFH 12.1, OS 17.7 TPG) were observed. These results reflect an aggressive feature of postradiation sarcomas. CONCLUSION: Prognosis for patients diagnosed with postradiation sarcoma is poor due to its aggressiveness. However, even if sarcoma occurs after irradiation in more than two fields in a single patient, improvements in prognosis are anticipated with appropriate chemotherapies and wide resection.     

原发性软组织恶性纤维组织细胞瘤的MRI影像诊断（附15例报告）

目的：探讨原发性软组织恶性纤维组织细胞瘤（MFH）的MRI影像诊断。方法：回顾性分析15例经手术病理证实的原发于软组织的恶性纤维组织细胞瘤患者的影像学资料,15例患者均行MRI检查,有7例行CT检查,8例行增强扫描。结果：原发于软组织恶性纤维组织细胞瘤15例,肿瘤形态多样,以卵圆形、分叶状多见。病变于T1WI呈低信号或等信号,T2WI呈高信号或等信号为主混杂信号影,7例病变内可见低信号之分隔影。13例病变内见坏死或黏液样改变,2例伴出血。明确有包膜3例。增强扫描均呈显著不均匀强化。结论：MRI对原发性软组织恶性纤维组织细胞瘤的诊断具有重要价值,是首选的影像学检查方法。     

Primary Malignant Fibrous Histiocytoma of the Stomach: (A Case Report)

A case of primary malignant fibrous histiocytoma (MFH) of the stomach is reported. The patient was a 51-year-old man who had a laparotomy for a malignant gastric tumour diagnosed by radiological studies and endoscopic examination. An inoperable gastric malignancy was found at operation. Histopathological studies of tissue biopsied at surgery showed characteristic light microscopic findings and strong positivity for alpha-1-antitrypsin by immunoperoxidase technique indicating the diagnosis of malignant fibrous histiocytoma. The absence of any other soft tissue or retroperitoneal tumour strongly indicated the stomach is the primary site.     

Malignant fibrous histiocytoma of the breast with axillary lymph node involvement

This case history describes a 70-year-old female patient with a malignant fibrous histiocytoma (MFH) of the breast with secondary involvement of the skin and axillary lymph node metastases. We think that in the case of a MFH of the breast a radical or modified radical mastectomy should be pursued instead of a simple mastectomy.     

Malignant fibrous histiocytoma of the lung

Primary malignant fibrous histiocytoma (MFH) of the lung is very rare. To date, only 32 reports of 63 cases of primary MFH of the lung have appeared in English, excluding tumors arising from the pulmonary arteries and pleura. We describe a patient with primary MFH of the lung who developed brain metastasis and involvement of pulmonary great vessels. In addition, we reviewed previously reported cases to establish the clinical characteristics and most appropriate management of primary pulmonary MFH. When disease is sufficiently limited, complete resection remains the mainstay of treatment.