Primary Renal Malignant Fibrous Histiocytoma Four-Case Report and Review of the Literature

OBJECTIVE To evaluate the diagnosis and treatment for primary renal malignant fibrous histiocytoma, a rare tumor arising from the kidney.METHODS The clinical and pathological data from 4 cases of malignant fibrous histiocytoma of the kidney detected in our hospital are described.One case of special interest involved a giant cell subtype, the first to be reported in the oncology literature. The clinicopathologic features and prognostic factors of this tumor were analysed and summarized after reviewing 55 documented cases in the English and Chinese literature.RESULTS A palpable mass (71.2%), emaciation (54.2%) and pain (54,2%) were common manifestations in renal MFH, Of all the cases, 51 were identified as a storiform-pleomorphic subtype by pathologists. Inconsideration of all the prognosis related factors, the residual tumor and high TNM stage predicted a shortened survival duration, but the symptom of a fever served as a better prognostic factor.CONCLUSION Malignant fibrous histiocytoma which arises from the kidney is a rare pathologic type, and possesses a high tendency towards local recurrence and distant metastasis. Despite the poor prognosis, early detection and radical surgery can prolong survival in selected cases.     

腹膜后巨大恶性纤维组织细胞瘤误诊1例并文献复习

目的观察腹膜后恶性纤维组织细胞瘤的临床表现、病理形态及免疫组化特点，吸取误诊教训。方法通过对1例腹膜后恶性纤维组织细胞瘤的临床表现、光镜形态及多次免疫组化结果进行观察，明确诊断，并复习相关文献。结果恶性纤维组织细胞瘤多发于中老年男性。根据镜下所见及第一次免疫组化结果曾诊断为巨大恶性神经鞘膜瘤。后又经多次免疫组化结果S-100（-），NF（-），排除恶性神经鞘膜瘤的可能；经多次专家会诊及远程会诊，确诊为腹膜后巨大恶性纤维组织细胞瘤。结论原发性腹膜后恶性肿瘤比较多样化，因此他的确诊依赖于镜下形态和可靠的免疫组化结果，这些经验教训值得大家借鉴。     

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.     

乳腺恶性纤维组织细胞瘤1例报告并文献复习

目的：探讨乳腺原发性恶性纤维组织细胞瘤（MFH）的临床病理学、免疫组化特征和预后。方法：对手术切除一例乳腺MFH进行病理常规切片及免疫组化观察,并结合文献复习。结果：瘤组织由纤维细胞、纤维母细胞、黄色瘤样细胞组成。免疫组化CD68、Vimentin阳性表达,Ki67增殖指数38．38％。结论：MFH根据其分类及亚分类、分级、瘤体大小、部位、肿瘤深度,评估预后。     

乳腺恶性纤维组织细胞瘤1例报告并文献复习

目的:探讨乳腺原发性恶性纤维组织细胞瘤(MFH)的临床病理学、免疫组化特征和预后。方法:对手术切除一例乳腺MFH进行病理常规切片及免疫组化观察,并结合文献复习。结果:瘤组织由纤维细胞、纤维母细胞、黄色瘤样细胞组成。免疫组化CD68、Vimentin阳性表达,Ki67增殖指数38.38%。结论:MFH根据其分类及亚分类、分级、瘤体大小、部位、肿瘤深度,评估预后。     

Malignant fibrous histiocytoma: report of five cases and a review of the literature.

Five cases of malignant fibrous histiocytoma are presented and the relevant literature is reviewed. All the patients had locally extensive tumor or eventual recurrent or metastatic disease. Determining malignancy by histologic criteria is difficult. Therefore, histologic, gross, and clinical behavior of the tumor is important. Though the role of radiation therapy and chemotherapy is not yet established, it appears that at least wide "cancer resection," if possible, is the treatment of choice. The unusually high incidence of nonresectable, recurrent, or metastatic disease in retroperitoneal histiocytomas raises the question as to whether all three modalities of therapy should be used in the treatment of tumors at this particular site.     

Primary laryngeal malignant fibrous histiocytoma: a case report

Malignant fibrous histiocytoma (MFH) of the upper respiratory tract is rare. We report a case of laryngeal MFH in a 78-year-old man. The patient died 8 months after the first surgical treatment. The clinicopathologic features are described, and the importance of electron microscopy and immunohistochemical techniques in the differential diagnosis with so-called "sarcomatoid carcinoma" is stressed. The tumor has an aggressive biological behavior and tends to recur and metastasize.     

Malignant fibrous histiocytoma of maxilla — a report of five cases and review of literature

Malignant fibrous histiocytoma of the maxilla is a rare malignant tumor of the bone. Affection of maxilla does not occur very commonly. In this report five cases of fibrous histiocytoma have been presented along with a review of literature.     

Myxoid malignant fibrous histiocytoma of the ovary: a case report

A 46-year-old woman presented with whole abdominal discomfort, and imaging revealed a 3-cm-sized ill-defined ovarian mass with extensive peritoneal carcinomatosis. Histologic examination showed malignant fibrous histiocytoma of ovary with predominant myxoid stroma. Microscopic examination showed a highly cellular neoplasm composed of fibroblast-like cells with a predominant myxoid stroma and high pleomorphism and mitotic activity. Immunohistochemically, the tumor was negative for smooth muscle actin, desmin, S-100, pancytokeratin, c-kit, epithelial membrane antigen, and calretinin. Malignant fibrous histiocytoma of ovary is extremely rare, with only six previously reported cases. To the best of our knowledge, the myxoid type of malignant fibrous histiocytoma of ovary has not been previously reported in the English literature except for a case arising in a dermoid cyst of ovary. We present the case and briefly discuss the differential diagnosis.     

左股骨下段原发性恶性纤维组织细胞瘤脑室内转移1例报告及文献复习

目的: 探讨骨原发性恶性纤维组织细胞瘤(MFH)的临床特点及诊断.方法: 报告1例原发性股骨下端MFH并脑室转移病例结合文献复习.结果: MFH临床表现无特征性,临床及影像学诊断困难,MFH的诊断主要依赖于组织病理学资料,治疗后易出现局部复发和远处转移.结论: 若患者出现骨痛,局部肿块,影像学上如出现虫蚀状或大片状溶骨性破坏,巨大软组织肿块,无骨膜反应,应考虑到本病.     

Malignant fibrous histiocytoma of the heart. A case report and review of the literature

A case report of a 28-year-old woman with malignant fibrous histiocytoma (MFH) of the left atrium is presented, and the six previous reports of this rare cardiac tumor are reviewed. A tendency for malignant fibrous histiocytoma of the heart to occur in the left atrium of young women is suggested; this sarcoma's usual location is in the soft tissue of elderly men. The apparent predilection for the left atrium is unique among cardiac malignancies. Careful pathologic study is necessary to differentiate the uniformly fatal MFH of the heart from the more common benign atrial myxoma.     

Postirradiation malignant fibrous histiocytoma in a patient with squamous carcinoma of the uterine cervix--an autopsy case

An autopsy case of postirradiation sarcoma is described. The tumor, a malignant fibrous histiocytoma, occurred in the radiation fied 7 years after postoperative external beam radiation therapy (6000 rad) for squamous cell carcinoma of the cervix. To our knowledge, 11 cases of postirradiation malignant fibrous histiocytoma have been reported in the literature and only one occurrence of this neoplasm after treatment for cervical cancer has been described. We review the literature on postirradiation sarcoma with special attention to malignant fibrous histiocytoma.     

An autopsied case of malignant fibrous histiocytoma of the spleen

An auspsied case of malignant fibrous histiocytoma (MFH) is reported. The patient, a 53-year-old male had developed splenomegaly 1 year before admission. On admission, hepatosplenomegaly and ascites were seen, with prominent leucocytosis in the peripheral blood, and the patient died of cachexia. On autopsy, the primary neoplasma was found in the spleen, with metastasis to the liver, vertebrae, lymph nodes and peritoneum. Histologically, to tumor showed a storiform arrangement of tumor cells, with foamy or hemosiderin-faden cytoplasm in places, corresponding to MFH. As far as we know, there have been no reports of primary MFH of the spleen in Japan.     

Chemotherapy of malignant fibrous histiocytoma: a Southwest Oncology Group report.

Malignant fibrous histiocytoma is a rare tumor, which constitutes 3-4% of the soft tissue sarcomas. It occurs with maximum frequency in the sixth and seventh decades of life and has a distinct male preponderance. In two-thirds of the patients an extremity is the primary site and approximately one-half develop local recurrences and one-half, distant metastases. Response to combination chemotherapy occurred in 33%, a rate similar to that seen in other sarcomas.     

骨良性纤维组织细胞瘤的影像学表现(附4例报道及文献复习)

目的:探讨骨良性纤维组织细胞瘤的影像学特点,以提高对该病的影像学诊断水平及鉴别诊断能力.方法:收集4例骨良性纤维组织细胞瘤患者的临床及影像学资料,结合近年来国内外相关文献进行回顾性分析.结果:4例骨良性纤维组织细胞瘤均表现为类圆形或椭圆形骨质破坏区,边界清楚,有完整硬化环,环壁厚薄较均匀,无骨膜反应.2例为多发病变,病变累及双侧股骨及髂骨;2例MRI检查T1WI等信号,T2WI不均匀高信号,其中1例病变周围可见软组织肿块;1例CR及CT检查提示病灶内有骨性分隔和软组织密度影.结论:骨良性纤维组织细胞瘤影像学上具有一定特异性,认识其表现有助于对该病的诊断和鉴别诊断.     

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.     

Myxoid malignant fibrous histiocytoma of the bladder

Although the most common soft tissue sarcoma of adulthood, malignant fibrous histiocytoma (MFH) is an extremely rare tumor of the urinary bladder. Only three well-documented cases have been reported in the world literature. The patient presented in this report represents the first case of the myxoid variant to develop in the urinary bladder. Whereas all previous patients with MFH of the bladder had intermittent hematuria, this patient's chief complaint was bladder outlet obstruction due to extension of the tumor into the prostate. He was managed with radical cystoprostatectomy, postoperative radiation therapy to the tumor bed, and adjuvant chemotherapy using doxorubicin. The patient tolerated the therapy well and was disease-free at the 3-year follow-up visit. The histogenesis, clinical features, pathologic characteristics, and treatment considerations of this rare bladder tumor are discussed in detail.