Malignant (fibrous) histiocytoma of bone--fact or fancy?.

Malignant (fibrous) histiocytoma is currently defined as a malignant primary bone tumor that contains a mixture of fibrogenic cells and cells that are histologically similar but which appear to be histiocytic. In this type of histiocytoma the nuclei are often indented; cytoplasm is usually abundant and may be slightly foamy; nucleoli are often large; and multinucleated malignant cells are usually a prominent feature. Many dedifferentiated chondrosarcomas, osteosarcomas, and fibrosarcomas of bone contain areas that resemble what we regard as malignant (fibrous) histiocytoma. When the entirety of a malignant tumor of bone fits the outlined histologic pattern, the designation of malignant (fibrous) histiocytoma seems appropriate. From 158 fibrosarcomas of bone and 962 osteosarcomas of bone in our files, 35 tumors were segregated because they appeared to be properly designated as malignant (fibrous) histiocytoma. A wide age range was represented by affected patients, and a large variety of bones harbored these tumors. Approximately one-third of patients eligible for 5-year follow-up were long-term, symptom-free survivors. Four deaths occurred from the tumor after more than 5 years, and one patient had radiographic evidence of pulmonary metastasis 7 years after amputation. Radiation therapy has been curative in at least two cases. The correct designation for these tumors in the light of current knowledge is malignant tumor, consistent with malignant (fibrous) histiocytoma.     

Metastatic intracerebral malignant fibrous histiocytoma from the lung

Malignant fibrous histiocytoma usually develops in the extremities, peritoneal or retroperitoneal space and the thigh. The occurrence in the lung and brain is quite rare. We report two cases of metastatic intracerebral malignant fibrous histiocytoma from the lung. The first patient developed cerebral metastasis six years after treatment of the primary site, and the second one died two months after the diagnosis of generalized metastases. Both were quite resistant to radiation therapy.     

Benign Fibrous Histiocytoma in Hodgkin's Disease

A case of benign fibrous histiocytoma in a patient with previous Hodgkin's disease is reported. Benign fibrous histiocytoma consists of a proliferation of benign histiocytes in addition to inflammatory cells and occasional Reed-Sternberg-like cells. Hodgkin's disease is considered a malignant disorder of the monocyte-histiocyte cell line but also includes benign inflammatory cells as part of its histological picture. The possibility that these two entities are biologically related is considered. The pathogenic relationship between both entities is discussed in the context of the immune dysregulation associated with Hodgkin's disease.     

Radiation-induced intracranial malignant fibrous histiocytoma.

An autopsy case of radiation-induced intracranial malignant fibrous histiocytoma (fibroxanthosarcoma) is reported. The tumor developed in the region of the sella turcica 11 years after high dose radiotherapy of a chromophobe adenoma of the pituitary. The tumor had infiltrated the base of the brain as well as the base of the skull. Metastases were not found. The tumor was composed of an admixture of bizarre fibroblasts, histiocytes and giant cells, xanthoma cells and siderophages, with a storiform fibrous stroma. This appears to be the first documented instance of a malignant fibrous histiocytoma occurring intracranially after local x-irradiation.     

Aneurysmal ("angiomatoid") fibrous histiocytoma of the skin

Seventeen cases are reported of a variety of cutaneous fibrous histiocytoma, which we have designated as aneurysmal ("angiomatoid") fibrous histiocytoma. These lesions differ from the classical cutaneous fibrous histiocytoma in both their clinical presentation and pathologic features. Clinically, they may be larger than the usual cutaneous fibrous histiocytoma, are blue, black, or dark red, and have a cystic consistency. They are most commonly located on the extremities and may be associated with symptoms of pain and rapid growth. The clinical diagnosis of fibrous histiocytoma is seldom considered in the differential diagnosis, which may include malignant melanoma, hemangioma, neurofibroma, and nonspecific cyst. Histologically, the lesions are characterized by the presence of large, blood-filled tissue spaces, which, at times, account for up to one half their size. These spaces lack an endothelial lining, being surrounded and lined by histiocytes, many of which contain hemosiderin pigment, fibroblasts, and foam cells. The solid portions of the tumor have the usual features of a cutaneous fibrous histiocytoma. This "angiomatoid" lesion is closely allied to what has been termed "hemosiderin histiocytoma," which appears to be a precursor stage in its formation. The presence of extravasated erythrocytes in combination with a spindle-cell stroma may lead to an erroneous diagnosis of Kaposi's sarcoma. This cutaneous tumor has architectural and cytologic similarities to its malignant soft tissue counterpart recently described as angiomatoid malignant fibrous histiocytoma. However, unlike the latter, the cutaneous lesion is benign and lacks the prominent inflammatory infiltrate, pleomorphic appearance, and systemic manifestations of its soft tissue counterpart. The distinctive clinical and pathologic features of the cutaneous lesion serve to separate it as a specific variant of the cutaneous fibrous histiocytomas.     

髋臼周围肿瘤切除术后重建

目的探讨对髋臼周围肿瘤手术切除及重建骨盆稳定性的方法，最大限度保留肢体功能，减少复发。方法2002～2005年15例髋臼周围肿瘤患者，其中软骨肉瘤8例，恶性纤维组织细胞瘤3例，Ewing肉瘤2例，转移瘤2例；6例配合化疗，4例行瘤段灭活再植，1例行瘤段灭活再植＋人工全髋置换，10例行人工假体置换（包括全髋）。结果1例术后死亡，1例感染，清创后股骨头旷置；14例术后随访9～33个月，4例软骨肉瘤复发，其他病例均能保留患肢部分功能和骨盆稳定性。结论恶性纤维组织细胞瘤、Ewing肉瘤化疗有效者保肢率高，局部复发率低；软骨肉瘤复发率高；瘤段灭活再植因骨愈合时间长，影响功能；股骨头旷置肢体短缩，跛行明显；人工假体置换可最大限度地保留肢体功能；单纯转移瘤为提高生存质量，减轻痛苦，可考虑手术治疗，但年龄大，全身脏器应急能力差者，手术应慎重。     

Establishment and characterization of neoplastic cells from a malignant fibrous histiocytoma. A possible stem cell line

Using the semisolid agar method, neoplastic three clones were isolated from a malignant fibrous histiocytoma. All clones represented morphologically only one type of cells having fine structure similar to that of histiocyte with multiple filopodia. The clones carrying Fc- and C3-surface receptors showed marked immunophagocytosis. They were positive for acid phosphatase, nonspecific esterase, and alpha-1-antitrypsin. These clones were able to display the potential for production of collagenous matrix. Moreover, inoculations of the each clone into nude mice resulted in productions of malignant fibrous histiocytoma with pleomorphic pattern. These tumors were composed morphologically of various types of cells such as immature, histiocyte-like, fibroblast-like, and multinucleated giant cells. These morphologic alterations of histiocytes occurred in all of the three clones in vitro. These findings suggest that the cloned cells established from the malignant fibrous histiocytoma are neoplastic histiocytes with capability to form various types of cells, a possible stem cell.     

Pleomorphic leiomyosarcoma of the soft parts: a reassessment by histology and immunohistochemistry of pleomorphic soft tissue sarcomas

Histologically it is often difficult to differentiate malignant fibrous histiocytoma (MFH) from leiomyosarcoma. Sixty-three cases formerly diagnosed as pleomorphic soft tissue sarcomas were examined by immunohistochemistry. We identified three different types according to its positivity for the myogenic markers: A) positive cells with a fascicular pattern, 13 cases (26%); B) a small number of scattered positive cells, 29 cases (57%); C) negative for markers, 9 cases (17%). We reassessed 51 MFHs as follows: A type as pleomorphic leiomyosarcoma, B+C types as MFH. Pleomorphic leiomyosarcomas were also positive for collagen type IV around the compactly arranged individual tumor cells. An imunohistochemical analysis and detection of fascicular structures are thus considered to be necessary in order to distinguish between leiomyosarcoma and MFH.     

Malignant giant cell tumor of soft parts. An ultrastructural study of four cases.

Four cases of malignant giant cell tumor of soft parts (MGCT) were studied ultrastructurally. Most of the cells in three cases were identified as undifferentiated mesenchymal cells and mononuclear monohistiocytic cells. The osteoclastlike cells seemed to arise from fusion of monohistiocytic cells. Immature fibroblastic cells, occasionally containing filaments with densities, were also present. They were scarce in three cases and predominant in the fourth. It was concluded that MGCT is a mesenchymal sarcoma with a bimorphic--monohistiocytic and fibroblastic--differentiation. MGCT is therefore considered a special variant of malignant fibrous histiocytoma.     

Benign and malignant fibrous histiocytomas of the soft tissues: functional characterization of the cultured cells

Cell cultures were carried out from fresh tumor tissues obtained from seven cases of histiocytic tumors of the soft tissues including four of malignant fibrous histiocytoma, two of benign fibrous histiocytoma, and one of giant cell tumor of tendon sheath. The cultured cells were studied by light and electron microscopy, rosette formation for surface receptors, immune phagocytosis, and enzyme cytochemistry. The culture of each tumor revealed a mixture of histiocyte-like cells, fibroblast-like cells, amd intermediate forms. Bizarre giant cells were numerous in the early cultures of malignant fibrous histiocytoma, but they were absent in the cultures of benign fibrous histiocytoma and giant cell tumor of tendon sheath. In all the seven cases the cultured cells exhibited several features characteristic of histiocytes: (1) surface receptors for the Fc-portion of immunoglobulin G; (2) surface receptors for the third component of complement; (3) immune phagocytosis of opsonized erythrocytes; and (4) lysosomal enzymes including alpha-naphthyl butyrate esterase, and acid phosphatase. These findings suggest that the histiocytic tumors of the soft tissues in discussion actually comprise the cells that have the functional property of histiocyte. The tumor cells in the surgical specimens presented essentially the same ultrastructural and enzyme histochemical characteristics as those of the cultured cells.     

Transthoracic fine-needle aspiration biopsy of pulmonary spindle cell and mesenchymal lesions: a study of 61 cases

BACKGROUND: Spindle cell and mesenchymal lesions of the lung encompass a wide variety of benign and malignant conditions. However, to the authors' knowledge, because of their rarity, few reports concerning their cytologic findings are available in the literature. The current review emphasizes the cytomorphologic features, differential diagnosis, and potential pitfalls associated with these lesions. METHODS: Seven hundred seventy-nine percutaneous lung fine-needle aspiration (FNA) specimens were retrieved from the authors' cytopathology files over a period of 5 years. Sixty-one cases (7.8%) in which a spindle cell component was the dominant or key feature were identified. The authors reviewed the cytologic smears, immunocytochemical studies, and corresponding surgical material and clinical information. RESULTS: Of these 61 aspirates, 33 (54%) were reactive processes (31 granulomas, 1 organizing pneumonia, and 1 inflammatory pseudotumor). Five cases (0.8%) were benign neoplasms (2 hamartomas, 2 solitary fibrous tumors, and 1 schwannoma). Twenty-three cases (38%) were malignant neoplasms (8 cases were primary tumors [including 5 carcinomas with spindle cell or sarcomatoid features, 1 spindle cell carcinoid tumor, 1 leiomyosarcoma, and 1 synovial sarcoma] and 15 cases were secondary tumors [including 9 melanomas, 2 leiomyosarcomas, 1 malignant fibrous histiocytoma, 1 meningioma, 1 sarcomatoid renal cell carcinoma, and 1 uterine malignant mixed müllerian tumor]). A specific diagnosis was rendered in 52 cases (85%). No false-positive cases were encountered but there was one false-negative case. One patient who was diagnosed with granulomatous inflammation on FNA was found to have nonsmall cell lung carcinoma on subsequent transbronchial biopsy. No malignant cells were identified in the smears on review. The FNA from the organizing pneumonia was interpreted as a solitary fibrous tumor whereas the inflammatory pseudotumor was diagnosed as granulomatous inflammation. The FNA from one pulmonary hamartoma initially was considered to be nondiagnostic. One solitary fibrous tumor and the schwannoma were diagnosed as smooth muscle tumor and spindle cell tumor, not otherwise specified, respectively. Among the malignant tumors, the primary synovial sarcoma and one of the metastatic malignant melanomas initially were interpreted as primitive neuroectodermal tumor/Ewing sarcoma and poorly differentiated carcinoma, respectively. CONCLUSIONS: Spindle cell lesions of the lung rarely are encountered on transthoracic lung FNA and are comprised of a wide variety of benign and malignant entities. By correlating clinical and radiologic data, cytologic findings, and ancillary studies, a high diagnostic accuracy rate can be achieved with FNA.     

Malignant fibrous histiocytoma of bone. A review of 13 cases and an ultrastructural study

Observations on 13 patients with primary malignant fibrous histiocytoma of bone were reported. Included were nine male and four female patients, ranging in age from 6 to 81 years (mean, 44.8 years). Pain was the most common complaint. The interval from the first symptom to the initial treatment varied from 2 months to 20 years. Seven tumors arose in the knee region. Roentgenologically, most of the lesions presented with an osteolytic and destructive appearance. Histologically, highly variable morphologic features existed. The storiform-pleomorphic pattern was found in every tumor, although it was not necessarily pathognomonic for malignant fibrous histiocytoma of bone. Surgery, radical or incomplete, was the primary treatment for all but one patient. Lymph node metastasis was present in three. Five patients died of the disease from 3 to 79 months after the diagnosis (mean, 28 months), all exhibiting metastasis to the lung. Gaucher's body-like structure demonstrated in the electron microscopic study shows the histiocytic quality of malignant fibrous histiocytoma of bone.     

Malignant fibrous histiocytoma. An electron microscopic study.

Sixteen cases of malignant fibrous histiocytoma are presented. Electron microscopy of 15 cases demonstrated fibroblast-like and mononuclear and multinucleated histiocyte-like cells. A small capillary was at the center of all storiform areas examined. Ultrastructural examination can be diagnostically useful within the context of a narrow differential diagnosis by conventional microscopy and the ability, by electron microscopy, to eliminate other mesenchymal cell types. In 13 cases, follow-up information was available from 18 months to 9 years following histological diagnosis. Five patients are alive and 8 patients have died, including two non-tumor related deaths. In 3 cases follow-up was less than 4 months. The biologic behavior of the tumor in this series was generally not related to histopathological parameters. The issue of histogenesis is largely unresolvable. Ultrastructural studies of various types of fibrous histiocytomas, suggesting cells of origin other than histiocytes, give credence to the concept that the histiocyte may represent a morphologic state of a given mesenchymal cell rather than a particular cell type.     

Malignant fibrous histiocytoma (malignant fibrous xanthoma: xanthosarcoma) of bone.

Seven cases of malignant fibrous histiocytoma of bone are documented. In addition to typical histologic features such as the presence of a storiform pattern, histiocytic and fibrocytic cell types and severe cellular atypism, in five cases there were areas in which osteoclast-type giant cells were prominent and the lesion resembled a giant cell tumor. Three of the seven patients developed pulmonary metastases within two years of amputation, one developed extensive local recurrence including soft tissue and vascular invasion, and the other three are without evidence of disease but have been followed for only short periods.     

Sarcomatoid carcinoma of the lung. Immunohistochemical and ultrastructural studies of 14 cases.

The authors retrospectively reviewed data regarding 14 patients with sarcomatoid carcinomas of the lung seen and treated at M.D. Anderson Cancer Center from 1955 to 1986. The following were the histologic criteria for inclusion in the study: (1) the concurrent presence of malignant epithelial and sarcomatoid spindle cell components, and (2) positive immunoreactivity for antikeratin antibody or ultrastructural demonstration of epithelial differentiation in sarcomatoid tumors in which the epithelial component was inconspicuous. For the sarcomatoid components, the most frequent pattern was malignant fibrous histiocytoma, which was present in ten tumors. An unclassified sarcomatoid pattern was found in two cases and a fibrosarcomatous pattern in two remaining cases. Clinically, the median patient age was 59 years; 12 patients were male and 2 were female; 13 were smokers and 1 used snuff. Three patients had Stage I, ten had Stage III, and one had Stage IV disease. One patient with Stage I, seven with Stage III, and one with Stage IV disease died of their carcinomas 2 to 26 months after diagnosis (median survival time 12 months). All patients who had lymph node metastases at presentation died of disease. The authors concluded the following: (1) patients with sarcomatoid carcinoma of the lung usually presented at an advanced stage; (2) lymph node metastasis, as with a usual carcinoma of the lung, is an important prognostic factor; and (3) for all lung tumors with a sarcomatoid pattern, especially a malignant fibrous histiocytoma pattern, extensive samples should be obtained and immunoperoxidase or ultrastructural studies done to identify epithelial differentiation.     

Increased incidence of brain metastases in sarcoma patients

Eleven cases of brain metastases that developed in 114 sarcoma patients are presented. Two of 11 patients presented with brain metastasis at the time of diagnosis and the other nine developed them later. The high incidence of brain metastases in patients with rhabdomyosarcoma (26%) and malignant fibrous histiocytoma (27%), two types of tumor which supposedly metastasize rarely to the brain, is remarkable. The increased incidence of brain metastases may be related to longer survival of sarcoma patients and to the inability of AMN and other drugs used in the treatment of sarcomas to cross the blood-brain barrier. Preventive treatment of brain metastases with drugs active in the CNS or with radiotherapy following the diagnosis of pulmonary metastases, could be useful, especially in patients with rhabdomyosarcoma and malignant fibrous histiocytoma.     

10例肺原发恶性纤维组织细胞瘤临床分析

目的研究肺原发恶性纤维组织细胞瘤的治疗方法及其预后。方法回顾性分析经手术病理证实的肺原发恶性纤维组织细胞瘤10例,其中5例单纯手术切除,3例术后放射治疗,2例术后化疗。结果10例患者中,6例生存1年以上,其中4例生存均超过2年,且3例超过3年。1年生存率为60%,2年生存率为40%。局部复发1例,远地转移1例,局部复发加远地转移3例,复发时间1～18个月。结论肺原发恶性纤维组织细胞瘤的预后较差,主要治疗手段为外科手术,术后放射治疗和化疗的疗效尚不肯定。     

Clinico-morphological analysis of bone fibrosarcomas (a retrospective study)

The data on 35 cases of bone fibrosarcoma treated at the Center clinic in 1955-1982 are presented. A retrospective morphological investigation left diagnosis unchanged in 17 cases (48.6%). In 9 cases diagnosis was changed to fibrous histiocytoma of the bone, in 2--parostal sarcoma and in one case--"dedifferentiated" chondrosarcoma. In 6 cases, diagnosis could not be improved and neoplasms were identified as poly-morphocellular sarcoma. Clinically, a more favorable course and prognosis are distinguishing features of fibrosarcoma as compared with osteogenic sarcoma and malignant fibrous histiocytoma.     

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。     

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。