Malignant fibrous histiocytoma

Summary The ultrastructural findings in 17 cases of malignant fibrous histiocytoma (MFH) are described. The tumors consisted of fibroblast-like cells and histiocyte-like cells in different proportions in different cases. Intermediate, undifferentiated, xanthomatous and multinucleated giant cells were also identified. In 12 of 17 cases myofibroblasts were evident. Acid phosphatase activity was detected cytochemically in the Golgi zone, endoplasmic reticulum and lysosomes (GERL) mainly within histiocyte-like cells, in three cases. These observations indicate that the GERL of the tumor cells are engaged in the formation of lysosomes. The polymorphic cellular composition, including undifferentiated cells, lends support to the concept that the MFH originates from a primitive multipotent undifferentiated mesenchymal cell.Supported by Grants-in-aid for Cancer Research from the Ministry of Health and Welfare (52-1), and from the Ministry of Education, Science and Culture (401057) of Japan and by a Grant from the Fukuoka-ken Anti-Cancer Association. 1976Presented at the 69th Annual Meeting of the Japanese Pathological Society on June 26, 1980, Sapporo     

Malignant fibrous histiocytoma of bone: initial diagnosis by aspiration biopsy cytology

Aspiration biopsy cytology by fine needle is becoming a popular diagnostic procedure for soft-tissue lesions; it also can be used successfully to diagnose some lesions from bone. The value of this procedure for making the initial diagnosis is illustrated by a case in which a malignant fibrous histiocytoma from a skull bone afflicted with Paget's disease was aspirated and interpreted as a sarcoma. This was later confirmed and subclassified by histology and electron microscopy from the tissue.     

Primary pleomorphic malignant fibrous histiocytoma of the heart

Primary pleomorphic malignant fibrous histiocytoma of the heart is rare. The present study was performed to study the clinical and pathological features of the disease. We describe two rare cases of primary cardiac malignant fibrous histiocytoma and review the published individual data of the patients. Both patients complained of dyspnea, and underwent palliative tumor resection. However, they died several months after surgery. A thorough literature review with clinical presentations, diagnostic features, treatment, and outcomes was done. We have for the first time analyzed the factors related to the survival of malignant fibrous histiocytoma. It is usually difficult to make an appropriate preoperative diagnosis. Despite complete surgical resection and aggressive chemotherapy and radiotherapy, the prognosis is still poor.     

Malignant fibrous histiocytoma of the lung

Summary A case of malignant fibrous histiocytoma of the lung is reported. The tumour margin was well circumscribed, showing an expanding border and no capsule. The main part of the tumour was composed of spindle-shaped fibroblast-like cells arranged in broad fascicles with a partially storiform pattern. Other parts of the tumour were arranged in a haphazard pattern, containing many mononucleated and multinucleated giant cells. Ultrastructurally six differnt cell types were encountered. The dominant type was a fibroblast-like cell; also present were many giant cells and some histiocyte-like cells, together with their intermediate forms, and few undifferentiated mesenchymal cells. We consider this tumour to have developed from the peribronchial connective tissue; it has the same cellular composition as the malignant counterpart arising in soft tissues.     

Malignant fibrous histiocytoma arising in a recurrent chordoma

Summary We present the case of a sacrococcygeal chordoma which recurred 15 years after the radical removal as a soft tissue tumor in the gluteal musculature. This tumor consisted of two parts: a chordoma without symptoms of aggressive cellular proliferation and a malignant fibrous histiocytoma. During the following 4 years several local recurrences of the malignant fibrous histiocytoma occurred in the gluteal musculature. The patient finally died of lung metastases. No chordoma tumor tissue was found in the lungs, in the gluteal musculature or in the sacrococcygeal bone area. Histology including electron microscopy revealed no proof of a transition of chordoma into malignant fibrous histiocytoma. It must be assumed that the secondary soft tissue tumor originated from residual chordoma cells which were implanted during the operation of the primary tumor. It remains unclear whether the malignant fibrous histiocytoma arose from mesenchymal stromal cells within the chordoma or directly from primitive neuroectodermal chorda cells which possess the ability to differentiate into a variety of cell types including mesenchymal cells.     

Malignant fibrous histiocytoma of bone: an ultrastructural study

An ultrastructural study of a case of malignant fibrous histiocytoma of bone in a 16-year-old skeletally mature female is presented. There were multiple metastatic bone lesions and a marked hypercalcemia. The cell population was similar to that found in soft tissue malignant fibrous histiocytoma lesions and comprised undifferentiated cells, fibroblastlike cells, histiocytes, and multinucleated giant cells. Occasional myofibroblasts and transitional cells with histiocytic and fibroblastic components were seen. The histiocytes were characterized by prominent Golgi bodies. Although there were many fibroblastlike cells, the rough endoplasmic reticulum was rarely as extensive or as well organized as in normal fibroblasts. The giant cells did not have the ultrastructural characteristics of osteoclasts, i.e., the clear zones and ruffled borders. Zonula adherens (belt desmosome) junctions were seen, but in general, intercellular junctions were poorly developed as to length and extent.     

Angiomatoid malignant fibrous histiocytoma

Summary A case of an angiomatoid malignant fibrous histiocytoma is presented. The electron microscopic findings demonstrate the presence of a variety of tumor cell types including smooth and striated muscle cells. This indicates that malignant fibrous histiocytoma originates from a pluripotent primitive mesenchymal cell.     

Malignant fibrous histiocytoma of the pancreas

An extremely rare case of malignant fibrous histiocytoma In the pancreas Is reported. A 70-year-old man complained of upper abdominal discomfort. A tumor in the head of the pancreas was demonstrated by ultrasonography and computed tomography. The surgical specimen revealed a relatively well demarcated tumor, 9 × 7 × 6.5cm in size. Microscopically, fibroblastic, histiocytic, and muitinucleated giant tumor cells were observed in the myxoid area, but some tumor cells had proliferated in a storiform-pleomorphlc pattern. Immunohtstochemically, some tumor cells were positive for lysozyme, α-1-antitrypsin, α-1-antichymotrypsin, and vimentin. Electron microscopically, tumor cells showed a combination of fibroblastic and histiocytic features. The patient Is currently well with no evidence of recurrence or metastasis 22 months after operation.     

骨恶性纤维组织细胞瘤13例临床病理分析

目的 观察骨恶性纤维组织细胞瘤(BMFH)病理形态，进一步探讨其组织形态学发生、病理学诊断及鉴别诊断。方法 通过形态学观察、免疫组织化学应用并结合有关电镜文献资料及相关临床病理资料进行综合分析。结果 BMFH由骨原始间充质细胞发生并具有组织细胞、原纤维细胞形态学特征和免疫学表型及中间型细胞(过渡细胞)超微结构特点和多项分化潜能。结论 BMFH从形态学上须与骨纤维肉瘤、恶性骨巨细胞瘤及特殊类型骨肉瘤相鉴别，从组织学发生上彼此间也有着密切的联系，并以此为依据为术前细针穿刺病理诊断的可靠性及治疗方案的选择提供参考。     

Osteosarcoma with features mimicking malignant fibrous histiocytoma

Summary Three osteosarcomas (OS) with features resembling malignant fibrous histiocytoma (MFH) were selected and investigated to identify any clinico-pathological similarities. In all cases there was no significant difference from conventional OS on the radiography and laboratory data. The appearance of MFH-like features within the whole tumour tissue varied from 7% to 55%. It was composed of spindle-shaped cells arranged in short irregular fascicles and a storiform pattern admixed with osteoclast-like giant cells, but devoid of neoplastic osteoid. Such spindle-shaped cells had a poorly developed rough endoplasmic reticulum and expressed a strong alkaline phosphatase activity as well as vimentin. A series of allografts to athymic mice using the MFH-like tissues also showed histologically a proliferation of plump spindle-shaped cells with a storiform pattern lacking osteoclast-like giant cells, and intensely positive for alkaline phosphatase. These findings indicate that the MFH-like features are identified as modulated OS. The constituting cells are most likely to be poorly developed with possible phenotypic alteration in the maturation stage of osteoblastic cell lineage, but different from conventional MFH of bone as regards their distinct histochemical pattern.     

Malignant fibrous histiocytoma of chest wall

Primary malignant fibrous histiocytoma (MFH) of the chest wall is rare. We report a case of primary MFH arising from the chest wall, which was thought to be a metastasis or myeloma. The imaging study revealed a single mass of the chest wall involving a rib. Resection and chest wall reconstruction was done. The histologic diagnosis was storiform-pleomorphic primary MFH. Although MFH of the chest wall is an uncommon pathology, it should be considered in the differentiation of a single bony destructive lesion involving the rib with a soft tissue component.     

Malignant fibrous histiocytoma of the esophagus

A 78-year-old man presented with an esophageal polyp that was confirmed by Immunohistochemistry and electron microscopy to be malignant fibrous histiocytoma. The tumor was comprised of a proliferation of spindle-shaped cells admixed with bizarre giant cells. These tumor cells were immunoreactive for smooth muscle actin, vimentin, α-1-arrti-chymotrypsin and CD68. Electron microscopic examination revealed the myoflbroblastic and histtocytic features of the tumor cells. No elements of epithelial or myogenic differentiation were found in the tumor. Malignant fibrous histiocytoma of the esophagus is extremely rare, with 10 cases being documented so far in the literature. The differential diagnosis of pteomorphic tumors of the esophagus is discussed.     

Inflammatory malignant fibrous histiocytoma of kidney: a case report

Among the renal sarcomas, inflammatory malignant fibrous histiocytoma (MFH) is an extremely rare presentation. A 45-year-old woman presented with acute retention urine following an episode of gross hematuria. Computerized tomography showed a solid mass at the lower pole of the left kidney. The patient underwent left nephrectomy. Histologically and immunohistochemically, the tumor was diagnosed as an inflammatory subtype of MFH. Histological appearances of inflammatory MFH vary widely and frequently overlap with benign reactive conditions such as Xanthogranulomatous pyelonephritis (XGPN) and malignant lesions, e.g. malignant lymphoma and, less frequently, a sarcomatoid variant of renal cell carcinoma. It is important, though difficult, to differentiate inflammatory MFH from these lesions. Careful morphological examination and immunohistochemical findings of the lesion are of great value, in particular in excluding it from its mimics. We discuss the pathological features and challenges involved in differentiating inflammatory MFH from its masquerader.     

An ultrastructural study of malignant fibrous histiocytoma of the larynx

Malignant fibrous histiocytoma (MFH) of the vocal cord occurring in a 46-year-old male was studied immunohistochemically and ultrastructurally. The tumor consisted of the two main areas, pleomorphic and storiform. Immunohistochemical staining was strongly positive for α1-antitrypsin and α1-antichymotrypsin, and negative for desmin, keratin, myoglobin, actin, carcinoembryonic antigen, and S100 protein. Ultrastructurally, this MFH consisted of four different types of cells which were either fibroblast-like, histiocyte-like, myofibroblast-like, or with features of both the fibroblast and histiocyte types. Transition forms between the four types of cells were also found. This suggests that cells of MFH are derived from the same undifferentiated stem cells.     

An immunohistochemical study of differentiation in malignant fibrous histiocytoma

Immunohistochemistry was used to examine 10 cases of malignant fibrous histiocytoma. Malignant cells in all cases expressed vimentin and in eight there was co-expression of either desmin or neurofilament, both of these being present in four cases. In addition, cytokeratin was found in one case. In each tumour, a population of small cells was identified which had the staining characteristics of benign macrophages, and this was distinct from the tumour cells. This study supports the concept that malignant fibrous histiocytoma is a tumour of mesenchymal cells rather than of histiocytes and emphasizes the diversity of its cytostructure.     

Inflammatory fibrous histiocytoma of the penis

A 62-year-old man presented with a penile mass which was diagnosed histologically as an inflammatory fibrous histiocytoma. The predominant inflammatory cells in the tumour were eosinophils. The tumour regressed after radiotherapy but recurred eight months later, when it showed very few inflammatory cells. At no time did this patient have an elevated white cell count. This tumour has not previously been reported on the penis, nor have eosinophils been described as the principal inflammatory cell in these tumours.     

Atypical fibrous histiocytoma of the scrotum

Atypical fibrous histiocytoma is a rare neoplasm. A scrotal location for this tumor is even more unusual. We report a case of a 90-year-old man with scrotal atypical fibrous histiocytoma. Our case had histologic features consistent with those cases previously reported in the literature. The tumor consists of cells with large hyperchromatic irregular nuclei, bizarre multinucleated cells (monster cells), and xanthomatous cells with large prominent nuclei set in a background of classic fibrous histiocytoma. Rare mitotic figures are identified. Immunohistochemical studies showed the tumor cells to be positive for vimentin, smooth muscle actin, desmin, KP-1, factor XIIIa, and MIB-1 (less than 10%). In addition to the expected immunohistochemical studies, the tumor stained diffusely positive for CD117. To our knowledge, this is the first report of atypical fibrous histiocytoma of the scrotum.     

A correlative cytologic and histologic study of malignant fibrous histiocytoma: an analysis of 40 cases examined by fine-needle aspiration cytology

A correlative cytologic and histologic study of 40 cases of histologically highly pleomorphic malignant fibrous histiocytoma (MFH) is presented. The fine-needle aspiration biopsy was performed preoperatively, and a diagnosis of malignant soft-tissue tumor could be established in all cases. The cytologic and histologic features corresponded well with each other. The two main cell types were mono- and multinucleated, large polymorphic, often bizarre, histiocyte-like cells and atypical fibroblast-like cells. For a correct diagnosis of pleomorphic MFH, it is important to recognize atypical large polymorphic tumor cells showing signs of phagocytosis: prominent cytoplasmic vacuolization, cell debris or even well-preserved cells within the tumor cell cytoplasm. Phagocytic activity was easily demonstrated in air-dried and May-Grünwald Giemsa-stained material. The differential diagnosis of MFH as opposed to other soft-tissue sarcomas and pleomorphic carcinomas is discussed.     

Fine-needle aspiration cytology of angiomatoid malignant fibrous histiocytoma

Angiomatoid malignant fibrous histiocytoma (AMFH) is a rare, low-grade malignant mesenchymal neoplasm that affects mostly the extremities of children and young adults. Excisional surgery is the adequate treatment. The cytologic, immunocytologic, and histologic features noted in two patients having AMFH are presented. Cytologic smears showed histiocyte-like cells dispersed and in clusters, in close relation with eosinophilic mesenchymal fragments in a bloody background with lymphocytes. The tumor cells showed mild to moderate anisocariosis, often with nucleolus and vast, fragile cytoplasm. A fibroblastic-like spindle to ovoid cell population was also present in one patient. Immunohistochemical results are most consistent with myofibroblastic cell differentiation. When accompanied by adequate clinical information and ancillary techniques, a specific preoperative cytologic diagnosis is possible.