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 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 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.     

Benign fibrous histiocytoma of the renal capsule.

The first case of benign fibrous histiocytoma of the renal capsule is reported in a male aged 44 years. The tumor had its point of origin in the renal capsule. Histologically, the tumor was composed of intersecting fascicles of fibroblastic cells forming a loose crisscross or "storiform" pattern. Electron microscopic studies of tumor cells revealed intermediate filaments and membrane-bound collagen fibers which continued to extracellular collagen bundles. This deep seated fibrous histiocytoma had a more prominent storiform pattern and fewer secondary elements such as xanthoma cells than cutaneous ones.     

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.     

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.     

Malignant fibrous histiocytoma of the lung

A 75-year-old woman was admitted to a hospital for diagnosis of pulmonary infarction and died during treatment. An autopsy revealed a tumor 5 cm in diameter in the hilus of the left lung, spreading into the posterior mediastinum, and a metastasis was also found in the right lung. Histologically, this tumor consisted of two kinds of cells; one of fibroblast-like cells and the other of histiocyte-like cells, showing a storiform pattern. Furthermore, a positive staining for alpha 1-antitrypsin, but negative for CEA, keratin or S-100 protein was seen. Therefore, it was diagnosed as malignant fibrous histiocytoma (MFH) originating in the hilus of the left lung. In addition, many foci of hemorrhagic infarction due to metastasis and infiltration of the tumor into the pulmonary arteries were observed in the right lung. MFH is one of the rarest primary tumors of the lung.     

Malignant fibrous histiocytoma of bone

Summary Malignant fibrous histiocytoma (MFH) of bone is a well-defined tumor by light microscopy but no agreement has been achieved concerning its histogenesis. We present the light and electron microscopic findings of four cases of MFH of bone. In case 1 multiple bone tumors were observed and in case 4 the tumor developed after irradiation. It was our aim to document the cytological variability and to arrange the findings in a histogenetic concept of primary intraosseous MFH. We observed some undifferentiated cells but mainly histiocyte- and fibroblast-like cells including intermediate forms, and several types of giant cells. We should emphasize the fact that there were also some large cells with a light microscopic resemblance to rhabdomyoblasts and with electron microscopic characteristics of myoblastic differentiation. From the ultrastructural point of view, therefore, MFHs seem to derive from a primitive mesenchymal stem cell rather than from the ordinary histiocyte. It is suggested that osteosarcoma and MFH of bone may have a common progenitor cell but it is important to make a clear clinico-pathological distinction between the tumors because of differing biological behavior.     

Malignant fibrous histiocytoma of the larynx]

A rare case of larynx malignant fibrous histiocytoma is presented. Histologically and ultrastructurally, the tumour was similar to malignant fibrous histiocytoma of other organs. The patient was followed up for 2 years after surgical treatment and preoperative irradiation. No recurrence and metastases were observed.     

Malignant fibrous histiocytoma of the soft tissues

Clinico-morphological characterization of the malignant fibrous histiocytoma (MFH) of soft tissues in 142 patients is presented. According to the authors' data MFH is the most frequent tumour among soft tissue malignant neoplasms (15,7%) in adult patients. The age of patients is predominantly from 40 to 70, the most frequent site is a lower limb (thigh). A typical morphological variant of MFH clearly dominates (73%): there is a tendency to the development of an inflammatory variant at the retroperitoneal tumour site. Recurrences in all group of patients (104) who were followed-up were in 68,3%, metastasis in 45,2%, the 5-year survival in 59,6% of cases. A clear-cut dependence of these indices upon the depth of tumour location soft tissues, node size, tumour site is noted. In view of pronounced domination of a MFH typical variant the final conclusion on the feasibility of separation of other variants can be only made on the basis of a much greater number of cases.     

Malignant fibrous histiocytoma of the parotid gland

We described a rare malignant fibrous histiocytoma of the parotid gland (MFH) in a 63-year-old woman. During six months the tumour size became 10 cm in diameter with skin ulceration. The tumour was examined morphologically, by immunohistochemistry and molecular biology methods - FASAY and CGH. The histology revealed a storiform-pleomorphic type of MFH with high mitotic rate. The FASAY method identified a non-mutated p53 gene. The chromosomal changes were identified by the CGH method and 6 cytogenetic changes were found in the tumour cells (deletions at 8p12-p22, 13q32-qter, 14q24-qter, and gains of chromosomal material at 5p, 8q12-q23, and Xq25-qter). The patient died shortly after the beginning of chemotherapy. Autopsy revealed brain and cerebellar haemorrhage. No other tumour foci were proved. In view of short course of disease we lack the data about the influence of the non-mutated p53 gene on the prognosis and therapy.     

Malignant fibrous histiocytoma of the maxillary sinus

A case of malignant fibrous histiocytoma arising primarily in the left maxillary sinus is described. The patient, a 39-year-old male, who had suffered from sinusitis for 20 years, began to have paresthesia or sharp pain of the left side of the face and toothaches of the left maxilla. At operation a white fibrous tumor developing extensively from the lateral wall to the upper and medial walls of the left maxillary sinus and into the ethmoidal sinus was noted. Following a gradual progression of dyspnea, he died approximately one year after the onset in spite of radiation therapy and anticancer chemotherapy. An autopsy revealed recurrence of the tumor in the left maxillary sinus with wide-spread metastases to the lungs, pleurae, pancreas, kidneys and bone marrows. The direct cause of death was respiratory failure due to extensive growths of the pulmonary and pleural metastases.     

Malignant fibrous histiocytoma of the heart.

We describe a 54-year-old man with a recurrent malignant fibrous histiocytoma in the left atrium. During the patient's first hospitalization, the tumor clinically presented as a typical atrial myxoma and was removed by routine procedure. Histologically, it was diagnosed as sarcoma, probably rhabdomyosarcoma. Nine months later the patient was readmitted because of recurrence. This time, the tumor, along with interatrial septum and a part of the anterior atrial wall, was excised by means of cardiac explantation and reimplantation. On light microscopic, immunohistochemical, and electron microscopic examination, the tumor was classified as a storiform-pleomorphic type of malignant fibrous histiocytoma. No other therapeutic procedures were performed, and 11 months after the second surgery the patient died of massive hemorrhage from a duodenal ulcer. A recurrent tumor in the left atrium and several distant metastases were found at autopsy.     

Malignant fibrous histiocytoma of bone.

Existence of an entity called malignant fibrous histiocytoma of bone was emphasized, based on two of our own cases experienced recently and on previous reports related to this tumor. Histologically the tumor resembles markedly the malignant fibrous histiocytoma originating in the soft part. In addition, an attempt was made to clarify its biological behavior, histological subclassification and criteria to be malignant and differential diagnoses from several other bone diseases which sometimes mimic this malignant bone tumor.     

Malignant fibrous histiocytoma arising in the liver

A unique type of hepatic sarcoma in an adult was composed of pleomorphic fibrohistiocytic cells with benign cystic structures. The malignant mesenchymal component was morphologically similar to malignant fibrous histiocytoma of the soft tissue. The cystic epithelial structures appeared to be formed by the cystic transformation of the entrapped bile ducts and ductules. Malignant fibrous histiocytoma can arise in the liver, but the original architecture of the liver may affect or modify the histologic characteristics of the sarcoma.     

Leukaemoid reaction and eosinophilia in “Inflammatory fibrous histiocytoma”

Summary A case of retroperitoneal inflammatory fibrous histiocytoma associated to a marked granulocytic blood reaction and high eosinophilic count is reported. The paraneoplastic nature of the haematological findings and their probable prognostic role are discussed. Ultrastructurally the tumor does not differ significantly from non inflammatory fibroxanthosarcomas.