Myofibrosarcoma of the bone: a clinicopathologic study.
Myofibroblastic tumors are fairly recently established soft tissue neoplasms. Although most of them appear to be benign, myofibrosarcoma of the soft tissue, seemingly their malignant counterpart, have been reported. We describe the clinicopathologic and radiologic features of four cases of myofibrosarcoma arising from the bone. All but one of the patients were women ranging in age from 60 to 71 years. Two tumors occurred in the metaphyses of distal femurs and the others arose in the iliac bones. On radiologic examination all tumors exhibited well-demarcated lytic destructive lesions without periosteal reaction. Two tumors were localized in the bone, whereas the other two extended into surrounding soft tissues. Histologically, all tumors were composed principally of a mixture of a cell-rich fascicular area and a hypocellular fibrous area. In the former area tumor cells had rather eosinophilic spindle-shaped wavy cytoplasm and were arranged in interlacing fascicles and small storiform patterns with variable numbers of inflammatory cells. Tumors occasionally showed prominent pleomorphism, and large cells with hyperchromatic nuclei were seen. In contrast, hypocellular areas had various features, including collagenous, hyalinous scar-like and rarely keloid-like areas. Focal coagulation necroses were present in all but one tumor. Immunohistochemically, the tumors were positive for vimentin, muscle actin (HHF35), alpha-smooth muscle actin, calponin, and desmin, whereas all of them were negative for high molecular weight caldesmon. On follow-up there was one fatal case with distant metastases, whereas the clinical courses of other cases after wide resection were excellent. Myofibrosarcoma of the bone has distinctive histopathologic features, which should be distinguished from those of other bone tumors with myoid differentiation.
Available from: Ruchika Gupta
- "Myofibroblasts are mesenchymal cells showing characteristics of both fibroblasts and smooth muscle cells. In addition to its role in wound healing, they have been described in soft tissue tumours like myofibroblastoma, angiomyofibroblastoma, myofibromatosis and inflammatory myofibroblastic tumour [1,2]. Myofibroblastic sarcoma was characterized as a distinct neoplasm in 1998 by Mentzel et al. . "
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ABSTRACT: Primary myofibroblastic sarcoma of the bone is a rare spindle cell tumour with, to the best of our knowledge, only eight cases reported in the available English language literature. The disease's rarity and its low-grade features make an accurate diagnosis difficult in most cases. The differential diagnoses of this unusual tumour include various benign entities as well as other sarcomas. Due to the difference in prognosis, a precise pathologic diagnosis is essential, which requires a combination of thorough morphologic examination, immunohistochemistry and electron microscopy wherever available.
We report the case of a 38-year-old Indian woman with a lytic lesion in her left femur. The tumour was associated with cortical destruction and soft tissue extension. A biopsy from the soft tissue component showed features suggestive of a low-grade malignant mesenchymal tumour. Excision of the tumour was performed and histopathological examination showed a low-grade spindle cell sarcoma with collagenous stroma. Expressions of vimentin and smooth muscle actin were also noted. Ultrastructural examination confirmed its myofibroblastic nature. A final diagnosis of low-grade myofibroblastic sarcoma of the left femur was thus rendered.
Low-grade myofibroblastic sarcoma is one of the rarer osseous spindle cell sarcomas depicting a favourable prognosis in the cases reported so far. Its diagnosis requires ancillary techniques like immunohistochemistry and electron microscopy. To the best of our knowledge, we report the ninth case in the literature and the first case from our subcontinent.
Available from: PubMed Central
- "Other locations have also been described: the salivary gland , nasal skin  and the vulva . Painless growth of a large mass is typical of intraosseous tumours . With other locations some signs may occur such as fever, chills, leukocytosis or meningeal irritation (cerebral tumour), and more aggressive growth has been observed in the abdomen. "
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ABSTRACT: Low-grade myofibroblastic sarcoma is a rare entity, which mostly develops in the soft tissues of the head and neck. Within the oral cavity lingual lesions are the most common. It tends to recur locally rather than to metastasise.
We present a 54-year-old man with a one-year history of buccal oedema. He also had arterial hypertension and clinical examination revealed distension of the left mandibular ramus with laminar deflection in the area of the retromolar triangle.
We present a rare intramandibular encapsulated lesion that caused diagnostic difficulties. Our diagnostic methods included immunohistochemistry and molecular investigations. We emphasise the uncommon location of this tumour type.
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