Myofibrosarcoma of the bone: a clinicopathologic study.
ABSTRACT 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.
SourceAvailable from: Tsuyoshi Saito[Show abstract] [Hide abstract]
ABSTRACT: INTRODUCTION: Low-grade myofibroblastic sarcoma (myofibrosarcoma) is described to be a distinct atypical myofibroblastic tumor often with fibromatosis-like features and predilection for head and neck. Low-grade myofibroblastic sarcoma of bone is extremely rare. PRESENTATION OF CASE: A 50-year-old woman was admitted to our hospital because she had experienced right knee pain for 2 years. Plain radiography showed a honeycombed lesion on the right distal femur, and computed tomography showed a bone tumor with cortex destruction invading the soft tissue. A biopsy specimen from the intraosseous lesion showed a hypocellular area of spindle cell proliferation with dense collagen deposition, which is reminiscent of a histological feature of desmoplastic fibroma. However, histological examination of the extraosseous lesion indicated a slightly hypercellular area containing scattered spindle-shaped atypical cells with enlarged nuclei, suggestive of low-grade sarcoma. Spindle-shaped atypical cells were immunohistochemically positive for SMA. A final diagnosis of low-grade myofibroiblastic sarcoma of the bone was made from a surgically resected specimen. DISCUSSION: The patient was alive and well with no evidence of disease at 15 months after the surgery without any additional therapy. CONCLUSION: Extensive sampling of a tumor may be necessary to determine the true nature of the tumor and to make an accurate diagnosis.11/2012; 4(2):195-199. DOI:10.1016/j.ijscr.2012.11.017
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ABSTRACT: Cutaneous spindle cell tumors share the common feature of appearing as spindle-shaped cells on light microscopy. Their pathogenesis, presentation, and prognosis are highly variable, and numerous techniques for workup and treatment have been reported. We performed an analysis of the available scientific literature in order to codify the clinical, immunohistochemical, and biologic features of these tumors and to provide insight into the most effective practices for their management, with a focus on Mohs micrographic surgery (MMS). In this article, the clinical and histopathological characteristics of dermatofibrosarcoma protuberans, atypical fibroxanthoma, malignant fibrous histiocytoma, spindle cell squamous cell carcinoma, superficial leiomyosarcoma, desmoplastic melanoma, cutaneous angiosarcoma, and myofibrosarcoma are described, and methods for diagnosis, workup, treatment, and surveillance are evaluated. Cutaneous spindle cell neoplasms are diverse in origin, presentation, and behavior. Immunostaining assists in differentiating among the various types. Further workup is sometimes indicated to characterize local invasion or assess for metastatic disease. Surgery is typically the first-line treatment, and MMS is associated with low recurrence rates and a tissue-sparing advantage for many tumors. Adjuvant treatments, including radiation therapy, molecular-targeted therapy, and conventional chemotherapy, are sometimes indicated, and close clinical surveillance is required after treatment.Dermatologic Surgery 01/2012; 38(6):825-50. DOI:10.1111/j.1524-4725.2012.02296.x · 1.56 Impact Factor
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ABSTRACT: Low-grade myofibroblastic sarcoma (LGMS) is a distinct mesenchymal myofibroblastic malignancy. The tumor may occur at a variety of sites, but is particularly associated with the head and neck. Of the two maxillary sarcomas that were analyzed in the present study, one was misdiagnosed as an inflammatory myofibroblastic tumor during pre-operative excision biopsy, and later presented with a different immunophenotype upon recurrence. Representative paraffin blocks from formalin-fixed tissues were selected from each patient and designated as case 1 and case 2. Immunohistochemical studies were performed on 3-μm thick sections using primary antibodies against α-smooth muscle actin (α-SMA), muscle-specific actin (MSA), desmin, vimentin, calponin, h-caldesmon, fibronectin, cytokeratin, cluster of differentiation 34 (CD34), S-100 protein, anaplastic lymphoma kinase (ALK), epithelial membrane antigen (EMA) and Ki-67. Immunohistochemistry was performed using the streptavidin-biotin-peroxidase complex method. The tumor cells from the two maxillary LGMSs, including the recurrent lesion, were positive for vimentin and fibronectin, and negative for S-100 protein, CD34, EMA, h-caldesmon, ALK, MSA and calponin. The tumor cells from case 1 demonstrated positive staining for α-SMA protein and negative staining for desmin. By contrast, the tumor cells from the primary lesion in case 2 presented with negative staining for α-SMA and positive staining for desmin, while the cells of the recurrent lesion were α-SMA-positive and desmin-negative. The present study concluded that cases of LGMS with immunoprofile alterations are predictive of relatively poor prognoses.Oncology letters 02/2015; 9(2):619-625. DOI:10.3892/ol.2014.2790 · 0.99 Impact Factor