Subcutaneous ossifying fibromyxoid tumor

Department of Surgical Pathology, The University of Texas M.D. Anderson Cancer Center, Houston, TX 77030, USA.
Journal of Cutaneous Pathology (Impact Factor: 1.58). 12/2006; 33(11):749-53. DOI: 10.1111/j.1600-0560.2006.00540.x
Source: PubMed


Ossifying fibromyxoid tumor (OFMT) is an uncommon neoplasm occurring predominantly in the deep soft tissues of the trunk and proximal extremities. The lineage of this rare tumor to date is still uncertain. We present a case of a subcutaneous OFMT that recurred 8 years after initial resection. The histological findings of the primary and recurrent lesions are compared along with the histologic features possibly associated with aggressiveness. Dermatopathologists should consider OFMT in the differential diagnosis of subcutaneous neoplasms with a myxoid matrix.

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    ABSTRACT: Ossifying fibromyxoid tumor (OFT) is a unique soft tissue tumor of uncertain histogenesis. The majority of reported cases (approximately 220) have pursued a benign clinical course. However, recent literature has emphasized the existence of morphologically atypical and clinically malignant examples of this tumor and proposed guidelines for assessment of biologic potential. In the present study, we evaluated 104 cases of OFT from the Armed Forces Institute of Pathology, accessioned between the years 1970 and 2007. Herein, OFT was strictly defined as a tumor with lobular architecture, predominantly epithelioid cell morphology, a low level of atypia, corded and trabecular growth patterns, moderate amounts of myxocollagenous matrix, and often, focal peripheral metaplastic bone formation. Tumors that lacked conventional morphology were excluded. The exclusion group included cutaneous mixed tumors, low-grade fibromyxoid sarcomas, and extraskeletal osteosarcomas. The OFTs occurred in 64 men and 40 women with a median age of 50 years (range, 21 to 81 y). The tumor size ranged from 0.7 to 17 cm (median, 3 cm). The mitotic rate varied from 0 to 41 mitotic figures per 50 HPFs (median, 2/50 HPFs). Tumor cell nuclei typically contained small, distinct nucleoli, and necrosis was infrequent (11/104). The great majority of tumors (67/71, 94%) were positive for S100 protein, whereas only occasional examples had (focal) positivity for desmin, glial fibrillary acidic protein, and an AE1/AE3 keratin cocktail. Local recurrences were documented in 9 of 41(22%) living patients, usually 10 or more years after primary surgery, but there were no metastases. A mitotic rate of >2 mitotic figures/50 HPFs was a risk factor for local recurrence, but necrosis, tumor size, the presence of satellite nodules, and positive margins were not. When OFT is strictly defined by the criteria noted above, there is potential for local recurrence, but there seems to be little or no risk for metastasis.
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    ABSTRACT: Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue tumor of uncertain histogenesis, occurring predominantly in deep soft tissues of the extremities. Typically, OFMT presents in adults on the extremities or trunk, as a deep soft tissue mass. Less appreciated is the fact that OFMT may also present as a mass in the superficial subcutis or dermis. We herein report a female who presented with an asymptomatic subcutaneous nodule on the left thigh for 3 years, and who was diagnosed as having typical ossifying fibromyxoid tumor, by unique histopathologic and immunohistochemical studies. Most reported cases have pursued a benign clinical course. However, recent literature emphasized the existence of morphologically atypical and clinically malignant cases of OFMTs. Pathologic criteria for malignancy have been proposed, and reclassification of these tumors as tumors of intermediate malignancy, raise our attention while coping with OFMT clinically.
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