Ossifying Fibromyxoid Tumor: Invariable Ultrastructural Features and Diverse Immunophenotypic Expression

Department of Orthopaedic Surgery, Akita University, Akita, Akita, Japan
Ultrastructural Pathology (Impact Factor: 1.08). 06/2007; 31(3):233-9. DOI: 10.1080/01913120701351157
Source: PubMed


Ossifying fibromyxoid tumor (OFMT) is a rare enigmatic soft tissue tumor, the origin of which is still uncertain. The authors report on 3 cases of OFMT arising in the trunk and head and neck regions of adults. Two recurred and one was suspected to have metastasis. All tumors consisted of multiple nodules, in which round or polygonal tumor cells were arranged in sheets or cords within a fibromyxoid background. Characteristic shell-like bone tissues were recognized in all tumors. Based on the grading system proposed by Folpe et al., 2 cases were designated as malignant OFMT and 1 as typical. In addition to S-100 protein, cytokeratin and neuronal markers (neurofilament, CD56 or CD57) were detected in 1 and 2 tumors, respectively. The salient and invariable ultrastructural features included reduplicated basal laminas, which seem to be crucial for the diagnosis.

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    ABSTRACT: Ossifying fibromyxoid tumors were first described in 1989. They are composed of small round cells dispersed in a myxoid matrix and contain bone, osteoid, and collagen. Bone invasion is very rare. Our case was unusual because of the spinal location of the tumor and its invasive features including destruction of the cortex of the vertebral body, and invasion of the spinal canal causing a radioculopathy. The clinical presentation, radiologic features, and histopathologic findings are described, and the relevant literature is reviewed.
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    ABSTRACT: Ossifying fibromyxoid tumor (OFMT) is a rare, recently described entity. As such, there is a paucity of information in the literature regarding this neoplasm. According to most reports, the tumor usually develops subcutaneously in the soft tissues of the extremities. Malignant forms of the tumor are far more rare than their benign counterparts. We present a new case of a malignant OFMT of the parapharyngeal space in a 33-year-old Pakistani man. The tumor was excised, and the patient did well with no complications. This case represents a rare occurrence of OFMT of the parapharyngeal space.
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    ABSTRACT: Ossifying fibromyxoid tumor of soft tissues (OFMT) is considered a rare mesenchymal neoplasm. Its main histological features are sheets and ill-defined lobules of rounded bland cells within a fibromyxoid background and a thick collagenous capsule with an incomplete rim of lamellar bone. This lesion occurs mostly in the soft tissues of the lower extremities and limb girdles. In this paper, we describe a mesenchymal tumor removed from the right thigh of a 41 year-old-woman. The neoplasm differed histologically from typical forms of OFMT for areas of moderate cellularity and atypia, nuclear enlargement and small nucleoli. Focally, stromal tongues of osteoid were centrally and irregularly located within the lesion with evident spindling of tumor cells around them. The mitotic activity was low (up to 19 per 50 HPF) and atypical figures were rarely seen. The tumor was positive to S-100 protein, vimentin, CD10, CD56, CD99, ASMA, calponin and collagen IV. Rare elements were positive for cytokeratin AE1/AE3. To the best of our knowledge, this is the first case of atypical OFMT reported to be positive for calponin. The patient is currently alive and well with no evidence of disease at 96 months following surgery. In spite of low-grade histology, OFMT has high local recurrence rate and low metastatic potential, primarily in the lungs, even several years after surgical removal. The recognition of this entity is important. In this report the authors address differential diagnosis and enigmatic histogenesis of this neoplasm.
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