Ossifying fibromyxoid tumor: a study of 6 cases of atypical and malignant variants

免疫组织化学 病理 恶性肿瘤 结蛋白 核异型性 肉瘤 组织学 异型性 荧光原位杂交 生物 医学 波形蛋白 染色体 生物化学 基因
作者
Kossivi Dantey,Karen E. Schoedel,Oleksandr Yergiyev,Richard L. McGough,Alka Palekar,Uma Rao
出处
期刊:Human Pathology [Elsevier BV]
卷期号:60: 174-179 被引量:15
标识
DOI:10.1016/j.humpath.2016.10.012
摘要

Ossifying fibromyxoid tumors (OFMT) of soft parts are rare, slow-growing tumors that have potential for local recurrence and may metastasize. While OFMT originally was considered benign, several cases of malignant OFMT have been documented. There is no universally accepted risk stratification, although this study emphasizes the importance of utilizing histology, immunohistochemistry and FISH in establishing the diagnosis. Herein, we describe six cases of atypical and malignant OFMT with differences in morphologic features, 5 of which display the proposed morphological criteria for malignancy. The patients were mostly male (M = 5, F = 1) with an age range of 33–69 years. The tumors arose from the extremities (3 cases), the shoulder (1 case), the head and neck area (1 case), and the paraspinal area (1 case). One tumor had high grade and overtly sarcomatous changes, while another invaded the underlying clavicle. Two cases showed cytological atypia and necrosis. Fluorescence in situ hybridization (FISH) detected rearrangement of the PHF1 gene in 5 cases. All cases were positive for EAAT4 and actin by immunohistochemistry, while negative for desmin. Three tumors were immunoreactive for S100 protein. INI-1 immunohistochemical staining was conserved in all but 2 cases in which a mosaic loss of expression was noted. All but two patients are currently alive and free of disease.
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