Weiss Vivian L, Antonescu Cristina R, Alaggio Rita, Cates Justin M, Gaskin David, Stefanovici Camelia, Coffin Cheryl M
1 Department of Pathology, Microbiology, and Immunology, Vanderbilt University, Nashville, TN, USA.
Pediatr Dev Pathol. 2013 Nov-Dec;16(6):425-31. doi: 10.2350/13-06-1353-CR.1. Epub 2013 Aug 6.
Myxoinflammatory fibroblastic sarcoma (MIFS), originally described as a low-grade malignant soft-tissue tumor in adults, has recently been reported in children and in non-acral sites. This report describes the clinicopathologic features of a series of 5 MIFS in children and adolescents (3 males, 2 females), ranging in age from 5 to 17 years (mean, 13 years). These tumors presented as small, superficial, slowly growing soft-tissues masses of the scalp, neck, middle finger, forearm, and thigh. Histologically, the tumors were composed of spindled and plump polygonal cells with prominent nuclear pleomorphism, nuclear pseudoinclusions; large eosinophilic nucleoli; myxoid foci intermingled with spindled foci; and an accompanying inflammatory infiltrate of lymphocytes, plasma cells, and variable neutrophils. Immunohistochemical analysis revealed variable reactivity for CD34 and smooth muscle actin in the tumor cells. Genetic analysis in 3 cases showed no rearrangements of TGFBR3 or MGEA5. Follow up in 4 cases revealed no recurrence or metastasis. These 5 cases of childhood and adolescent MIFS demonstrate an expanded age range and topographic distribution and a favorable outcome. The differential diagnosis and importance of recognizing this rare neoplasm in young patients are discussed.
黏液炎性纤维母细胞肉瘤（MIFS）最初被描述为成人的一种低级别恶性软组织肿瘤，最近在儿童及非肢端部位也有报道。本报告描述了一系列5例儿童及青少年MIFS（3例男性，2例女性）的临床病理特征，年龄范围为5至17岁（平均13岁）。这些肿瘤表现为头皮、颈部、中指、前臂和大腿处小的、浅表的、生长缓慢的软组织肿块。组织学上，肿瘤由梭形和饱满的多边形细胞组成，具有明显的核多形性、核假包涵体；大的嗜酸性核仁；黏液样灶与梭形灶相互交织；并伴有淋巴细胞、浆细胞和不同数量中性粒细胞的炎性浸润。免疫组化分析显示肿瘤细胞对CD34和平滑肌肌动蛋白的反应性各异。3例的基因分析显示未发现TGFBR3或MGEA5重排。4例随访未发现复发或转移。这5例儿童及青少年MIFS显示出年龄范围和部位分布的扩大以及良好的预后。讨论了年轻患者中鉴别诊断及认识这种罕见肿瘤的重要性。
