Desai S R, Dombale V D, Janugade H B
Department of Pathology, KIMS, Karad, Maharashtra.
Indian J Pathol Microbiol. 2005 Jul;48(3):379-80.
Infantile fibromatosis represents the childhood counter part of musculoaponeurotic fibromatosis & arises as a solitary mass in skeletal muscle, adjacent fascia, aponeurosis or periosteum. The lesion is extremely rare. Microscopically it exists in two forms diffuse (mesenchymal) & desmoid. The less common desmoid form rarely occurs in infancy. Immunophenotype shows vimentin positivity with variable positivity with muscle markers. The differential diagnosis of this type is infantile fibrosarcoma. The tumor may locally recur if inadequately excised. We report a case of infantile fibromatosis of desmoid type occurring in 10 months male child for its extreme rarity.
婴儿纤维瘤病是肌筋膜纤维瘤病在儿童期的对应病症,表现为骨骼肌、相邻筋膜、腱膜或骨膜内的孤立肿块。该病变极为罕见。显微镜下,它有两种形式,弥漫性(间质性)和硬纤维瘤样。较罕见的硬纤维瘤样形式在婴儿期很少发生。免疫表型显示波形蛋白阳性,肌肉标志物呈不同程度阳性。这种类型的鉴别诊断是婴儿纤维肉瘤。如果切除不充分,肿瘤可能局部复发。我们报告一例10个月大男童发生的硬纤维瘤样婴儿纤维瘤病,因其极为罕见。