Buccoliero Anna Maria, Ghionzoli Marco, Castiglione Francesca, Paglierani Milena, Baroni Gianna, Messineo Antonio, Taddei Gian Luigi
Pathology Unit, Anna Meyer Children Hospital, Florence, Italy.
Department of Pediatric Surgery, Anna Meyer Children Hospital, Florence, Italy.
Pathol Res Pract. 2014 Dec;210(12):1152-5. doi: 10.1016/j.prp.2014.03.011. Epub 2014 Apr 8.
Inflammatory myofibroblastic tumor is an uncommon tumor regarded as "intermediate malignancy". We present the clinical, pathological and molecular features of a mesenteric inflammatory myofibroblastic tumor in a 9-month-old male infant. The patient was referred to Anna Meyer Children Hospital of Florence, Italy, for an asymptomatic abdominal mass measuring about 7cm. The lesion was radically excised, and the postoperative course was uneventful. Histologically, the tumor was composed of spindle cells immunopositive for vimentin and desmin admixed with an inflammatory infiltrate. Rearrangement of ALK gene was demonstrated by FISH and immunohistochemistry (cytoplasmic, perinuclear and punctate immunocoloration). The peculiar punctate ALK immunocoloration suggested a possible unusual ALK gene rearrangement involving the CLTC gene.
炎性肌纤维母细胞瘤是一种被视为“中间型恶性”的罕见肿瘤。我们报告了一名9个月大男婴肠系膜炎性肌纤维母细胞瘤的临床、病理和分子特征。该患者因一个约7厘米大小的无症状腹部肿块被转诊至意大利佛罗伦萨的安娜·迈耶儿童医院。病变被根治性切除,术后过程顺利。组织学上,肿瘤由波形蛋白和结蛋白免疫阳性的梭形细胞组成,并伴有炎性浸润。通过荧光原位杂交(FISH)和免疫组织化学(细胞质、核周和点状免疫染色)证实了ALK基因重排。ALK点状免疫染色的独特表现提示可能存在涉及CLTC基因的异常ALK基因重排。