Luo Jean J, Baksh Fabien K, Pfeifer John D, Eastman James T, Beyer Frederick C, Dehner Louis P
The Lauren V. Ackerman Laboratory of Surgical Pathology, Barnes-Jewish and St Louis Children's Hospitals, Washington University Medical Center, St Louis, MO, USA.
Pediatr Dev Pathol. 2008 Jan-Feb;11(1):46-9. doi: 10.2350/07-01-0220.1.
Mucinous cystic neoplasms (MCNs) make up a morphologic family of similar appearing tumors arising in the ovary and various extraovarian sites, including the pancreas, hepatobiliary tract, paratesticular soft tissues, and mesentery. Other than the uncommon mucinous cystadenoma of the ovary presenting in adolescence, MCNs are rarely seen by the pediatric pathologist. The present case is a 5-year-old boy with an abdominal mass appearing to arise in the mesentery of the small intestine. Because of its unresectability, a generous biopsy was performed and disclosed a MCN with focal complex papillary architecture in the absence of appreciable cytologic atypia or invasion into the wall. Like other MCNs, this tumor had an inhibin-positive, ovarian-like stroma that was nonreactive for estrogen and progesterone receptors. Only 1 other case of a mesenteric MCN has been reported to date in a child and none in a male. The MCN of the mesentery joins other, somewhat more common cystic lesions of the omentum and mesentery presenting in childhood.
黏液性囊性肿瘤(MCNs)构成了一组形态学上相似的肿瘤家族,它们出现在卵巢以及包括胰腺、肝胆管、睾丸旁软组织和肠系膜在内的各种卵巢外部位。除了青春期出现的罕见的卵巢黏液性囊腺瘤外,儿科病理学家很少见到MCNs。本病例是一名5岁男孩,腹部有一肿块,似乎起源于小肠系膜。由于无法切除,进行了广泛的活检,结果显示为一个MCN,具有局灶性复杂乳头结构,未见明显的细胞学异型性或侵犯肠壁。与其他MCNs一样,该肿瘤有抑制素阳性、类似卵巢的间质,对雌激素和孕激素受体无反应。迄今为止,仅报道过1例儿童肠系膜MCN,尚无男性病例报道。肠系膜MCN与儿童期出现的其他一些较常见的大网膜和肠系膜囊性病变相同。