Geramizadeh Bita, Bahador Ali, Ganjei-Azar Parvin, Asadi Ali
Pathology Department, Medical School of Shiraz University, Shiraz 71344, Iran.
J Pediatr Surg. 2005 Mar;40(3):572-4. doi: 10.1016/j.jpedsurg.2004.11.031.
Gastrointestinal stromal tumor (GIST) is very rare in infancy. Most of the reported cases in the literature are in adults; some are in children but there are a few reported cases in the literature. The present case is a 6-day-old female neonate presenting with lethargy, poor feeding, constipation, abdominal distention, and rectal bleeding. She was operated on with the impression of intestinal obstruction, and right hemicolectomy was performed on her. Surgical specimen showed a well-defined and round 3-cm mass in the cecal area. Diagnosis was made by histologic and immunohistochemical studies which showed a GIST. The tumor showed positive vimentin and c-kit but negative for all other markers (desmin, actin, S100, NSE, and CD-34). So the case was an undifferentiated GIST. After 1 year of follow-up the patient was completely normal.
胃肠道间质瘤(GIST)在婴儿期非常罕见。文献报道的大多数病例发生在成人中;一些发生在儿童中,但文献中报道的病例较少。本病例为一名6日龄女婴,表现为嗜睡、喂养困难、便秘、腹胀和直肠出血。她接受了手术,初步诊断为肠梗阻,并进行了右半结肠切除术。手术标本显示盲肠区域有一个边界清晰的3厘米圆形肿块。通过组织学和免疫组化研究确诊为GIST。肿瘤波形蛋白和c-kit呈阳性,但所有其他标志物(结蛋白、肌动蛋白、S100、NSE和CD-34)均为阴性。因此,该病例为未分化GIST。随访1年后,患者完全正常。