Soti Khiabani Mahsa, Monajemzadeh Maryam, Raji Hojatollah, Zamani Fatemeh, Vaseie Mohammad, Pak Neda
Children's Medical Center, Pediatrics Center of Excellence, Tehran, Iran.
Department of Pediatric Emergency, Tehran University of Medical Sciences, Tehran, Iran.
Iran J Pathol. 2024 Winter;19(1):132-136. doi: 10.30699/ijp.2024.2003653.3122. Epub 2023 Dec 29.
The occurrence of rectosigmoid junction inflammatory myofibroblastic tumor (IMT) is uncommon in children. This is a rare form of mesenchymal tumor, belonging to the category of soft tissue tumors, and can be found at any anatomical site from the central nervous system to the gastrointestinal tract. Our patient was a 10-year-old male subject complaining of lack of defecation and constipation. The patient had decreased the frequency of defecation and constipation about two weeks before his referral and had not improved despite the use of laxatives. The abdomen was completely distended and there was no tenderness or guarding in the examination. Several airfluid levels are shown on the abdominal X-ray. In the ultrasound, free fluid was reported in the interlobular and pelvic spaces. The patient was transferred into the operating room. A tumor of the rectosigmoid junction was detected. Histopathologic studies showed evidence of IMT. IMT is a rare neoplasm of unknown origin, which may occur in various sites of the body. Complete surgical removal is usually curative, but early detection of recurrence is required. Treatment options include chemotherapy, radiation therapy, and immunotherapy. Further investigations are needed to improve the understanding and management of this rare tumor.
直肠乙状结肠交界处炎性肌纤维母细胞瘤(IMT)在儿童中并不常见。这是一种罕见的间叶性肿瘤,属于软组织肿瘤范畴,可发生于从中枢神经系统到胃肠道的任何解剖部位。我们的患者是一名10岁男性,主诉排便困难和便秘。患者在转诊前约两周排便频率降低且出现便秘,尽管使用了泻药仍未改善。腹部完全膨隆,检查时无压痛或肌紧张。腹部X线显示多个气液平面。超声检查报告在小叶间和盆腔间隙有游离液体。患者被转入手术室。检测到直肠乙状结肠交界处有一个肿瘤。组织病理学研究显示为IMT。IMT是一种起源不明的罕见肿瘤,可发生于身体的各个部位。完整的手术切除通常可治愈,但需要早期检测复发情况。治疗选择包括化疗、放疗和免疫治疗。需要进一步研究以增进对这种罕见肿瘤的理解和管理。