背景:纤维瘤或硬纤维瘤是相对罕见的源自肌膜神经系统的肿瘤。这种肿瘤没有特定的临床症状,有时会被误诊为其他疾病,例如胃肠道间质瘤(GIST)。
方法:一名28岁男子因腹部肿块而去北京协和医学院就诊,无腹痛或腹胀。
方法:考虑影像学特征和临床表现,该肿块在手术前主要被诊断为GIST.在手术过程中,在回盲部肠系膜下发现了这个位置,外观呈灰白色,坚韧的质地,流动性差,这与GIST的性质不一致。手术后,病理检查和个体免疫组织化学结果表明,该病变与腹膜后纤维瘤病合并慢性淋巴结炎化脓性炎症的诊断相符。
方法:因此,我们决定进行肿瘤切除,右半结肠切除术,十二指肠部分切除术,剖腹手术的肠吻合术,但正确的输尿管被保留了.切除肿瘤后,肠段的末端连续缝合。
结果:患者没有经历术中或术后并发症,术后3天出院。每3个月进行定期随访体格检查,如腹部超声和计算机断层扫描,在整个12个月内没有观察到复发的证据。
结论:总而言之,腹内纤维瘤病是一种极其罕见的肿瘤,必须与其他消化道肿瘤区分开来,病理和免疫组织化学检查是诊断的关键部分。纤维瘤病的早期诊断对于结果至关重要。广泛切除肿块可以最大程度地减少复发的风险。
BACKGROUND: Fibromatoses or desmoid tumors are relatively rare tumors derived from the musculoaponeurotic system. This tumor has no specific clinical symptoms and it is sometimes misdiagnosed as other diseases such as gastrointestinal stromal tumors (GISTs).
METHODS: A 28-year-old man visited Peking Union Medical College for a tangible abdominal mass without abdominal pain or distention.
METHODS: Considering the imaging characteristics and clinical manifestation, this mass was primarily diagnosed as GIST before surgery. During the surgery, the occupancy was found under the ileocecal mesentery, with grayish white appearance, tough texture, and poor mobility, which was not consistent with the character of the GIST. After the surgery, pathological examination and individual immunohistochemistry results demonstrated that the lesion was compatible with the diagnosis of retroperitoneal fibromatosis with purulent inflammation of chronic lymphadenitis.
METHODS: Therefore, we decided to perform tumor mass resection, right colon resection, partial duodenum resection, and intestinal anastomosis on laparotomy, but the right ureter was retained. After excision of the tumor, the ends of the intestine segment were continuously sutured.
RESULTS: The patient experienced no intraoperative or postoperative complications, and was discharged 3 days after surgery. Periodic follow-up physical examinations such as the abdominal ultrasound and computed tomography were performed each 3 months, and no evidence of recurrence was observed during the whole 12 months.
CONCLUSIONS: To sum up, intra-abdominal fibromatosis is an extremely rare tumor that must be differentiated from other tumors of the digestive tract, and pathological and immunohistochemical examination is a critical part of the diagnosis. Early diagnosis of fibromatosis is essential for the outcome. Extensive resection of the mass minimizes the risk of relapse.