Fujiwara Sho, Koyamada Nozomi, Miyazawa Koji, Saiki Yuriko, Horii Akira, Miyazaki Shukichi
Department of Surgery, Iwate Prefectural Chubu Hospital, 17-10 Murasakino, Kitakami, , Iwate, 024-8507, Japan.
Department of Molecular Pathology, Tohoku University School of Medicine, Sendai, Miyagi, 980-8575, Japan.
Surg Case Rep. 2024 Jan 29;10(1):28. doi: 10.1186/s40792-024-01827-8.
Young women with NF1 are at a high risk of developing breast cancer. Although they are at risk for abdominal tumors, such as gastrointestinal stromal tumors and neuroendocrine tumors, follow-up strategies for other tumors after breast cancer have not yet been established. Here, we present a case of duodenal neuroendocrine tumor found during follow-up after bilateral mastectomy for breast cancer with type 1 neurofibromatosis (NF1), for which pancreaticoduodenectomy (PD) and lymphadenectomy were performed.
A 46-year-old woman with NF1 was referred to our hospital for treatment of a duodenal submucosal tumor. Her previous operative history included bilateral mastectomy for breast cancer: right total mastectomy and left partial mastectomy performed 9 and 5 years ago, respectively. Her daughter was confirmed to have NF1, but her parents were unclear. Although she had no recurrence or symptoms during the follow-up for her breast cancer, she wished to undergo 18-fluorodeoxyglucose-positron emission tomography (FDG-PET) for systemic screening. FDG-PET demonstrated FDG accumulation in the duodenal tumor with a maximum standardized uptake value of 5.78. Endoscopy revealed a 20-mm-diameter tumor in the second duodenal portion, and endoscopic biopsy suggested a NET G1. We performed PD and lymphadenectomy for complete. She was doing well without recurrence and was followed up with PET tomography-computed tomography.
Early detection of gastrointestinal tumors is difficult, because most of them are asymptomatic. Gastrointestinal screening is important for patients with NF1, and PD with lymphadenectomy is feasible for managing duodenal neuroendocrine tumors, depending on their size.
患有1型神经纤维瘤病（NF1）的年轻女性患乳腺癌的风险很高。尽管她们也有患腹部肿瘤的风险，如胃肠道间质瘤和神经内分泌肿瘤，但乳腺癌后其他肿瘤的随访策略尚未确立。在此，我们报告一例在双侧乳房切除术后随访期间发现的十二指肠神经内分泌肿瘤病例，该患者患有1型神经纤维瘤病（NF1），为此实施了胰十二指肠切除术（PD）和淋巴结清扫术。
一名46岁患有NF1的女性因十二指肠黏膜下肿瘤被转诊至我院治疗。她既往的手术史包括双侧乳腺癌乳房切除术：分别于9年前和5年前进行了右侧全乳切除术和左侧部分乳房切除术。她的女儿被确诊患有NF1，但她的父母情况不明。尽管她在乳腺癌随访期间没有复发或症状，但她希望接受18氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）进行全身筛查。FDG-PET显示十二指肠肿瘤有FDG积聚，最大标准化摄取值为5.78。内镜检查发现十二指肠第二部有一个直径20毫米的肿瘤，内镜活检提示为G1级神经内分泌肿瘤（NET）。我们进行了PD和淋巴结清扫术以彻底切除肿瘤。她恢复良好，无复发，并接受PET断层扫描-计算机断层扫描随访。
胃肠道肿瘤早期难以发现，因为它们大多无症状。胃肠道筛查对NF1患者很重要，根据十二指肠神经内分泌肿瘤的大小，PD联合淋巴结清扫术对于治疗此类肿瘤是可行的。
