Kitada Tomoya, Masui Toshihiko, Kasai Yosuke, Uchida Yuichiro, Ogiso Satoshi, Ito Takashi, Ishii Takamichi, Seo Satoru, Katsuragawa Hiroyuki, Uemoto Shinji
Department of Surgery, Graduate School of Medicine, Kyoto University, 54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto, 606-8507, Japan.
Department of Diagnostic Pathology, Graduate School of Medicine, Kyoto University, 54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto, 606-8507, Japan.
Surg Case Rep. 2021 Mar 19;7(1):72. doi: 10.1186/s40792-021-01155-1.
Although duodenal neuroendocrine neoplasms (DuNENs) usually have indolent phenotypes, some DuNENs exhibit aggressive clinical manifestations. Tumor size > 1 cm, lymph node metastasis, and high grade have been associated with poor prognosis. However, preoperative risk evaluation is often difficult, because Ki-67 index on biopsy is frequently underestimated due to the intratumor heterogeneity. Here, we present a case of a subcentimeter DuNEN with a low Ki-67 index on endoscopic biopsy, who developed lymph node metastasis and high-grade liver metastasis.
The patient was a 52-year-old female who presented an epigastric pain. Esophagogastroduodenoscopy revealed a duodenal submucosal lesion with a size of 8 mm. The endoscopic biopsy showed DuNEN with a Ki-67 index of 3.3% (G2 categorized by the World Health Organization 2019 classification). We performed an open partial duodenectomy with adjacent lymph node dissection. Pathological examination of the resected specimens revealed a Ki-67 index of 13.5% (G2) in the "hot spot" and lymph node metastasis. A hepatic low-density area detected on preoperative contrast-enhanced computed tomography appeared to be a liver metastasis on postoperative gadoxetic acid-enhanced magnetic resonance imaging. Subsequently, we performed a laparoscopic partial hepatectomy. Pathological examination of the liver specimen showed a metastatic neuroendocrine tumor with a Ki-67 index of 27.5% (NET-G3). The patient has been alive for 14 months since the hepatectomy.
This case shows the possibility of high malignant potential of DuNEN even if the primary lesion is < 1 cm and has a low Ki-67 index on biopsy.
尽管十二指肠神经内分泌肿瘤（DuNENs）通常具有惰性表型，但一些DuNENs表现出侵袭性临床表现。肿瘤大小>1 cm、淋巴结转移和高级别与预后不良相关。然而，术前风险评估往往很困难，因为活检时Ki-67指数常因肿瘤内异质性而被低估。在此，我们报告一例内镜活检时Ki-67指数低的亚厘米级DuNEN病例，该病例发生了淋巴结转移和高级别肝转移。
患者为一名52岁女性，出现上腹部疼痛。食管胃十二指肠镜检查发现一个8 mm大小的十二指肠黏膜下病变。内镜活检显示为DuNEN，Ki-67指数为3.3%（根据世界卫生组织2019年分类为G2）。我们进行了开放性部分十二指肠切除术及相邻淋巴结清扫术。切除标本的病理检查显示“热点”区域Ki-67指数为13.5%（G2）且有淋巴结转移。术前对比增强计算机断层扫描检测到的肝脏低密度区域在术后钆塞酸增强磁共振成像上显示为肝转移。随后，我们进行了腹腔镜部分肝切除术。肝脏标本的病理检查显示为转移性神经内分泌肿瘤，Ki-67指数为27.5%（NET-G3）。自肝切除术后，患者已存活14个月。
该病例表明，即使原发性病变<1 cm且活检时Ki-67指数低，DuNEN仍有可能具有高恶性潜能。
