Yachida Tatsuo, Nakajima Takeshi, Nonaka Satoru, Nakamura Keiko, Suzuki Haruhisa, Yoshinaga Shigetaka, Oda Ichiro, Moriya Yoshihiro, Masaki Tsutomu, Saito Yutaka
*Endoscopy Division ‡Colorectal Surgery Division, National Cancer Center Hospital, Tokyo †Department of Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Kagawa, Japan.
J Clin Gastroenterol. 2017 May/Jun;51(5):407-411. doi: 10.1097/MCG.0000000000000555.
After colorectal cancer and desmoid tumors, duodenal adenocarcinoma is the next leading cause of death in familial adenomatous polyposis (FAP) patients, but it has not been thoroughly investigated.
To investigate the clinical course of duodenal neoplasia, including adenoma and cancer, we investigated 77 Japanese FAP patients treated at the National Cancer Center Hospital, Tokyo, Japan. We evaluated the clinicopathologic features, Spigelman severity score, and management of duodenal neoplasms. Data were acquired from a prospectively enrolled database.
Fifty-one (66%) of the 77 FAP patients had duodenal neoplasia during this observational period, and 47 of 51 patients had extra-ampulla duodenal neoplasia; 42 (58%) had duodenal neoplasms (extra-ampulla), 4 had duodenal adenomas with high-grade dysplasia (HGD), and 1 had invasive carcinoma. Among the 45 patients (extra-ampulla) with duodenal adenoma with HGD or low-grade dysplasia, 8 (18%) patients were treated using endoscopic resection (ER). During the short observation period, ER was performed only in HGD cases. None of the patients died from duodenal neoplasia. In total, during the surveillance period, duodenal HGD was detected in 5 (63%) of 8 patients graded as Spigelman stage IV; HGD was not detected in stage 0 (n=33), I (n=0), II (n=12), or III (n=20) patients.
Short-interval endoscopic surveillance and appropriate ER may help prevent duodenal invasive carcinoma. In addition, there was little development of invasive carcinoma during the follow-up. The Spigelman classification is beneficial for the risk assessment of duodenal neoplasia in Japanese FAP patients.
在结直肠癌和硬纤维瘤之后，十二指肠腺癌是家族性腺瘤性息肉病（FAP）患者的下一个主要死亡原因，但尚未得到充分研究。
为了研究十二指肠肿瘤（包括腺瘤和癌）的临床病程，我们调查了在日本东京国立癌症中心医院接受治疗的77例日本FAP患者。我们评估了十二指肠肿瘤的临床病理特征、斯皮格尔曼严重程度评分及处理方式。数据来自前瞻性登记数据库。
在这个观察期内，77例FAP患者中有51例（66%）发生十二指肠肿瘤，其中51例患者中有47例发生壶腹外十二指肠肿瘤；42例（58%）有十二指肠肿瘤（壶腹外），4例有高级别异型增生（HGD）的十二指肠腺瘤，1例有浸润性癌。在45例（壶腹外）有HGD或低级别异型增生的十二指肠腺瘤患者中，8例（18%）患者接受了内镜切除（ER）。在短观察期内，ER仅在HGD病例中进行。没有患者死于十二指肠肿瘤。总体而言，在监测期内，8例斯皮格尔曼IV期患者中有5例（63%）检测到十二指肠HGD；在0期（n = 33）、I期（n = 0）、II期（n = 12）或III期（n = 20）患者中未检测到HGD。
短间隔内镜监测和适当的ER可能有助于预防十二指肠浸润性癌。此外，随访期间浸润性癌进展很少。斯皮格尔曼分类有助于对日本FAP患者十二指肠肿瘤进行风险评估。
