Shimura Yuhi, Kanaji Shingo, Urakawa Naoki, Yamamoto Masashi, Utsumi Masako, Takiguchi Gousuke, Hasegawa Hiroshi, Matsuda Yoshiko, Yamashita Kimihiro, Matsuda Takeru, Oshikiri Taro, Nakamura Tetsu, Suzuki Satoshi, Kakeji Yoshihiro
Division of Gastrointestinal Surgery, Department of Surgery, Graduate School of Medicine, Kobe University, 7-5-2 Kobeshi, Chuou-ku, Kusunoki-cho, Kobe, Hyogo, 650-0017, Japan.
Surg Case Rep. 2020 May 24;6(1):112. doi: 10.1186/s40792-020-00877-y.
We report a rare case of gastrointestinal stromal tumors (GISTs) in Carney's triad, successfully treated using laparoscopic gastrectomy with lymph node dissection after chemotherapy.
A 21-year-old woman presented to our hospital for treatment of recurrent GISTs. The patient had been admitted for treatment 11 years prior, with black stools being the chief presenting complaint at that time. On examination at that time, multiple submucosal tumors in the pyloric antrum and multiple pulmonary tumors had been observed. She underwent open partial gastrectomy, and the diagnosis of GISTs was confirmed. She was administered tyrosine kinase inhibitors to treat lung metastases from 2 months after surgery. Due to the increasing size of the lung tumors, a right upper lobectomy was performed 9 years after the index gastric surgery. Histopathological examination of the lung specimen, in combination with re-examination of the gastric specimens, was indicative of incomplete-type Carney's triad. Eleven years after the index gastric surgery, multiple GISTs were observed in her entire stomach. Tumor biopsy revealed a succinate dehydrogenase deficiency, confirming the diagnosis of recurrent GISTs. For treatment, the patient underwent laparoscopic completion gastrectomy, with D1 plus lymph node dissection.
This is a first case report of completion gastrectomy performed laparoscopically for the treatment of GISTs associated with incomplete-type Carney's triad. The recurrent GISTs developed over a protracted period of 11 years from the index gastric surgery to tumor recurrence.
我们报告了一例罕见的卡尼三联征中的胃肠道间质瘤(GIST),在化疗后通过腹腔镜胃切除术加淋巴结清扫成功治疗。
一名21岁女性因复发性GIST到我院就诊。该患者11年前曾因黑便为主诉入院治疗。当时检查发现幽门窦多发黏膜下肿瘤及多发肺肿瘤。她接受了开放性部分胃切除术,确诊为GIST。术后2个月开始给予酪氨酸激酶抑制剂治疗肺转移。由于肺肿瘤增大,在首次胃手术后9年进行了右上肺叶切除术。肺标本的组织病理学检查结合胃标本的重新检查,提示为不完全型卡尼三联征。首次胃手术后11年,在她的整个胃内观察到多发GIST。肿瘤活检显示琥珀酸脱氢酶缺乏,确诊为复发性GIST。为进行治疗,患者接受了腹腔镜全胃切除术及D1+淋巴结清扫。
这是首例关于腹腔镜全胃切除术治疗不完全型卡尼三联征相关GIST的病例报告。复发性GIST从首次胃手术到肿瘤复发历时11年。