Tuberous sclerosis patient with neuroendocrine carcinoma of the esophagogastric junction: A case report

被引:3
作者
Ishida, Natsuki [1 ]
Miyazu, Takahiro [1 ]
Tamura, Satoshi [1 ]
Suzuki, Satoshi [2 ]
Tani, Shinya [2 ]
Yamade, Mihoko [1 ]
Iwaizumi, Moriya [3 ]
Osawa, Satoshi [2 ]
Hamaya, Yasushi [1 ]
Shinmura, Kazuya [4 ]
Sugimura, Haruhiko [4 ]
Miura, Katsutoshi [5 ]
Furuta, Takahisa [6 ]
Sugimoto, Ken [1 ]
机构
[1] Hamamatsu Univ Sch Med, Dept Med 1, 1-20-1 Handayama, Hamamatsu, Shizuoka 4313192, Japan
[2] Hamamatsu Univ Sch Med, Dept Endoscop & Photodynam Med, Hamamatsu, Shizuoka 4313192, Japan
[3] Hamamatsu Univ Sch Med, Dept Lab Med, Hamamatsu, Shizuoka 4313192, Japan
[4] Hamamatsu Univ Sch Med, Dept Tumor Pathol, Hamamatsu, Shizuoka 4313192, Japan
[5] Hamamatsu Univ Sch Med, Dept Hlth Sci, Hamamatsu, Shizuoka 4313192, Japan
[6] Hamamatsu Univ Sch Med, Clin Res Ctr, Hamamatsu, Shizuoka 4313192, Japan
关键词
Tuberous sclerosis complex; Neuroendocrine carcinoma; Neuroendocrine tumor; mTOR inhibitor; Esophagogastric junction; Chemotherapy; Case report; MIXED ADENONEUROENDOCRINE CARCINOMA; CHEMOTHERAPY; TUMORS; MANEC;
D O I
10.3748/wjg.v26.i45.7263
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
BACKGROUND Tuberous sclerosis complex (TSC) is a rare inherited disease with non-cancerous tumor growths in the skin, brain, kidneys, heart, and lungs. The co-occurrence of neuroendocrine neoplasm (NEN) with TSC is even rarer. There have been few reports on the relationship between TSC and neuroendocrine tumors (NETs), and fewer on the relationship between TSC and neuroendocrine carcinoma (NEC), a subtype of NEN. This is the first reported case of NEC occurring at the esophagogastric junction in a patient with TSC. CASE SUMMARY A 46-year-old woman visiting our hospital for the treatment of TSC was admitted to the emergency department with tarry stools and dizziness. Computed tomography scans revealed thickness of the gastric cardia, multiple metastatic lesions of the liver, and enlarged lymph nodes near the lesser curvature of the stomach. Esophagogastroduodenoscopy revealed a type 3 tumor located from the esophagogastric junction to the fundus, and the pathological diagnosis by biopsy was NEC. The patient was treated with seven courses of cisplatin + irinotecan, followed by eight courses of ramucirumab + nab-paclitaxel, one course of nivolumab, and two courses of S-1 + oxaliplatin. Twenty-three months after the first treatment, the patient died because of disease progression and deterioration of the general condition. CONCLUSION This case of NEC occurring in a patient with TSC indicates a difference in the occurrence of NETs and NECs.
引用
收藏
页码:7263 / 7271
页数:9
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