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

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 neuroend...

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Autores principales: Ishida, Natsuki, Miyazu, Takahiro, Tamura, Satoshi, Suzuki, Satoshi, Tani, Shinya, Yamade, Mihoko, Iwaizumi, Moriya, Osawa, Satoshi, Hamaya, Yasushi, Shinmura, Kazuya, Sugimura, Haruhiko, Miura, Katsutoshi, Furuta, Takahisa, Sugimoto, Ken
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Baishideng Publishing Group Inc 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7723665/
https://www.ncbi.nlm.nih.gov/pubmed/33362382
http://dx.doi.org/10.3748/wjg.v26.i45.7263
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author Ishida, Natsuki
Miyazu, Takahiro
Tamura, Satoshi
Suzuki, Satoshi
Tani, Shinya
Yamade, Mihoko
Iwaizumi, Moriya
Osawa, Satoshi
Hamaya, Yasushi
Shinmura, Kazuya
Sugimura, Haruhiko
Miura, Katsutoshi
Furuta, Takahisa
Sugimoto, Ken
author_facet Ishida, Natsuki
Miyazu, Takahiro
Tamura, Satoshi
Suzuki, Satoshi
Tani, Shinya
Yamade, Mihoko
Iwaizumi, Moriya
Osawa, Satoshi
Hamaya, Yasushi
Shinmura, Kazuya
Sugimura, Haruhiko
Miura, Katsutoshi
Furuta, Takahisa
Sugimoto, Ken
author_sort Ishida, Natsuki
collection PubMed
description 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.
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spelling pubmed-77236652020-12-24 Tuberous sclerosis patient with neuroendocrine carcinoma of the esophagogastric junction: A case report Ishida, Natsuki Miyazu, Takahiro Tamura, Satoshi Suzuki, Satoshi Tani, Shinya Yamade, Mihoko Iwaizumi, Moriya Osawa, Satoshi Hamaya, Yasushi Shinmura, Kazuya Sugimura, Haruhiko Miura, Katsutoshi Furuta, Takahisa Sugimoto, Ken World J Gastroenterol Case Report 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. Baishideng Publishing Group Inc 2020-12-07 2020-12-07 /pmc/articles/PMC7723665/ /pubmed/33362382 http://dx.doi.org/10.3748/wjg.v26.i45.7263 Text en ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved. http://creativecommons.org/licenses/by-nc/4.0/ This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
spellingShingle Case Report
Ishida, Natsuki
Miyazu, Takahiro
Tamura, Satoshi
Suzuki, Satoshi
Tani, Shinya
Yamade, Mihoko
Iwaizumi, Moriya
Osawa, Satoshi
Hamaya, Yasushi
Shinmura, Kazuya
Sugimura, Haruhiko
Miura, Katsutoshi
Furuta, Takahisa
Sugimoto, Ken
Tuberous sclerosis patient with neuroendocrine carcinoma of the esophagogastric junction: A case report
title Tuberous sclerosis patient with neuroendocrine carcinoma of the esophagogastric junction: A case report
title_full Tuberous sclerosis patient with neuroendocrine carcinoma of the esophagogastric junction: A case report
title_fullStr Tuberous sclerosis patient with neuroendocrine carcinoma of the esophagogastric junction: A case report
title_full_unstemmed Tuberous sclerosis patient with neuroendocrine carcinoma of the esophagogastric junction: A case report
title_short Tuberous sclerosis patient with neuroendocrine carcinoma of the esophagogastric junction: A case report
title_sort tuberous sclerosis patient with neuroendocrine carcinoma of the esophagogastric junction: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7723665/
https://www.ncbi.nlm.nih.gov/pubmed/33362382
http://dx.doi.org/10.3748/wjg.v26.i45.7263
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