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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...
Autores principales: | , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Baishideng Publishing Group Inc
2020
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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. |
format | Online Article Text |
id | pubmed-7723665 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Baishideng Publishing Group Inc |
record_format | MEDLINE/PubMed |
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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