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Gastroesophageal Junction Neuroendocrine Carcinoma

MedGen UID:
926576
•Concept ID:
C4289044
•
Neoplastic Process

Definition

An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the gastroesophageal junction. According to the size of the malignant cells, the prominence of the nucleoli, and the amount of cytoplasm, it is classified either as small or large cell neuroendocrine carcinoma. [from NCI]

Recent clinical studies

Etiology

Histology Shift in Esophageal Cancer Between Biopsies and Resections After Neoadjuvant Therapy: A Pilot Study.
Hou T, Yang Z, Zhang Q, Zhang X, Liao X, Lin J
Int J Surg Pathol 2024 Aug;32(5):920-925. Epub 2023 Oct 30 doi: 10.1177/10668969231208029. PMID: 37899731
Pathological Diversity of Gastric Cancer from the Viewpoint of Background Condition.
Abe H, Ushiku T
Digestion 2022;103(1):45-53. Epub 2021 Oct 8 doi: 10.1159/000519337. PMID: 34628409
Surgical Resection of Rare Esophageal Cancers.
Saddoughi SA, Taswell J, Harmsen WS, Inra ML, Blackmon SH, Nichols FC 3rd, Cassivi SD, Wigle DA, Shen KR, Allen MS
Ann Thorac Surg 2016 Jan;101(1):311-5. Epub 2015 Aug 13 doi: 10.1016/j.athoracsur.2015.06.015. PMID: 26277558
The Multidisciplinary Team Conference's Decision on M-Staging in Patients with Gastric- and Gastroesophageal Cancer is not Accurate without Staging Laparoscopy.
Strandby RB, Svendsen LB, Fallentin E, Egeland C, Achiam MP
Scand J Surg 2016 Jun;105(2):104-8. Epub 2015 Aug 10 doi: 10.1177/1457496915598760. PMID: 26261200
Comparison of gastro-oesophageal junction carcinomas in Chinese versus American patients.
Huang Q, Fan X, Agoston AT, Feng A, Yu H, Lauwers G, Zhang L, Odze RD
Histopathology 2011 Aug;59(2):188-97. doi: 10.1111/j.1365-2559.2011.03924.x. PMID: 21884197

Diagnosis

High-grade mixed neuroendocrine non-neuroendocrine neoplasm of the gastroesophageal junction: A rare case report and review of literature.
Sulaiman M, Agarwal S, Mandal A, Kumar S, Das P
Indian J Pathol Microbiol 2022 Oct-Dec;65(4):918-920. doi: 10.4103/ijpm.ijpm_143_21. PMID: 36308207
Tuberous sclerosis patient with neuroendocrine carcinoma of the esophagogastric junction: A case report.
Ishida N, Miyazu T, Tamura S, Suzuki S, Tani S, Yamade M, Iwaizumi M, Osawa S, Hamaya Y, Shinmura K, Sugimura H, Miura K, Furuta T, Sugimoto K
World J Gastroenterol 2020 Dec 7;26(45):7263-7271. doi: 10.3748/wjg.v26.i45.7263. PMID: 33362382Free PMC Article
Surgical Resection of Rare Esophageal Cancers.
Saddoughi SA, Taswell J, Harmsen WS, Inra ML, Blackmon SH, Nichols FC 3rd, Cassivi SD, Wigle DA, Shen KR, Allen MS
Ann Thorac Surg 2016 Jan;101(1):311-5. Epub 2015 Aug 13 doi: 10.1016/j.athoracsur.2015.06.015. PMID: 26277558
From the radiologic pathology archives: esophageal neoplasms: radiologic-pathologic correlation.
Lewis RB, Mehrotra AK, Rodriguez P, Levine MS
Radiographics 2013 Jul-Aug;33(4):1083-108. doi: 10.1148/rg.334135027. PMID: 23842973
Barrett's metaplasia and adenocarcinoma of the esophagus and gastroesophageal junction.
Thompson JJ, Zinsser KR, Enterline HT
Hum Pathol 1983 Jan;14(1):42-61. doi: 10.1016/s0046-8177(83)80045-8. PMID: 6832749

Therapy

PD-1 blockade in deficient mismatch repair mixed adenoneuroendocrine carcinoma of the stomach: new hope for an orphan disease.
Riccò B, Salati M, Reggiani Bonetti L, Dominici M, Luppi G
Tumori 2020 Dec;106(6):NP57-NP62. Epub 2020 Sep 2 doi: 10.1177/0300891620952845. PMID: 32878569
A Rare Case of Mixed Adenoneuroendocrine Carcinoma (MANEC) of the Gastroesophageal Junction with HER2/neu Overexpression and Distinct Orbital and Optic Nerve Toxicity after Intravenous Administration of Cisplatin.
Golombek T, Henker R, Rehak M, Quäschling U, Lordick F, Knödler M
Oncol Res Treat 2019;42(3):123-127. Epub 2019 Feb 23 doi: 10.1159/000495218. PMID: 30799422
New model for gastroenteropancreatic large-cell neuroendocrine carcinoma: establishment of two clinically relevant cell lines.
Krieg A, Mersch S, Boeck I, Dizdar L, Weihe E, Hilal Z, Krausch M, Möhlendick B, Topp SA, Piekorz RP, Huckenbeck W, Stoecklein NH, Anlauf M, Knoefel WT
PLoS One 2014;9(2):e88713. Epub 2014 Feb 14 doi: 10.1371/journal.pone.0088713. PMID: 24551139Free PMC Article
Unusual case of small cell gastric carcinoma: case report and literature review.
Richards D, Davis D, Yan P, Guha S
Dig Dis Sci 2011 Apr;56(4):951-7. Epub 2010 Sep 18 doi: 10.1007/s10620-010-1404-1. PMID: 20848201

Prognosis

Large cell neuroendocrine carcinoma at the esophagogastric junction. An extremely rare entity.
García-Redondo M, Ruiz Pardo J, Sánchez Fuentes PA, Vidaña Márquez E, Belda Lozano R, Pérez-Rodríguez Á, Gorostiaga Í, Reina Duarte Á
Rev Esp Enferm Dig 2023 Dec;115(12):719-720. doi: 10.17235/reed.2023.9505/2023. PMID: 36809887
Tuberous sclerosis patient with neuroendocrine carcinoma of the esophagogastric junction: A case report.
Ishida N, Miyazu T, Tamura S, Suzuki S, Tani S, Yamade M, Iwaizumi M, Osawa S, Hamaya Y, Shinmura K, Sugimura H, Miura K, Furuta T, Sugimoto K
World J Gastroenterol 2020 Dec 7;26(45):7263-7271. doi: 10.3748/wjg.v26.i45.7263. PMID: 33362382Free PMC Article
Surgical Resection of Rare Esophageal Cancers.
Saddoughi SA, Taswell J, Harmsen WS, Inra ML, Blackmon SH, Nichols FC 3rd, Cassivi SD, Wigle DA, Shen KR, Allen MS
Ann Thorac Surg 2016 Jan;101(1):311-5. Epub 2015 Aug 13 doi: 10.1016/j.athoracsur.2015.06.015. PMID: 26277558
Unusual case of small cell gastric carcinoma: case report and literature review.
Richards D, Davis D, Yan P, Guha S
Dig Dis Sci 2011 Apr;56(4):951-7. Epub 2010 Sep 18 doi: 10.1007/s10620-010-1404-1. PMID: 20848201
Neuroendocrine in Barrett's mucosa and adenocarcinomas of the gastroesophageal junction.
Koppert LB, Wijnhoven BP, Tilanus HW, Stijnen T, Van Dekken H, Dinjens WN
Int J Surg Pathol 2004 Apr;12(2):117-25. doi: 10.1177/106689690401200204. PMID: 15173916

Clinical prediction guides

Histology Shift in Esophageal Cancer Between Biopsies and Resections After Neoadjuvant Therapy: A Pilot Study.
Hou T, Yang Z, Zhang Q, Zhang X, Liao X, Lin J
Int J Surg Pathol 2024 Aug;32(5):920-925. Epub 2023 Oct 30 doi: 10.1177/10668969231208029. PMID: 37899731
A case of large cell neuroendocrine carcinoma exhibiting rhabdoid features in the esophagogastric junction.
Ichimata S, Aoyagi D, Takehana T, Uehara T, Shiozawa S
Pathol Int 2019 Aug;69(8):481-487. Epub 2019 Jun 24 doi: 10.1111/pin.12807. PMID: 31237049
Poorly Differentiated Medullary Phenotype Predicts Poor Survival in Early Lymph Node-Negative Gastro-Esophageal Adenocarcinomas.
Treese C, Sanchez P, Grabowski P, Berg E, Bläker H, Kruschewski M, Haase O, Hummel M, Daum S
PLoS One 2016;11(12):e0168237. Epub 2016 Dec 28 doi: 10.1371/journal.pone.0168237. PMID: 28030564Free PMC Article
Functional outcomes and quality of life after proximal gastrectomy with esophagogastrostomy using a narrow gastric conduit.
Ronellenfitsch U, Najmeh S, Andalib A, Perera RM, Rousseau MC, Mulder DS, Ferri LE
Ann Surg Oncol 2015 Mar;22(3):772-9. Epub 2014 Sep 12 doi: 10.1245/s10434-014-4078-7. PMID: 25212836
New model for gastroenteropancreatic large-cell neuroendocrine carcinoma: establishment of two clinically relevant cell lines.
Krieg A, Mersch S, Boeck I, Dizdar L, Weihe E, Hilal Z, Krausch M, Möhlendick B, Topp SA, Piekorz RP, Huckenbeck W, Stoecklein NH, Anlauf M, Knoefel WT
PLoS One 2014;9(2):e88713. Epub 2014 Feb 14 doi: 10.1371/journal.pone.0088713. PMID: 24551139Free PMC Article

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