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. 2024 Mar 7;31(6):432-436.
doi: 10.1159/000536674. eCollection 2024 Dec.

Gastric Mixed Neuroendocrine-Non-Neuroendocrine Neoplasm: An Unusual Tumor and Its Presentation in a Young Adult

Tânia Carvalho  1 , Andreia Coutada  2 , Manuel Jácome  2 , Dália Fernandes  1
Affiliations

Gastric Mixed Neuroendocrine-Non-Neuroendocrine Neoplasm: An Unusual Tumor and Its Presentation in a Young Adult

Tânia Carvalho et al. GE Port J Gastroenterol. .

Abstract
in English, Portuguese

Introduction: Gastric cancer is the fourth most common cause of cancer death, with more than 90% of the cases being adenocarcinomas. Among the diverse subtypes, mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN) is one of the rarest types. Gastric cancer can manifest with significant bleeding in up to 5% of patients.

Case presentation: The authors present a case of a healthy 26-year-old male who experienced two episodes of major upper gastrointestinal bleeding that were resolved with endoscopic treatment. During the second endoscopy, a 15-mm nodular subepithelial lesion was identified at the gastroesophageal junction. Endoscopic ultrasound revealed a homogeneous and hypoechoic lesion with well-defined limits in the deep mucosa. Histological examination of the biopsies showed an adenocarcinoma. The patient later underwent a distal esophagectomy and a total gastrectomy, followed by chemotherapy. Histological examination of the surgical specimen showed a mixed adenoneuroendocrine carcinoma composed of an adenocarcinoma with tubular/glandular pattern and signet ring cells and a large cell-type neuroendocrine carcinoma. The neoplasia had infiltrated the outer muscular layers of the stomach and had disseminated to 3 regional lymph nodes, leading to its classification as stage IIb. Two years following the treatment, there is no evidence of recurrence. All genetic tests applied were negative.

Discussion: A MiNEN occurs when both neuroendocrine and non-neuroendocrine components represent at least 30% of the lesion. Due to its rarity, epidemiology and standard treatment are not well established because most data published are from case reports. In this context, we present a compelling case study, highlighting the patient's young age, the rarity of this specific cancer, and its uncommon presentation.

Introdução: O cancro gástrico é a quarta causa mais comum de morte por cancro, sendo, em mais de 90% dos casos, adenocarcinoma. Entre os vários subtipos de tumores gástricos, a neoplasia mista neuroendócrina e não-neuroendócrina (MiNEN) é dos mais raros. O cancro gástrico pode-se manifestar como hemorragia digestiva major em até 5% dos doentes.

Apresentação do caso: Os autores apresentam o caso de um jovem saudável de 26 anos que se apresentou com dois episódios de hemorragia digestiva major tratadas endoscopicamente. Na segunda endoscopia observou-se uma lesão subepitelial nodular com 15 mm na junção esofagogástrica que, na ecoendoscopia, apresentava-se como uma lesão homogénea e hipoecóica, com limites bem definidos, na dependência da mucosa profunda. As biópsias da lesão revelaram presença de adenocarcinoma. O doente foi submetido a uma gastrectomia total com esofagectomia distal e, posteriormente, a quimioterapia. Na peça cirúrgica foi identificado um carcinoma misto adenoneuroendócrino composto por um adenocarcinoma com padrão tubular/glandular e células em anel de sinete e um carcinoma neurodendócrino de células grandes. O tumor invadia a camada muscular externa do estômago e apresentava 3 adenopatias locais, sendo classificado como estadio IIb. Após 2 anos de seguimento, não há sinais de recidiva. Todos os testes genéticos realizados foram negativos. Um MiNEN é definido pela presença de um componente neuroendócrino e um não-neuroendócrino, sendo que cada um representa, pelo menos, 30% da lesão. A epidemiologia e o tratamento mais adequado ainda não estão bem estabelecidos, dado que é um tumor raro e a maioria da informação disponível advém de casos clínicos. Neste contexto, os autores apresentam um caso clínico em que se destaca a idade jovem do doente, a raridade do tumor e e a sua apresentação incomum.

Keywords: Gastric cancer; Mixed neuroendocrine-non-neuroendocrine neoplasm; Upper gastrointestinal bleeding.

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Conflict of interest statement

The authors have no conflicts of interest to declare.

Figures

Fig. 1.
Fig. 1.
Forrest Ib ulcer with active oozing at the cardia treated with diluted adrenaline and polidocanol injection since clip placement in the vessel was unsuccessful.
Fig. 2.
Fig. 2.
15-mm nodular subepithelial lesion at the gastroesophageal junction with ulceration in the proximal margin of the gastric mucosa.
Fig. 3.
Fig. 3.
Homogeneous and hypoechoic lesion of 15 × 6 mm and well-defined limits, originated at the deep mucosa without local adenopathy’s.
Fig. 4.
Fig. 4.
Mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN), composed of an adenocarcinoma and a large cell neuroendocrine carcinoma. In mixed adenoneuroendocrine carcinoma (MANEC), each component constitutes more than 30% of the neoplasm. a Tubular/glandular pattern (right) and poorly cohesive cells (left). b Synaptophysin positive. c Chromogranin positive (focally). d Cam5.2 positive. e Ki-67 proliferation index more than 80%.
See this image and copyright information in PMC

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