Korean J Gastroenterol.  2019 Dec;74(6):349-355. 10.4166/kjg.2019.74.6.349.

Mixed Neuroendocrine-non-neuroendocrine Neoplasm of the Stomach that is Distributed in Depth on the Same Tumor: Inconsistent with the Definition of Mixed Adenoneuroendocrine Carcinoma in the 2010 World Health Organization Classification of Tumors of the Digestive System

• Jeong J
• Jung K
• Kim JH
• Kim SE
• Moon W
• Park MI
• Park SJ

• KMID: 2469910
• DOI: http://doi.org/10.4166/kjg.2019.74.6.349

Abstract

A mixed adenoneuroendocrine carcinoma (MANEC) of the stomach is a rare disease entity that was first defined by the World Health Organization (WHO) classification (2010) for tumors of the digestive system. According to the WHO classification (2010), MANEC is referred to as a tumor with both neuroendocrine and non-neuroendocrine neoplasms; each component of the tumor should be at least 30%. On the other hand, this cut-off value lacks clinical evidence and does not explain the characteristics and heterogeneity of this tumor. A 66-year-old male diagnosed with early gastric cancer (EGC) at a community hospital was referred to the Kosin University Gospel Hospital for further evaluation of gastric cancer. Esophagogastroduodenoscopy and EUS performed at the Kosin University Gospel Hospital revealed a sub-mucosal tumor-like component. In addition, a re-biopsy revealed a neuroendorine tumor at different depths of the same tumor. The final pathologic-diagnosis through surgery revealed a mixed neuroendocrine-non-neuroendocrine neoplasm, which is inconsistent with the definition of MANEC. Clinicians should consider EUS when a tumor has atypical endoscopic findings, even if EGC has already been diagnosed.

Keyword

Stomach neoplasms; Adenocarcinoma; Neuroendocrine tumors; World Health Organization

MeSH Terms

Adenocarcinoma
Aged
Classification
Digestive System*
Endoscopy, Digestive System
Global Health*
Hand
Hospitals, Community
Humans
Male
Neuroendocrine Tumors
Population Characteristics
Rare Diseases
Stomach Neoplasms
Stomach*
World Health Organization*

Figure

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  Fig. 1 Esophagogastroduodenoscopy findings at an outside hospital. The picture showed a 1.4×0.8 cm sized mucosal elevation with central ulceration at the greater curvature side of the distal antrum.

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  Fig. 2 (A) Pathology findings of the outside hospital; it showed a moderately differentiated adenocarcinoma (H&E, ×40). (B) H&E, ×100 on the site of adenocarcinoma.

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  Fig. 3 Esophagogastroduodenoscopy findings at Kosin University Gospel Hospital showing a 2.0×0.8 cm sized mucosal elevation with central ulceration. The lesion also had an irregular surface and asymmetric hyperemic mucosal-change.

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  Fig. 4 Findings of endoscopic ultrasound at Kosin University Gospel Hospital showing a 1.3×0.4 cm sized isoechoic, and hypoechoic mass-like lesion with an ovoidal shape. Some parts of the lesion invaded the submucosal layer.

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  Fig. 5 According to the endoscopic biopsy at Kosin University Gospel Hospital, the pictures showed the characteristic results of immunohistochemistry shown in the neuroendocrine tumor (A: CD56, ×100; B: chromogranin, ×100; C: synaptophysin, ×100).

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  Fig. 6 Pathology results from a surgical specimen. The low magnification microscopy image showed that tubular adenocarcinoma and neuroendocrine carcinoma were distributed with depth (A: H&E, ×40; B: tubular adenocarcinoma, H&E, ×100; C: neuroendocrine tumor with 2 mitoses, ×100; D: Ki-57 index, 20–30%; E: immunohistochemistry result of chromogranin, diffuse weak positive, ×100).

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