J Pathol Transl Med.  2018 Mar;52(2):126-129. 10.4132/jptm.2017.09.16.

Multiple Neuroendocrine Tumors in Stomach and Duodenum in a Multiple Endocrine Neoplasia Type 1 Patient

• Kim B
• Yang HK
• Kim WH

• KMID: 2408514
• DOI: http://doi.org/10.4132/jptm.2017.09.16

Abstract

A 67-year-old woman with a history of subtotal parathyroidectomy, distal pancreatectomy, and total splenectomy 23 years prior underwent surgical gastric resection for neuroendocrine tumors of the stomach and duodenum. Meticulous examination of the entire stomach and duodenum revealed multiple scattered, minute neuroendocrine tumors. To the best of our knowledge, this is the first case report of a patient diagnosed with gastroduodenal neuroendocrine tumors associated with multiple endocrine neoplasia type 1 (MEN 1) in whom complete histologic mapping of the whole gastrectomy specimen was performed. The presence of MEN 1-associated neuroendocrine tumors in the stomach is very rare, but should be considered in patients diagnosed with MEN 1 who present with a new tumor in the stomach.

Keyword

Multiple endocrine neoplasia type 1; Stomach neoplasms; Neuroendocrine tumors; Histologic mapping

MeSH Terms

Aged
Duodenum*
Female
Gastrectomy
Humans
Male
Multiple Endocrine Neoplasia Type 1*
Multiple Endocrine Neoplasia*
Neuroendocrine Tumors*
Pancreatectomy
Parathyroidectomy
Splenectomy
Stomach Neoplasms
Stomach*

Figure

• 

  Fig. 1. (A) Two subepithelial masses were found in the duodenum on esophagogastroduodenoscopy. (B) A neuroendocrine tumor with a diffuse pattern. (C) Immunohistochemistry of cytokeratin. (D) Immunohistochemistry of chromogranin A. (E) Abdominal computed tomography reveals a 6-mm-sized nodule (arrow) in the duodenal bulb.

• 

  Fig. 2. Histologic features of neuroendocrine tumors. (A) Neuroendocrine dysplasia in the submucosal layer of the duodenum. (B) Neuroendocrine tumor in a ribbon-like growth pattern. (C) Metastatic lymph node in station 3. (D) Immunohistochemistry of Ki-67.

• 

  Fig. 3. Histologic mapping of the entire specimen revealed 19 neuroendocrine lesions in the stomach and duodenum (red, neuroendocrine tumor; blue, neuroendocrine dysplasia).

Reference

1. Kouvaraki MA, Lee JE, Shapiro SE, et al. Genotype-phenotype analysis in multiple endocrine neoplasia type 1. Arch Surg. 2002; 137:641–7.

2. de Laat JM, van der Luijt RB, Pieterman CR, et al. MEN1 redefined, a clinical comparison of mutation-positive and mutation-negative patients. BMC Med. 2016; 14:182.

3. Thakker RV. Multiple endocrine neoplasia type 1 (MEN1) and type 4 (MEN4). Mol Cell Endocrinol. 2014; 386:2–15.

4. Feliberti E, Perry RR, Vinik A. Multiple endocrine neoplasia type I and MEN II. In : De Groot LJ, Chrousos G, Dungan K, editors. Endotext. South Dartmouth: MDText.com Inc;2000.

5. Turner JJ, Christie PT, Pearce SH, Turnpenny PD, Thakker RV. Diagnostic challenges due to phenocopies: lessons from multiple endocrine neoplasia type1 (MEN1). Hum Mutat. 2010; 31:E1089–101.

6. Grin A, Streutker CJ. Neuroendocrine tumors of the luminal gastrointestinal tract. Arch Pathol Lab Med. 2015; 139:750–6.

7. Wang GG, Yao JC, Worah S, et al. Comparison of genetic alterations in neuroendocrine tumors: frequent loss of chromosome 18 in ileal carcinoid tumors. Mod Pathol. 2005; 18:1079–87.

8. Christopoulos C, Papavassiliou E. Gastric neuroendocrine tumors: biology and management. Ann Gastroenterol. 2005; 18:127–40.

9. Falchetti A. Genetic screening for multiple endocrine neoplasia syndrome type 1 (MEN-1): when and how. F1000 Med Rep. 2010; 2:14.

10. Tsikitis VL, Wertheim BC, Guerrero MA. Trends of incidence and survival of gastrointestinal neuroendocrine tumors in the United States: a seer analysis. J Cancer. 2012; 3:292–302.

11. Li TT, Qiu F, Qian ZR, Wan J, Qi XK, Wu BY. Classification, clinicopathologic features and treatment of gastric neuroendocrine tumors. World J Gastroenterol. 2014; 20:118–25.

12. Sato Y, Hashimoto S, Mizuno K, Takeuchi M, Terai S. Management of gastric and duodenal neuroendocrine tumors. World J Gastroenterol. 2016; 22:6817–28.