J Pathol Transl Med.  2016 Nov;50(6):482-485. 10.4132/jptm.2016.02.27.

Goblet Cell Carcinoid of the Rectum in a Patient with Neurofibromatosis Type 1

Affiliations
  • 1Department of Pathology, Korea University Ansan Hospital, Ansan, Korea. nausika@korea.ac.kr

Abstract

No abstract available.


MeSH Terms

Carcinoid Tumor*
Goblet Cells*
Humans
Neurofibromatoses*
Neurofibromatosis 1*
Rectum*

Figure

  • Fig. 1. The rectum shows a combined occurrence of goblet cell carcinoid (GCC, empty arrows) and neuroendocrine tumors (filled arrows) as well as neurofibromatosis (A). GCC is mostly composed of small nests of signet-ring-like goblet cells (B), and the cytoplasmic mucin is readily identified with alcian blue pH 2.5 (C). (D) Synaptophysin immunostain clearly shows a few scattered neuroendocrine cells in GCC.

  • Fig. 2. Neuroendocrine tumors (NETs) consist of small uniform cells which exclusively form trabecular structures (A) and diffusely stained with synaptophysin (B). In the vicinity of both goblet cell carcinoid and NETs, proliferation of slender spindle cells morphologically compatible with neurofibromatosis (C) is evident with S-100 protein immunostain (D).

  • Fig. 3. The submucosal tumor in the jejunum is composed of bland-looking spindle cells (A) which highly expressed c-Kit by immunohistochemistry (B).


Reference

1. Agaimy A, Vassos N, Croner RS. Gastrointestinal manifestations of neurofibromatosis type 1 (Recklinghausen’s disease): clinicopathological spectrum with pathogenetic considerations. Int J Clin Exp Pathol. 2012; 5:852–62.
2. Rindi G, Klimstra DS, Arnold R, et al. Nomenclature and classification of neuroendocrine neoplasms of the digestive system. Bosman FT, Carneiro F, Hruban RH, Theise ND. WHO classification of tumours of the digestive system. 4th ed. Lyon: IARC Press;2010. p. 13–4.
3. Gui X, Qin L, Gao ZH, Falck V, Harpaz N. Goblet cell carcinoids at extraappendiceal locations of gastrointestinal tract: an underrecognized diagnostic pitfall. J Surg Oncol. 2011; 103:790–5.
Article
4. Hofler H, Kloppel G, Heitz PU. Combined production of mucus, amines and peptides by goblet-cell carcinoids of the appendix and ileum. Pathol Res Pract. 1984; 178:555–61.
5. Stancu M, Wu TT, Wallace C, Houlihan PS, Hamilton SR, Rashid A. Genetic alterations in goblet cell carcinoids of the vermiform appendix and comparison with gastrointestinal carcinoid tumors. Mod Pathol. 2003; 16:1189–98.
Article
6. Yamabuki T, Omi M, Yonemori A, et al. Goblet cell carcinoid of the rectum with lymph node metastasis: report of a case. Surg Today. 2011; 41:1284–9.
Article
7. Gregersen T, Holt N, Gronbaek H, Vogel I, Jørgensen LJ, Krogh K. Goblet cell carcinoid in a patient with neurofibromatosis type 1: a rare combination. Case Rep Gastrointest Med. 2012; 2012:185730.
Article
8. Maertens O, Prenen H, Debiec-Rychter M, et al. Molecular pathogenesis of multiple gastrointestinal stromal tumors in NF1 patients. Hum Mol Genet. 2006; 15:1015–23.
Article
9. Stewart W, Traynor JP, Cooke A, et al. Gastric carcinoid: germline and somatic mutation of the neurofibromatosis type 1 gene. Fam Cancer. 2007; 6:147–52.
Article
Full Text Links
  • JPTM
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr