Massive gastric juvenile-type polyposis: a clinicopathological analysis of 22 cases

Raul S. Gonzalez, Volkan Adsay, Rondell Graham, Stuti G. Shroff, Michael M. Feely, Michael G. Drage, David N. Lewin, Eric A. Swanson, Rhonda K. Yantiss, Pelin Bağci, Alyssa M. Krasinskas

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

Aims: Massive gastric polyposis is a rare entity that is often associated with juvenile polyposis syndrome (JPS). The aim of this study was to evaluate the clinicopathological features of 22 patients with abundant gastric juvenile-type or hyperplastic-like polyps. Methods and results: The study included 12 males and 10 females with a median age of 48 years (range: 13–79 years). Fourteen (64%) patients carried a diagnosis of JPS, and three had prior gastrointestinal adenocarcinomas. Patients without known JPS presented at an older median age (60 years versus 40 years; P = 0.0068). Clinical symptoms included nausea, vomiting, and abdominal pain; 23% of patients were asymptomatic. Eighteen cases showed complete or near-complete carpeting of the gastric mucosa by innumerable polyps, ranging from a few millimetres to ~100 mm. Most polyps formed long, bulbous projections and had characteristic histological features, including a smooth outer contour, prominent stromal oedema, and widely spaced, often cystically dilated glands lined by foveolar epithelium; some polyps had less stroma and more hyperplastic foveolar epithelium. All had normal underlying or adjacent mucosa. Four (18%) cases harboured adenocarcinoma, and seven (32%) others showed dysplasia. SMAD4 immunohistochemical staining showed patchy loss in polyps from 19 of 20 cases tested. Five of six (84%) patients tested had a germline SMAD4 mutation. Conclusions: Massive gastric juvenile-type polyposis can occur in patients with and without known JPS, and may mimic different conditions, such as other polyposis syndromes and Ménétrier disease. Pathologists play an important role in disease classification, as some patients lack a family or personal history of JPS, have few if any colonic polyps, and may not harbour diagnostic germline mutations.

Original languageEnglish (US)
Pages (from-to)918-928
Number of pages11
JournalHistopathology
Volume70
Issue number6
DOIs
StatePublished - May 1 2017

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Polyps
Germ-Line Mutation
Adenocarcinoma
Epithelium
Colonic Polyps
Gastric Mucosa
Gastric Polyposis
Nausea
Abdominal Pain
Vomiting
Edema
Stomach
Mucous Membrane
Juvenile polyposis syndrome
Staining and Labeling

Keywords

  • gastric polyps
  • genetics
  • juvenile polyposis syndrome
  • Ménétrier disease
  • SMAD4

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology

Cite this

Gonzalez, R. S., Adsay, V., Graham, R., Shroff, S. G., Feely, M. M., Drage, M. G., ... Krasinskas, A. M. (2017). Massive gastric juvenile-type polyposis: a clinicopathological analysis of 22 cases. Histopathology, 70(6), 918-928. https://doi.org/10.1111/his.13149

Massive gastric juvenile-type polyposis : a clinicopathological analysis of 22 cases. / Gonzalez, Raul S.; Adsay, Volkan; Graham, Rondell; Shroff, Stuti G.; Feely, Michael M.; Drage, Michael G.; Lewin, David N.; Swanson, Eric A.; Yantiss, Rhonda K.; Bağci, Pelin; Krasinskas, Alyssa M.

In: Histopathology, Vol. 70, No. 6, 01.05.2017, p. 918-928.

Research output: Contribution to journalArticle

Gonzalez, RS, Adsay, V, Graham, R, Shroff, SG, Feely, MM, Drage, MG, Lewin, DN, Swanson, EA, Yantiss, RK, Bağci, P & Krasinskas, AM 2017, 'Massive gastric juvenile-type polyposis: a clinicopathological analysis of 22 cases', Histopathology, vol. 70, no. 6, pp. 918-928. https://doi.org/10.1111/his.13149
Gonzalez, Raul S. ; Adsay, Volkan ; Graham, Rondell ; Shroff, Stuti G. ; Feely, Michael M. ; Drage, Michael G. ; Lewin, David N. ; Swanson, Eric A. ; Yantiss, Rhonda K. ; Bağci, Pelin ; Krasinskas, Alyssa M. / Massive gastric juvenile-type polyposis : a clinicopathological analysis of 22 cases. In: Histopathology. 2017 ; Vol. 70, No. 6. pp. 918-928.
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abstract = "Aims: Massive gastric polyposis is a rare entity that is often associated with juvenile polyposis syndrome (JPS). The aim of this study was to evaluate the clinicopathological features of 22 patients with abundant gastric juvenile-type or hyperplastic-like polyps. Methods and results: The study included 12 males and 10 females with a median age of 48 years (range: 13–79 years). Fourteen (64{\%}) patients carried a diagnosis of JPS, and three had prior gastrointestinal adenocarcinomas. Patients without known JPS presented at an older median age (60 years versus 40 years; P = 0.0068). Clinical symptoms included nausea, vomiting, and abdominal pain; 23{\%} of patients were asymptomatic. Eighteen cases showed complete or near-complete carpeting of the gastric mucosa by innumerable polyps, ranging from a few millimetres to ~100 mm. Most polyps formed long, bulbous projections and had characteristic histological features, including a smooth outer contour, prominent stromal oedema, and widely spaced, often cystically dilated glands lined by foveolar epithelium; some polyps had less stroma and more hyperplastic foveolar epithelium. All had normal underlying or adjacent mucosa. Four (18{\%}) cases harboured adenocarcinoma, and seven (32{\%}) others showed dysplasia. SMAD4 immunohistochemical staining showed patchy loss in polyps from 19 of 20 cases tested. Five of six (84{\%}) patients tested had a germline SMAD4 mutation. Conclusions: Massive gastric juvenile-type polyposis can occur in patients with and without known JPS, and may mimic different conditions, such as other polyposis syndromes and M{\'e}n{\'e}trier disease. Pathologists play an important role in disease classification, as some patients lack a family or personal history of JPS, have few if any colonic polyps, and may not harbour diagnostic germline mutations.",
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