Do sporadic Peutz-Jeghers polyps exist? Experience of a large teaching hospital

Ashlie L. Burkart, Todd Sheridan, Marc Lewin, Hubert Fenton, Nilofar J. Ali, Elizabeth A Montgomery

Research output: Contribution to journalArticle

Abstract

Most types of sporadic gastrointestinal (GI) polyps vastly outnumber their syndromic counterparts. In contrast, the incidence of sporadic Peutz-Jeghers polyps (PJP) is unknown. We examined all potential PJP seen at our hospital over a 22-year (y) period to assess the incidence of sporadic PJP. The pathology database of a large hospital was searched for "Peutz-Jeghers polyp(s)," yielding 121 polyps from 38 patients. The polyps were reviewed by 3 pathologists to confirm the diagnosis. Clinical information to confirm or refute a diagnosis of Peutz-Jeghers syndrome (PJS) was collected. Of the 102 polyps included after histologic review, 94 polyps arose in patients meeting the World Health Organization criteria for PJS. These PJS polyps were eliminated from further analysis. Clinical information was obtained for the remaining 8 patients with potential "sporadic" PJP (1 to 50 y; mean=14 y; median=4 y). Of the 8 potential sporadic PJP, only 3 polyps from 3 patients had unequivocal PJP histologic features, all from the small intestine. All 3 patients had clinical histories suggesting syndromic PJP although they did not meet World Health Organization criteria, that is, 2 developed pancreatic cancer, 1 had bilateral "ovarian cystic masses" and a glomus tympanicum tumor, and 1 had strong family history of GI malignancies. The 5 remaining patients each had a colonic polyp with features suggestive, but not definitely diagnostic of, PJP. In these cases, prolapse lesions could not be excluded. One patient had a history of high-grade dysplasia in a tubulovillous adenoma in the colon at 53 years, but no family cancer history. Another had a family GI cancer history. Another had a history of pituitary adenoma at age 39, and the last had ductal breast carcinoma diagnosed 4 years before the discovery of the polyp. Our findings suggest that if sporadic PJP exist, they are extremely rare. Moreover, our data suggest that individuals with a single PJP may have a cumulative lifetime risk of cancer similar to those with the syndrome.

Original languageEnglish (US)
Pages (from-to)1209-1214
Number of pages6
JournalAmerican Journal of Surgical Pathology
Volume31
Issue number8
DOIs
StatePublished - Aug 2007

Fingerprint

Polyps
Teaching Hospitals
Peutz-Jeghers Syndrome
Glomus Tympanicum Tumor
Colonic Polyps
Carcinoma, Ductal, Breast
Neoplasms
Gastrointestinal Neoplasms
Prolapse
Incidence
Pituitary Neoplasms
Pancreatic Neoplasms

Keywords

  • Hamartomatous polyps
  • LKB1
  • Peutz-Jeghers polyp
  • Small intestine
  • Sporadic polyps
  • STK11

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine

Cite this

Do sporadic Peutz-Jeghers polyps exist? Experience of a large teaching hospital. / Burkart, Ashlie L.; Sheridan, Todd; Lewin, Marc; Fenton, Hubert; Ali, Nilofar J.; Montgomery, Elizabeth A.

In: American Journal of Surgical Pathology, Vol. 31, No. 8, 08.2007, p. 1209-1214.

Research output: Contribution to journalArticle

Burkart, Ashlie L. ; Sheridan, Todd ; Lewin, Marc ; Fenton, Hubert ; Ali, Nilofar J. ; Montgomery, Elizabeth A. / Do sporadic Peutz-Jeghers polyps exist? Experience of a large teaching hospital. In: American Journal of Surgical Pathology. 2007 ; Vol. 31, No. 8. pp. 1209-1214.
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