Peutz-Jeghers Syndrome Associated With Functioning Ovarian Tumor

Christian, Charles L.; McLoughlin, Thomas G.; Cathcart, Edward R.; Eisenberg, M. Michael

doi:10.1001/jama.1964.03070230071027

Cited by 36 publications

(4 citation statements)

References 8 publications

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“…Her mother had pigmentation of her lips, but no evidence or symptoms of GI polyposis, and the rest of the family history was unremarkable. She underwent an exploratory laparotomy, and was found to have multiple polyps of the small intestine, stomach, duodenum, and a mass on the left ovary that turned out to be a granulosa-theca cell tumor22. Other authors have described ovarian tumors in patients with PJS64,136,157, and Scully et al concluded that up to 5% of females with PJS develop an unusual tumor of the ovary, called ovarian sex cord tumor with annular tubules, which are microscopic and tend to be bilateral and multifocal132.…”

Section: Peutz-jeghers Syndrome (Pjs)mentioning

confidence: 99%

Hamartomatous Polyposis Syndromes

Calva

Howe

2008

Surgical Clinics of North America

127

116

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SynopsisSince the histological description of the hamartomatous polyp in 1957 by Horrilleno et al., several different syndromes have been described with the propensity to develop these polyps in the upper and lower GI tracts. These include Juvenile Polyposis, Peutz-Jeghers syndrome, hereditary mixed polyposis syndrome, and the PTEN hamartoma tumor syndromes (Cowden and Bannayan-RileyRuvalcaba syndromes), which are autosomal-dominantly inherited, and Cronkhite-Canada syndrome, which is acquired. The clinical aspects, the molecular pathogenesis, the organ systems affected, the risks of cancer, and the management of these hamartomatous polyposis syndromes will be reviewed in this paper. Although the incidence of these syndromes is low, it is important for clinicians to recognize these disorders in order to prevent morbidity and mortality in these patients, and to perform presymptomatic testing in patients at risk.

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Section: Peutz-jeghers Syndrome (Pjs)mentioning

confidence: 99%

Hamartomatous Polyposis Syndromes

Calva

Howe

2008

Surgical Clinics of North America

127

116

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“…11 Cervical adenocarcinoma and ovarian (granulosa cell) tumors are not a rare finding in PJS female patients. 12 A small number of cancers affecting lung, gall bladder, bile duct, basal cells, and blood stem cells have been described in PJS patients. 3,4,13 Recently, germline mutations in LKB1 have been associated with PJS.…”

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confidence: 99%

LKB1 Somatic Mutations in Sporadic Tumors

Avizienyte

Loukola

Roth

et al. 1999

The American Journal of Pathology

116

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“…In women with Peutz-Jeghers syndrome there is an association with benign and malignant ovarian neoplasms, in particular granulosa cell tumours affecting the young reproductive age group (Christian, McLoughlin, Cathcart & Eisenberg, 1964, Dozois et af. 1970, Christian 1971.…”

Section: Discussionmentioning

confidence: 99%

Peutz‐Jeghers syndrome with pseudoinvasion of hamartomatous polyps and multiple epithelial neoplasms

Bolwell

James

1979

Histopathology

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The risk of malignant change developing in the hamartomatous polyps in Peutz-Jeghers syndrome is widely held to be negligible. However an association with tumours of the upper gastro-intestinal tract, ovary and other diverse multiple neoplasms is now recognized. Previously reported cases of malignant change in Peutz--Jeghers polyps may represent 'pseudoinvasion' and we report such a case. This was associated with carcinomas of the bile ducts, left tonsil and a papillary adenoma of the pancreatic duct. It lends further support to the view that there may be a genetic predisposition to the development of neoplasms in this condition. Pathologists and clinicians must be aware of this entity of 'pseudoinvasion' in order to avoid unnecessarily radical surgery in Peutz-Jeghers syndrome.

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Peutz-Jeghers Syndrome Associated With Functioning Ovarian Tumor

Cited by 36 publications

References 8 publications

Hamartomatous Polyposis Syndromes

Hamartomatous Polyposis Syndromes

LKB1 Somatic Mutations in Sporadic Tumors

Peutz‐Jeghers syndrome with pseudoinvasion of hamartomatous polyps and multiple epithelial neoplasms

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