TITLE

Colonic Polyposis and Neoplasia in Cowden Syndrome

AUTHOR(S)
STANICH, PETER P.; OWENS, VICTORIA L.; SWEETSER, SETH; KHAMBATTA, SHEREZADE; SMYRK, THOMAS C.; RICHARDSON, RONALD L.; GOETZ, MATTHEW P.; PATNAIK, MRINAL M.
PUB. DATE
June 2011
SOURCE
Mayo Clinic Proceedings;Jun2011, Vol. 86 Issue 6, p489
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
OBJECTIVE: To identify and describe the frequency, hlstologlc features, and clinical outcome of colon polyposis and neoplasla In Cowden syndrome-a rare familial hamartoma tumor syndrome associated with mutations In the PTEN gene. PATIENTS AND METHODS: Patients with a clinical diagnosis of PTEN hamartoma tumor syndrome-Cowden phenotype were ret. rospectlvely identified and studied. Only those who underwent colonoscopy or colon pathologlc interpretation were included In the final analysis. RESULTS: From 1994 to 2009, 13 patients met study Inclusion criteria. Of the 10 patients who underwent colonoscopy, 9 (90%; 95% confidence Interval [CI], 57%-100%) had polyps, and 7(70%; 95% CI, 39%-90%) were estimated to have more than 50 polyps. Pathologic findings of the colon were reviewed in 11 patients, and the spectrum of tumors included hamartomatous, inflammatory, adonomatous, ganglioneuromatous, hyperplastic, and juvenile polyps Of the 13 patients, 2(15%; 95% CI, 3%-43%) had left-sided adenocarcinoma without microsatellite instability. Five (38%) of the 13 patients underwent colectomy secondary to polyp dysplasla. CONCLUSION: Patients with Cowden syndrome have a heavy colon polyp burden with a wide pathologic spectrum, both benign and malignant. The colon polyposls results in a previously unreported morbidity with a high colectomy rate.
ACCESSION #
62094793

 

Related Articles

  • hamartoma.  // Taber's Cyclopedic Medical Dictionary (2009);2009, Issue 21, p1003 

    An encyclopedia entry for "hamartoma," which refers to a tumor from new growth of normal tissues, as well as an entry for "multiple hamartoma," is presented.

  • RE: Cowden Syndrome and PTEN Hamartoma Tumor Syndrome: Systematic Review and Revised Diagnostic Criteria. MIN-HAN TAN; CHARIS ENG // JNCI: Journal of the National Cancer Institute;Jun2014, Vol. 106 Issue 6, p1 

    A letter to the editor is presented in response to the article "Cowden Syndrome and the PTEN Hamartoma Tumor Syndrome: Systematic Review and Revised Diagnostic Criteria," by R. Pilarski and colleagues published in a 2013 issue.

  • RE: Cowden Syndrome and PTEN Hamartoma Tumor Syndrome: Systematic Review and Revised Diagnostic Criteria. PILARSKI, ROBERT; BURT, RANDALL; KOHLMAN, WENDY; PHO, LANA; SHANNON, KRISTEN M.; SWISHER, ELIZABETH // JNCI: Journal of the National Cancer Institute;Jun2014, Vol. 106 Issue 6, p1 

    A response from the authors of the article "Revised Diagnostic Criteria for Cowden Syndrome and the PTEn Hamartoma Tumor Syndrome" is presented.

  • Testicular Hamartomas and Epididymal Tumor in a Cowden Disease: A Case Report. Rasalkar, Darshana D.; Paunipagar, Bhawan K. // Case Reports in Medicine;2010, p1 

    Testicular hamartomas (TH) is a benign condition. An association of TH with Cowden disease (CD) is known. Ultrasound features of hamartomas are often diagnostic. We present a case of Cowden disease with TH and an epididymal tumor. Imaging features of TH and its differentials has been discussed....

  • PTEN loss in the continuum of common cancers, rare syndromes and mouse models. Hollander, M. Christine; Blumenthal, Gideon M.; Dennis, Phillip A. // Nature Reviews Cancer;Apr2011, Vol. 11 Issue 4, p289 

    PTEN is among the most frequently inactivated tumour suppressor genes in sporadic cancer. PTEN has dual protein and lipid phosphatase activity, and its tumour suppressor activity is dependent on its lipid phosphatase activity, which negatively regulates the PI3K-AKT-mTOR pathway. Germline...

  • Leiomyomatous hamartoma of the incisive papilla. Corrêa, Luciana; Lotufo, Mônica; Martins, Marília Trierveiler; Sugaya, Norberto; de Sousa, Suzana Cantanhede Orsini Machado // Journal of Clinical Pediatric Dentistry;Winter2001, Vol. 25 Issue 2, p157 

    A case of unusual hamartoma in a six-year-old otherwise healthy Brazilian girl is reported, with emphasis on histological and immunohistochemical features. A mass observed in the incisive papilla was detected whose appearance was similar to congenital epulis or fibroma. Histological findings...

  • Laparoscopic Excision of a Symptomatic Brunneroma. Carboni, Fabio; Valle, Mario; Garofalo, Alfredo; Mariantonia, Carosi // American Surgeon;Feb2011, Vol. 77 Issue 2, pE37 

    The article presents a case study of a 73 year old male patient who presented to physicians with a three month history of asthenia, epigastric pain and dysphagia. A discussion of diagnostic testing which was conducted on the patient, and resulted in him having to undergo a laparoscopic excision...

  • Cowden's disease, Cowden's syndrome.  // Taber's Cyclopedic Medical Dictionary (2009);2009, Issue 21, p538 

    An encyclopedia entry for "Cowden's disease, Cowden's syndrome," which refers to multiple hamartoma, is presented.

  • Hamartoma esplénico. Reporte de caso. Soto-Medina, Carlos Alberto; Mier-Escurra, Erik Antonio; Treviño-Garza, Francisco; Ripa-Galván, Paola // Cirugia y Cirujanos;may/jun2014, Vol. 82 Issue 3, p328 

    Background: Hamartoma is a slow-growing, rare mixed benign tumor. In general, it does not produce symptoms, so it is more commonly found as an incentaloma during autopsies or laparotomies. Incidence of splenic hamartomas is low, representing 0.001% of the general population. Clinical case: We...

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics