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01.12.2014 | Original Article | Ausgabe 4/2014

Familial Cancer 4/2014

Cribriform-morular variant of papillary thyroid carcinoma: an indication to screen for occult FAP

Zeitschrift:
Familial Cancer > Ausgabe 4/2014
Autoren:
Rachel A. Levy, Vanessa W. Hui, Rupa Sood, Stephanie Fish, Arnold J. Markowitz, Richard J. Wong, José G. Guillem
Wichtige Hinweise
Presented, in part, at the Annual Meeting of The Collaborative Group of the Americas on Inherited Colorectal Cancer, October 7–8, 2013, Anaheim, CA.

Abstract

Cribriform-morular variant (CMV) is a rare subtype of papillary thyroid carcinoma (PTC) that is associated with familial adenomatous polyposis (FAP). Given the high likelihood for multi-organ malignancies in FAP patients, this study explores the yield of diagnosing occult FAP among CMV-PTC patients. Institutional database was searched in order to identify patients with pathologically-confirmed CMV-PTC from 2000 to 2012. Medical records were reviewed, and clinical and pathological features were analyzed. Eleven cases of CMV were identified from 6,901 patients with PTC, for a prevalence of 0.16 %. All 11 patients were female. The median age at CMV-PTC diagnosis was 36 years (range 18–46). Two patients had pre-existing FAP at the time of PTC diagnosis. The other nine patients were referred for colonoscopy and/or genetic testing. Six patients underwent colonoscopy and one (17 %) was diagnosed with FAP based on polyposis phenotype and genetic testing. The mean age of patients at the time of CMV-PTC diagnosis was younger in the FAP group (23 years, range 18–34) than in the sporadic group (37 years, range 25–46). All three patients with FAP-associated CMV-PTC had multicentric tumors, while all five sporadic patients did not. Our study found that approximately one-sixth of patients with CMV-PTC may have occult FAP. Patients with FAP-associated CMV-PTC appear to be younger and more likely to have multicentric tumors than those with sporadic CMV-PTC. Due to the increased risk of malignancy in patients with FAP, patients with CMV-PTC should be referred for colonoscopy and/or genetic evaluation for FAP.

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