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


Authors: Levy, R. A.; Hui, V. W.; Sood, R.; Fish, S.; Markowitz, A. J.; Wong, R. J.; Guillem, J. G.
Article Title: Cribriform-morular variant of papillary thyroid carcinoma: an indication to screen for occult FAP
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. © 2014, Springer Science+Business Media Dordrecht.
Keywords: adolescent; adult; middle aged; young adult; pathology; carcinoma; thyroid neoplasms; thyroid tumor; complication; familial adenomatous polyposis; hereditary colorectal cancer; adenomatous polyposis coli; colon polyposis; papillary thyroid carcinoma; cribriform-morular variant; humans; human; female
Journal Title: Familial Cancer
Volume: 13
Issue: 4
ISSN: 1389-9600
Publisher: Springer  
Date Published: 2014-12-01
Start Page: 547
End Page: 551
Language: English
DOI: 10.1007/s10689-014-9732-5
PUBMED: 24934245
PROVIDER: scopus
DOI/URL:
Notes: Export Date: 2 September 2015 -- Source: Scopus
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MSK Authors
  1. Arnold J Markowitz
    139 Markowitz
  2. Jose Guillem
    414 Guillem
  3. Richard J Wong
    420 Wong
  4. Stephanie Anne Fish
    25 Fish
  5. Rachel Allison Levy
    3 Levy
  6. Vanessa W Hui
    7 Hui
  7. Rupa   Sood
    4 Sood