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

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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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The authors declare no conflicts of interest associated with this manuscript.

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Authors and Affiliations

  1. Colorectal Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, Room C-1077, New York, NY, 10065, USA

    Rachel A. Levy, Vanessa W. Hui, Rupa Sood & José G. Guillem

  2. Medicine/Endocrinology Service, New York, NY, USA

    Stephanie Fish

  3. Medicine/Gastroenterology and Nutrition Service, New York, NY, USA

    Arnold J. Markowitz

  4. Surgery/Head and Neck Service, Memorial Sloan Kettering Cancer Center, New York, NY, USA

    Richard J. Wong

Authors
  1. Rachel A. Levy
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  2. Vanessa W. Hui
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  3. Rupa Sood
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  4. Stephanie Fish
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  5. Arnold J. Markowitz
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  6. Richard J. Wong
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  7. José G. Guillem
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Correspondence to José G. Guillem.

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Levy, R.A., Hui, V.W., Sood, R. et al. Cribriform-morular variant of papillary thyroid carcinoma: an indication to screen for occult FAP. Familial Cancer 13, 547–551 (2014). https://doi.org/10.1007/s10689-014-9732-5

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  • DOI: https://doi.org/10.1007/s10689-014-9732-5

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