Cribriform-morular variant of papillary thyroid carcinoma: a distinctive type of thyroid cancer.
The aim of this systematic review is to study the features of cribriform-morular variant of papillary thyroid carcinoma (CMV-PTC) by analysing the 129 documented cases in the English literature. The disease occurred almost exclusively in women. The median age of presentation for CMV-PTC was 24 years. Slightly over half of the patients with CMV-PTC had familial adenomatous polyposis (FAP). CMV-PTC presented before the colonic manifestations in approximately half of the patients with FAP. Patients with FAP often have multifocal tumours in the thyroid. Microscopic examination of CMV-PTC revealed predominately cribriform and morular pattern of cancer cells with characteristic nuclear features of papillary thyroid carcinoma. Psammoma body is rare. On immunohistochemical studies, β-catenin is diffusely positive in CMV-PTC. The morular cells in CMV-PTC are strongly positive for CD10, bcl-2 and E-cadherin. Pre-operative diagnosis of CMV-PTC by fine-needle aspiration biopsy could be aided by cribriform architecture, epithelial morules and β-catenin immunostaining. Mutations ofAPCgene are found in the patients with CMV-PTC associated with FAP. In addition, mutations inCTNNB1,RET/PTCrearrangement andPI3K3CAmutations have been reported.BRAFmutation is negative in all CMV-PTC tested. Compared to conventional papillary thyroid carcinoma, CMV-PTC had a lower frequency of lymph node metastases at presentation (12%) and distant metastases (3%) as well as lower recurrence rates (8.5%) and patients' mortality rates (2%). To conclude, patients with CMV-PTC have distinctive clinical, pathological and molecular profiles when compared to conventional papillary thyroid carcinoma.
Cancer Molecular PathologySchool of Medicine and Menzies Health Institute Queensland, Griffith University, Gold Coast, Australia a.lam@griffith.edu.au.
Alfred King-Yin
AK
Lam
Cancer Molecular PathologySchool of Medicine and Menzies Health Institute Queensland, Griffith University, Gold Coast, Australia.
Nassim
N
Saremi
D002291:Carcinoma, Papillary / Q000145:classification* / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism; D006801:Humans; D011379:Prognosis; D000077273:Thyroid Cancer, Papillary; D013964:Thyroid Neoplasms / Q000145:classification* / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism
cribriform-morular; papillary carcinoma; review; thyroid