Cribriform-morular variant of papillary thyroid carcinoma: a distinctive type of thyroid cancer

Abstract

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 of APC gene are found in the patients with CMV-PTC associated with FAP. In addition, mutations in CTNNB1, RET/PTC rearrangement and PI3K3CA mutations have been reported. BRAF mutation 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.

The new 4th edition World Health Organization classification for thyroid tumors, Asian perspectives

“…Histologically, it is characterized by round squamoid structures called morules and colloid production is absent in the follicle lumen (Fig. a) . Cribriform‐morular variant of 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%) (Table ) …”

“…3a). 123,124,128,129 Cribriformmorular variant of 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%) ( Table 2). 129…”

Sclerosing mucoepidermoid carcinoma with eosinophilia of the thyroid: Case report of a rare lesion with novel genetic mutation

“…APC gene mutations have been observed in patients with familial adenomatous polyposis (FAP) who have papillary thyroid carcinoma . There is a strong correlation between cribriform‐morular variant papillary thyroid carcinoma and APC gene mutations …”

“…[25][26][27] There is a strong correlation between cribriform-morular variant papillary thyroid carcinoma and APC gene mutations. 28,29 This case report describes a case of a rare thyroid tumor, SMECE, that mimics a primary salivary gland neoplasm. Notable ultrasound, gross, cytologic, and histopathologic findings are reviewed.…”

Multi-omics Signatures and Translational Potential to Improve Thyroid Cancer Patient Outcome

“…These variants are associated with high rates of metastasis and thyroid cancer-related mortality [10,11], and thus are sometimes categorized as poorly differentiated thyroid cancer (PDTC). The cribriform-morular variant PTC is a characteristic variant associated with familial adenomatous polyposis (FAP) [12].…”