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

Levy, Rachel A.; Hui, Vanessa W.; Sood, Rupa; Fish, Stephanie; Markowitz, Arnold J.; Wong, Richard J.; Guillem, José G.

doi:10.1007/s10689-014-9732-5

Cited by 24 publications

(22 citation statements)

References 23 publications

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“…However, the authors did not present the prevalence of CMV-PTC. The third large series of CMV-PTC was reported by Levy and coworkers in Memorial Sloan Kettering Cancer Centre in the USA in 2014 (Levy et al 2014). The authors identified 11 patients with CMV-PTC of 6901 patients with papillary thyroid carcinoma in the period from 2001 to 2012.…”

Section: Epidemiologymentioning

confidence: 97%

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

Lam¹,

Saremi²

2017

Endocrine-Related Cancer

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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.

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Section: Epidemiologymentioning

confidence: 97%

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

Lam¹,

Saremi²

2017

Endocrine-Related Cancer

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“…Alternatively, histologically similar morular structures have been reported in the cribriform-morular variant of papillary thyroid carcinoma (CMVPTC) [32]. Although sporadic examples of CMVPTC have been reported, this rare histopathologic subtype is strongly associated with FAP syndrome [33,34]. Specifically, 1-2 % of the patients with FAP develop papillary thyroid carcinoma and 20-40 % of these tumors are diagnosed as CMVPTC [35,36].…”

Section: Discussionmentioning

confidence: 93%

“…Specifically, 1-2 % of the patients with FAP develop papillary thyroid carcinoma and 20-40 % of these tumors are diagnosed as CMVPTC [35,36]. The presence of a cribriform-morular architectural pattern in thyroid cancer has been considered an indicator for occult FAP necessitating appropriate patient evaluation [34,37]. It appears possible that germline or acquired mutations in genes of the Wnt/b-catenin pathway, such as APC, may correlate with a morular phenotype in a variety of benign or malignant neoplasms arising in the thyroid gland or odontogenic tissues.…”

Section: Discussionmentioning

confidence: 99%

Orthokeratinized Odontogenic Cyst with an Associated Keratocystic Odontogenic Tumor Component and Ghost Cell Keratinization and Calcifications in a Patient with Gardner Syndrome

Argyris

Koutlas

2016

Head and Neck Pathol

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Gardner syndrome (GS) is caused by mutations in the APC and besides adenomatous colorectal polyps includes such manifestations as osteomas, epidermoid cysts (ECs) and occasionally multiple pilomatricomas. More than 50 % of ECs in patients with GS exhibit pilomatricoma-like ghost cell keratinization. The latter may be explained by the fact that the development of both GS and pilomatricoma is driven by activation of the Wnt/b-catenin signaling pathway. A 62-year-old, Caucasian male with history of GS presented with a unilocular, mixed radiopaque/radiolucent mandibular lesion causing divergence and external root resorption of involved teeth. Histopathologically, the lesion was composed of two cystic components, an orthokeratinized odontogenic cyst (OOC) and a smaller one with characteristics of keratocystic odontogenic tumor (KCOT) featuring, focally, ghost cells and an epithelial morule-like structure. Dystrophic calcifications essentially similar to those seen in pilomatricomas were observed in the fibrous connective tissue wall. The KCOT and OOC epithelia revealed strong and diffuse cytokeratin (AE1/AE3) and b-catenin immunoreactivity. CD10 positive immunostaining was seen in the keratin and superficial spinous cell layers in both OOC and KCOT. The intraepithelial and mural ghost cells showed a cytokeratin (?), b-catenin and CD10 (-) immunophenotype. The diagnosis of OOC with ghost cell calcifications in association with KCOT was rendered. The patient was lost to follow-up. Although a coincidental co-existence cannot be excluded, ghost cell calcifications mimicking pilomatricoma-like changes in an unusual odontogenic cyst combining OOC and KCOT features as seen in this patient with GS may be explained by the common molecular mechanisms underlying the pathogenesis of cutaneous pilomatricomas and GS.

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“…Recent data have shown that FAP patients are usually diagnosed with TC at a younger age than the general population [27], in which the median age of diagnosis is 50 years (range 45–54) [4]. Similarly, in PR, the median age of diagnosis for TC in the general population in males and females is 54 and 50 years, respectively [5].…”

Section: Discussionmentioning

confidence: 99%

Risk of thyroid cancer among Caribbean Hispanic patients with familial adenomatous polyposis

et al. 2015

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Background & Aims Familial adenomatous polyposis (FAP) is an inherited form of colorectal cancer (CRC) characterized by hundreds of adenomatous polyps in the colon and rectum. FAP is also associated with thyroid cancer (TC), but the lifetime risk is still unclear. This study reports the standardized incidence ratio of TC in Hispanic FAP patients. Methods TC incidence rates in patients with FAP were compared with the general population through direct database linkage from the Puerto Rico Central Cancer Registry (PRCCR) and the Puerto Rico Familial Colorectal Cancer Registry (PURIFICAR) between the periods of January 1, 2006 to December 31, 2013. The study population consisted of 51 Hispanic patients with FAP and 3,239 with TC from the general population. The Standardized incidence ratio (SIR) was calculated using the Indirect Method, defined as observed TC incidence among patients with FAP in PURIFICAR’s cohort (2006–2013) divided by the expected TC incidence based on the PR population rates (2006–2010). SIR values were estimated by sex (male, female and overall). This study received IRB approval (protocol # A2210207). Results In Hispanic patients with FAP the standardized incidence ratio (SIR) (95% CI) for TC was 251.73 (51.91 – 735.65), with higher risk for females 461.18 (55.85–1665.94) than males 131.91 (3.34–734.95). Conclusions Hispanic FAP patients are at a high risk for TC compared to the general population. Our incidence rates (SIR) are higher than previous studies, suggesting that this community may be at a higher risk for TC than previously assumed. Implementation of clinical surveillance guidelines and regular ultrasound neck screening in Hispanic FAP patients is recommended.

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

Cited by 24 publications

References 23 publications

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

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

Orthokeratinized Odontogenic Cyst with an Associated Keratocystic Odontogenic Tumor Component and Ghost Cell Keratinization and Calcifications in a Patient with Gardner Syndrome

Risk of thyroid cancer among Caribbean Hispanic patients with familial adenomatous polyposis

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