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Wells SA Jr, Pacini F, Robinson BG, Santoro M (2013) Multiple endocrine neoplasia type 2 and familial medullary thyroid carcinoma: an update.
Thyroid Off J Am Thyroid Assoc 25(6):567–610. Wells SA Jr, Asa SL, Dralle H et al (2015) Revised american thyroid association guidelines for the management of medullary thyroid carcinoma. SR of literature indicates that MEN2A-related CLA is more frequent in women and presents a high penetrance, being the second most frequent manifestation of the syndrome, preceded only by MTC.ĭavies L, Welch HG (2006) Increasing incidence of thyroid cancer in the United States, 1973–2002. The overall reported prevalence of medullary thyroid carcinoma, CLA, pheochromocytoma and hyperparathyroidism was 94, 51, 30 and 16 %, respectively. Most interesting, the standardized CLA prevalence was higher in women (2.3/1.0, P < 0.005).
#Cla 2a review skin#
The mean age reported by patients to initial skin lesion suggestive to CLA was 20 ± 13 years. The mean age of MEN2A diagnosis was 31 ± 17 years, with 77 % women. The literature search resulted in 20 publications that contributed with 25 MEN2A families and 214 individuals. The median age at MEN2A diagnosis in our cohort was 25 (13–41) years, 68 % were women and all harbored codon 634 RET mutations. Thirty-eight patients with MEN2A-related CLA followed at our institution were evaluated. Here, we describe 3 MEN2A-related CLA kindred and perform a systematic review (SR) of the literature on clinical, biochemical and molecular characteristics of MEN2A-related CLA patients. Multiple endocrine neoplasia type 2A (MEN2A) may be rarely associated with cutaneous lichen amyloidosis (CLA), a skin lesion located in the interescapular region.
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