Primary hyperparathyroidism as first manifestation in multiple endocrine neoplasia type 2A: an international multicenter study.

Multiple endocrine neoplasia type 2A (MEN 2A) is a rare syndrome caused by RET germline mutations and has been associated with primary hyperparathyroidism (PHPT) in up to 30% of cases. Recommendations on RET screening in patients with apparently sporadic PHPT are unclear. We aimed to estimate the prevalence of cases presenting with PHPT as first manifestation among MEN 2A index cases and to characterize the former cases. An international retrospective multicenter study of 1085 MEN 2A index cases. Experts from MEN 2 centers all over the world were invited to participate. A total of 19 centers in 17 different countries provided registry data of index cases followed from 1974 to 2017. Ten cases presented with PHPT as their first manifestation of MEN 2A, yielding a prevalence of 0.9% (95% CI: 0.4-1.6). 9/10 cases were diagnosed with medullary thyroid carcinoma (MTC) in relation to parathyroid surgery and 1/10 was diagnosed 15 years after parathyroid surgery. 7/9 cases with full TNM data were node-positive at MTC diagnosis. Our data suggest that the prevalence of MEN 2A index cases that present with PHPT as their first manifestation is very low. The majority of index cases presenting with PHPT as first manifestation have synchronous MTC and are often node-positive. Thus, our observations suggest that not performing RET mutation analysis in patients with apparently sporadic PHPT would result in an extremely low false-negative rate, if no other MEN 2A component, specifically MTC, are found during work-up or resection of PHPT.S D received a national grant (AZV 16-32665A).S

Diagnostic and Therapeutic Approaches to Thyroid Nodules in Turkey

Objective: This study aimed to evaluate the approaches to thyroid nodules adopted by various specialists and centers in Turkey. Material and Methods: The questionnaire designed by Ralf Paschke for International Thyroid Congress (2010) was provided to 400 (264 general surgeons, 58 endocrine surgeons, and 78 endocrinologists) physicians from Turkey. Education and research hospitals (n=110), state hospitals (n=84), university hospitals (n=122), and private hospitals (n=84) were included. An index case was provided and questions regarding diagnostic and therapeutic/follow-up strategies were asked. Results: The index case was a 35-year-old man presented with swallowing discomfort. His thyroid-stimulating hormone level was 0.5 mIU/L. Thyroid ultrasonography (US) revealed a 13 mm right nodule (RN) and an 18 mm left nodule (LN). The most frequently queried information by physicians about the case was the result of fine needle aspiration biopsy (FNAB) (38.5%) and scintigraphy+FNAB (25.5%). Routine calcitonin measurement was advised by 33.5% of specialists. US and scintigraphy were detailed as; showed intranodular flow, microcalcification in the solid, and normoactive RN and solid hypoechoic, and hypoactive for the LN. FNAB was recommended by 68.5% of specialists for both nodules. Surgery without FNAB was recommended by 9% (n=36) of specialists, mainly by general surgeons (n=32). Specialists from private hospitals recommended surgical management for benign nodules, more frequently than state and university hospitals (p<0.01). Conclusion: Not only invasive and noninvasive diagnostic tests but also the treatment and follow-up strategies varied among general surgeons, endocrine surgeons, and endocrinologists in Turkey. The surgical approach for benign nodules was more frequently preferred in private hospitals

Comparison of Early Total Thyroidectomy with Antithyroid Treatment in Patients with Moderate-Severe Graves' Orbitopathy: A Randomized Prospective Trial

Background: The optimal therapeutic choice for Graves' hyperthyroidism in the presence of moderate-severe Graves' orbitopathy (GO) remains controversial. Objectives: We aimed to compare GO course in patients with moderate-severe GO treated with early total thyroidectomy (TTx) versus antithyroid drug (ATD) regimens, in a prospective, randomized manner. Methods: Forty-two patients with moderate-severe GO were enrolled. A total of 4.5 g of pulse corticosteroids were given intravenously to all patients before randomization. Patients in the first group were given TTx, whereas patients in the second group were treated with ATDs. TSH was kept between 0.4 and 1 mIU/l. The clinical course of GO was evaluated with proptosis, lid aperture, clinical activity score (CAS), and diplopia. Results: Eighteen and 24 patients were randomized to the TTx and ATD groups, respectively. Thyroid autoantibodies decreased significantly, and there were significant improvements in proptosis, lid aperture, and CAS in the TTx group. While in the ATD group the decrement in thyroid autoantibodies was not significant, there were significant improvements in proptosis and CAS. When the TTx group was compared with the ATD group, anti-TPO, anti-Tg, and TSH-receptor antibodies were significantly decreased in the TTx group (p < 0.01), but there was no significant difference with respect to proptosis, lid aperture, CAS, and diplopia between the two groups during a median (min.-max.) follow-up period of 60 months (36-72). Conclusion: Although no definitive conclusions could be drawn from the study, mainly due to limited power, early TTx and the ATD treatment regimens, followed by intravenous pulse corticosteroid therapy, seemed to be equally effective on the course of GO in this relatively small group of patients with moderate-severe GO during a median (min.-max.) follow-up period of 60 months (36-72). (C) 2016 European Thyroid Association Published by S. Karger AG, Base

Comparison Of Early Total Thyroidectomy With Antithyroid Treatment In Patients With Moderate-Severe Graves' Orbitopathy: A Randomized Prospective Trial

Background: The optimal therapeutic choice for Graves' hyperthyroidism in the presence of moderate-severe Graves' orbitopathy (GO) remains controversial. Objectives: We aimed to compare GO course in patients with moderate-severe GO treated with early total thyroidectomy (TTx) versus antithyroid drug (ATD) regimens, in a prospective, randomized manner. Methods: Forty-two patients with moderate-severe GO were enrolled. A total of 4.5 g of pulse corticosteroids were given intravenously to all patients before randomization. Patients in the first group were given TTx, whereas patients in the second group were treated with ATDs. TSH was kept between 0.4 and 1 mIU/l. The clinical course of GO was evaluated with proptosis, lid aperture, clinical activity score (CAS), and diplopia. Results: Eighteen and 24 patients were randomized to the TTx and ATD groups, respectively. Thyroid autoantibodies decreased significantly, and there were significant improvements in proptosis, lid aperture, and CAS in the TTx group. While in the ATD group the decrement in thyroid autoantibodies was not significant, there were significant improvements in proptosis and CAS. When the TTx group was compared with the ATD group, anti-TPO, anti-Tg, and TSH-receptor antibodies were significantly decreased in the TTx group (p < 0.01), but there was no significant difference with respect to proptosis, lid aperture, CAS, and diplopia between the two groups during a median (min.-max.) follow-up period of 60 months (36-72). Conclusion: Although no definitive conclusions could be drawn from the study, mainly due to limited power, early TTx and the ATD treatment regimens, followed by intravenous pulse corticosteroid therapy, seemed to be equally effective on the course of GO in this relatively small group of patients with moderate-severe GO during a median (min.-max.) follow-up period of 60 months (36-72). (C) 2016 European Thyroid Association Published by S. Karger AG, BaselWo