Long-Term Quality of Life and Pregnancy Outcomes of Differentiated Thyroid Cancer Survivors Treated by Total Thyroidectomy and I 131

Introduction. Differentiated thyroid cancer (DTC) is rare and confers good prognosis. Long-term health related quality of life (HRQoL) and pregnancy outcomes are not well known in subjects treated during adolescence and young adulthood. Methods. Cross-sectional analysis of HRQoL and global self-esteem, using SF-36 and ISP-25 surveys, and of pregnancy outcomes in female survivors of DTC treated by total thyroidectomy and I131 before age of 25 years. Results. Forty-five of 61 patients (74%) responded to the survey. Cumulative I131 activity was ≤3.85 GBq in 18 subjects and >3.85 GBq in 27 subjects. Mean time from diagnosis was 7.6 ± 5.2 years for the group ≤ 3.85 GBq versus 16.9 ± 11.6 years for the group > 3.85 GBq (P<0.05). No significant alteration in long-term HRQoL and global self-esteem was observed. Thirty pregnancies after I131 were noted in patients from the group > 3.85 GBq and 10 in patients from the group ≤ 3.85 GBq. Frequency of miscarriages was of 17% (group > 3.85 GBq) and 10% (group ≤ 3.85 GBq) with 9 and 24 live births, respectively. No congenital malformations or first year mortality was noted. Conclusion. Long-term HRQoL, global self-esteem, and pregnancy outcomes are not affected in young female survivors of DTC

18F-FDOPA PET/CT Uptake Parameters Correlate with Catecholamine Secretion in Human Pheochromocytomas

International audienceBackground: 18 F-FDOPA positron emission tomography/ computed tomography (PET/CT) is a sensitive nuclear imaging technology for the diagnosis of pheochromocytomas (PHEO). However, its utility in determining predictive factors for the secretion of catecholamines remains poorly studied. Methods: Thirty-nine histologically confirmed PHEO were included in this retrospective single-center study. Patients underwent 18 F-FDOPA PET/CT before surgery, with an evaluation of several uptake parameters (standardized uptake values [SUV max and SUV mean ] and the metabolic burden [MB] calculated as follows: MB = SUV mean × tumor volume) and measurement of plasma and/or urinary metanephrine (MN), normetanephrine (NM), and chromogranin A. Thirty-five patients were screened for germline mutations in the RET, SDHx, and VHL genes. Once resected, primary cultures of 5 PHEO were used for real-time measurement of catechol-amine release by carbon fiber amperometry. Results: The MB of the PHEO positively correlated with 24-h urinary excre-tion of NM (r = 0.64, p < 0.0001), MN (r = 0.49, p = 0.002), combined MN and NM (r = 0.75, p < 0.0001), and eventually plasma free levels of NM (r = 0.55, p = 0.006). In the mutated patients (3 SDHD, 2 SDHB, 3 NF1, 1 VHL, and 3 RET), a similar correlation was observed between MB and 24-h urinary combined MN and NM (r = 0.86, p = 0.0012). For the first time, we demonstrate a positive correlation between the PHEO-to-liver SUV max ratio and the mean number of secretory granule fusion events of the corresponding PHEO cells revealed by amperometric spikes (p = 0.01). Conclusion: While the 18 F-FDOPA PET/CT MB of PHEO strongly correlates with the concentration of MN, amperometric recordings suggest that 18 F-FDOPA uptake could be enhanced by overactivity of cat-echolamine exocytosis

FACTS-international year 2

Summary Objectives:  To compare alendronate 70 mg once weekly (OW) with risedronate 35 mg OW with respect to change in bone mineral density (BMD), biochemical markers and upper gastrointestinal (UGI) tolerability over 24 months. Methods:  This was a 12-month extension to the Fosamax® Actonel® Comparison Trial international study (FACTS). Postmenopausal women with osteoporosis randomly assigned to either alendronate 70 mg OW or risedronate 35 mg OW for the 12-month base study continued taking the same double-blind study medication. Efficacy measurements were BMD at the hip trochanter, lumbar spine, total hip, and femoral neck and levels of four bone turnover markers at 24 months. The primary hypothesis was that alendronate would produce a greater mean per cent increase from baseline in hip trochanter BMD at 24 months. Results:  Trochanter BMD increased significantly from baseline to month 24 in both groups, with a significantly larger increase with alendronate: adjusted mean treatment difference of 1.50% (95% confidence interval: 0.74%, 2.26%; p < 0.001). Similar results were seen at all BMD sites. Significant geometric mean per cent decreases (p < 0.001) from baseline were seen for all four bone turnover markers in both groups, with significantly larger decreases (p < 0.001) with alendronate: adjusted mean treatment differences ranged from 8.9% to 25.3%. No significant differences were seen in incidence of UGI or other adverse events. Conclusions:  Alendronate 70 mg OW yielded significantly greater BMD gains and larger decreases in bone turnover marker levels than risedronate 35 mg OW over 24 months, with no difference in UGI tolerability

Long-term prognosis of patients with pediatric pheochromocytoma

A third of patients with paraganglial tumors, pheochromocytoma, and paraganglioma, carry germline mutations in one of the susceptibility genes, RET, VHL, NF1, SDHAF2, SDHA, SDHB, SDHC, SDHD, TMEM127, and MAX. Despite increasing importance, data for long-term prognosis are scarce in pediatric presentations. The European-American-Pheochromocytoma-Paraganglioma-Registry, with a total of 2001 patients with confirmed paraganglial tumors, was the platform for this study. Molecular genetic and phenotypic classification and assessment of gene-specific long-term outcome with second and/or malignant paraganglial tumors and life expectancy were performed in patients diagnosed at <18 years. Of 177 eligible registrants, 80% had mutations, 49% VHL, 15% SDHB, 10% SDHD, 4%NF1, and one patient each in RET, SDHA, and SDHC. A second primary paraganglial tumor developed in 38% with increasing frequency over time, reaching 50% at 30 years after initial diagnosis. Their prevalence was associated with hereditary disease (P=0.001), particularly in VHL and SDHD mutation carriers (VHL vs others, P=0.001 and SDHD vs others, P=0.042). A total of 16 (9%) patients with hereditary disease had malignant tumors, ten at initial diagnosis and another six during follow-up. The highest prevalence was associated with SDHB (SDHB vs others, P<0.001). Eight patients died (5%), all of whom had germline mutations. Mean life expectancy was 62 years with hereditary disease. Hereditary disease and the underlying germline mutation define the long-term prognosis of pediatric patients in terms of prevalence and time of second primaries, malignant transformation, and survival. Based on these data, gene-adjusted, specific surveillance guidelines can help effective preventive medicine.publishersversionPeer reviewe

Place de la chirurgie radioguidée dans la prise en charge des récidives et des métastases des cancers différenciés de la thyroïde

La chirurgie radioguidée est une technique qui permet au chirurgien d'identifier une lésion marquée en préopératoire par un isotope radioactif, visant à orienter le geste, mais aussi réduire la taille de l'incision, la durée de l'intervention et donc la morbidité. Deux types de sondes peropératoires peuvent être utilisées : avec cristal à scintillations ou semi-conducteur. Validée pour la détection du ganglion sentinelle dans les mélanomes et les cancers du sein, appliquée également pour la détection des adénomes parathyroïdiens et des ostéomes ostéoïdes, nous nous sommes intéressés à la place de la CRG dans les récidives et métastases des CDT. Notre étude a porté sur 5 patients traités par thyroïdectomie totale et irathérapie pour CDT et dont le suivi a révélé des récidives ou des métastases par un taux élevé de Tg, un BCT positif, voire une imagerie positive. Après administration de 100 mCi d'iode 131 chaque patient a bénéficié d'une scintigraphie cervico-thoracique suivie le lendemain d'une détection peropératoire pour l'ablation des lésions iodofixantes. Une scintigraphie postopératoire permet de vérifier la disparition des foyers. La sonde a aidé à localiser les lésions à l'exception d'1 cas où le foyer était peu visible à l'examen préopératoire et la sonde n'émettait pas de signal. L'imagerie comme le Pet-scan semble complémentaire de la scintigraphie dans 1 cas en améliorant la localisation d'une lésion cervicale. Les résultats obtenus à moyen terme dans notre série apparaissent meilleurs lorsque la détection peropératoire survient tôt dans la prise en charge. Le taux de rémission atteint 40%. Une CRG pourrait se discuter dès qu'un BCT se révèle positif mais avec son inconfort pour le patient et l'irradiation cumulée pouvant représenter un facteur limitant. Le petit nombre de patients inclus dans cette étude ne permet pas de préciser à quel moment de la prise en charge des récidives ou métastases de CDT se place la détection peropératoire mais aux vues des résultats un arbre décisionnel peut-être proposé.NANCY1-SCD Medecine (545472101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF