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Pneumocystis pneumonia (PcP) is an opportunistic infection occurring in immunocompromised patients. Cushing's syndrome (CS) impairs the immune system, and several authors have reported PcP in patients with CS. The present study aimed to characterize PcP occurring in a CS context and its management in French tertiary centers, in order to highlight the similarities in clinical presentation and treatment according to whether prophylaxis is implemented or not.This was a multicenter retrospective study conducted in several French University Hospitals and Cancer Centers. Patients with PcP and confirmed CS regardless of etiology were included. We excluded patients with other known causes of acquired immunodeficiency with increased risk of PcP.Twenty-five patients were included. CS etiology was neoplastic in 84.0% of cases. CS clinical presentation associated predominant catabolic signs (76.0%), hypokalemia (91.7%) and lymphopenia (89.5%). CS was intense in most patients, with mean plasma cortisol levels at diagnosis of 2.424±1.102nmol/L and urinary free cortisol10× the upper limit of normal in 85.0%. In all patients, PcP onset followed introduction of cortisol blockers, at a median 5.5 days. Patients were treated with 1 to 3 cortisol blockers, mainly metyrapone (88%), which significatively lowered plasma cortisol levels to 667±541nmol/L at the onset of PcP (P0.001). PcP occurred in 7 patients despite prophylaxis. Finally, 60.0% patients were admitted to intensive care, and 20.0% died of PcP.High mortality in patients with PcP implies that clinicians should be better informed about this rare infectious complication. Prophylaxis remains controversial, requiring comparative studies.

Objective After temozolomide failure, no evidence-based treatment is available for pituitary carcinomas (PCs) and aggressive pituitary tumors (APTs). To date, only 12 cases treated with immune-checkpoint inhibitors (ICIs) have been published, showing encouraging efficacy. Predictive factors of response are lacking. Here, we aimed to assess the real-life efficacy and predictors of response to ICIs in PCs and APTs. Design and methods This study is a multicentric, retrospective, observational cohort study, including all PCs and APTs treated with ICIs in France up to March 2022. PD-L1 immunohistochemistry and CD8+ T cell infiltration were evaluated centrally. Results Six PCs (four corticotroph and two lactotroph) and nine APTs (five corticotroph and four lactotroph) were included. The real-life efficacy of ICIs was lower than previously published data. Three corticotroph tumors (33.3%) showed partial response, one (11.1%) stable disease, while five (55.6%) progressed. One lactotroph tumor (16.7%) showed partial response, one (16.7%) stable disease, while four (66.7%) progressed. PCs responded far better than APTs, with 4/6 PCs showing partial response compared to 0/9 APTs. Corticotroph tumors responded slightly better than lactotroph tumors. In the four responsive corticotroph tumors, PD-L1 staining was negative and CD8+ T cell infiltration attained a maximum of 1% in the tumor center. Conclusions Confirmation of the presence or absence of metastases is necessary before starting ICIs. After temozolomide failure, ICIs appear as a good therapeutic option for PCs, especially for corticotroph carcinomas. Negative PD-L1 staining and very low CD8+ T cell infiltration in the tumor center should not preclude ICI administration in corticotroph carcinomas. Significance statement This is the first study to assess the real-life efficacy of ICIs in pituitary carcinomas (PCs) and aggressive pituitary tumors. We also assessed potential predictors of response and are the first to assess the predictive value of CD8+ cell infiltration. We identified the tumor type as a major predictor, ICIs proving far more effective in treating PCs. Our study provides evidence that ICIs are a good option after temozolomide failure for PCs (four of six responded), especially for corticotroph carcinomas (three of four responded). We also provide evidence that negative PD-L1 staining and very low CD8+ cell infiltration in the tumor center should not preclude ICI administration in corticotroph carcinomas. Moreover, our findings point toward the need to systematically perform extension workup before starting ICIs.

Context Cortisol-lowering drugs may not restore a normal cortisol secretion in Cushing disease (CD). Objective This work aimed to assess the long-term cortisol exposure in medically treated CD patients using hair-cortisol (HF) and hair-cortisone (HE) measurement. Methods This multicenter prospective study included 3 groups of female patients: CushMed = 16 treated with a stable cortisol-lowering drug dosage and normal urinary free cortisol (UFC); CushSurg = 13 cured by pituitary surgery; CushBla = 15 receiving stable recommended doses of hydrocortisone following bilateral adrenalectomy. Patients were evaluated for 3 months with their usual treatments. Two late-night saliva and 24-hour urine samples were collected monthly in CushMed, and at study end in CushSurg and CushBla patients. A 3-cm hair sample was collected at study end from all patients. Main outcome measures included clinical score and centralized measurement of UFC, late-night salivary cortisol (LNSF), late-night salivary cortisone (LNSE), HE, HF. Results Despite having almost all UFCs normalized, CushMed patients exhibited increased HE as compared to CushSurg controls (P = .003). CushMed patients also had increased clinical score (P = .001), UFC (P = .03), LNSF, LNSE (P = .0001), and variability in the latter parameters (P = .004). CushBla patients had increased HF and HE, contrasting with LNSEs similar to CushSurg patients. Six of 15 CushMed patients exhibited increased HE concentrations and had increased antihypertensive drug dosage compared to CushMed patients with normal HE (P = .05). Conclusion Despite normalized UFCs, a subset of medically treated CD patients displays an altered circadian rhythm of serum cortisol. A single HE measurement identifies chronic mild persistent hypercortisolism and could replace multiple saliva analyzes to monitor medical treatments in CD patients once UFC is normalized.

Craniopharyngiomas are tumors located in the hypothalamic region which leads to obesity in about 50% of cases. Long-term efficacy and safety of bariatric surgery are lacking in this peculiar population. The aim of this study is to determine the 5-year weight loss and resolution of type 2 diabetes (T2D) after bariatric surgery in patients operated on craniopharyngioma who had developed hypothalamic obesity.This is a multicenter french retrospective case-control study. Subjects with craniopharyngioma (n = 23) who underwent sleeve gastrectomy (SG) (n = 9) or Roux-en-Y gastric bypass (RYGB) (n = 14) (median age 35 years [25;43] and BMI 44.2 kg/mTWL% after 1 and 5 years was lower in the craniopharyngioma group than in the control group: 23.1 [15.4; 31.1] (23/23) vs 31.4 [23.9; 35.3] at 1 year (p = 0.008) (46/46) and 17.8 [7.1; 21.9] (23/23) vs 26.2 [18.9; 33.9] at 5 years (p = 0.003) (46/46). After RYGB, TWL% was lower in the craniopharyngioma group compared to the control group (p 0.001) and comparable after SG both at 1 and 5 years. No difference between the two groups was observed in T2D remission rate and in early and late adverse events. No hormonal deficiency-related acute disease was reported.Bariatric surgery induced a significant weight loss in the craniopharyngioma group at 1 and 5 years, but less than in common obesity. SG may be more effective than RYGB but this remains to be demonstrated in a larger cohort.

International audience; Cushing's syndrome is defined by prolonged exposure to glucocorticoids, leading to excess morbidity and mortality. Diagnosis of this rare pathology is difficult due to the low specificity of the clinical signs, the variable severity of the clinical presentation, and the difficulties of interpretation associated with the diagnostic methods. The present consensus paper by 38 experts of the French Society of Endocrinology and the French Society of Pediatric Endocrinology and Diabetology aimed firstly to detail the circumstances suggesting diagnosis and the biologic diagnosis tools and their interpretation for positive diagnosis and for etiologic diagnosis according to ACTH-independent and -dependent mechanisms. Secondly, situations making diagnosis complex (pregnancy, intense hypercortisolism, fluctuating Cushing's syndrome, pediatric forms and genetically determined forms) were detailed. Lastly, methods of surveillance and diagnosis of recurrence were dealt with in the final section.

Context Prospective studies have demonstrated the efficacy of osilodrostat in Cushing disease. No study has evaluated osilodrostat in a series of patients with paraneoplastic Cushing syndrome/ectopic adrenocorticotropin syndrome (PNCS/EAS). Objective This work aimed to evaluate in France the real-world efficacy and safety of osilodrostat in patients with PNCS/EAS. Methods A total of 33 patients with PNCS/EAS with intense/severe hypercortisolism were involved in this retrospective, multicenter, real-world study. Patients received osilodrostat between May 2019 and March 2022 at a median initial dose (range) of 4 mg/day (1-60) and maximum dose, 20 mg/day (4-100), first under patient then cohort temporary authorizations and after marketing authorization. Regimens used titration (n = 6), block and replace (n = 16), or titration followed by block and replace (n = 11). Results In 11 patients receiving osilodrostat as first-line monotherapy, median 24-hour urinary free cortisol (24h-UFC) decreased dramatically (from 26 × upper limit of normal [ULN; 2.9-659] to 0.11 × ULN [0.08-14.9]; P < .001). In 9 of them, 24h-UFC normalization was achieved in 2 weeks (median). Thirteen additional patients were previously treated with classic steroidogenesis inhibitors but 10 of these 13 were not controlled. In these patients, osilodrostat monotherapy, used as second line, induced a significantly decreased of 24h-UFC (from 2.6 × ULN [1.1-144] to 0.22 × ULN [0.12-0.66]; P < .01). Nine additional patients received osilodrostat in combination with another anticortisolic drug, decreasing 24h-UFC from 11.8 × ULN (0.3-247) to 0.43 × ULN (0.33-2.4) (P < .01). In parallel, major clinical symptoms/comorbidities improved dramatically with improvement in blood pressure, hyperglycemia, and hypokalemia, allowing the discontinuation or dose reduction of patient treatments. Adrenal insufficiency (grade 3-4) was reported in 8 of 33 patients. Conclusion Osilodrostat is a rapidly efficient therapy for PNCS/EAS with severe/intense hypercortisolism. Osilodrostat was generally well tolerated; adrenal insufficiency was the main side effect.

Resume Objectifs La plupart des etudes retrouvent une majoration du risque de fractures vertebrales chez les patients acromegales, malgre une densite minerale osseuse normale. Nous avons etudie la prevalence des fractures vertebrales chez les patients acromegales de moins de 80 ans suivis au CHU de Nantes. Methode Etude transversale monocentrique realisee au CHU de Nantes de 1988 a 2018. Cinquante patients (18 femmes, 32 hommes) d’un âge median de 52,3 ans (27 -78 ans) ont ete inclus. Les fractures vertebrales etaient evaluees sur des radiographies conventionnelles des rachis lombaire et thoracique de face et de profil, selon la methode semi-quantitative de Genant. Nous avons egalement etudie les deformations vertebrales autres que les fractures, en utilisant 3 criteres : presence d’osteophytes, presence de pincement discal et aspect cuneiforme des vertebres. Nous avons analyse la densite minerale osseuse par absorptiometrie bi-photonique et le statut hormonal. Resultats Trois patients (6 %) presentaient une fracture vertebrale : une fracture de grade 1 et 2 fractures de grade 2 selon la classification de Genant, chez deux patients osteoporotiques et un patient osteopenique. Leurs deficits ante-hypophysaires etaient substitues. En considerant les deformations franches (osteophyte ou pincement discal ≥ grade 2 ou aspect cuneiforme), le rachis dorsal etait deforme chez 22 patients (44 %) et le rachis lombaire chez 21 patients (42 %). Conclusions Les patients acromegales presentaient une faible prevalence de fractures vertebrales, mais un nombre important de deformations vertebrales. Nous supposons que cette forte prevalence de deformations franches pourrait expliquer la forte prevalence de fractures vertebrales rapportee precedemment.

To determine efficacy and safety of thermal ablation (TA) for the local treatment of lung metastases of thyroid cancer. We retrospectively studied 47 patients from 10 centers treated by TA (radiofrequency, microwaves, and cryoablation) over 10 years. The endpoints were overall survival (OS), local efficacy, complications (CTCAE classification), and factors associated with survival. OS curves after first TA were built using the Kaplan–Meier method and compared with the log-rank test. A total of 107 lung metastases during 75 sessions were treated by radiofrequency (n = 56), microwaves (n = 9), and cryoablation (n = 10). Median follow-up time after TA was 5.2 years (0.2–13.3). OS was 93% at 2 years (95% confidence interval (CI): 86–94) and 79% at 3 years (95% CI: 66–91). On univariate and multivariate analysis with a Cox model, histology was the only significant factor for OS. OS at 3 years was 94% for follicular, oncocytic, or papillary follicular variant carcinomas, compared to 59% for papillary, medullary, insular or anaplastic carcinomas (P = 0.0001). The local control rate was 98.1% at 1 year and 94.8% at 2, 3, 4, and 5 years. Morbidity was low with no major complications (grade 4 and 5 CTCAE) and no complications in 29 of 75 sessions (38.7%). TA is a useful, safe and effective option for local treatment of lung metastases from thyroid carcinoma. Prolonged OS was obtained, especially for lung metastases from follicular, oncocytic, or papillary follicular variant carcinomas. Achieving disease control with TA delays the need for systemic treatment.

Objectives Patients with acromegaly appear to be at increased risk of vertebral fractures despite normal bone mineral density. We investigated the prevalence of vertebral fractures in a cohort of acromegalic patients under 80 years of age. Methods Monocentric cross-sectional study performed at Nantes University Hospital from 1988 to 2018. Fifty patients (18 females, 32 males) with a median age of 52.3 years (range: 27–78) were included. Radiological vertebral fractures were evaluated on conventional lumbar and thoracic spine radiographs using Genant's semiquantitative fracture assessment. We studied qualitative abnormalities of the spine using three criteria: osteophytes, disc-space narrowing and wedge-shaped vertebrae. We analysed bone mineral density and endocrine status. Results Three patients (6%) had a vertebral fracture: one grade 1 and two grade 2 according to Genant's assessment, with two osteoporotic and one osteopenic patients. They had no unsubstituted pituitary deficiency. Considering the frank deformations (osteophyte or disc narrowing ≥ grade 2 or wedge-shaped), the thoracic spine was deformed in 22 patients (44%) and the lumbar spine in 21 patients (42%). Conclusion Acromegalic patients had a low prevalence of vertebral fractures but had a significant amount of vertebral deformations. We speculate that this high prevalence of frank deformations could explain the previously reported high prevalence of vertebral fractures.

Context When an SDHx mutation is identified in a patient with a pheochromocytoma (PCC) or a paraganglioma (PGL), predictive genetic testing can detect mutation carriers that would benefit from screening protocols. Objective To define the tumor detection rate in a large cohort of asymptomatic SDHX mutation carriers. Design and Setting Retrospective multicentric study in 6 referral centers. Patients Between 2005 and 2019, 249 asymptomatic SDHx (171 SDHB, 31 SDHC, 47 SDHD) mutation carriers, with at least 1 imaging work-up were enrolled. Results Initial work-up, including anatomical (98% of subjects [97–100% according to center]) and/or functional imaging (67% [14–90%]) detected 48 tumors in 40 patients. After a negative initial work-up, 124 patients benefited from 1 to 9 subsequent follow-up assessments (mean: 1.9 per patient), with a median follow-up time of 5 (1–13) years. Anatomical (86% [49–100 %]) and/or functional imaging (36% [7–60 %]) identified 10 new tumors (mean size: 16 mm [4–50]) in 10 patients. Altogether, 58 tumors (55 paraganglioma [PGL], including 45 head and neck PGL, 2 pheochromocytoma [PCC], 1 gastrointestinal stromal tumor [GIST]), were detected in 50 patients (22 [13%] SDHB, 1 [3.2%] SDHC, and 27 [57%] SDHD), with a median age of 41 years old [11–86], 76% without catecholamine secretion and 80% during initial imaging work-up. Conclusions Imaging screening enabled detection of tumors in 20% of asymptomatic SDHx mutation carriers, with a higher detection rate in SDHD (57%) than in SDHB (13%) and SDHC (3%) mutation carriers, arguing for a gene-by-gene approach. Prospective studies using well-defined protocols are needed to obtain strong and useful data.

International audience; Le nombre d’interventions de chirurgie bariatrique a été multiplié par 4 ces 10 dernières années en raison de l’épidémie des cas d’obésité, qui a triplé dans la population générale lors des quatre dernières décennies. De façon concomitante, le nombre de patients obèses ou ayant été opérés d’une chirurgie bariatrique candidats à une chirurgie thyroïdienne a donc considérablement augmenté. La proportion des patients obèses représente actuellement 10 à 20 % des patients thyroïdectomisés dans les cohortes françaises. De plus, la fréquence des pathologies thyroïdiennes bénignes et malignes semble être influencée par l’obésité, unhazard ratio d’environ 1,8 pour les cancers thyroïdiens ayant été mis en évidence chez la femme obèse. Si l’obésité ne semble pas influencer la morbidité postopératoire spécifique de la thyroïdectomie, un antécédent de chirurgie bariatrique malabsorptive doit inciter à la plus grande prudence en cas de chirurgie thyroïdienne, car il expose à un risque important d’hypocalcémie sévère (plus de 60 % des cas dans certaines études) nécessitant une supplémentation intraveineuse dans environ 20 % des cas. Dans cette dernière situation, une supplémentation vitamino-calcique périopératoire est indispensable. Par ailleurs, les doses de lévothyroxine à visée substitutive devraient aussi être adaptées chez les patients obèses, calculées non pas à partir du poids réel, mais à partir du poids idéal. L’objectif de cette revue est de discuter l’influence de l’obésité sur l’évolution des pathologies nodulaires et néoplasiques thyroïdiennes, sur la morbidité de la chirurgie thyroïdienne ainsi que sur les traitements médicamenteux postopératoires. L’impact de la chirurgie bariatrique sur ces différentes étapes est également discuté.

Resume La decouverte d’une masse surrenalienne est une situation de plus en plus frequente. Si leur decouverte se fait parfois a l’occasion de signes cliniques, la plupart des tumeurs surrenaliennes sont des incidentalomes, decouverts sur un scanner ou une IRM realisee pour une indication non surrenalienne. Cependant, toute masse retroperitoneale supra-renale n’est pas toujours d’origine surrenalienne et peut ainsi etre d’origine extra-surrenalienne. Le pourcentage de lesions operees et attribuees a tort a une origine surrenalienne representerait environ 3,5 % des surrenalectomies. Ces diagnostics differentiels incluent principalement des tumeurs retroperitoneales (lymphomes, sarcomes, tumeurs neurogeniques, tumeurs germinales), malignes dans 2 cas sur 3, plus rarement des anomalies vasculaires ou malformatives, et la plupart du temps situes a gauche en raison de la localisation adjacente d’une plus grande variete de structures anatomiques. Si la surrenalectomie unilaterale est source de peu de morbidite, la meconnaissance de ces diagnostics peut entrainer une prise en charge inadaptee : chirurgie trop conservatrice en cas de sarcome par exemple. Certaines lesions ont une presentation caracteristique a l’imagerie (lymphangiome kystique, angiomyolipome par exemple). Dans d’autres cas, les dosages hormonaux sont indispensables, et d’autres examens (IRM, ponction-biopsie, tomographie a emissions de positons au 18-Fluorodeoxyglucose) peuvent corriger le diagnostic. C’est a l’issue de cette demarche diagnostique qu’une prise en charge adaptee, chirurgicale ou non, peut etre realisee. L’objet de cette revue est d’exposer les principaux diagnostics de masses supra renales pouvant etre confondues avec d’authentiques masses surrenaliennes, de preciser leurs caracteristiques ainsi que leur prise en charge therapeutique.

Persistent primary hyperparathyroidism (PHPT) occurs in 2.5% to 15% of cases after parathyroidectomy. Few studies have evaluated the best pre-reoperative imaging approaches for persistent sporadic PHPT. This retrospective multicenter study aimed to evaluate the benefit of a second pre-reoperative 99mTc-methoxy-isobutyl-isonitrile (MIBI) scintigraphy for patients with persistent PHPT who had a 99mTc-MIBI before their initial surgery. The study enrolled 50 patients with persistent sporadic PHPT who had reoperation between 2006 and 2016 in three French University Hospitals (Angers, Nantes, and La Pitie Salpetriere-Paris). Preoperative 99mTc-MIBI scan was performed before each operation. After the reoperation, 42 patients (84%) were cured. By the second 99mTc-MIBI, 31 patients (62%) had a removed gland identified. A new pathologic gland was identified by a second 99mTc-MIBI in 25 patients (50%), and this imaging permitted correction of an initial surgical error in six patients (12%). A second 99mTc-MIBI showed a sensitivity of 63%, a specificity of 89%, a positive predictive value (PPV) of 78%, and a negative predictive value (NPV) of 80%. A concordant second 99mTc-MIBI and ultrasonography (17 patients) showed a sensitivity of 70%, a specificity of 81%, a PPV of 70%, and an NPV of 81%. Performing a second 99mTc-MIBI scan permitted 62% of the persistent PHPT patients to be cured, allowing identification of new pathologic glands in 50% of the cases and correction of an initial surgical error in 12% of the cases, with high specificity and PPV. These results reinforce the fact that a second 99mTc-MIBI scan should be performed at first intention before reoperation of patients with persistent PHPT, regardless of the result from the initial 99mTc-MIBI scan.

Context Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors explained by germline or somatic mutations in about 70% of cases. Patients with SDHB mutations are at high risk of developing metastatic disease, yet no reliable tumor biomarkers are available to predict tumor aggressiveness. Objective We aimed at identifying long noncoding RNAs (lncRNAs) specific for PPGL molecular groups and metastatic progression. Design and Methods To analyze the expression of lncRNAs, we used a mining approach of transcriptome data from a well-characterized series of 187 tumor tissues. Clustering consensus analysis was performed to determine a lncRNA-based classification, and informative transcripts were validated in an independent series of 51 PPGLs. The expression of metastasis-related lncRNAs was confirmed by RT-qPCR. Receiver operating characteristic (ROC) curve analysis was used to estimate the predictive accuracy of potential markers. Main Outcome Measure Univariate/multivariate and metastasis-free survival (MFS) analyses were carried out for the assessment of risk factors and clinical outcomes. Results Four lncRNA-based subtypes strongly correlated with mRNA expression clusters (chi-square P-values from 1.38 × 10–32 to 1.07 × 10–67). We identified one putative lncRNA (GenBank: BC063866) that accurately discriminates metastatic from benign tumors in patients with SDHx mutations (area under the curve 0.95; P = 4.59 × 10–05). Moreover, this transcript appeared as an independent risk factor associated with poor clinical outcome of SDHx carriers (log-rank test P = 2.29 × 10–05). Conclusion Our findings extend the spectrum of transcriptional dysregulations in PPGL to lncRNAs and provide a novel biomarker that could be useful to identify potentially metastatic tumors in patients carrying SDHx mutations.

BACKGROUND Malignant pheochromocytoma and paraganglioma (MPP) are characterized by prognostic heterogeneity. Our objective was to look for prognostic parameters of overall survival (OS) in MPP patients. PATIENTS AND METHODS Retrospective multicenter study of MPP characterized by a neck-thoraco-abdomino-pelvic CT or MRI at the time of malignancy diagnosis in European centers between 1998 and 2010. RESULTS One hundred sixty-nine patients from 18 European centers were included. Main characteristics of patients with MPP were: primary pheochromocytoma in 53% of patients; tumor- or hormone-related symptoms in 57% or 58% of cases; positive plasma or urine hormones in 81% of patients; identification of a mutation in SDHB in 42% of cases. Metastatic sites included bone (64%), lymph node (40%), lung (29%), and liver (26%); mean time between initial and malignancy diagnosis was 43 months (range, 0 to 614). Median follow-up was 68 months and median survival 6.7 years. Using univariate analysis, better survival was associated with head and neck paraganglioma, age

Design Hypercortisolism during pregnancy is a risk factor for prematurity. Long-term exposure to hypercortisolism may lead to permanent comorbidities, such as hypertension or diabetes, even after remission. Our aim was to determine whether women with a history of Cushing’s disease (and being eu-, hypo- or hypercortisolic at the time of pregnancy) had the same risks of comorbidities, and especially prematurity, during pregnancy. Methods It was a retrospective multicentric study focusing on mothers with a history of Cushing’s disease or diagnosed during pregnancy, followed in French tertiary referral centers. We compared the outcomes of pregnancies depending on the cortisolic status at the time of pregnancy. Results A total of 60 patients (78 pregnancies including 21 with hypercortisolism, 32 with hypocortisolism and 25 in eucortisolism in 25) were evaluated. The overall rate of preterm birth was 24.3%, with a peak in women diagnosed during pregnancy (62.5%), a high risk in hypercortisolic (33%) and hypocortisolic (19.3%), and a low risk (8%) in eucortisolic women Gestational diabetes and hypertension were observed in 21% and 10.4% of the whole cohort, with a higher risk in hypercortisolic women. Cesarean delivery was performed in 33.7% of the cohort. Conclusions Being non-eucortisolic at the time of pregnancy increases the risk of prematurity and comorbidities compared to the general population. Women with a history of Cushing’s disease should thus be carefully monitored during pregnancy. The high rate of cesarean delivery emphasizes the fact that these pregnancies should always be considered at risk.

Objective Adrenal ganglioneuromas are rare, differentiated, neuroblastic tumors that originate from the peripheral sympathetic nervous system. Because of their rarity, information is limited, derived from small cases series. Our objective was to characterize this tumor and provide help for its management. Methods A retrospective multicenter analysis of adrenal ganglioneuromas from 20 French centers belonging to the COMETE network and one Belgian center. Results Among the 104 cases identified, 59.6% were women (n = 62/104), median age at diagnosis was 29 years, with 24 pediatric cases. 60.6% (n = 63/104) were incidentalomas. Ganglioneuromas were non-secreting tumors in 90.8% of cases (n = 89/98), whereas the preoperative hormonal evaluation was indeterminate for 9.2% of patients (n = 9/98). CT imaging, performed on 96 patients, revealed large tumors (median diameter of 50 mm) with a non-contrast density > 10 Hounsfield units in 98.1% (n = 52/53) and calcifications in 64.6% of cases (n = 31/48). Increased uptake on 123I-MIBG scintigraphy and 18F-FDG-PET/CT was observed in 26.7% (n = 8/30) and 42.2% (n = 19/45) of the tumors, respectively. All 104 patients underwent surgery. No recurrence was observed among the 42 patients who had an imaging follow-up (mean 29.6 months, median 18 months (4–156)). Conclusion Adrenal ganglioneuromas are large tumors, mostly nonfunctioning, without benign imaging features. Although the duration of follow-up was limited in our series, no recurrence was identified. A review of the literature confirms the absence of postoperative recurrence. Based on all available data, in the absence of special circumstances (genetic form, uncertain histological diagnosis), long-term follow-up is not necessary after complete surgery for patients with an adrenal ganglioneuroma.

Serum carcinoembryonic antigen (CEA) is a tumor marker especially used to follow a patient with colorectal cancer. However, it is non-specific and could be increased in several cancers and some benign conditions. We report the case of a 70-year-old man followed since 2014 for a left colon adenocarcinoma with the persistence of an increased CEA. There was no evidence of recurrence, but a right lobar thyroid nodule without a significantly increased uptake was incidentally discovered on the CT scan of 18F-fluorodeoxyglucose (18F-FDG) PET/CT. We suspected a medullary thyroid carcinoma (MTC) explaining the persistent elevation of CEA. Plasma calcitonin levels were 47 ng/L (N < 10). Fine needle aspiration cytology found atypia of undetermined significance and the patient was reluctant to undergo surgery without any further exploration. We performed a 18F-fluorodihydroxyphenylalanine (18F-FDOPA) PET/CT preoperatively which revealed a punctiform focus of the right thyroid lobe corresponding to a pT1aN1aMxR0 medullary thyroid carcinoma, histopathologically confirmed. This case highlights that despite the potential usefulness of 18F-FDG PET/CT in case of an unknown source of elevated CEA this imaging may be falsely negative as in the case of MTC and should lead to further explorations.

International audience; Androgen receptor gene (AR) mutations are responsible for androgen insensitivity syndrome (AIS) presenting with a clinical phenotype that ranges from gynaecomastia and/ or infertility in mild AIS (MAIS) to complete testicular feminisation in complete AIS. We report a novel AR gene mutation in two unrelated adult patients with MAIS and we studied its functional impact using 3D modelling. Patient 1, referred for infertility, presented with gynaecomastia, mild hypospadias and bilateral testicular hypotrophy contrasting with high testosterone levels, an elevated FSH, an elevated androgen sensitivity index (ASI) and oligoasthenoteratospermia. In vitro fertilisation and intracytoplasmic sperm injection resulted in a successful twin pregnancy. Patient 2 referred for a decrease in athletic performance had surgically treated gynaecomastia, oligoasthenospermia, high testosterone levels and an elevated ASI. Despite his impaired spermogram, he fathered two children without assisted reproductive technology. AR gene sequencing in the two patients revealed a common novel missense mutation, Ala699Thr, in exon 4 within the ligand-binding domain. 3D modelling studies showed that this mutation may impact dimer stability upon ligand binding or may affect allosteric changes upon dimerisation. This study illustrates the value of structural analysis for the functional study of mutations and expands the database of AR gene mutations.

A 61-year-old woman presenting with hyperthyroidism received 131I therapy for a toxic adenoma diagnosed by 123I scintigraphy. Six months later, the patient had a relapse of hyperthyroidism, and 123I scintigraphy showed a mirror image of the first scintigraphy: a high and diffuse uptake in the thyroid gland except for the previously treated nodule. Graves disease was confirmed by elevated thyrotropin receptor antibodies. The patient was cured by a second radioiodine therapy. Radioiodine-induced Graves disease may occur within 6 months of 131I treatment of toxic adenoma and can be treated with a second line of 131I.

The incidence of obesity in the general population has tripled in the past four decades. The number of bariatric surgeries performed annually has quadrupled in the past ten years due to this obesity epidemic. Concomitantly, the number of patients who are obese or who have undergone bariatric surgery and have become candidates for thyroid surgery has also increased considerably. Among patients undergoing thyroidectomy in the French population, the proportion of obese patients currently represents 10 to 20%. In addition, the frequency of benign and malignant thyroid pathologies seems to be increased by obesity, with a Hazard Ratio of around 1.8 for thyroid cancers in obese women. While obesity does not seem to influence the specific post-operative morbidity of thyroidectomy, a history of malabsorptive bariatric surgery should encourage the greatest caution in patients who need to undergo thyroid surgery since it is associated with a significant risk of severe hypocalcemia (>60% in some studies) requiring intravenous calcium supplementation in about 20% of cases. In the latter situation, peri-operative vitamin D-calcium supplementation is essential. In addition, the replacement dose of Levothyroxine (T4) intended for replacement must also be adapted in obese patients, calculated not from the actual weight but from the ideal weight. The objective of this review is to discuss the influence of obesity on the evolution of nodular and neoplastic thyroid pathologies, on the morbidity of thyroid surgery as well as on post-operative drug treatments. The impact of bariatric surgery on these different aspects is also discussed.

The clinical utility of rituximab (RTX) in Graves’ orbitopathy (GO) treatment remains controversial since the discrepant results from 2 prospective randomized studies (Stan M et al. J Clin Endocrinol Metab 2015; Salvi M et al. J Clin Endocrinol Metab 2015). The aim of this study was to assess in real life the characteristics and the clinical outcomes of patients with GO treated with RTX in cases of corticosteroid resistance or corticosteroid dependence. Multicenter French retrospective study including patients with moderate-to-severe GO requiring second-line treatment with RTX. Patients were classified according to three main baseline characteristics: clinical inflammation (CAS ≥ 3), oculomotor limitation, and visual dysfunction. Patients were considered as responders if, at 24 weeks (week 24), at least 1 of these 3 parameters improved with no worsening elsewhere. Forty patients were included (65% smokers, 38% dysthyroidism). Thirty-two patients were treated with RTX alone (one patient excluded owing to side effects): 64.5% had favorable responses at week 24 and significant reduction in baseline CAS (3.29 ± 1.6) at 12 weeks (1.93 ± 1.1; P

Context The behavior of locally advanced pheochromocytoma (LAP) remains unknown. Objective We characterized the population with LAP and recurrence-free survival (RFS). Methods This retrospective multicentric study was run within the ENDOCAN-COMETE network and French Group of Endocrine Tumors (GTE) from 2003 to 2018, including patients from 11 French referral centers with LAP as defined by capsular invasion, vascular invasion, adipose tissue invasion, and/or positive locoregional lymph nodes at diagnosis without evidence of distant metastasis. The main outcome measure was recurrence, defined as tumor reappearance, including local site and/or distant metastasis. The primary endpoint was RFS analysis; secondary endpoints were characterization, overall survival (OS), and prognostic factors of recurrence. Results Among 950 patients, 90 (9%) exhibited LAP criteria and 55 met inclusion criteria (median age, 53 years; 61% males; 14% with germline mutation; 84% with catecholamine excess). LAP was defined by 31 (56%) capsular invasions, 27 (49%) fat invasions, 6 (11%) positive lymph nodes, and 22 (40%) vascular invasions. After median follow-up of 54 months (range, 6-180), 12 patients (22%) had recurrences and 3 (5%) died of metastatic disease. Median RFS was 115 months (range, 6-168). Recurrences were local in 2 patients, distant in 2, and both local and distant in 8 patients. Median OS of patients was not reached. Size above 6.5 cm (P = 0.019) and Ki-67 > 2% (P = 0.028) were identified as independent significant prognostic factors in multivariate analysis. Conclusion LAP represents 9% of pheochromocytoma’s population and has a metastatic behavior. This study paves the way for future pathological TNM classification.

Discovery of an adrenal mass is nowadays a frequent situation. While adrenal tumors can cause a variety of symptoms, more often than not they are diagnosed incidentally on imaging exams such as CT-scan or MRI performed for another purpose. However, any retroperitoneal supra-renal mass can have an extra-adrenal origin. Indeed, operated non-adrenal masses initially but wrongly diagnosed as an adrenal disease represent about 3.5% of adrenalectomies. These differential diagnoses principally include retroperitoneal tumors that are malignant in two thirds of cases (lymphomas, sarcomas, neurogenic or germinal tumors), and more rarely vascular anomalies or congenital malformations, which are most frequently left-sided due to the wide variety of anatomical structures surrounding the left adrenal gland. Several lesions can originate from the adrenal gland or be located near the gland (paraganglioma, ganglioneuroma). Even though unilateral adrenalectomy is associated with low morbidity, ignorance of these differential diagnoses can cause ill-adapted management; overly conservative surgery in case of sarcoma is one example. Some of these lesions have characteristic clinical or imaging features (cystic lymphangioma, angiomyolipoma…). In other cases, assessment of hormonal secretion is required and additional exams (MRI, percutaneous biopsy, PET-scan with 18-Fluorodeoxyglucose) can correct an erroneous diagnosis. The above diagnostic approach allows appropriate management (with or without surgery). The purpose of this review was to highlight the main differential diagnoses of adrenal masses, to describe their characteristics, and to discuss their therapeutic management.

Background Voice disorders are frequent after thyroidectomy. We report the long-term voice quality outcomes after thyroidectomy using the voice handicap index self-questionnaire. Methods Eight hundred patients who underwent total thyroidectomy between 2014 and 2017 in 7 French hospitals were prospectively included. All patients filled in voice handicap index questionnaires, preoperatively and 2 and 6 months after surgery. Results Median (range) voice handicap index scores were significantly increased at month 2 (4 [0; 108]) compared to preoperative values (2 [0; 76]) and were unchanged at month 6 (2 [2; 92]). Clinically significant voice impairment (voice handicap index score difference ≥18 points) was reported in 19.7% at month 2 and 13% at month 6. Thirty-seven (4.6%) had postoperative vocal cord palsy. In patients with vocal cord palsy compared to those without, median voice handicap index scores were increased at month 2 (14 [0; 107] vs 4 [0; 108]; P = .0039), but not at month 6 (5 [0; 92] vs 2 [0; 87]; P = .0702). Clinically significant impairment was reported in 38% vs 19% at month 2 (P = .010), and in 19% vs 13% at month 6 (P = .310). Thyroid weight, postoperative hypocalcemia, vocal cord palsy, and absence of intraoperative neuromonitoring utilization were associated with an increased risk of clinically significant self-perceived voice impairment at month 2. Conclusion Thyroidectomy impairs patients’ voice quality perception in patients with and without vocal cord palsy.

Thyroid pathologies are the most common forms of endocrinopathy under anticancer immunotherapy. Frequency ranges from 3% to 22% for hypothyroidism and 1% to 11% for thyrotoxicosis. Risk is higher with anti-PD-1 than anti-CTLA-4 treatment and higher again with associated treatment. Pathophysiology mainly consists in silent inflammatory thyroiditis, which accounts for the usual presentation of transient thyrotoxicosis followed by hypothyroidism. Therapeutic strategy usually consists in monitoring with or without symptomatic treatment in case of thyrotoxicosis, and levothyroxine replacement therapy in case of symptomatic hypothyroidism or TSH > 10 mIU/L. Screening for dysthyroidism should be systematic ahead of treatment and before each immunotherapy injection for the first 6 months, then at a lower rhythm. It comprises clinical assessment and TSH assay. Onset of thyroid dysfunction should not interrupt immunotherapy, being mainly transient, easy to treat and mild. Teamwork between oncologists and endocrinologists improves screening and management, so as better to accompany the patient during treatment.

Postthyroidectomy voice disorders can occur without any recurrent laryngeal nerve injury, and probably are the most frequent complication after thyroidectomy. We report the long-term voice quality outcomes after total thyroidectomy without vocal cord palsy using a simple self-assessment tool: the voice handicap index self-questionnaire.This observational prospective multicenter study included 203 patients from the "ThyrQoL" study (ClinicalTrial NCT02167529), who underwent total thyroidectomy between October 2014 and August 2015 in 3 French Hospitals (Nantes, La Roche-sur-Yon, and Limoges). Exclusion criteria included confirmed malignant disease, age18 years, and preoperative voice troubles with confirmed vocal cord palsy. Direct flexible laryngoscopy was performed after surgery. Nineteen patients with a postoperative vocal cord palsy were excluded from analysis.One hundred and seventy-six patients with no vocal cord palsy were analyzed. Voice handicap index scores were significantly altered on postoperative month 2 compared with preoperative values (7.02 ± 11.56 vs 14.41 ± 19.44; P.0001). Voice handicap index scores were not significantly different on postoperative month 6 compared with preoperative values (7.02 ± 11.56 vs 7.61 ± 14.02; P=.381). Thirty-six patients (20.5%) described significant voice impairment 2 months after total thyroidectomy. Nine patients (5.7%) still experienced significant discomfort at 6 months.Twenty percent of patients had initial voice impairment at 2 months postthyroidectomy, with a progressive recovery to preoperative levels at 6 months with6% with persistent voice complaints.

Objectifs Decrire les caracteristiques cliniques, pathologiques et pronostiques des patients traites en France pour TNE renale. Materiel et methodes Nous avons collecte les donnees cliniques et pathologiques des patients traites pour une TNE renale histologiquement prouvee dans les centres de reference entre 1995 et 2019. Resultats 27 patients ont ete inclus, l’âge median etait de 52 ans. Les TNEs etaient toutes sporadiques, se developpaient sur malformation renale en fer a cheval dans 22 %, etaient kystiques dans 26 %. Aucune n’etait fonctionnelle, 58 % ont ete diagnostiquees fortuitement. La taille tumorale mediane etait de 60 mm, une extension metastatique a ete observee dans 29 %. Les TNEs etaient de grade 1 et 2 dans 50 % et 50 %. Apres un suivi median de 74,5 mois, 9 (33 %) recidives ont ete reportees. 9 (33 %) deces ont ete notes. Les survies sans rechute et survie globales medianes etaient de 102 et 109 mois. La rechute etait significativement associee avec des tumeurs plus volumineuses. Une moins bonne survie etait associee a des tumeurs symptomatiques et la presence de metastase au diagnostic. En cas de maladie avancee, une reponse partielle a ete observee chez 3 des 4 patients traites par radiotherapie interne vectorisee et 2 des 9 patients traites par chimiotherapie. Conclusions Il s’agit de la plus grande serie de TNEs renales. Ces tumeurs semblent etre sporadiques, non fonctionnelles, et G1 ou G2. Le pronostic reste correct avec une survie mediane proche de 10 ans. La taille tumorale et la presence de metastase semblent pronostiques.

Objectifs Analyser retrospectivement la sensibilite de la TEP/TDM aux analogues de la somatostatine marques au Gallium-68 (TEP/TDM-68Ga-SMSa) dans l’exploration des paragangliomes (PGL) et pheochromocytomes (PHEO), seule, et comparativement aux autres traceurs TEP. Materiels et methodes La TEP/TDM-68Ga-SMSa etait realisee en plus des examens d’imagerie morphologique (TDM et/ou IRM) dans l’exploration de PGL/PHEO prouves histologiquement. Une hyperfixation superieure au bruit de fond environnant etait consideree pathologique et comparee au gold-standard : anatomopathologie, positivite d’au moins une autre modalite d’imagerie ou donnees du suivi. Si realisees, les donnees des TEP/TDM-18F-DOPA et TEP/TDM-18FDG etaient analysees. Resultats 40 patients consecutifs, 20 femmes et 20 hommes, d’âge median 55 ans, 19 porteurs d’une mutation germinale SDH (1 SDHA, 12 SDHB, 2 SDHC, 4 SDHD) ont ete explores, 18 pour le bilan initial et 22 pour le suivi. Au total, 197 lesions ont ete confirmees : 59 primitifs/recidives locales et 138 metastases. Les sensibilites par-patient et par-lesion etaient de 95 %, 96 %, 86 % et 92 %, 93 % et 84 % respectivement, pour les TEP/TDM-68Ga-SMSa, TEP/TDM -18F-DOPA et TEP/TDM -18FDG. Les 16 faux-negatifs du 68Ga-SMSa etaient 5 metastases pulmonaires, 5 metastases hepatiques, 3 PGL sous-diaphragmatiques, 1 recidive locale de PHEO et 2 adenopathies. La TEP/TDM-18FDG detectaient 8 de ces lesions et la TEP/TDM-18F-DOPA 9 des 10 lesions explorees. Conclusion Notre etude confirme la sensibilite elevee de la TEP/TDM-68Ga-SMSa pour le bilan lesionnel des PGL/PHEO et montre certaines limites. Ainsi, pour les maladies multifocales/metastatiques et les PGL sous-diaphragmatiques, de meilleures performances etaient obtenues en associant a la TEP/TDM-68Ga-SMSa d’autres traceurs, notamment la 18F-DOPA.

Introduction Les adenomes somatotropes non controles par chirurgie et/ou Octreotide/Lanreotide requierent d’autres traitements. Objectif Evaluer les pratiques dans l’acromegalie non controlee par le traitement initial. Methode Questionnaire en ligne aux membres de la SFE entre juin 2020 et fevrier 2021. Resultats Cent quatorze connexions (2/3 F, 1/2 : centre hospitalier, 1/2 > 50 ans) ; 40 dossiers completes. Au diagnostic : 20 H/20 F, symptomatiques (60%), diabetiques (25%), hypertendus (37%), SAOS (47%). IGF1 : 3,7 LSN (1,2–8,2), hyperprolactinemie (31%). Macroadenomes (87%) invasifs au niveau du sinus caverneux (60%). Operes dans 70% des cas, radiotherapie complementaire dans 25% des cas. Octreotide/Lanreotide : 34/40, dose maximale : 85% des cas, arret : 50% des cas (intolerance [30%]/inefficacite [70%]). Pasireotide : 12/40, dose maximale : 58%, arret : 42% des cas (intolerance [50%]/inefficacite [50%]). Cabergoline : 15/40 (dose moyenne : 1,5 mg/semaine), arret : 47% des cas (inefficacite [86%]/intolerance [14%]). Pegvisomant : 11/40 (dose moyenne : 63 mg/semaine), en cours : 91%, arret pour intolerance : 1/11. Injection quotidienne sauf un (bihebdomadaire) ; 5 a posologie optimale, 4 en cours d’augmentation, 1 refus d’augmentation. Au dernier bilan : IGF I > 1,2 LSN : 45%, prolactine normale : 100% (19/19), envahissement sinus caverneux a l’IRM : 72%. Pas de changement de traitement pour 19 car : asymptomatique (n = 9), IGF1 « acceptable » (n = 15), refus du patient (n = 3), « ne sait pas quoi faire de plus » (n = 1). 52,5% presentes en RCP pour valider le choix therapeutique (n = 17) ou demander conseil (n = 4).

La pneumocystose est une complication infectieuse grave de l’hypercortisolisme endogene (HE) severe sans recommandation specifique de prevention primaire d’apres les societes savantes actuelles. Notre etude avait pour objectif de decrire les caracteristiques communes des patients ayant un HE complique de pneumocystose et les modalites de prevention realisees dans les centres experts. Les patients avec une immunodepression acquise ou innee connue pour etre a risque de pneumocystose etaient exclus. Un total de 21 patients de 11 centres differents (12 femmes, 9 hommes ; âge moyen : 54,4 ± 19,0 ans) ont ete inclus. L’etiologie de l’HE etait neoplasique chez 76,2 % des patients (16/21 : 13 TNE, 3 corticosurrenalomes). 71,4 % des patients presentaient une forme catabolique d’HE. Au diagnostic, la kaliemie moyenne etait a 2,66 ± 0,79 mmol/L, une lymphopenie etait observee chez 75 % des patients et 62 % avaient un CLU > 10N. Chez 71,4 % des patients, les premiers signes de pneumocystose sont apparus dans les 7 jours apres l’introduction d’un traitement anticortisolique (mediane : 5,5 jours). La cortisolemie moyenne a 8 h au diagnostic d’HE etait de 782 ± 396 ng/mL tandis qu’elle etait a 257 ± 201 ng/mL au moment de la pneumocystose (p = 0,0001). Un total de 14,4 % des patients sont morts lors de la pneumocystose. Cette etude permet de mieux caracteriser les patients avec un HE complique de pneumocystose et de discuter l’interet d’une prophylaxie. Le risque de pneumocystose parait particulierement eleve dans les 7 jours apres l’introduction d’un traitement anticortisolique et notamment lors de la restauration de l’eucortisolisme.

Introduction/objectifs Le diagnostic differentiel entre maladie de Cushing (MC) et secretion ectopique d’ACTH (SEA) peut necessiter un catheterisme des sinus petreux (KTSP). L’utilisation de la prolactine est controversee et non standardisee du fait des multiples equations proposees pour evaluer la selectivite du prelevement, le diagnostic et la lateralisation de l’adenome. Notre objectif etait d’etudier l’apport de la prolactine dans ces indications. Methodes Analyse retrospective: 54 catheterismes (CHU Nantes 2012–2021). Application des equations proposees dans les 3 indications: gradient centro-peripherique (C/P) de prolactine, gradient C/P d’ACTH normalise sur la prolactine ou non et gradient intersinus d’ACTH normalise sur la prolactine ou non. Comparaison aux resultats d’imagerie et de chirurgie. Resultats Le gradient C/P de prolactine concluait a une mauvaise selectivite pour 14 KTSP suggerant a tort 6 SEA au lieu de MC (faux negatifs-FN). Le gradient d’ACTH entrainait 6 FN evites par normalisation mais induisait 3 faux positifs-FP(MC au lieu de SEA). Le gradient intersinus d’ACTH concordait dans 48 % des cas avec l’imagerie (55,6 % apres normalisation) et 48 % avec la chirurgie (normalisation ou non). Conclusion Le gradient C/P de prolactine est utile pour declencher une relecture du veinogramme en cas de prelevement non selectif. Le gradient d’ACTH normalise permet alors de limiter les FN. L’etablissement d’un seuil adapte aux techniques de dosages est neanmoins necessaire pour limiter les FP. Le gradient intersinus ne permet pas de localiser l’adenome. Nous proposons un arbre decisionnel pour l’interpretation clinique.

We report the case of a patient who presented with multiple brown tumors as the inaugural manifestation of primary hyperparathyroidism. Tc-99m hexakis methoxyisobutylisonitrile (99mTc-MIBI) scintigraphy demonstrated increased radiotracer uptake by the bone lesions. The patient was a 65-year-old male who sought advice for a swelling on his right shin. An osteolytic lesion was visible on the radiograph. A bone biopsy showed a benign tumor containing abundant osteoclastic cells. Laboratory abnormalities included hypercalcemia (3.63mmol/L with 1.91mmol/L ionized calcium), hypophosphatemia (0.38mmol/L), and parathyroid hormone elevation (880.8pg/mL; N: 10-70). Serum 25-OH Vitamin D level was lower than 4ng/mL (N: 30-60). An 18-FDG PET/CT scan identified numerous high-uptake bone lesions. By 99mTc-MIBI scintigraphy, a large high-uptake mass was seen in the left parathyroid gland, as well as high-uptake lesions throughout the skeleton, which were less numerous than those seen by 18-FDG PET/CT. Ultrasonography of the neck visualized a mass consistent with an adenoma in the left parathyroid gland. Brown tumors are bone lesions whose diagnosis should be considered in patients with clinical and laboratory evidence of hyperparathyroidism, once a malignant disease is ruled out. Our case report suggests that 18-FDG PET/CT may be more sensitive than whole-body 99mTc-MIBI scintigraphy in detecting brown tumors.

Introduction Le ganglioneurome (GN) est une tumeur neuroblastique differenciee, rare, issue du systeme nerveux sympathique peripherique. Il se developpe aux depens de la glande surrenale dans 20 a 25 % des cas. L’objectif de cette etude est de caracteriser les GN surrenaliens et d’etudier le risque de recidive apres chirurgie. Materiel/patients et methodes Etude retrospective multicentrique des cas de GN surrenaliens dans 20 centres francais appartenant au reseau COMETE et un centre belge. Resultats Parmi les 106 cas recenses, 60,4 % sont des femmes (N = 64/106), l’âge median au diagnostic est de 29 ans avec 25 cas pediatriques. 60,4 % (N = 64/106) des GN sont des incidentalomes. Les tumeurs ne secretent pas dans 90 % des cas (N = 90/100), le bilan hormonal preoperatoire est douteux pour 10 % des patients (N = 10/100). Au niveau radiologique, ce sont des tumeurs de grande taille (mediane a 50 mm) avec une densite spontanee > 10 UH dans 98 % des cas (N = 54/55) et des calcifications dans 65,3 % des cas (N = 32/49). Un hypermetabolisme en scintigraphie 123I-MIBG et en TEP 18FDG est retrouve dans respectivement 25,8 % (N = 8/31) et 41,3 % (N = 19/46) des tumeurs. 104 patients ont ete operes. Parmi les 42 patients ayant eu un suivi d’imagerie de plus de 3 mois (suivi moyen de 29,6 mois ; mediane 18 mois [4–156]), aucune recidive radiologique n’est retrouvee. Discussion Les GN surrenaliens sont des tumeurs de grande taille, classiquement non secretantes, ne possedant pas les caracteristiques radiologiques de benignite. Cependant, aucune recidive n’a ete identifiee dans notre cohorte. La revue des donnees de la litterature conforte l’absence de recidive post-operatoire.

Introduction Les inhibiteurs de tyrosine kinases (ITK) ont montre leur efficacite dans le cancer thyroidien refractaire. Cependant, ces therapies sont associees a une perte de poids. Des etudes ont montre que le niveau de PTHrp etait associe a une perte de poids dans differents cancers. L’hypoparathyroidie post-thyroidectomie et les ITK peuvent modifier le niveau de PTH. Dans cette etude, nous avons evalue si le niveau de PTH serique pouvait influencer la perte de poids sous ITK chez les patients ayant un cancer thyroidien. Methodologie Il s’agissait d’une etude multicentrique retrospective chez des patients atteints d’un cancer thyroidien traite par ITK. Nous avons evalue la correlation entre le niveau de PTH et la perte de poids sous traitement et compare la variation des parametres nutritionnels sous ITK selon la presence ou non d’une hypoparathyroidie post-thyroidectomie. Resultats 159 patients ont ete inclus avec un suivi median de 12 ± 16 mois. 17,6 % des patients presentaient une hypoparathyroidie. Une correlation positive entre le niveau de PTH serique et le pourcentage de perte de poids a ete trouvee apres 6 mois de traitement (r = 0,48, p = 0,02). L’albumine serique et l’indice nutritionnel PNI etaient egalement diminues a la fin du suivi chez les sujets avec un niveau detectable de PTH uniquement (p Conclusion Dans notre etude, la perte de poids pendant le traitement d’un cancer thyroidien par ITK pourrait etre liee, en partie, au niveau de PTH. La correction des facteurs augmentant la PTH tels que la carence en vitamine D devrait etre envisagee.