October 2008
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18 Reads
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30 Citations
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October 2008
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18 Reads
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30 Citations
June 2008
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87 Reads
Le réseau RENATEN se propose de structurer l’offre de soins pour les patients atteints de tumeurs endocrines, sporadiques ou héréditaires, sur le territoire français. Après avoir rappelé les objectifs et les finalités du réseau, cet article précise les particularités diagnostiques, évolutives et thérapeutiques des tumeurs endocrines, digestives ou non (thyroïde, parathyroïdes, surrénales, bronches). The RENATEN network has been created in order to organize in a structured manner the management of patients with endocrine tumours, whether sporadic or hereditary, throughout the entire French territory. The objectives and aims of the network are presented first, followed by a description of the diagnostic, evolutive, and therapeutic specificities of endocrine tumours, of digestive origin or not (thyroid, parathyroid, adrenals, lungs).
May 2008
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40 Reads
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81 Citations
Clinical Endocrinology
(18)F-DOPA has emerged as a promising tool in the localization of chromaffin-tissue-derived tumours. Interestingly, phaeochromocytomas (PHEO) are also FDG avid. The aim of this study was to retrospectively evaluate the results of (18)F-FDOPA and/or (18)F-FDG-PET in patients with PHEO and paragangliomas (PGLs) and to compare the outcome of this approach with the traditional therapeutic work-up. Nine patients with non-MEN2 related PHEO or PGL were evaluated. At the time of the PET studies, the patients were classified into three groups based on their clinical history, conventional and SPECT imaging. The groups were malignant disease (n = 5, 1 VHL), apparently unique tumour site in patients with previous surgery (n = 1, SDHB) and multifocal tumours (n = 3, 1 VHL, 1 SDHD). (18)F-FDOPA and (18)F-FDG-PET PET/CT were then performed in all patients. PET successfully identified additional tumour sites in five out of five patients with metastatic disease that had not been identified with SPECT + CI. Whilst tumour tracer uptake varied between patients it exhibited a consistently favourable residence time for delayed acquisitions. (18)F-FDOPA uptake (SUVmax) was superior to (18)F-FDG uptake in cases of neck PGL (three patients, four tumours). If only metastatic forms and abdominal PGLs were considered, (18)F-FDG provided additional information in three cases (two metastatic forms, one multifocal disease with SDHD mutation) compared to (18)F-FDOPA. Our results suggest that tumour staging can be improved by combining (18)F-FDOPA and (18)F-FDG in the preoperative work-up of patients with abdominal and malignant PHEOs. (18)F-FDOPA is also an effective localization tool for neck PGLs. MIBG however, still has a role in these patients as MIBG and FDOPA images did not completely overlap.
November 2007
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18 Reads
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10 Citations
Annales d Endocrinologie
Multiple endocrine neoplasia type 2 (MEN2) is an hereditary disease with a prevalence of 1/5000. Three phenotypic variants have been identified: MEN2A associates medullary thyroid carcinoma (MTC) to pheochromocytoma in about 20-50% of cases and to primary hyperparathyroidism in 5-20% of cases; MEN2B associates MTC to pheochromocytoma in 50% of cases, to marphanoid habitus and to mucosal and digestive ganglioneuromatosis whereas in familial isolated medullary thyroid carcinoma (FMTC), the other components of the disease are absent. In MEN2, natural history of the disease and a common embryologic origin (neural crest) may explain the phenotypes observed in the organ involved, beginning from the stage of hyperplasia to adenoma and cancer. MEN2 is an inherited autosomal dominant disease with a complete penetrance, related to germline mutation in the proto-oncogene RET. MTC represent the most frequent circumstance of diagnosis. Pheochromocytoma and HPT may reveal the disease unfrequently and are systematically associated to undiagnosed MTC which is present yet. Analysis of the RET gene allows to confirm the diagnosis of MEN2 by identifying the causal germline mutation. Management of MEN2 patients include thyroidectomy associated to cervical central and bilateral lymph nodes dissection for MTC, unilateral adrenalectomy for unilateral pheochromocytoma or bilateral adrenalectomy when both glands are involved, and selective resection of pathologic parathyroid glands for HPT. Familial genetic screening detects at risk subjects who will develop the disease and allows to manage them at the earliest stage of the disease by perform early or prophylactic thyroidectomy such giving them the best chance of cure. Prognosis of MEN2 is mainly related to the stage-dependant prognosis of MTC, thus pointing the necessity of a complete thyroid surgery for index cases with MTC and the earliest thyroidectomy for screened at risk subjects.
November 2007
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16 Reads
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13 Citations
Annales d Endocrinologie
Medullary thyroid carcinoma (MTC) is developed from thyroid C cells that secrete calcitonin (CT). MTC represents 5-10% of thyroid cancers with a 1-2% incidence in nodular thyroid diseases. Diagnosis is usually made by a solitary nodule often associated to nodal metastasis and confirmed by a high basal CT level which represents its biological marker. MTC may present as a sporadic form and in about 30% of case as a familial form as a part of multiple endocrine neoplasia syndrome, an hereditary dominant inherited disease related to germline mutation of the proto-oncogene RET. Both biological (CT) and genetic (RET) markers allows the optimal diagnosis and treatment of MTC; the former allows screening and early diagnosis of MTC by routinely CT measurements in nodular thyroid diseases that make the adequate and complete surgery required to be performed. The former leads to diagnose familial MTC and to identify at risk subjects in whom early or prophylactic surgery may be performed. Treatment of MTC is based on the complete surgical resection: total thyroidectomy associated to central and laterocervical nodal dissection. For locally advanced or metastatic MTC, complete cervical surgery is required and needs to be associated to other systemic treatments: as chemotherapy is not very efficient, radioimmunotherapy and RET target gene therapy (mainly tyrosine kinase inhibitors) appears as possible valuable therapeutic options for the future. Prognosis of MTC is mainly related to both the stage of the disease and the extend of the initial surgery. Ten-year survival is about 80% when the patients are not surgically cured and reaches 95% when the biological marker CT is normalized after surgery.
October 2007
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37 Reads
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9 Citations
Annales d Endocrinologie
Medullary thyroid carcinoma (MTC) is developed from thyroid C cells that secrete calcitonin (CT). MTC represents 5–10% of thyroid cancers with a 1–2% incidence in nodular thyroid diseases. Diagnosis is usually made by a solitary nodule often associated to nodal metastasis and confirmed by a high basal CT level which represents its biological marker. MTC may present as a sporadic form and in about 30% of case as a familial form as a part of multiple endocrine neoplasia syndrome, an hereditary dominant inherited disease related to germline mutation of the proto-oncogene RET. Both biological (CT) and genetic (RET) markers allows the optimal diagnosis and treatment of MTC; the former allows screening and early diagnosis of MTC by routinely CT measurements in nodular thyroid diseases that make the adequate and complete surgery required to be performed. The former leads to diagnose familial MTC and to identify at risk subjects in whom early or prophylactic surgery may be performed. Treatment of MTC is based on the complete surgical resection: total thyroidectomy associated to central and laterocervical nodal dissection. For locally advanced or metastatic MTC, complete cervical surgery is required and needs to be associated to other systemic treatments: as chemotherapy is not very efficient, radioimmunotherapy and RET target gene therapy (mainly tyrosine kinase inhibitors) appears as possible valuable therapeutic options for the future. Prognosis of MTC is mainly related to both the stage of the disease and the extend of the initial surgery. Ten-year survival is about 80% when the patients are not surgically cured and reaches 95% when the biological marker CT is normalized after surgery.
March 2007
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3 Reads
Diabetes & Metabolism
October 2005
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8 Reads
Annales d Endocrinologie
October 2005
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9 Reads
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1 Citation
Annales d Endocrinologie
October 2005
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8 Reads
Annales d Endocrinologie
... PPGLs classically exhibit a marked 18 F-DOPA uptake, especially those associated with polycythemia, those that belong to cluster 2 PPGLs, and HNPGLs (Fig. 4). 18 F-DOPA PET/CT has a sensitivity approaching 100% and a very high specificity (>95%) for these tumors; however, it is substantially lower for metastatic tumors (37,39,(44)(45)(46)(47)(48)(49)(50)(51). The main advantage over other tracers (eg, in comparison to 123 I-MIBG and labeled-SSA) relies on the low to moderate uptake by healthy adrenals, thus allowing more specific detection of PCC than 123 I-MIBG. ...
October 2008
... In 2004 Lonser et al. described a 40-year-old man with VHL disease showing bilateral endolymphatic-sac tumors [3]. This type of tumor is present in 10-16% of cases of VHL disease [4,5], which occurs in 1 out of 36,000 births [6]. VHL is an autosomal dominant disease resulting from deletions or mutations in the VHL gene located on the short arm of chromosome 3 [3 p25-p26]. ...
January 1998
Annales d Endocrinologie
... Patients with normal ACE and undetectable calcitonin are considered biochemically cured and have an excellent prognosis [18]. Postoperatively, basal calcitonin levels can determine if the surgical procedure was complete; a value lower than 10pg/ml confirms biological remission [25]. ...
Reference:
Medullary Thyroid Carcinoma
October 2007
Annales d Endocrinologie
... Previous study [13] reported that non-biochemical cure correlated with metastatic lymph nodes in the lateral neck. Patients who achieved biochemical remission after initial treatment were associated with a low risk of recurrence (3 %) [14] compared with those who failed to achieve biochemical cure [15]. In the literature, the standard of biochemical cure is not uniform due to the varying sensitivity, specificity, and reference values of different Ctn test kits. ...
September 2001
Clinical Endocrinology
... Extremely reactive iodide is kept under control by a strong antioxidant defense system in the thyrocyte cytoplasm (Song et al., 2007). Later on, hormone production starts on the border between the follicular epithelial apical membrane and colloid, where TPO localized in the apical membrane (Kotani & Ohtaki, 1987;Fayadat et al., 1998) oxidizes iodide to iodine and incorporates it in the tyrosine residues on Tg (Dunn & Dunn, 2001). TSH stimulation leads both to increased synthesis and secretion of TH. ...
November 1998
Endocrinology
... Fu et al. 10.3389/fonc.2023.1216394 ...
December 1998
Thyroid: official journal of the American Thyroid Association
... It is noteworthy that the majority of lesions associated with the MEN2/RET abnormality are NETs outside of the gastrointestinal system. VHL disease is an autosomal dominant syndrome whose cardinal features include a predisposition to renal cancers, retinal and/or cerebellar hemangioblastoma, pheochromocytoma, and cystic and/or pancreatic endocrine tumors [39]. Ten percent to fifteen per cent of patients with VHL develop pancreatic islet or ductal endocrine cell tumors [40] and more than 50% exhibit multiple tumors. ...
February 1998
Annales d Endocrinologie
... In the latter, due to inherited (autosomal dominant) REarranged during Transfection (RET) protooncogene alteration (9)(10)(11)(12), MTC can be associated (multiple endocrine neoplasia type IIA and IIB -MEN IIA and MEN IIB) or not (familial medullary thyroid carcinoma -F M T C ) , w i t h o t h e r e n d o c r i n e n e o p l a s i a s u c h a s pheochromocytoma (PHEO) and/or hyperparathyroidism due to parathyroid hyperplasia or multiple adenomatosis (PTHAd) (13). MTC in children is extremely rare and it can be found almost exclusively in inherited cases of MEN II (14)(15)(16)(17). ...
December 1999
European Journal of Endocrinology
... Individuals with MEN2B have a 50% risk of developing PHEOs, which are benign tumors [100]. In children, PHEOs may develop as early as age 12 years with a mean age of about 15 years [106,107]. The tumors generally present earlier in MEN2B than in MEN2A and are more frequently bilateral in nature [108]. ...
February 2001
European Journal of Endocrinology
... Cela devrait conduire, petit à petit, à la suppression des examens non informatifs et à l'ajout d'analyses complémentaires indispensables. Le principe étant de n'utiliser que ce qui est strictement nécessaire à la décision médicale par le respect, au plus près, des recommandations des bonnes pratiques élaborées et validés par les différents groupes d'experts [2][3][4]6,8,12,17,20,22,24,30,32]. ...
May 2002
Annales de biologie clinique