F. Sebag

Influence of Experience on Performance of Individual Surgeons in Thyroid Surgery: Prospective Cross Sectional Multicentre Study

Objective To determine the association between surgeons’ experience and postoperative complications in thyroid surgery. Design Prospective cross sectional multicentre study. Setting High volume referral centres in five academic hospitals in France. Participants All patients who underwent a thyroidectomy undertaken by every surgeon in these hospitals from 1 April 2008 to 31 December 2009. Main outcome measures Presence of two permanent major complications (recurrent laryngeal nerve palsy or hypoparathyroidism), six months after thyroid surgery. We used mixed effects logistic regression to determine the association between length of experience and postoperative complications. Results 28 surgeons completed 3574 thyroid procedures during a one year period. Overall rates of recurrent laryngeal nerve palsy and hypoparathyroidism were 2.08% (95% confidence interval 1.53% to 2.67%) and 2.69% (2.10% to 3.31%), respectively. In a multivariate analysis, 20 years or more of practice was associated with increased probability of both recurrent laryngeal nerve palsy (odds ratio 3.06 (1.07 to 8.80), P=0.04) and hypoparathyroidism (7.56 (1.79 to 31.99), P=0.01). Surgeons’ performance had a concave association with their length of experience (P=0.036) and age (P=0.035); surgeons aged 35 to 50 years had better outcomes than their younger and older colleagues. Conclusions Optimum individual performance in thyroid surgery cannot be passively achieved or maintained by accumulating experience. Factors contributing to poor performance in very experienced surgeons should be explored further.

Quality of life changes and clinical outcomes in thyroid cancer patients undergoing radioiodine remnant ablation (RRA) with recombinant human TSH (rhTSH): a randomized controlled study

Background  Recombinant human TSH (rhTSH) has become the modality of choice for radioiodine remnant ablation (RRA) in low‐risk thyroid cancer patients.

The role of 18F‐FDOPA and 18F‐FDG–PET in the management of malignant and multifocal phaeochromocytomas

Background  18F‐DOPA has emerged as a promising tool in the localization of chromaffin‐tissue‐derived tumours. Interestingly, phaeochromocytomas (PHEO) are also FDG avid.

Limited Value of 18F-F-DOPA PET to Localize Pancreatic Insulin-Secreting Tumors in Adults with Hyperinsulinemic Hypoglycemia

CONTEXT Fluorine-18-L-dihydroxyphenylalanine positron emission tomography (18F-FDOPA PET) imaging is increasingly used in the workup of neuroendocrine tumors. It has been shown to be an accurate tool in the diagnosis of congenital hyperinsulinism, but limited information is available on its value in adult disease. OBJECTIVE, PATIENTS, AND DESIGN: The objective of this study was to review our experience with 18F-FDOPA PET imaging in six consecutive patients with hyperinsulinemic hypoglycemia (HH) (four solitary insulinomas, one diffuse beta-cell hyperplasia, one malignant insulinoma). 18F-FDOPA uptake was also evaluated in 37 patients (43 procedures) without HH or other pancreatic neuroendocrine tumors, which acted as a control group. RESULTS Using visual analysis, 18F-FDOPA-PET proved positive in only one case (a multiple endocrine neoplasia type 1 related insulinoma). In diffuse beta-cell hyperplasia, the pancreatic uptake was similar to controls. In the patient with liver metastases, the extent of disease was underestimated. The pancreatic uptake was not statistically different between controls and hyperinsulinemic patients. The main limitation for identifying insulinomas or beta-cell hyperplasia in adults appears to be to the 18F-FDOPA uptake and retention in the whole pancreas. This drawback is potentially circumvented in focal hyperplasia in newborns due to a lower aromatic amino acid decarboxylase expression in the extralesional pancreatic parenchyma. CONCLUSIONS 18F-FDOPA PET is of limited value in localizing pancreatic insulin secreting tumors in adult HH. Our results contrast with the referential study and require further analysis.

Intraoperative diagnosis and treatment of parathyroid cancer and atypical parathyroid adenoma

Distinction of parathyroid cancer from atypical parathyroid adenoma (APA) at operation is difficult. The aim of this study was to determine whether parathyroid cancer and APA have different operative findings and long‐term outcomes.

First Report of Bilateral Pheochromocytoma in the Clinical Spectrum of HIF2A-Related Polycythemia-Paraganglioma Syndrome

CONTEXT Molecular genetic research has so far resulted in the identification of 10 well-characterized susceptibility genes for hereditary pheochromocytoma (PHEO) or paraganglioma (PGL). Recently, a new syndrome characterized by multiple PGLs and somatostatinomas associated with congenital polycythemia due to somatic mutations in HIF2A has been reported. OBJECTIVE The aim of the study was to define the genetic defect in a new case of bilateral PHEO and multiple PGLs associated with congenital polycythemia. PATIENT A female patient presented with neonatal polycythemia (treated by phlebotomies, 1 session approximately every 4 mo), mildly enlarged cerebral ventricles, and bilateral PHEO and multiple PGLs. There was no family history of any neuroendocrine tumor or polycythemia. Surgical removal of the tumors only temporarily normalized plasma erythropoietin (Epo) levels and discontinued phlebotomies. No germline mutations were initially detected in the SDHB, SDHC, SDHD, VHL, and PHD2 genes, known to be associated with polycythemia. The PHEOs presented with a typical noradrenergic biochemical phenotype. RESULTS A heterozygous missense mutation (c.1589C>T) was identified in exon 12 of HIF2A, resulting in an alanine 530 substitution in the HIF-2α protein with valine (A530V). This somatic mutation was detected in the tissue from 1 PHEO and 1 PGL, with no HIF2A germline mutation found. This mutation led to stabilization of HIF-2α and hence a gain-of-function phenotype, as in previously published studies. CONCLUSION This case represents the first association of a somatic HIF2A gain-of-function mutation with PHEO and congenital polycythemia, and it alerts physicians to perform proper genetic screening in patients presenting with multiple norepinephrine-producing PHEOs and polycythemia. This report also extends the previous findings of a new syndrome of only multiple PGLs, somatostatinomas, and polycythemia to multiple PHEOs.

Iodine Biokinetics and Radioiodine Exposure after Recombinant Human Thyrotropin-Assisted Remnant Ablation in Comparison with Thyroid Hormone Withdrawal

CONTEXT A few prospective studies have evaluated the use of recombinant human TSH (rhTSH) for radioiodine remnant ablation. OBJECTIVE Our objective was to compare the effects of the both TSH regimens on iodine biokinetics in the thyroid remnant, dosimetry, and radiation protection. DESIGN We conducted a prospective randomized study. MATERIALS AND METHODS Eighty-eight patients were enrolled for radioiodine ablation to either the hypothyroid or rhTSH arms. A whole-body scan was performed at 48 and 144 h after therapy. Dose rates were assessed at 24, 48, and 144 h. Urinary samples were obtained during the first 48 h. Thyroglobulin was assessed before and after therapy. Iodine biokinetics in the remnants were calculated from gamma-count rates. Radiation-absorbed dose was calculated using OLINDA software. Exposure estimation was based on a validated model. RESULTS The effective half-life in the remnant thyroid tissue was significantly longer after rhTSH than during hypothyroidism (P = 0.01), whereas 48-h (131)I uptakes and residence times were similar. After therapy, thyroglobulin release (a marker of cell damage) was lower in the rhTSH arm. The mean total-body effective half-life and residence time were shorter in patients treated after rhTSH. Residence time was also lower for the colon and stomach. Absorbed dose estimates were lower in the rhTSH arm for the lower large intestine, breasts, ovaries, and the bone marrow. Dose rates at the time of discharge were lower in the rhTSH group with a reduction in cumulative radiation exposure to contact persons. CONCLUSIONS In comparison with thyroid hormone withdrawal, rhTSH is associated with longer remnant half-life of radioactive iodine while also reducing radiation exposure to the rest of the body and also to the general public who come in contact with such patients.

Functional characterization of nonmetastatic paraganglioma and pheochromocytoma by (18) F-FDOPA PET: focus on missed lesions.

To evaluate the clinical value of 18F‐fluorodihydroxyphenylalanine (18F‐FDOPA) PET in relation to tumour localization and the patients genetic status in a large series of pheochromocytoma/paraganglioma (PHEO/PGL) patients and to discuss in detail false‐negative results.

Leçons retenues après 274 surrénalectomies laparoscopiques

Aims: To define the role of minimally invasive videoassisted surgery in the surgical management of adrenal disease and discuss the respective indications of the trans and retroperitoneal video assisted approaches. Materials and Methods: During the last 8 years (1994–2001), 330 adrenalectomies were performed in 305 patients: 274 (83%) laparoscopic approaches and 56 (17%) open approach. Open surgery was reserved for patients presenting with large or malignant tumours (29 cases), multiple and/or extraadrenal phaeochromocytomas (13 cases), previous intraabdominal intestinal surgery (10 cases), and in those requiring concomitant intraabdominal surgery (4 cases). Laparoscopic adrenalectomy was performed using the lateral transperitoneal approach for 89 Conn’s syndrome, 67 Cushing’s syndrome, 2 virilising tumours, 51 phaeochromocytomas and 65 non secretory tumours greater than 4 cm in diameter. Nineteen patients underwent bilateral adrenalectomy. Results: There were no deaths. Twenty patients (7.3%) had a complication. Eleven cases required open conversion (4%) because of difficulties with dissection (8 cases), peroperative suspicion of malignancy (2 cases), and one pneumothorax. The average size of tumours was 34 mm (7–110 mm). There were 18 malignant tumours (6.5%): 8 adrenocortical carcinomas, 1 leiomyosarcoma, and 9 metastases. All patients with hormonally secreting tumours were cured of their endocrinopathy. There was 1 death secondary to hepatic metastases in a patient with an adrenocortical carcinoma. Conclusion: Most adrenal tumours are suitable for video assisted excision. The only absolute contraindication is an invasive carcinoma requiring an extended excision. The lateral, transperitoneal approach is the most suitable for tumours greater than 5–6 cm in diameter. Both the transperitoneal or retroperitoneal approaches are suitable for smaller tumours depending on operator choice and experience. However in the presence of a large right lobe of liver or previous intraabdominal surgery the retroperitoneal approach may be preferable.

Comparison of [111In]pentetreotide‐SPECT and [18F]FDOPA‐PET in the localization of extra‐adrenal paragangliomas: the case for a patient‐tailored use of nuclear imaging modalities

Aims and methods  The aim of this prospective study was to compare the diagnostic value of [18F]FDOPA‐PET and [111In]pentetreotide‐SPECT somatostatin receptor scintigraphy (SRS) in patients with nonmetastatic extra‐adrenal paragangliomas (PGLs). Twenty‐five consecutive unrelated patients who were known or suspected of having nonmetastatic extra‐adrenal PGLs were prospectively evaluated with SRS and [18F]FDOPA‐PET. 131I‐MIBG and [18F]FDG‐PET were added to the work‐up in patients with a personal or familial history of PGL, predisposing mutations, abdominal PGLs, metanephrine hypersecretion and abdominal foci on SRS and/or [18F]FDOPA‐PET.