Background
Metastases from follicular thyroid carcinoma (FTC) are usually blood borne and far less to lymph nodes (LN). The present study was designed to evaluate the factors that are associated with ...LN metastases in patients operated on for FTC.
Methods
A retrospective review of 70 patients (25 men; mean age, 47 (range, 14–92) years) operated on between January 1995 and December 2005 for FTC was undertaken. All patients had histopathology study of the regional LN and postoperative radioiodine scintigraphy. According to LN invasion, they were divided into two groups in which various parameters were compared.
Results
The length of follow-up was 52 (range 17–108) months. A total of 375 LN were examined from ipsilateral neck side and 87 LN from the contralateral side. LN metastases were found in five (7%) cases: all ipsilateral. Comparing data from the patients with and without LN metastases, respectively, age was 60 and 47 years (
p
= 0.12), male/female ratio was 1/4 and 24/41 (
p
= 0.44), tumor size was 68 and 37 mm (
p
< 0.01), tumor differentiation (well/moderate) was 3/2 and 25/19 (
p
= 0.89), distant metastases were detected in 1 and 8 cases (
p
= 0.72) .The tumor size was >40 mm in 35 (50%) patients. All patients with LN metastases were found in this group (5/35; 14%).
Conclusions
Follicular thyroid carcinoma results in metastases to regional lymph nodes in 7% of cases but only to the ipsilateral neck side. Tumor size is the only factor that impacts it. We do not recommend lymph node dissection in follicular thyroid carcinomas <4 cm.
Background
Hereditary hyperparathyroidism has been reported to occur in 5–10 % of cases of primary hyperparathyroidism in the context of multiple endocrine neoplasia (MEN) types 1, 2A and 4; ...hyperparathyroidism-jaw tumour (HPT-JT); familial isolated hyperparathyroidism (FIHPT); familial hypocalciuric hypercalcaemia (FHH); neonatal severe hyperparathyroidism (NSHPT) and autosomal dominant moderate hyperparathyroidism (ADMH). This paper aims to review the controversies in the main genetic, clinical and pathological features and surgical management of hereditary hyperparathyroidism.
Methods
A peer review literature analysis on hereditary hyperparathyroidism was carried out and analyzed in an evidence-based perspective. Results were discussed at the 2015 Workshop of the European Society of Endocrine Surgeons devoted to hyperparathyroidism due to multiple gland disease.
Results
Literature reports scarcity of prospective randomized studies; thus, a low level of evidence may be achieved.
Conclusions
Hereditary hyperparathyroidism typically presents at an earlier age than the sporadic variants. Gene penetrance and expressivity varies. Parathyroid multiple gland involvement is common, but in some variants, it may occur metachronously often with long disease-free intervals, simulating a single-gland involvement. Bilateral neck exploration with subtotal parathyroidectomy or total parathyroidectomy + autotransplantation should be performed, especially in MEN 1, in order to decrease the persistent and recurrent hyperparathyroidism rates; in some variants (MEN 2A, HPT-JT), limited parathyroidectomy can achieve long-term normocalcemia. In FHH, surgery is contraindicated; in NSHPT, urgent total parathyroidectomy is required. In FIHPT, MEN 4 and ADMH, a tailored case-specific approach is recommended.
Objectives: The aims of this study were to determine the performance of each variable, to define the optimal diagnostic thresholds and to determine the relative value of assaying chromogranin A ...(CgA). Design: Prospective study. Methods: Two groups of patients were studied: a control group of 71 patients and a group of 63 patients with a histologically-proven pheochromocytoma (52 pheochromocytomas and 14 paragangliomas). Fourteen of the patients had a family history of the disease. Eleven variables were assayed in each patient, i.e. the plasma and urinary concentrations of amines and their derivatives, and the CgA serum concentration. Results: The study of the control group showed that all the serum assays gave false positive results (from 6 to 23%), as did four of the six urinary assays (from 2.9 to 12.3%). The areas under the receiver operating characteristic curves varied from 0.689 to 0.992. The variables relating to the epinephrine pathway were significantly less expressed in the hereditary diseases than in the sporadic cases. The diagnostic thresholds of the three most efficient variables have been raised. Conclusions: Plasma determinations of metanephrines are now an easy and convenient tool for the diagnosis of pheochromocytoma. However, in our study the best specificity was obtained with the urinary tests rather than with the plasma assays while the highest sensitivities were for the normetanephrine assays. The assay of CgA was highly efficient in diagnosing pheochromocytomas in the absence of renal insufficiency. By combining it with fractionated metanephrine assays, the sensitivities of the latter were increased.
Background. Our aim was to assess long-term results after less than total parathyroidectomy for hyperparathyroidism in multiple endocrine neoplasia type 1. Methods. Of 1888 patients undergoing ...operation at our institution for primary hyperparathyroidism between 1972 and 2001, 83 (4.4%) had multiple endocrine neoplasia type 1. Outcome data were available for 79; 66 underwent subtotal parathyroidectomy, 55 (83%) of these with bilateral thymectomy. In 13 patients, only grossly enlarged glands were resected (mean 1.1 per patient) as the syndrome of multiple endocrine neoplasia type 1 was not yet evident or the initial exploration was performed elsewhere. Results. Follow-up has been 48 ± 51 months (mean + SD). Intraoperative serum PTH assay decay in 20 patients was suggestive of cure in 18 patients, none of whom required reoperation. Nine patients (11%) required reoperation (3 required reoperation twice) after a mean interval of 77 ± 53 months. Subtotal parathyroidectomy resulted in a lesser reoperation rate than resection of grossly enlarged glands (7% vs 30%, P =.02). At the time of review, 63 patients (80%) were normocalcemic, 10 (13%) hypocalcemic (2 after unsuccessful delayed autograft), and 7% hypercalcemic (none after reoperation). By Kaplan-Meier analysis, the rate of surgical cure (patients who are nonhypercalcemic) is 60% and 51% at 10 and 15 years, respectively. Conclusion. Subtotal parathyroidectomy reduces the need for reoperation. Selective reoperation leads to long-lasting biochemic cure. (Surgery 2002;132:1119-25.)
The French and Belgian GENEM study group’s multiple endocrine neoplasia type I (MEN‐I) database was used to evaluate trends in clinical presentation, surgical treatment of primary hyperparathyroidism ...(pHPT) (n=245), and prognostic factors for hypercalcemia correction among 256 MEN‐I cases. The patients were retrieved through the GENEM network from various Belgian and French instititutions with the help of genetics laboratories. Among the 245 pHPT patients (96%), 42% were men. The mean age at the time of diagnosis was 39.5 ± 13.3 years. Trends were studied for three periods: before 1986, from 1986 to 1990, and thereafter. After 1990 MEN‐I patients were more often diagnosed with isolated pHPT (8%, 11%, 28%, for the three periods, respectively; p=0.002); it was seen more often in screened patients (31%, 28%, 53%; p=0.001), more often among those in already known MEN‐I families (64%, 45%, 72%; p=0.005), and among those with lower preoperative calcemia (2.93, 2.87, 2.79 mmol/L; p=0.001). The age at pHPT diagnosis remained constant throughout the study. The percentage of cervical explorations dropped during the entire study (87%, 87%, 53%; p<0.0001). After 1985 the percentage of subtotal parathyroidectomies increased (25%, 59%, 51%; p=0.0004). Pathology disclosed more hyperplasias (59%, 85%, 74%; p=0.008). Postoperative hypercalcemia decreased (47%, 15%, 19%; p<0.0001); and postoperative hypocalcemia increased nonsignificantly (5%, 15%, 15%; p=0.1). Subtotal parathyroidectomy odds ratio (OR) 13, no MEN‐I family background (OR 3), and the most recent study period (>1985) (OR 3) were significant predictive factors of hypercalcemia correction according to the multivariate analysis. This is the first multicentric study on the management of MEN‐I‐related pHPT. Immediate postoperative hHPT cure increased, but only 80% of the operated patients were cured after 1990. Fifteen percent were hypocalcemic. Because MEN‐I‐related hHPT cure remains difficult to achieve, we advocate that subtotal parathyroidectomies be performed in specialized centers.
Résumé
Introduction. A partir de 245 cas d’hyperparathyroïdie (pHPT) enregistrés au groupe d’étude franco‐belge du GENEM (96%), (256 cas de NEM1), on a analysé l’évolution de la présentation clinique, le résultat après traitement chirurgical et de son effet sur la maladie pHPT. Population et méthodes. Les patients ont été retrouvés grâce au réseau multicentrique du GENEM et avec l’aide des laboratoires d’analyse génétique. Parmi les 245 cas de pHPT, 42% étaient des hommes. L’âge moyen au moment du diagnostic a été de 39,5 ± 13,3 ans. Trois périodes ont été étudiées: avant 1986, de 1986 à 1990, et après 1990. Résultats. Après 1990, il s’agissait plus souvent d’pHPT isolées (8%‐11%‐28%, 0; p=0,002), plus souvent dépistées (31%‐28%‐53%, p=0,001), plus souvent au sein de familles NEM1 connues (64%‐45%‐72%, respectivement: p=0,005), et avec une calcémie plus basse (2,93‐2,87‐2,79, respectivement; mmol/L, p=0,001). L’âge au moment du diagnostic de pHPT n’a pas changé au cours du temps. Le pourcentage de patients opérés pour exploration cervicale a diminué (87%‐87%‐53%, respectivement; p<0,0001). Après 1985, le pourcentage de parathyroïdectomies subtotales a augmenté (25%‐59%‐51%, respectivement: p=0,0004). L’anatomo‐pathologie a mis en évidence plus d’hyperplasies (59%‐85%‐74%, respectivement; p=0,008). L’hypercalcémie post‐opératoire a diminué (47%‐15%‐19%, respectivement; p<0,0001) et l’hypocalcémie a augmenté non significativement (5%‐5% – 15%, respectivement; p=0,1). La parathyroïdectomie subtotale (Odds ratio=13), l’absence d’autre atteinte familiale (Odds ratio=3), et la prise en charge après 1985 (Odds ratio=3) ont été les facteurs indépendants prédictifs d’un contrôle de la pHPT en analyse multivariée. Conclusion. Il s’agit de la première analyse multicentrique de la prise en charge de la pHPT des NEM1. Après 1990, la correction de la pHPT a été plus efficace mais seulement dans 80% des cas. Quinze pourcent des patients étaient hypocalcémiques. Ces constatations poussent à opérer les pHPT des NEM1 en centres spécialisés pour réaliser une parathyroïdectomie subtotale.
Resumen
Introducción: Se utilizó, para analizar los casos de hiperparatiroidismo (pHPT) (n=245) registrados como MEN L (n=256), el grupo de estudio franco‐belga del GENEM. Se analizaron: La evolución clinica y el tratamiento quirúrgico y, sus efectos sobre la pHPT. Material y métodos: Los pacientes se descubrieron gracias a la red multicéntrica del GENEM y con la ayuda de laboratorios de análisis genético. Entre los 245 pHPT (96%), 42% eran varones. La edad media, en el momento del diagnóstico, fue de 39.5 ± 13.3 años. Su evolución se estudió en tres periodos de tiempo: antes de 1986, entre 1986 y 1990 y después de 1990. Resultados: Después de 1990 los casos de MEN 1 se diagnosticaron sobre todo por determinaciones aislada de pHPT (8%‐11%‐28%, p=0.002), se descubrieron con más frecuencia (31%‐28%‐53%, p=0.001), en especial en el seno de familias conocidas por padecer un MEN 1 (64%‐45%‐72%, p=0.005) y con calcemias más bajas (2.93‐2.87‐2.79 mmol/L, p=0.001). La edad, al efectuar el diagnóstico, no se ha modificado a lo largo del tiempo. El porcentaje de enfermos operados ha disminuido (87%‐87%‐53%, p<0.0001). A partir de 1985 el porcentaje de paratiroidectomias subtotales aumentó (25%‐59%‐51%, p=0.0004). Los estudios anatomopatológicos revelaron una mayor incidencia de hiperplasias (59%‐85% ‐74%, p=0.008). La hipercalcemia postoperatoria ha disminuido (47%‐15%‐19%, p<0.0001) y la hipocalcemia aumentado, pero no significativamente (5%‐5%‐15%, p=0.1). En un análisis multivariante la paratiroidectomia subtotal (Odds ratio=13), la falta de antecedentes familiares de MEN 1 (Odds ratio=3) y la operación más precoz a partir de 1985 (Odds ratio=3), son los factores predictivos más significantes de la corrección de la hipercalcemia. Conclusión: Se trata del primer análisis multicéntrico del tratamiento del sÍndrome de MEN 1 referido a la pHPT. La curación del hHPT aumentó en el postoperatorio inmediato, pero sólo el 80% de los pacientes operados estaban curados después de 1990. 15% de los pacientes estaban hipocalcémicos. Dado que la curación de enfermos con síndrome de MEN 1 relacionado con hHPT es dificil, se aconseja realizar una paratiroidectomía subtotal en centros especializados.
Background
Laparoscopic adrenalectomy (LA) has become the standard technique for most indications. The aim of this study was to determine the predictive factors of intra- and postoperative ...complications in order to inform the orientation of patient to a surgeon with more experience in adrenal surgery.
Methods
From January 1994 to December 2013, 520 consecutive patients benefited from LA at Huriez Hospital, Lille, France. Each complication was graded according to the Dindo–Clavien-grade scale. The predictive factors of complications were determined by logistic regression.
Results
Fifty-two surgeons under the supervision of 5 senior surgeons (individual experience >30 LA) participated. Postoperative complications with a grade of ≥2 occurred in 52 (10 %) patients (29 (5.6 %) medical, 19 (3.6 %) surgical, and 4 (0.8 %) mixed complications) leading to 12 (2.3 %) reoperations. There was no postoperative death. Intraoperative complication happened in 81 (15.6 %) patients responsible for conversion to open adrenalectomy (OA) odds ratio (OR) 13.9, CI 95 % 4.74–40.77,
p
< 0.001. History of upper mesocolic or retroperitoneal surgery was predictive of intraoperative complication (OR 2.02, 1.05–3.91,
p
= 0.036). Lesion diameter ≥45 mm was predictive of intraoperative complication (OR 1.94, 1.19–3.15,
p
= 0.008), conversion to OA (OR 7.46, 2.18–25.47,
p
= 0.001), and adrenal capsular breach (OR 4.416, 1.628–11.983,
p
= 0.004). Conversion to OA was the main predictive factor of postoperative complications (OR 5.42, 1.83–16.01,
p
= 0.002). Under adequate supervision, the surgeon’s individual experience and initial adrenal disease were not considered predictive of complications.
Conclusion
Lesion diameter over 45 mm is the determinant parameter for guidance of patients to surgeons with more extensive experience.
Abstract
Context
Heterozygous germline pathogenic variants found in succinate dehydrogenase (SDH) complex genes predispose to hereditary paraganglioma (PGL) syndromes. No mosaicism has yet been ...reported in this setting.
Design and Participant
We describe the clinical history of a case of SDH complex, subunit B (SDHB) mosaicism. A 24-year-old woman who developed a cardiogenic shock during dental surgery was diagnosed with a functional para-aortic PGL, which produced predominantly norepinephrine and its metabolites. The tumor was removed and showed a loss of SDHB expression by immunohistochemistry. Four years after initial laparotomy, the patient had a rapid cardiac decompensation during her second pregnancy, despite negative imaging 10 months before. Two recurrent functional PGLs were found and surgically removed. Initial genetic analysis performed by Sanger sequencing did not reveal any germline pathogenic variant in SDHB, VHL, SDHD, SDHC, SDHAF2, RET, MAX, and TMEM127. Next-generation sequencing performed on tumor- and blood-extracted DNAs highlighted the presence of a mosaic rare variant in SDHB (c.557G>A, p.Cys186Tyr) with an allelic ratio of 15% in the blood DNA.
Conclusions
We report the full clinical description of a proband with SDHB mosaicism associated with a functional, recurrent PGL. This case strengthens the necessity to complete the genetic analysis with methodologies able to identify germline mosaicism, especially in the case of early disease onset.
We reported a case of succinate dehydrogenase complex B mosaicism and showed that it is responsible for a functional paraganglioma with recurrence and early onset.
Background
Laparoscopic adrenalectomy (LA) is the standard treatment for benign adrenal lesions. The laparoscopic approach has also been increasingly accepted for adrenal metastases but remains ...controversial for adrenocortical carcinoma (ACC). In a retrospective cohort study we compared the outcome of LA versus open adrenalectomy (OA) in the treatment of stage I and II ACC.
Methods
This was a double cohort study comparing the outcome of patients with stage I/II ACC and a tumor size <10 cm submitted to LA or OA at Lille University Hospital referral center from 1985 to 2011. Main outcomes analyzed were: postoperative morbidity, overall survival, and disease-free survival.
Results
Among 111 consecutive patients operated on for ACC, 34 met the inclusion criteria. LA and OA were performed in 13 and 21 patients, respectively. Baseline patient characteristics (gender, age, tumor size, hormonal secretion) were similar between groups. There was no difference in postoperative morbidity, but patients in LA group were discharged earlier (
p
< 0.02). After a similar follow-up (66 ± 52 for LA and 51 ± 43 months for OA), Kaplan–Meier estimates of disease-specific survival and disease-free survival were identical in both groups (
p
= 0.65,
p
= 0.96, respectively).
Conclusions
LA was associated with a shorter length of stay and did not compromise the long-term oncological outcome of patients operated on for stage I/II ACC ≤ 10 cm ACC. Our results suggest that LA can be safely proposed to patients with potentially malignant adrenal lesions smaller than 10 cm and without evidence of extra-adrenal extension.
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.
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