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Viel, Giovanni (2017) Adrenal surgery: five years of experience. [Tesi di dottorato]

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Abstract (inglese)

Background
Adrenal disease is a heterogeneous group of diseases characterized by specific peculiarities. The development of diagnostic methods and the use of genetics in recent years have enabled us to better define each of the syndromes that originate from the adrenal gland. Although in literature there are many publications that analyze single aspects of each pathological condition, few publications allow us an overview.
The aim of this study is to analyze 5 years of endocrine surgery experience to highlight the main characteristics of the adrenal disease and the better surgical approach.

Materials and methods
The study is a retrospective analysis of prospectively collected data from January 2012 to October 2016 at the Endocrine Surgery Unit of the University of Padua, Italy. The experience is analyzed in term of diagnostic and surgical approach, in relation to the different adrenal tumors. Biochemical exams, imaging and genetic features are evaluated. The different surgical approaches are described.

Results
Two hundred twelve patients underwent surgical treatment for adrenal tumors. Ninety-four patients (38.7%) came from other regions than Veneto. Primary aldosteronism (PA) was diagnosed in 71 cases, Hypercortisolism in 43 cases (Cushing Sindrome -CS n=16, adrenocorticotropic hormone independent macronodular adrenal hyperplasia -AIMAH n=9, Cushing Disease -CD n=7, Subclinical Cushing Sindrome -SCS n=6, PA and SCS n=2), Pheocromocythoma (P) in 33 cases, Non Secreting Tumors (NST) in 39 cases, adrenocortical cancer (K) in 9 cases, metastasis in 19 cases, CS in ectopic ACTH secreting tumor in 1 case and virilizing tumor in 1 case.
Genetic analysis reported mutation in ARMC5 gene in 2 patients with AIMAH diagnosis. Mutations in VHL, SDHD, RET, MAX and NF1 were described in patients with P. The surgical approach depends on preoperative diagnosis, genetic analysis and team experience. Cortical sparing adrenalectomy was reserved for a case of familial P. Retroperitoneoscopic procedures were performed in prevalence in patients with PA (20 cases) but also in one patient with K. Two laparoscopic adrenalectomy were performed in presence of K.

Conclusions
Surgical treatment is the final answer to a clinical, endocrinological, radiological, genetics and surgical evaluation. The complexity of the adrenal tumor classification represents itself an indication at multidisciplinary approach exclusively in high-volume centers in order to determine the most appropriate treatment.

Abstract (italiano)

Presupposti dello studio
La patologia surrenalica rappresenta un gruppo eterogeno di malattie caratterizzate da specifiche peculiarità. l'evoluzione delle metodiche diagnostiche e l'ausilio della genetica negli ultimi anni hanno permesso di definire meglio ciascuna delle sindromi che originano dalla ghiandola surrenalica. Benché nella letteratura scientifica siano numerose le pubblicazioni che analizzano singoli aspetti di ciascuna condizione patologica, poche sono le pubblicazioni che permettano di avere un visione d'insieme della problematica.
Obiettivo di questo studio è identificare le peculiarità delle patologie surrenaliche sottoposte a chirurgia negli ultimi 5 anni.

Materiali e metodi
Lo studio è un’analisi retrospettiva di dati raccolti in modo prospettico a partire da Gennaio 2012 a Ottobre 2016 presso l’Unità di Endocrinochirurgia dell’Università di Padova, Italia. L'esperienza di un singolo centro è stata analizzata in termini di iter diagnostico e approccio chirurgico in relazione alle differenti diagnosi di tumore surrenalico. Esami di laboratorio, accertamenti radiologici e caratteristiche genetiche sono state considerate. Sono stati descritti i diversi approcci chirurgici.

Risultati
212 pazienti provenienti da tutta Italia, 94 (38.7%) dei quali provenienti da regioni diverse dal Veneto, sono stati sottoposti a trattamento chirurgico per neoplasie surrenaliche. In 43 casi è stato diagnosticato ipercortisolismo (16 Cushing Sindrome -CS, 9 iperplasia surrenalica macronodulare ACTH indipendente, 7 malattia di Cushing -CD, 6 Sindrome di Cushing Subclinica -SCS, 2 Iperaldosteronismo primitivo -PA and SCS), 71 PA, 33 Feocromocitoma (P), 39 Tumori non secernenti (NST), 9 carcinoma corticosurrenalico (K), 19 Metastasi, 1 CS da ACTH ectopico, 1 tumore virilizzante. Le analisi genetiche hanno riportato mutazioni di ARMC5 gene in 2 pazienti con diagnosi di AIMAH. Mutazioni in VHL, SDHD, RET, MAX e NF1 sono state descritte in P. La surrenectomia "cortical sparing" è stata riservata ad un caso di feocromocitoma familiare. L'accesso retroperitoneoscopico è stato utilizzato prevalentementee in pazienti con PA (20 casi) ma anche in un caso di K. Inoltre 2 surrenectomie laparoscopiche sono state eseguite in presenza di diagnosi di K.

Conclusioni
Il trattamento chirurgico rappresenta la risposta finale ad una valutazione clinica, endocrinologica, radiologica, genetica e chirurgica. La complessità della classificazione dei tumori surrenalici, rappresenta di per se stessa un'indicazione all'approccio multidisciplinare, esclusivamente in centri ad alto volume al fine di garantire il trattamento più appropriato.

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Tipo di EPrint:Tesi di dottorato
Relatore:Iacobone, Maurizio
Dottorato (corsi e scuole):Ciclo 29 > Corsi 29 > IPERTENSIONE ARTERIOSA E BIOLOGIA VASCOLARE
Data di deposito della tesi:31 Gennaio 2017
Anno di Pubblicazione:31 Gennaio 2017
Parole chiave (italiano / inglese):Adrenal tumor/neoplasia surrenalica, Cushing sindrome/sindrome di Cushing, primary hyperaldosteronism/iperaldosteronismo primitivo, pheochromocytoma/feocromocitoma, adrenocortical carcinoma/carcinoma corticosurrenalico
Settori scientifico-disciplinari MIUR:Area 06 - Scienze mediche > MED/09 Medicina interna
Struttura di riferimento:Dipartimenti > Dipartimento di Medicina
Codice ID:10136
Depositato il:02 Nov 2017 16:42
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