Dipartimento dell’Emergenza e dei Trapianti d’OrganoSezione di Urologia e Trapianto di Rene

Università di Bari

La chirurgia del surrene

Pasquale Ditonno Michele Battaglia

Seminari di Fisiopatologia ChirurgicaBari – 10-11 Novembre 2011

ADRENALECTOMYANTERIOR APPROACH

1927

1936ADRENALECTOMY

POSTERIOR APPROACH

TRANSABDOMINALLAPAROSCOPIC

ADRENALECTOMY1991

ROUX in LausaneMAYO in Rochester

YOUNG

A SURGICAL HISTORY

Snow

1.Cagner2.Mercan 1992

1.LATERAL TRANSABD.2.POSTERIOR RETROPER.

LAPAROSCOPIC

1563– Eustachius describes the anatomy of the adrenalGland

1855 – Addison correlates clinical features of adrenaldisease with pathology found in autopsies

1886 – Frankel describes pheochromocytoma

1912 – Cushing presents clinical features ofHypercortisolism

1955 – Conn describes hyperaldosteronism

Advances during the last fifteen years have completely changed our understanding of and approach…

In the past the diagnosis was complex, time consuming and, sometimes, In the past the diagnosis was complex, time consuming and, sometimes, frustating process, especially with regard to localizationfrustating process, especially with regard to localization

Scint.Scan. US,CT,MR,

Interdisciplinary collaboration

Incidentaloma’s increased incidence

1986 2010

36,6% 58,2%

Indicazioni alla chirurgia

“Surgery of the adrenal gland consists of operative procedures to ‘correct endocrine abnormalities’ or to ‘treat malignant disease’.

When medical therapy is ineffective or does not exist for a particular adrenal disease, surgery becomes necessary.”

Chow and Blute, Surgery of the adrenal glands, Campbell Urology, 9th Ed

IS IT MALIGNANT?

IS IT METASTATIC?

IS IT FUNCTIONAL?

SURRENECTOMIA: LA NOSTRA ESPERIENZA (Gennaio 1978-Ottobre 2011)

386 Surrenectomie

188 dx – 188 sx – 10 Bilaterali184 M – 202 F – Età media: 54 aa (r: 14-82)

Distribuzione per tipo di accesso

lombotomico: 160 Anteriore: 26 Toraco-addominale: 5

Laparoscopico: 195

Laparoscopic adrenalectomy the “platinum standard”Cestari et al. Curr Opin Urol. 2005 Mar;15(2):69

Benign Adenoma 50%

Cyst 10%

Myelolipoma 10%

Pheochromocytoma 10%

Metastases 6-30%

Adrenal Cancer 0.01%

The incidental adrenal mass. Am J Med 1996

Major Causes

Posizione: Non sul rene, ma mediale al rene, sottodiaframmatica, dinanzi al pilastro laterale

Quadrilatero di Albarran

Fegato - Milza

Margine med.polo super.del rene

CavaAorta

Peduncolo renale

E’ contenuto in una propria loggia, inclusa in quella renale, senza tuttavia stretti rapporti

Tale loggia e’ separata dal polo superiore del rene da tessuto fibroadiposo ancorato al diaframma

Vie di accesso al surrene

• addominale

transperitoneale

extraperitoneale

• lombotomica

• toraco-freno-laparotomica

• posteriore

• laparoscopica ( trans- e retroperitoneale)

Approccio laparoscopico: i vantaggi

Analgesici

Hospital stay

Recovery Time

Soddisfazionedel paziente

Cosmesi

Elementi da considerare qualunque sia tipo di accesso

Tipo di patologia (Carcinoma)

Sede (mono, bilaterale, ectopica)Volume della lesione

Body Mass Index Friabilità dell’organo

Attenta emostasi Esperienza del chirurgo

Ampiezza del campo operatorio Dominio dei vasi Possibilità di estensione dell’accesso

La chirurgia del surreneLa chirurgia del surrene

L’approccio chirurgico

Patologia

Patients 1004

Non-Secretory 854 (85%)

Pheochromocytoma 42 (4.2%)

Sub-Clinical Cushings Syndrome 92 (9.2%)

Aldosterone-Producing Adenoma 15(1.6%)

Journal of Clinical Endocrinology & Metabolism 85 (2) 637-644, 2000

L’incidentaloma surrenalico

“ “ Yes, doctor, the abdominal scan was negative for the Yes, doctor, the abdominal scan was negative for the problem you were worried about, but we problem you were worried about, but we

found another one of those adrenal masses”found another one of those adrenal masses”

A.I.D.S.A.I.D.S.Adrenal incidentaloma discovered serendipitouslyAdrenal incidentaloma discovered serendipitously

Su 87.065 autopsie in 24 studi è riportata una incidenza del 6%.Nelle ecografie addominali una incidenza dello 0,6-1.3 %

Kloss RT et al. Endocr Rev 1995; 16:460

La probabilità della diagnosi correla con l’età:0.2 % in soggetti di età tra 20 e 29 aa

7 % in soggetti di età superiore ai 70 aa

Qual è l’approccio diagnostico ottimale?

Non esiste uno specifico algoritmo diagnosticoL’approccio diagnostico si basa su:esperienza clinica del medico indagini di laboratorio e di imaging

Young WF, NEJM 2007; 356

Sindrome di Cushing subclinica

Il termine subclinico si riferisce alla presenza di una secrezione autonoma di cortisolo in pazienti che non presentano i caratteristici segni dell’ipercortisolismoper un nodulo iperfunzionante capace di sopprimere il surrene controlaterale senza elevare il cortisolo sierico e determinare la classica sindrome

Se lo stato di soppressione ormonale non viene riconosciuto, dopo la surrenectomia può comparire una crisi addisoniana

Tuttavia questi pazienti spesso presentano gli effetti dellapersistente secrezione endogena di cortisolo:• ipertensione• obesità• diabete mellito• osteoporosi

Emral R. Endocr J 2003;50:399-408

Sindrome di Cushing subclinica

Strategie terapeutiche

Chirurgia

Wait and see

Pazienti giovaniPazienti sintomatici

Pazienti anzianiPazienti asintomatici

E’ un tumore raro con caratteristiche cliniche peculiari allo stesso tempo una condizione curabile e potenzialmente letale

Per curare la malattia:Per curare la malattia:•Diagnosi precoce•Localizzazione precisa•Appropriata preparazione anestesiologica pre and post-operatoria•Rimozione chirurgica completa

Fattori di rischio:Fattori di rischio:

•Rilascio di catecolamine imprevisto ed incontrollabile

•Possibilità di malattia multifocale e comportamento maligno

FEOCROMOCITOMA

Hypertension In Pheochromocytoma

• Paroxysmal in 48%--episodically secreted• Persistent in 29%-- continually secreted• Normal in 13%

FEOCROMOCITOMA

• Attacks of Headaches (80%)• Palpitations (64%)• Diaphoresis (57%)

Symptomatic Triad Of Headache, Sweating, And Tachycardia In A Hypertensive Patient

Sensitivity 90.9% And Specificity 93.8%

Feocromocitoma clinicamente silente

circa il 5% degli incidentalomi surrenalici

Dosaggio delle metanefrine frazionate e catecolamine nelle urine delle 24 oreImaging phenotype

Imaging

Alta densità alla TAC

Elevata vascolarizzazione

Ritardato washout del MC

Alta intensità nelle sequenze T2-pesate (RMN)

Surrenectomia per Feocromocitoma1959-2011

Approccio Transperitoneale 53

Approccio lombotomico 47

Laparoscopia (dal 2000) 13

Pz

Bambini 12

Adulti 101

Totale 113

Sede surrenalica 100

Sede extra-surr. 8

Bilaterale 6

Maligni 16

Rare tumor (0.5-2 case per million)

An extraordinarily aggressive malignancy with an overall poor prognosis

Despite aggressive surgical therapy, the actuarial 5-year survival for patients who undergo complete resection ranges 23% to 48%

Incomplete resection (including removal of adjacent, involved organs) is associated with a median survival of less than 1 year

Adrenal carcinoma

Dackiw AP, World J Surg 2001;25:914–926

• 342 Patients With Adrenal Incidentaloma Retrospectively Evaluated

• Tumor Diameter Averaged 2.5 cm

• Most Malignant Tumors Measured > 5 cm

• Removing All Tumors > 4 cm Would HaveRemoved Eight Benign Tumors For Every Carcinoma

The Mayo Clinic Study

Incidentally discovered adrenal tumors: an institutional perspective.Herrera MF; Grant CS; van HeerdenJA; Sheedy PF; Ilstrup DM. Surgery 1991 Dec;110(6):1014-21

• Masses > 6 cm Usually Are Treated Surgically

• Masses < 4 cm Are Generally Monitored

• Masses Between 4 And 6 cm:Criteria Other Than Size Should Be Considered In Making The Decision To Monitor Or Proceed To Surgery

• Experienced investigators now recommend excision of all tumors >4 cm

Mass

National Institutes Of HealthManagement Of The Clinically Inapparent Adrenal Mass (Incidentaloma) 2002

Assessment del potenziale maligno

In uno studio condotto su 2005 pazienti con incidentaloma surrenalico un carcinoma era presente nel 4,7% dei soggetti e il 2,5% risultava affetto da malattia metastatica

Young WF, Endocrinol Metab CNA 2000; 29:159

Dimensioni

Imaging phenotype

>4 cm 90% sensibilitàBassa specificità

Forma irregolareDensità mista alla TACElevata vascolarizzazioneRitardato washout del MCAlta intensità nelle sequenze T2-pesate (RMN)

Distribuzione delle neoplasie maligne in rapporto alle dimensioni

-

50

100

150

200

250

300

0-4 4-8 8-12 > 12

N° cases

Diametro (cm)

19 Malignancy

386 Surrenectomie

Initial case reports: early postop development of carcinomatosis in all 5 pts with an incidentally discovered primary AC undergone laparoscopy for a presumed benign adrenal mass (Conn’s [2]; Cushing’s [2]; virilizing tumor[1]).

3 of 5 suffered local recurrence, and 1 port-site recurrence, after 4-14 months after laparoscopy

Applicability of laparoscopy, with the possibility of tumor fracture or inadequate resection of adjacent organs, has been questioned

Ushiyama T, J Urol 1997;157:2239.Hofle G, Horm Res 1998;50:237–241.

Hamoir E, Ann Chir 1998;52:364–368.Deckers S, Horm Res 1999; 52:97–100.

Foxius A, Surg Endosc 1999;13:715–717.

When enthusiasm for laparoscopy turns to tragedy

An incidentally discovered primary Adrenal Carcinoma

MD Anderson Cancer Center:

at a median follow up of 28 months, comparison of recurrence rates for laparoscopic and open resection of AC.

Open group: 86% of patients recurred with 62% dying of mets and 24% alive with disease. Of these, 35% with local rec, 8% carcinomatosis, and remaining with mets.

Lap group: 6 pts 100% recurred, with higher percentage (83%) carcinomatosis At a follow up of 15 months, 66% pts died from mets, and 33% alive with disease

Open group: 6 pts with tumors <6 cm 4 of 6 were disease-free at 21 monthsLap group: 5 pts with tumors <6 cm developed local rec, distant, and/or peritoneal mets

In 2 of 6 cases, tumor fracture, rupture, or uncontrolled hemorrhage reported

Gonzalez RJ, Laparoscopic resection of adrenal cortical carcinoma: a cautionary note. Surgery 2005;138:1078–1086.

Gill recently described 31 pts undergone lap adrenalectomy for malignancy; 26 isolated adrenal mets, 6 primary incidental adrenal ca, and 1 malignant pheo.

The overall local recurrence rate was 23%.5 of 26 (19%) patients with adrenal mets2 of 6 (33%) with adrenal ca.

Patients with local rec also recurred at other (systemic) sites

Pts with local rec had a lower 3-year survival than pts without local recurrence (17% vs 66%, P 0.016).

Overall 5-year actuarial survival was 40% at a median follow up of 26 months.

Moinzadeh A, Gill IS. J Urol 2005;173:519–525.

A contemporary review of the literature between 1998 to 2004 reveals 25 cases of AC resected laparoscopically Local recurrence and/or intraperitoneal dissemination occurred in 10 of 25 (40%) pts

The disease-free interval averaged 34.1 months.

Henry JF, World J Surg 2002;26:1043–1047.Prager G, Arch Surg 2004;139:46–4961. Zeh HJ 3rd, Udelsman R.Ann

Surg Oncol 2003;10:1012–1017.Suzuki K. Biomed Pharmacother 2002; 56(suppl):139s-144s.

Kebebew E, Arch Surg 2002;137:948–951.Belldegrun A, Surg Gynecol Obstet 1986;163:203–208.

Valeri A, Surg Endosc 2002;16:1274–1279.

Despite arguments in favor of applying minimally invasive approaches

to the majority of adrenal lesions the laparoscopic resection of primary adrenal

malignancies remains controversial.

Gonzalez RJ, Surgery 2005;138:1078–1086.

Adrenal glands are common sites for mets in a number of primary cancers, but an isolated metastasis is rare

Resection of isolated adrenal mets of melanoma, lung, kidney, colon, and breast cancer may improve survival

In series of open adrenalectomy for mets, median survival of 30 months vs historical survival of 6-8 months without resection

Higashiyama M, Int Surg1994;79:124–129

Adrenal Metastasis

Adrenalectomy for mets from RCC are associated with the most favorable results

Lo CY, Br J Surg 1996;83:528–53

Recognition that most malignancies metastasize to the medullary portion, rather than cortex, rarely penetrate the capsule of the gland

To date, 8 series totaling 98 patients have reported the use of lap adrenalectomy for mets with no port-site recurrences and only 1 patient

(1%) developing peritoneal dissemination

laparoscopic resection less likely to result in tumor fracture,which predispose to local recurrence or intraperitoneal dissemination

Greene FL, CA Cancer J Clin 2007;57:130–146

DFS ranged from 42% to 91% over a mean followup interval of 8 to 26 months

Diverse considerazioni fanno tendere verso una aggressione laparoscopica nelle metastasi surrenaliche

Aumentata esperienza del chirurgo Migliore visione Immediato controllo dei vasi Possibilità di esplorare la cavità peritoneale per eventuali metastasi

Completo controllo del tumore primitivo

Metastasi isolata al surrene

Possibilità di resezione completa del surrene interessato

Chirurgia laparoscopica “organ sparing”

Accurata emostasi (coagulazione bipolare, colla di fibrina, EndoGIA, bisturi ad ultrasuoni)

Indicazioni: Piccoli AdenomiSindrome di CushingFeocromocitoma bilaterale

ConclusioniMigliore accuratezza delle tecniche diagnosticheIncremento incidentalomiNeoplasie maligne anche in piccole masse

La chirurgia rimane il gold standard nella cura delle masse surrenaliche ma con:

minori costi di degenzamigliori risultati esteticiminore morbidita’minori tempi operatori

sostituendo l’ approccio chirurgico tradizionale con quello laparoscopico

L’approccio multidisciplinare permette di ottenere i migliori risultati