Adrenocortical carcinoma surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

Overview

Surgery is the mainstay of treatment for adrenocortical carcinoma.

Surgery

• Surgery is the mainstay of treatment for adrenocortical carcinoma.
• Surgery can also be performed even in the case of the invasion into large blood vessels, such as the renal vein or inferior vena cava.
• A large percentage of patients are not surgical candidates.

Appropriate preoperative evaluation and operative planning are the most important to assure the best outcome.

Preoperative imaging should be obtained to evaluate the extent of the tumor.

Imaging should be done just before the surgery because ACC grow quickly and involvement of adjacent structures may change

Optimization of hormone excess in patients with functional masses especially those with Cushing’s syndrome

Patients widespread distant metastatic disease in multiple organs or those with multiple metastatic deposits in one organ system unable to be completely resected should not undergo adrenalectomy.

The primary tumor can instead be treated with external beam radiation for palliation along with other adjuncts to improve local symptoms and better control hormone excess,

if present (247).

Obstruction of the vena cava by tumor thrombus can lead to significant lower body edema, which leads to significant patient suffering. If tumor resection is not technically operable, vena cava stents can be placed, leading to temporary prevention of occlusion.

Debulking for control of hormone excess in the setting of known metastatic disease is also performed in some situations. The benefits of debulking must outweigh the risks of surgery in these patients who have poor wound healing and lengthy recovery periods due to preexisting debilitation

1.  Surgical approach

Adrenalectomy

1. Incision and exploration of the peritoneal cavity.

2. Evaluation of liver for metastasis

3. Containment A self-retaining retractor system with towels or laparotomy pads should be placed in such a way as

to exclude the rest of the peritoneal cavity from the area of the tumor and other organs requiring

resection.4. Mobilization of organs

adjacent to tumor

5. En bloc resection. Preserve any tissue overlying the tumor.

6. Regional lymphadenectomy

7. Provide intact en bloc specimen for pathologic review

8. Mark field to facilitate postsurgical external beam radiation therapy

9. Dictate operative report

2.  Lymph node dissection

The role of lymph node sampling or formal regional

lymph node dissection in the treatment of ACC remains

unknown (248). the lymphadenectomy is performed based on following the arterial supply of adrenal tumors

Because lymph nodes ideally should be removed as part of

the en bloc resection, surgeons need to individually balance

the increased risk due to extended surgery (eg, bleeding)

with the presumed benefit of radical lymph node dissection.

regional lymph node dissection improved tumor

staging ability and led to a more favorable oncological

outcome in patients with otherwise localized ACC. (249)

3. Open vs laparoscopic surgery

LA has

become the gold standard for resection of benign adrenal

masses, and it has been shown to result in significantly

lower morbidity, less pain, shorter hospital stays, and decreased

overall time to recovery when compared with

open adrenalectomy (OA).

Application of laparoscopic instruments to the tumor

can result in shedding of malignant cells that is undetectable

to the operating surgeon.

Minimizing direct contact with the tumor

surface is important so as not to abrade cells from the

tumor surface or enter the tumor capsule.

Some surgeons compromise by initiating adrenalectomies

laparoscopically to assess for evidence of intraperitoneal

metastasis or invasion of the adrenal gland into

other organs (246, 251).

Published data comparing the efficacy of LA vs OA for

ACC are limited.

At least 7 studies have been published since 2010 that

specifically address the topic of LA vs OA for ACC.

Two studies published by the M.D. Anderson Cancer Center

reported a recurrence rate of 86% in the OA group (154

patients) and 100% in the LA group (6 patients) (251,

257). Recent recommendations

by the American Association of Clinical Endocrinologists

and the American Association of Endocrine

Surgeons advocate OA by an experienced surgeon as the

procedure of choice (254).  Conversely, the European Society

of Endocrine Surgeons and European Society for

Medical Oncology suggest LA could be performed for

stage 1 and 2 ACC tumors less than 8 or 10 cm if an R0

resection is performed and surrounding periadrenal tissue

removed (255, 256).

Local recurrence and peritoneal carcinomatosis was

more common in the LA group. In a study by Leboulleux

et al (258), peritoneal carcinomatosis occurred in only

25% of patients treated by OA, as opposed to 60% of

patients who underwent LA.

In contrast, other studies reported evidence that LA

may be comparable to OA in patients with stage 1 and 2

ACC based on no significant difference in recurrence-free

survival (259, 260).

Surgical studies should focus on local and peritoneal

recurrence as indicators of quality of surgical resection,

because type of operative approach likely has a much

smaller role in the development of distant metastases. A

retrospective study from the University of Michigan reviewed

88 ACC patients, 17 of whom underwent LA, and

79% of the operations were performed at outside facilities,

and no laparoscopic operations for ACC were performed

at the University of Michigan, potentially introducing

a referral bias (262). Although overall recurrence

rates were similar and despite on average smaller tumors

in the LA group (7.0 cm) compared with the OA group

(12.3 cm), the LA group had a significantly earlier recurrence

(9.2 vs 19.2 months). Furthermore, there were more

R1 or R2 resections or notation of intraoperative tumor

spill (50% vs 18%). These data suggest that although LA

may be technically feasible (even for large tumors) (263,

264), the use of LA in ACC leads to a shorter disease-free

interval and a higher incidence of incomplete resections.

These results were confirmed in an extended follow-up

study of 110 patients undergoing OA and 46 undergoing

LA. After LA,30%had positive margins or intraoperative

tumor spill compared with 16% of OA patients despite

larger tumors and more stage 3 tumors. Overall survival

for patients with stage 2 ACC was longer in those undergoing

OA, and time to visible tumor bed recurrence or

peritoneal recurrence in stage 2 patients was shorter in LA

patients.

In summary, existing data are inconclusive and more

studies are needed to better judge the equivalence of LA to

OA. In accordance with the experience gained at the authors’

institution, a conservative approach using an open

approach is recommended for all adrenocortical lesions

that cannot be classified as benign before surgery.

4. Surgery for recurrent disease

Extent of disease and tempo of disease progression

guide the decision for reoperation in the setting of recurrence.

The number of organs involved by tumor at the time

of the first metastasis is a predictor of survival (243, 265).

In addition, University of Michigan data show the site of

first metastasis can also be used to predict survival,

with those having tumor recurrence in the peritoneum outside

the tumor bed having the worst survival.

Surgery is indicated in those patients with disease confined to 1 site or organ.

Beyond that, decisions regarding resection must be individualized. The type of initial operative resection is important to the decision-making process for reoperation.

Patients with tumor bed recurrence who have undergone

LA are much more likely to have disease too small to be

detected by imaging elsewhere in the peritoneal cavity

compared with those having undergone OA based on our

experience.

median survival of 74 months (5-year survival, 57%) in those undergoing complete second resections vs a median survival of 16 months (5-year survival, 0%) in those undergoing incomplete second resection. tumor grade nor additional nonsurgical treatment

Tumor grade influences the decision for reoperation

because it correlates with survival (243, 245).

In those with low-grade tumors, tempo of disease progression can be slower and lead to longer survival with resection of sites of recurrence or metastasis.

those patients with high-grade tumors, other sites of disease often appear quickly.

wait 3 months while treating with chemotherapy to assess for tumor responsiveness and/or tempo of progression. If progression is not rapid, surgery may proceed with greater benefit, whereas those with evidence of