contents
27.1 Introduction . . . 275 27.2 Indications . . . 275
27.3 Bilateral Pheochromocytoma . . . 275 27.4 Solitary Aldosteronomas . . . 277 27.5 Other Tumors . . . 277
27.6 Preoperative Evaluation . . . 277 27.7 Technique . . . 278
27.8 Follow-up . . . 283
27.9 Literature Summary . . . 284 27.10 Conclusions . . . 284
References . . . 285
27.1 Introduction
Partial adrenal resection, also known as adrenal-spar- ing surgery or subtotal adrenalectomy, is the removal of an adrenal tumor with preservation of the normal surrounding adrenal tissue. The aim of a partial adre- nal resection is to safely and completely resect the adrenal mass while leaving intact a functional portion of the gland. Partial adrenalectomy was initially de- scribed using the standard, open technique [8, 22], whereas recent reports refer to a laparoscopic ap- proach [7, 9, 10, 16, 25]. Laparoscopy lends itself nice- ly to partial adrenalectomy because of the high level of magnification that allows for a more detailed inspec- tion of the adrenal gland, aiding in the distinction be- tween the tumor mass and the normal adrenal tissue.
The focus of this chapter is on laparoscopic partial ad- renal resection, with a description of the indications, preoperative evaluation, operative technique, and postoperative follow-up of patients undergoing this procedure. Despite some reported successes with par- tial adrenalectomy, it is important for the reader to keep in mind that the current standard of care for pa- tients undergoing surgery for an adrenal mass is com- plete resection (Fig. 1).
27.2 Indications
The two most common disorders for which a partial- adrenal resection may be performed are bilateral pheo- chromocytoma and unilateral solitary aldosteronoma.
27.3 Bilateral Pheochromocytoma
Both the multiple endocrine neoplasia syndromes and von Hippel Lindau disease predispose to the develop- ment of pheochromocytomas of the adrenal medulla.
Approximately 50% of patients with MEN 2-A and MEN 2-B will develop pheochromocytomas, and un- like those with non-familial disease, patients with an inherited predisposition are far more likely to develop bilateral tumors [3]. These tumors are thought to slowly develop in the background of adrenal medul- lary hyperplasia [2]. Patients who develop bilateral pheochromocytomas require bilateral adrenal resec- tion, following which they are left with essentially no functional adrenal cortical tissue and require lifelong steroid replacement with the attendant side effects of steroids and risk of acute adrenal insufficiency. Be- cause these tumors involve only the adrenal medulla, it is an appealing concept to remove only the tumor it- self and the adrenal medullary tissue, and leave in place the steroid-producing adrenal cortex. Several cases of partial adrenal resection for patients with fa- milial pheochromocytoma have been described [8,10, 16,22].In those patients with synchronous tumors,the bilateral adrenal resection can be done at one opera- tion. In patients with a familial predisposition to pheochromocytoma who develop a unilateral tumor, a partial adrenal resection should still be considered, as approximately one-third of these patients will go on to develop a pheochromocytoma in the remaining gland [3]. The preoperative evaluation and technique of partial adrenal resection for bilateral pheochromo- cytoma is discussed later in the chapter.
Richard A. Hodin, Antonia E. Stephen
Normal
Inferior vena cava Tumor
Renal vein
Tumor
Fig. 1. Illustration of the technique of subtotal adrenalectomy. This was published in one of the earlier case reports describ-
ing open partial adrenal resection. In this case, a 48-year-old airline pilot, who was strongly opposed to requiring steroid
supplementation, underwent a bilateral partial adrenal resection for pheochromocytoma (IVC, inferior vena cava; RV, renal
vein). (From [22], with permission)
27.4 Solitary Aldosteronomas
Partial adrenalectomy has also been described for pa- tients with solitary cortical tumors, most commonly for aldosterone-producing tumors (Conn syndrome).
Unlike patients with bilateral pheochromocytomas, patients with unilateral adrenal tumors are not at risk of steroid dependence following complete resection, provided they have a normal,functioning gland on the other side. However, in the event of a tumor or hemor- rhage of the opposite adrenal gland,a partial resection of the aldosteronoma leaves the patient with func- tional adrenal tissue.Because aldosteronomas are usu- ally small (<2 cm),solitary,and eccentrically located at the margin of the adrenal gland, a limited resection is technically feasible. In addition, these tumors are al- most always benign and are unlikely to recur.As such, some authors have suggested that aldosteronomas can be safely enucleated, leaving the remainder of the gland and its blood supply undisturbed [11]. One re- port of laparoscopic adrenal-sparing surgery in 13 pa- tients with solitary aldosteronomas suggested exclu- sion criteria for partial resection as a diameter greater than 3 cm, tumors located in a concentric position in the gland, and tumors directly attached to the adrenal vein [9]. Using these criteria maximizes the chance of safe enucleation of the tumor with the preservation of a sufficient amount of well-vascularized adrenal tissue.
27.5 Other Tumors
Partial adrenalectomy has been described for patients with adrenal cortical tumors other than aldosterono- ma, including Cushing’s adenomas [7, 25]. Once again, commonly used inclusion criteria include a tumor size less than or equal to 3 cm. In one study, if the tumor was located in the central portion of the gland or the spared remnant was expected to be less than 50% of the total adrenal gland, a partial adrenal resection was not performed [7]. Another study identified the ideal size of a tumor appropriate for partial resection as 1.1–2.5 cm, citing both the difficulty in reliably locat- ing a tumor less than 1 cm in diameter within the gland, and the challenge of resecting a tumor greater than 2.5 cm while preserving functional adrenal tissue [25].
In general, a nonfunctional tumor is a rare indica- tion for partial adrenal resection. It may be indicated in selected cases of a benign tumor such as a gan- glioneuroma or myelolipoma [7]. However, the usual indication for removal of a nonfunctioning adrenal
mass is size greater than 4–5 cm or suspected malig- nancy,both contraindications to partial resection.The possibility of adrenocortical carcinoma or metastatic cancer of the adrenal gland is an absolute contraindi- cation to partial adrenal resection.
27.6 Preoperative Evaluation
The preoperative evaluation of patients being consid- ered for partial adrenalectomy is of paramount im- portance.As with all patients undergoing adrenal sur- gery, a complete hormonal evaluation is performed and localization studies are undertaken to identify the side of the tumor. Once this is done, there are several additional considerations in patients for whom a par- tial adrenal resection is considered. A thin slice (3–5 mm slice interval) computerized tomographic (CT) scan provides excellent detail of the structure of the adrenal and underlying abnormality.With current technology,CT can detect lesions as small as a few mil- limeters in diameter [26]. In cases where a partial re- section is considered, it is imperative to exclude addi- tional lesions or masses in the adrenal gland. For ex- ample, in a patient with a hormonally active tumor on the left side and more than one mass in the left adre- nal gland, it is impossible to determine which mass is productive. In that scenario, a complete adrenal resec- tion should be performed [7]. The location of the tumor within the gland can be helpful in planning the surgical approach; in general,anterior,peripherally lo- cated tumors are technically easier to resect than those located posteriorly or centrally within the gland. The CT should be done both unenhanced and with intra- venous contrast, as it is important to define preopera- tively the location of the tumor in relation to the main adrenal vein.In addition,delayed images are necessary to determine the likelihood of malignancy, an impor- tant consideration in the work-up of any adrenal mass, and of obvious importance in those considered for a partial resection. In order to minimize the chance of performing a partial resection for a malignancy, Jeschke et al. performed partial adrenal resection only on those patients with an adrenal mass with attenua- tion of 11 HU or less on the unenhanced images, and 37 HU or less on the delayed-enhanced images [9].
Several references [12, 14] are cited in the aforemen- tioned study as evidence for the reliability of these cri- teria in distinguishing an adenoma from a carcinoma.
In addition to CT, magnetic resonance imaging
(MRI) is commonly utilized in the work-up of pheo-
chromocytomas, which appear bright on T2-weighted
images.This can be helpful in identifying patients with MEN-2A or familial disorders who may have bilateral lesions. Preoperative MRI of patients with bilateral pheochromocytomas are shown in Fig.2 [10,25].Com- puted tomography, MRI, and nuclear scintigraphy all play an important role in the postoperative follow-up of these patients, as outlined later in the chapter.
27.7 Technique
The technique of laparoscopic adrenalectomy is well described in Chaps. 31–33. Patient positioning, trocar placement, and exposure of the adrenal gland in adre-
nal-sparing resection are the same as that for complete adrenal resection [18].Laparoscopy provides a view of the adrenal gland with parenchymal detail not possi- ble in the open approach, such that the tumor tissue is more readily identified. Both the transperitoneal and retroperitoneal approaches are described for partial adrenal resection.As is true in cases where a complete resection is performed, the transperitoneal approach, as popularized by Gagner and many others, is more commonly used [4]. However, it has been suggested that the retroperitoneal approach may better lend it- self to partial resections by providing a more detailed view of the adrenal and more precise differentiation between normal and abnormal tissue [25].
Intraoperatively, it is imperative to distinguish the normal adrenal tissue from the tumor itself, and to rule out additional lesions in the gland that may not have been apparent on preoperative studies. Intraop- erative ultrasound can be used to examine the entire adrenal gland and hence localize the tumor and define its relation to adrenal vessels and surrounding struc- tures. Intraoperative ultrasound has been described for both partial and complete adrenal resection [5, 7, 10, 16]. Once the adrenal gland is exposed, the intra- operative ultrasound probe is inserted through one of the 10-mm ports and the adrenal gland identified cephalad to the kidney. The tumor is visualized with- in the substance of the adrenal gland and, depending on the type, may appear hyper- or hypoechoic in rela- tion to the normal adrenal tissue [21].The extent of re- section needed and the size of a potential adrenal rem- nant can be accurately assessed. Synchronous lesions prohibiting a partial resection are identified. Follow- ing tumor resection, intraoperative ultrasound is used to inspect the gland to ensure a complete resection.
Due to the small size of many adrenal lesions,and what can be a subtle distinction between normal and ab- normal tissue, the use of intraoperative ultrasound when performing a partial adrenal resection is strong- ly recommended. An example of a preoperative and laparoscopic intraoperative ultrasound in a patient with an aldosterone-producing tumor is shown in Fig. 3 [21].
Several different techniques are described for tu- mor resection in adrenal-sparing surgery. It is gener- ally agreed that manipulation and dissection of the preserved remnant should be avoided as much as pos- sible. Many authors advocate preservation of the adre- nal vein and at least one of the arteries to ensure con- tinued blood supply and function of the remaining tis- sue [1, 7, 19]. In addition, division of the main adrenal vein may cause congestion of the remaining adrenal Fig. 2. a Preoperative magnetic resonance image revealing
bilateral adrenal masses in an 81-year-old woman with bilat- eral pheochromocytomas. (From [10], with permission).
b T2-weighted magnetic resonance image of a young man
with bilateral pheochromocytomas. (From [25], with per-
mission). Both of these patients subsequently underwent
bilateral partial adrenal resection
tissue and difficulty in obtaining adequate hemostasis [7]. In cases of pheochromocytoma, dissection of the gland while the vein is intact may lead to the release of excessive amounts of catecholamines and hemody- namic instability.This risk can be minimized with pre- and intraoperative pharmacologic management along with gentle handling of the tumor [9, 15]. Some au- thors contend that preservation of the main adrenal vein is unnecessary since adequate venous drainage will occur through collateral pathways. Kaouk et al., in a case report of bilateral partial adrenal resection for
pheochromocytoma, describe dividing the left main adrenal vein and preserving the right. There was no additional bleeding noted from the left side, and there were minimal hemodynamic fluctuations as a result of leaving the right vein intact [10]. In a study of 22 pa- tients who underwent laparoscopic partial adrenalec- tomy,the decision of whether or not to divide the main adrenal vein depended on the individual case, taking into account the location, size, and type of tumor. In that report, the adrenal vein was preserved in half of the cases (Table 1) [25].
Fig.3a,b. Ultrasound images of a patient with a right-sided aldosterone-secreting tumor.a Percutaneous ultrasound obtained
with the patient in the lateral decubitus position demonstrates a 2.6 cm adrenal mass above the superior pole of the right kid-
ney. b Intraoperative laparoscopic ultrasound (left panel) further localizes the tumor and clearly shows its relationship to the
inferior vena cava. On the right is a laparoscopic view of the ultrasound probe over the tumor. (From [21], with permission)
Table 1. A summary of the series published by Walz et al. Between 1994 and 1997, 22 patients underwent unilateral subtotal laparoscopic partial adrenal resection using the retroperitoneal approach. Division of the main adrenal vein was decided on an individual case basis, depending on the relationship of the vein to the adrenal tumor. Details of patient follow-up are in- cluded [25]
MEN: multiple endocrine neoplasia.
*Dark area: preserved part.
It should be emphasized that the tissue surrounding the gland must be adequately mobilized and the mar- gins well exposed prior to beginning a partial resection [9].In doing so,it should be kept in mind that excessive manipulation and dissection of the normal adrenal tis- sue can disrupt the blood supply and compromise its long-term function.A number of different instruments and techniques can be used for the tumor excision.One author describes outlining the edges of resection with the bipolar electrocautery in order to demarcate the re- section margin and minimize bleeding from the sur- face of the gland [9].Following cauterization of the sur- face, the tumor is resected with endoscissors. A fine endoscopic instrument with electrocautery,such as en- doscissors, allows for precise dissection along a plane
between normal tissue and tumor. Alternatively, the harmonic scalpel can be used for resection, and may provide better hemostasis (Fig. 4) [10, 25]. Neither the endoscissors nor the harmonic scalpel will provide ad- equate hemostasis for the main adrenal vein, which re- quires suture ligatures, clips, or a vascular stapler. In general, electrocautery or the harmonic scalpel is ade- quate for most of the small vessels, with only the main vein requiring a ligature or clip.
The vascular stapler can also be used for the resec- tion itself, to divide the tissue between the tumor and normal adrenal [7]. This may be most appropriate in cases of small, peripherally located lesions, and will generally achieve excellent hemostasis. Some argue, however, that the relatively large size of the endoscop- ic staplers do not allow for adequate precision in sep- arating normal from abnormal tissue in a gland as small as the adrenal and may risk sacrificing more normal adrenal parenchyma than necessary [9, 10]. In addition, authors who advocate the use of a stapler for resection warn that in patients with familial pheochro- mocytoma and adrenal medullary hyperplasia,the tis- sue may be too thick to safely accommodate division with a vascular stapler. Imai et al. published a report of five patients who underwent laparoscopic partial adrenalectomy using the vascular stapler.All had soli- tary tumors that were either aldosterone or weakly cortisol producing. Patient data and tumor size are shown in Table 2,and drawings depicting the tumor lo- cation within the adrenal are illustrated in Fig.5.All tu- mors were peripherally located, such that it was tech- nically feasible to perform the partial resection using the stapler [7].
An alternative method of resection has been de- scribed by Walther et al. After the laparoscopic ultra- Fig. 4. Technique of partial left adrenalectomy using the har-
monic scalpel. This patient had bilateral pheochromocy- tomas, and underwent bilateral laparoscopic partial resec- tions. The tumor is shown on the left side of the picture, while the arrows indicate the preserved adrenal remnant. (From [10], with permission)
Table 2. A summary of the five patients who underwent laparoscopic partial adrenal resection as published by Imai et al.These patient data emphasize that partial adrenal resection can be performed safely with a complete tumor resection [7]
Case Disease Age Sex Side Operating Blood loss
aTumor Margin
ctime (min) size
b(mm) (mm)
1 APA
d40 M R 210 Minimal 20 5
2 APA
d50 F R 132 Minimal 7 5
3 APA
d56 M L 120 Minimal 23 10
4 WFA
e70 F L 105 Minimal 26 9
5 WFA
e76 M R 145 Minimal 20 5
a
Estimated blood loss very little in all cases.
b
Maximal diameter of tumor.
c
Length between tumor margin and cutting edge.
d
Aldosterone producing adenoma.
e