Two tumours of the right adrenal gland treated videoscopically by the posterior retroperitoneal approach

Introduction
Pheochromocytomas contribute to as little as 0.2% of arterial hypertension; however, due to the potential threat of sudden release of catecholamines to the bloodstream, they are described as a biological time-bomb. Thus, there is a need for screening in all patients with signs and symptoms suggesting a possibility of hormonally active pheochromocy-toma, such as: headache, excessive sweating or palpitations [1]. An additional problem lies in differentiating benign from malignant adrenal tumours. Both presented reasons warrant surgical excision of all diagnosed pheochromocytomas [2].
The possibility of bifocal or multifocal pheo-chromocytomas is widely known in the literature [3]. Coincidence of pheochromocytoma with contralateral adrenal adenoma has rarely been reported [4-6]. Presence of both tumours within one gland has been described only in a few patients [7, 8] and so far there have been no reports on their simultaneous radical excision by a videoscopic posterior retroperitoneal approach.
Case report
A 49-year old female patient complained of arterial hypertension, palpitations and occasional collapses. Laboratory tests (Table I) showed elevated values of metanephrines and normetanephrines in daily urine, with no other hormonal or electrolytic abnormalities. On ultrasound scan, in the field of the right adrenal gland, a hypoechogenic tumour 29 × 34 mm was found, as well as a second heterogenic lesion 40 × 43 mm. Imaging studies were completed with computed tomography (Figure 1). It showed a focal lesion 40 × 41 × 37 mm with density of 12-15 Hounsfield units, with heterogeneous contrast enhancement, modelling the inferior vena cava. Additionally a hypodense 34 × 30 × 34 tumour lying posteriorly and cephalad in close proximity of the first one was visualised.
The patient was prepared during two weeks using an a-blocker (doxazosin) and underwent an elective operation. Videoscopic adrenalectomy by the posterior retroperitoneal approach (PRA) was performed (Figure 2). After clipping of the central vein, the right adrenal gland with both tumours was resected totally (Figures 3, 4). The postoperative period was uneventful. On the second postoperative day the drain was removed and the patient was discharged. The skin sutures were removed on the 7th postoperative day (Figure 5). Final pathological examination revealed pheochromocytoma without features of malignant lesion (Figure 6) and cortical adenoma (Figure 7).
Discussion
The presented case illustrates an extremely rare coincidence of two tumours of the ipsilateral adrenal gland. Only a few cases of pheochromocytoma and cortical adenoma in the same gland have been reported in the literature. Cotesta et al. [7] presented two cases of unilateral presence of both pathological types of adrenal tumours and their resection by an open approach. Hwang et al. [8] describes a patient with a small right cortical adenoma, not diagnosed preoperatively, lying next to a large pheochro-mocytoma, treated classically. Twice a left-sided adrenal tumour with two pathological components – partially that of pheochromocytoma and partially adenoma – has been reported [9, 10]. In one of those cases videoscopic adrenalectomy by PRA was performed [10].
Videoscopic technique is generally considered a gold standard in treatment of benign adrenal tumours [11]. It has many advantages over open surgery, such as: shorter hospitalization time, decreased postoperative morbidity, better cosmetics and quicker convalescence. Several methods of videoscopic approach are used: lateral transperitoneal adrenalectomy (LTA), anterior transperitoneal, lateral retroperitoneal and PRA [12]. Lateral transperitoneal adrenalectomy is the most commonly used [13]. Its main advantages are: large operative field and easy orientation based on landmarks known from open surgery [12]. The main difficulties in the trans-peritoneal approach are: obesity and adhesions after previous laparotomies [14].
In the presented case right videoscopic adrena-lectomy by PRA was performed using three trocars. This method has been popularized by Walz [15] and is presently used in a growing number of reference centres [13, 16-18]. A prospective randomized trial, conducted by Rubinstein et al. [14], showed that videoscopic retroperitoneal technique is comparable to transperitoneal one in aspects such as: operative time, estimated blood loss, analgesic requirements, hospital stay and the complication rate. The main limitation of both methods is the tumour size [19].
The main advantages of PRA are: direct access without need for mobilisation of abdominal organs, avoiding intra-abdominal adhesions and feasibility even in obese patients [18]. In a comparative study, Lombardi et al. [13] observed that patients returned to work faster after videoscopic adrenalectomy by PRA as compared to LTA. Rubinstein et al. [14] described postoperative hernias in 2 out of 25 pa-tients operated on using LTA, which was not observed in a group of 32 patients in whom PRA was performed.
As with every technique, PRA also has some minor drawbacks. Lombardi et al. [13] observed that values of arterial partial pressure of both carbon dioxide and oxygen were greater at the end of PRA as compared to LTA. Although it might bother the anaesthesiologist, increased carbon dioxide at completion of PRA does not have any negative impact on the postoperative period [13]. Another disadvantage of PRA is the need to become familiar with a new operative field, not known from open surgery [12]. However, once this is achieved, PRA turns out to be an easy and safe procedure. This was proven by Walz et al. [15], who performed 560 pro-cedures of PRA with no mortality. Standardization of the technique and gained experienced allowed Walz and his team to shorten the operative time to 40 ±15 min using 3 trocars and no postoperative drainage in most cases. In this setting even when bilateral adrenalectomy is needed, PRA has been advocated, especially in patients after previous laparotomies [13].
Application of modern instruments allows further minimization of morbidity and improvement of cosmesis by performing the PRA from single access [20], although the possibilities of wide use of this method are still limited. Also the recently introduced technique of natural orifice transluminal endoscopic surgery (NOTES) has been attempted during adrenalectomy in porcine and cadaver models [21, 22]. The first preclinical results demonstrate the feasibility and apparent safety of the procedure. Whether this will be confirmed in patients requires clinical studies.
The extent of resection may range from tumour enucleation to total adrenalectomy [23, 24]. As partial adrenalectomy becomes increasingly popular, our finding of two ipsilateral tumours emphasizes the importance of detailed preoperative imaging and evaluation of hormonal status.
In this paper, we report for the first time right adrenal pheochromocytoma and ipsilateral cortical adenoma, treated videoscopically by PRA. The presented case demonstrated a need for detailed preoperative diagnostics of all adrenal focal lesions. The performed operation proves that resection of bifocal right adrenal tumour by PRA is feasible and safe.
References
1. Pacak K, Linehan WM, Eisenhofer G, et al. Recent advances in genetics, diagnosis, localization, and treatment of pheo-chromocytoma. Ann Intern Med 2001; 134: 315-29.
2. Pacak K, Eisenhofer G, Ahlman H, et al.; International Symposium on Pheochromocytoma. Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005. Nat Clin Pract Endo-crinol Metab 2007; 3: 92-102.
3. Hoegerle S, Nitzsche E, Altehoefer C, et al. Pheochro-mocytomas: detection with 18F DOPA whole body PET – initial results. Radiology 2002; 222: 507-12.
4. Berbarie RF, Emmett M, Breslau NA. Quiz page. Secondary hypertension with contralateral pheochromocytoma and aldosteronoma. Am J Kidney Dis 2005; 46: A51, e1-3.
5. Morimoto S, Sasaki S, Moriguchi J, et al. Unique association of pheochromocytoma with contralateral nonfunctioning adrenal cortical adenoma. Am J Hypertens 1998; 11: 117-21.
6. Wolf A, Willenberg HS, Cupisti K, et al. Adrenal pheochro-mocytoma with contralateral cortisol-producing adrenal adenoma: diagnostic and therapeutic management. Horm Metab Res 2005; 37: 391-5.
7. Cotesta D, Petramala L, Serra V, et al. Pheochromocytoma associated with adrenocortical tumor in the same gland. Two case reports and literature review. Minerva Endocrinol 2006; 31: 183-9.
8. Hwang WR, Ma WY, Tso AL, et al. Pheochromocytoma and adrenocortical adenoma in the same gland. J Chin Med Assoc 2007; 70: 289-93.
9. Inoue J, Oishi S, Naomi S, et al. Pheochromocytoma associated with adrenocortical adenoma: case report and literature review. Endocrinol Jpn 1986; 33: 67-74.
10. Sato H, Igarashi H, Kishimoto Y, et al. Combined tumor consisting of non-functioning adrenocortical adenoma and pheochromocytoma in the same gland. Int J Urol 2002; 9: 398-401.
11. Wang DS, Terashi T. Laparoscopic adrenalectomy. Urol Clin North Am 2008; 35: 351-63.
12. Zacharias M, Haese A, Jurczok A, et al. Transperitoneal laparoscopic adrenalectomy: outline of the preoperative management, surgical approach, and outcome. Eur Urol 2006; 49: 448-59.
13. Lombardi CP, Raffaelli M, De Crea C, et al. Endoscopic adrenalectomy: Is there an optimal operative approach? Results of a single-center case-control study. Surgery 2008; 144: 1008-14.
14. Rubinstein M, Gill IS, Aron M, et al. Prospective, randomized comparison of transperitoneal versus retroperitoneal laparoscopic adrenalectomy. J Urol 2005; 174: 442-5.
15. Walz MK, Alesina PF, Wenger FA, et al. Posterior retroperitoneoscopic adrenalectomy – results of 560 proce-dures in 520 patients. Surgery 2006; 140: 943-8.
16. Barczyński M, Konturek A, Golkowski F, et al. Posterior retroperitoneoscopic adrenalectomy: a comparison between the initial experience in the invention phase and introductory phase of the new surgical technique. World J Surg 2007; 31: 65-71.
17. López PJ, Pierro A, Curry JI, Mushtaq I. Retroperitoneoscopic adrenalectomy: An early institutional experience. J Pediatr Urol 2007; 3: 96-9.
18. Perrier ND, Kennamer DL, Bao R, et al. Posterior retroperitoneoscopic adrenalectomy: preferred technique for removal of benign tumors and isolated metastases. Ann Surg 2008; 248: 666-74.
19. Ramacciato G, Paolo M, Pietromaria A, et al. Ten years of laparoscopic adrenalectomy: lesson learned from 104 procedures. Am Surg 2005; 71: 321-5.
20. Walz MK, Alesina PF. Single access retroperitoneoscopic adrenalectomy (SARA)-one step beyond in endocrine surgery. Langenbecks Arch Surg 2009; 394: 447-50.
21. Perretta S, Allemann P, Asakuma M, et al. Adrenalectomy using natural orifice translumenal endoscopic surgery (NOTES): A transvaginal retroperitoneal approach. Surg Endosc 2009; 23: 1390.
22. Zacharopoulou C, Nassif J, Allemann P, et al. Exploration of the retroperitoneum using the transvaginal natural orifice transluminal endoscopic surgery technique. J Minim Invasive Gynecol 2009; 16: 198-203.
23. Toutounchi S, Cieśla W, Krajewska E, et al. Wyłuszczenie laparoskopowe pojedynczego guza nadnercza u pacjentki z pierwotnym hiperaldosteronizmem. Videosurgery and other miniinvasive techniques 2007; 2: 164-7.
24. Walz MK, Peitgen K, Diesing D, et al. Partial versus total adrenalectomy by the posterior retroperitoneoscopic approach: early and long-term results of 325 consecutive procedures in primary adrenal neoplasias. World J Surg 2004; 28: 1323-9.