Urologia Internationalis
Urol Int 2008;81:244-246 DOI: 10.1159/000144071
Received: March 9, 2007 Accepted after revision: March 15, 2007
Cardiac and Caval Invasion of Left Adrenocortical Carcinoma
Ismet Yavascaoglua Mert Yilmazb Yakup Kordana
Departments of ªUrology and bCardiovascular Surgery, Faculty of Medicine, Uludag University, Bursa, Turkey
Key Words
Adrenocortical carcinoma . Thrombus . Invasion . Inferior vena cava
Abstract
Adrenocortical carcinoma (ACC) is a rare and highly malig- nant neoplasm. We present the case of a 51-year-old male patient with a left-sided ACC admitted to hospital with ipsi- lateral flank pain, weight loss, difficulty in breathing, ab- dominal discomfort and swelling and bilateral leg edema. Thoracoabdominal computed tomography revealed a huge adrenal mass with obvious tumor thrombus involvement of the inferior vena cava and right atrium. This is the first report describing caval and opposite side renal vein invasion of a left-sided ACC treated with grafting of the vessels. Histo- pathological examination of the tumors confirmed the diag- nosis of ACC. Postoperative recovery was uneventful. The patient received an adrenolytic agent, mitotane, postopera- tively and is alive with no evidence of recurrence after 2 years of follow-up. Copyright @ 2008 S. Karger AG, Basel
Introduction
Adrenocortical carcinoma (ACC) is a rare and highly malignant neoplasm with an incidence of 1-2 cases per million people per year worldwide [1]. These tumors are
highly malignant, with 50-70% exhibiting locally ad- vanced lesions at the time of presentation [2]. Resembling the renal cell adenocarcinoma, ACC also has the ability to develop venous tumor thrombus extension, and one third of primary ACC cases present with tumor throm- bus within the inferior vena cava (IVC) [3]. The identifi- cation of intracaval thrombus is paramount to the suc- cessful treatment of ACC, since complete local excision is the best therapy for advanced local disease [4]. By the evo- lution of cardiopulmonary bypass (CPB) and circulatory arrest the surgical removal of these thrombi becomes possible and bloodless dissection and inspection of the IVC has helped to reduce the risk of tumor thromboem- bolus [3].
Case Report
A 51-year-old man presented with a 6-month history of left- sided flank pain, weight loss, difficulty in breathing, abdominal discomfort, and swelling and bilateral leg edema. Abdominal computed tomography (CT) confirmed a 12 × 18 × 16 cm adre- nal mass with obvious involvement of the IVC. Magnetic reso- nance imaging (MRI) revealed a level III (intraatrial) tumor thrombus extending into the IVC and up into the right atrium (fig. 1). Hormonal evaluation was conducted, and all laboratory values were found within normal limits. The bone scan was nor- mal, but chest radiography and chest CT revealed two pulmonary nodules on the right side.
The patient was relatively young and suffering from the ob- structive symptoms of vena caval thrombus. The patient under-
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Mert Yilmaz, MD
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went a left thoracoabdominal radical nephrectomy, adrenalecto- my, and en bloc removal of level III thrombus (in the IVC and extending into the right atrium). During the operation, midline laparotomy incision was made and extended to the suprasternal notch. Then, midline sternotomy was performed. CPB was insti- tuted by using ascending aorta, superior vena cava, and right fem- oral vein cannulation. Systemic temperature was reduced till 26°C. The superior vena cava was snared and the right atrium opened. The tumor arising from the IVC was seen and freed from the atrial and IVC attachments. Unfortunately, there was a tumor invasion at the infrahepatic IVC, including orifices of the left re- nal and right renal vein. A short period of total circulatory arrest (8 min) was performed while the tumor together with right renal vein and a 6-cm segment of the IVC was excised. Polytetraflou- roethylene (PTFE) tube graft (20 mm) was anastomosed in an end-to-end fashion to the distal end of the IVC. The graft was clamped and CPB restarted. The saphenous vein graft was inter- posed between the right renal vein and the tube graft. The tube graft was then anastomosed in an end-to-end fashion to the prox- imal IVC. The right atrium was closed and the patient weaned off CPB at 37℃ in sinus rhythm without any inotropic support. Pa- thology confirmed ACC. The patient was discharged to home 9 days after surgery and MRI angiography revealed the functioning right renal vein (interposed saphenous vein graft) and IVC (PTFE graft interposed) (fig. 2). At last follow-up, 2 years after surgery, the patient was undergoing treatment with mitotane adjuvant chemotherapy (4.5 g/day) and was alive without any gross side ef- fect of mitotane and there is no evidence of recurrence and any change in the pulmonary nodules. And also, all the tumor-in- duced symptoms were almost completely regressed.
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Discussion
ACC is a rare, highly malignant tumor with poor prognosis related to delay in diagnosis and lack of effec- tive adjuvant treatment [5]. Although prognostic factors are unclear, curative surgical resection can improve sur- vival rate [3, 5, 6]. Involvement of the IVC is seen in 20- 30% of the patients [7]. However, there is no clear evi- dence that it signifies poor prognosis [5]. Moreover, Haak et al. [8] reported that total resection of the ACC provides better survival advantage over the subtotal resection (5- year survival: 49 vs. 9%, respectively).
Inferior vena caval tumor thrombus involvement can occur by either direct invasion or, more commonly, by intraluminal extension of the tumor thrombus via the adrenal or renal veins [6]. Due to the barrier effect of the intimal layer of the vena cava, the tumor thrombus usu- ally spreads via intraluminal extension, can quickly achieve supradiaphragmatic extension and grow in a mushroom fashion within the right atrium [3]. Inferior vena caval tumor thrombus extension is seen more com- monly on the right-sided tumors, since the right adrenal vein is shorter [6].
Chiche et al. [5], after reviewing the current literature, suggested palliative surgery to symptomatic patients
without severe comorbidity in whom complete locore- gional resection of primary ACC can be achieved. De- tailed scrunity of the literature revealed that there are few cases in which resection of the IVC with graft replace- ment was performed [9, 10]. In all these cases, the tumor was located on the right side and in 1 case tumor exten- sion was up to the right atrium [9]. Chiche et al. [5] re- ported 15 cases and reviewed the literature regarding this rare pathology. Only 3 cases (all were right-sided) re- quired resection of the vena cava and graft replacement, and none needed opposite side renal vein resection and graft replacement in this review. This is the first ACC case in which the IVC and opposite side renal vein were replaced.
Chiche et al. [5] also reported that 52.3% of patients with IVC extensions who underwent resection were alive
with metastasis or no evidence of recurrence after a mean follow-up time of 23.1 ± 20.4 months. Although the re- sponse rate of mitotane therapy has been reported to be up to 35% with mostly partial and transient responses, its clinical efficacy remains disputed [6]. However, in the lit- erature there are cases in which complete lasting remis- sion was achieved after mitotane treatment [6]. Despite mitotane’s significant side effects, such as lifelong steroid replacement, treatment with mitotane should be consid- ered for cases of metastatic disease.
In conclusion, a detailed preoperative workup should be done before attempting such an aggressive surgical procedure. In carefully selected patients who require pal- liation because of thrombus extension of ACC, surgical therapy might facilitate adjuvant therapies even in cases of metastases and may provide a survival benefit.
References
1 Dackiw AP, Lee JE, Gagel RF, Evans DB: Ad- renal cortical carcinoma. World J Surg 2001; 25:914-926.
2 Cohn K, Gottesman L, Brennan M: Adreno- cortical carcinoma. Surgery 1986;100:1170- 1177.
3 Hedican SP, Marshall FF: Adrenocortical carcinoma with intracaval extension. J Urol 1997;158:2056-2061.
4 Bodie B, Novick AC, Pontes JE, Straffon RA, Montie JE, Babiak T, Sheeler L, Schumacher P: The Cleveland Clinic experience with ad- renal cortical carcinoma. J Urol 1989;141: 257-260.
5 Chiche L, Dousset B, Kieffer E, Chapuis Y: Adrenocortical carcinoma extending into the inferior vena cava: presentation of a 15- patient series and review of the literature. Surgery 2006;139:15-27.
6 Figueroa AJ, Stein JP, Lieskovsky G, Skinner DG: Adrenal cortical carcinoma associated with venous tumour thrombus extension. Br J Urol 1997;80:397-400.
7 Cahill PJ, Sukov RJ: Inferior vena caval in- volvement by adrenal cortical carcinoma. Urology 1977;10:604-607.
8 Haak HR, Hermans J, van de Velde CJ, Lentjes EG, Goslings BM, Fleuren GJ, Krans HM: Optimal treatment of adrenocortical carcinoma with mitotane: results in a con- secutive series of 96 patients. Br J Cancer 1994;69:947-951.
9 Friedrich MG, Dill H, Unverdorben M, En- gels G, Scheele H, Bachmann K: Adrenal car- cinoma with intravenous extension into the tricuspid valvular plan in a patient with pat- ent foramen ovale. Eur Heart J 1994;15:708- 709.
10 Huguet C, Ferri M, Gavelli A: Resection of the suprarenal inferior vena cava. The role of prosthetic replacement. Arch Surg 1995;130: 793-797.
Erratum
In the article ‘Fibroepithelial polyp of distal ureter with periodic prolapse into bladder’ (Urol Int 2008;80:338-340), the name of the second author was misspelled. It should now read: Apostolos Kafetsoulis.
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