Abstract
Background
Most patients with adrenocortical cancer (ACC) continue to present with advanced disease. Invasion into the inferior vena cava (IVC) defines stage III disease and the management of such patients raises additional difficulties.
Method
A multicentre survey was organized by emailing a standardized proforma to members of the European Society of Endocrine Surgery (ESES). Anonymised retrospective clinical data were collected.
Results
Replies were received from 18 centres in nine countries. ACC with IVC invasion was encountered in 38 patients (18F:20M, age 15–84 years, median 54 years). There were 16 nonfunctioning tumours and 22 functioning tumours predominantly right-sided (26R:12L) and measuring 18–255 mm (median 115 mm). Fourteen patients had metastatic disease at presentation. Tumour thrombus extended in the prehepatic IVC (n = 21), subdiaphragmatic IVC (n = 6) or into the SVC/right atrium (n = 3). Open adrenalectomy was associated with resection of surrounding viscera in 24 patients (nephrectomy n = 16, liver resection n = 14, splenectomy n = 3, Whipple procedure n = 2). IVC was controlled locally (n = 27), at suprahepatic levels (n = 6) or necessitated cardiac bypass (n = 5). Complete resection (R0, n = 20) was achieved in the majority of patients, with a minority having microscopic persistent disease (R1, n = 7) or macroscopic residual disease (R2, n = 4). Perioperative 30-day mortality was 13% (n = 5). Postoperative Mitotane was used in 23 patients and chemotherapy in eight patients. Twenty-five patients died 2–61 months after their operation (median 5 months). Currently, 13 patients are alive at 2–58 months (median 16 months) with known metastatic disease (n = 7) or with no signs of distant disease (n = 6).
Conclusion
This dataset is limited by the lack of a denominator as it remains unknown how many other patients with ACC presenting with IVC invasion did not undergo surgery. The relatively low perioperative mortality and the long disease-free survival achieved by some patients should encourage surgeons with adequate experience to offer surgical treatment to patients presenting with advanced adrenocortical cancers.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Vassilopoulou-Sellin R, Schultz PN (2001) Adrenocortical carcinoma. Clinical outcome at the end of the 20th century. Cancer 92(5):1113–1121
Bilimoria KY, Shen WT, Elaraj D, Bentrem DJ, Winchester DJ, Kebebew E, Sturgeon C (2008) Adrenocortical carcinoma in the United States: treatment utilization and prognostic factors. Cancer 113(11):3130–3136
Icard P, Goudet P, Charpenay C, Andreassian B, Carnaille B, Chapuis Y, Cougard P, Henry JF, Proye C (2001) Adrenocortical carcinomas: surgical trends and results of a 253-patient series from the French Association of Endocrine Surgeons study group. World J Surg 25(7):891–897
Kebebew E, Reiff E, Duh QY, Clark OH, McMillan A (2006) Extent of disease at presentation and outcome for adrenocortical carcinoma: have we made progress? World J Surg 30(7):872–878
Aspinall SR, Imisairi AH, Bliss RD, Scott-Coombes D, Harrison BJ, Lennard TW (2009) How is adrenocortical cancer being managed in the UK? Ann R Coll Surg Engl 91(6):489–493
El-Dhuwaib Y, Lansdown M (2009) Adrenalectomy in England: most are done by surgeons who do less than 5 a year. BAETS abstract book
Koschker AC, Fassnacht M, Hahner S, Weismann D, Allolio B (2006) Adrenocortical carcinoma—improving patient care by establishing new structures. Exp Clin Endocrinol Diabetes 114(2):45–51
Chiche L, Dousset B, Kieffer E, Chapuis Y (2006) Adrenocortical carcinoma extending into the inferior vena cava: presentation of a 15-patient series and review of the literature. Surgery 139(1):15–27
Fassnacht M, Johanssen S, Quinkler M, Bucsky P, Willenberg HS, Beuschlein F, Terzolo M, Mueller HH, Hahner S, Allolio B (2009) Limited prognostic value of the 2004 International Union Against Cancer staging classification for adrenocortical carcinoma: proposal for a Revised TNM Classification. Cancer 115(2):243–250
Sturgeon C, Shen WT, Clark OH, Duh QY, Kebebew E (2006) Risk assessment in 457 adrenal cortical carcinomas: how much does tumor size predict the likelihood of malignancy? J Am Coll Surg 202(3):423–430
Favia G, Lumachi F, D’Amico DF (2001) Adrenocortical carcinoma: is prognosis different in nonfunctioning tumors? Results of surgical treatment in 31 patients. World J Surg 25(6):735–738
Brix D, Allolio B, Fenske W, Agha A, Dralle H, Jurowich C, Langer P, Mussack T, Nies C, Riedmiller H, Spahn M, Weismann D, Hahner S, Fassnacht M (2010) Laparoscopic versus open adrenalectomy for adrenocortical carcinoma: surgical and oncologic outcome in 152 patients. Eur Urol 201058(4):609–615
Turbendian HK, Strong VE, Hsu M, Ghossein RA, Fahey TJ 3rd (2010) Adrenocortical carcinoma: the influence of large vessel extension. Surgery 148(6):1057–1064
Conflicts of interest
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Mihai, R., Iacobone, M., Makay, O. et al. Outcome of operation in patients with adrenocortical cancer invading the inferior vena cava—a European Society of Endocrine Surgeons (ESES) survey. Langenbecks Arch Surg 397, 225–231 (2012). https://doi.org/10.1007/s00423-011-0876-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00423-011-0876-6