Radiation Oncology/Adrenocortical

Adrenocortical Carcinoma

Overview

• Rare tumors
  • Incidence 1 per 1,000,000
  • Represent 0.2% of all cancers
  • Up to 14% of adrenal incidentalomas
• Located in the adrenal cortex
• Produce steroids ~50% cases
• Equal in men:women
• Often associated with Beckwith-Wiedemann syndrome
• May be part of MEN I (hyperparathyroidism, endocrine pancreas tumors, pituitary adenomas, adrenal tumors, neuroendocrine tumors)

• UCSF, 2006 PMID 16680602 -- "Extent of disease at presentation and outcome for adrenocortical carcinoma: have we made progress?" (Kebebew E, World J Surg. 2006 May;30(5):872-8.)
  • SEER database review 1973-2000
  • Average age 51.2, 46% men, average tumor size 12 cm (only 4% <6cm)
  • Treatment: 88% surgical resection, EBRT used in 12%
  • Prognostic factors: low tumor grade, low stage, surgical resection
  • Conclusion: no change in presentation, diagnosis, stage over time

Staging

• Tumor (T)
  • T1: Tumor 5 cm or less in size; invasion absent
  • T2: Tumor greater than 5 cm in size; invasion absent
  • T3: Tumor outside adrenal in fat
  • T4: Tumor invading adjacent organs

• Lymph nodes (N)
  • N0: No positive lymph nodes
  • N1: Positive lymph nodes

• Metastases (M)
  • M0: No distant metastases
  • M1: Distant metastases

Treatment

• Surgical
• Adjuvant mitotane
• Use of RT controversial
• Perez 4th Edition:
  • Consider RT: preop in unresectable to downstage, postop with residual disease, postop with high risk features, primary RT for nonsurgical candidates, palliative
  • Dose 50-60 Gy to tumor/tumor bed, nodal drainage including contralateral periaortics
  • Shielding of contralateral kidney

• Italy/Germany, 2007 (1985-2005) PMID 17554118 -- "Adjuvant Mitotane Treatment for Adrenocortical Carcinoma." (Terzolo M, N Engl J Med. 2007 Jun 7;356(23):2372-2380.)
  • Retrospective. 177 patients at 8 centers in Italy and 47 centers in Germany. Radical surgery. Adjuvant mitotane in 47 Italian patients vs. no further treatment in 55 Italian and 75 German patients. Italian patients similar, German older and more Stage I-II
  • Outcome: RFS mitotane 42 months vs. Italian control 10 months vs. German control 25 months
  • Conclusion: Adjuvant mitotane may prolong recurrence-free survival
  • Editorial: While retrospective, well done, and provides compelling rationale for adjuvant low-dose mitotane

• Hahnemann, 1991 (1962-1990) PMID 1709336 -- "Radiation therapy for adjunctive treatment of adrenal cortical carcinoma." (Markoe AM, Am J Clin Oncol. 1991 Apr;14(2):170-4.)
  • Retrospective. 13 patients treated in 2 hospitals, 6 patients with primary/adjuvant RT
  • Conclusion: Postop RT may have improved survival

• Christie Hospital, 1987 (UK) (1968-1981) PMID 3121234 -- "Adrenal cortical carcinoma: survival after radiotherapy." (Magee BJ, Clin Radiol. 1987 Nov;38(6):587-8.)
  • Retrospective. 15 patients, 9 post op RT
  • Outcome: 10-year OS 33%
  • Prognostic: age at diagnosis, hormone production, complete resection

• Michigan, 2011 (1989-2008) PMID 20675074 -- "Adjuvant and definitive radiotherapy for adrenocortical carcinoma." (Sabolch A, Int J Radiat Oncol Biol Phys. 2011 Aug 1;80(5):1477-84.)
  • Retrospective. 58 patients, 10 post op RT
  • Outcome: Lack of radiotherapy use was associated with 4.7 times the risk of local failure compared with treatment regimens that involved radiotherapy
  • Conclusion: Adjuvant radiotherapy should be strongly considered after surgical resection.