Adrenal cortex adenocarcinoma is a rare cancer affecting the cortex of the adrenal glands. It has an incidence of 1-2 cases per million inhabitants per year.1 The disease incidence is greatest in the first decade of life and between the ages of 40 to 50 years.2
Treatment can be challenging for patients who present with advanced tumours and rare tumours, such as adrenal cortex adenocarcinoma, as they face a complex interplay of physical, social, psychological and spiritual sequelae.3 For these patients, there may be limited treatment options proven to be effective,3 and patients with metastatic cancer may eventually exhaust treatment options.4 Molecular profiling provides a more personalised approach to cancer management in that it can be used to identify biomarkers in tumour samples. This information can then be used to guide treatment for patients with limited treatment options.5
A 42-year-old male was diagnosed with right adrenocortical carcinoma in 2010. The diagnosis was based on histopathological analysis. Surgical resection of the lesion was performed in December 2010 and no tumour was observed in the surgical margins. Following surgical resection, the patient received mitotane from January 2011 to January 2012. However, the disease recurred with lung and bone metastases being observed. The patient had a grapefruit-sized lesion in the right chest wall between the ribs. This was causing severe pain, leading to a performance status of 3, and the patient was unable to walk unaided.
Initial diagnosis of right adrenocortical carcinoma was based on histopathological analysis. Computed tomography (CT) scans revealed disease recurrence with bone and lung metastases. At the time of progression, another biopsy was taken from the chest wall. This was sent for tumour profiling (Caris Molecular Intelligence). Tumour profiling revealed that the tumour had a low expression of thymidylate synthase (TS) and high expression of topoisomerase I (TOPO1). The detection of these biomarkers led to the decision to treat the tumour with FOLFIRI.
The patient received two lines of therapy with the best known standard regimens prior to molecular profiling of the tumour. Firstly, after the initial surgical resection of the tumour, the patient received mitotane from January 2011 to January 2012. After the disease recurred with lung and bone metastases, cisplatin and etoposide were administered, but no response was observed. When low expression of TS and high expression of TOPO1 were observed with tumour profiling, the patient received FOLFIRI.
Figure 1. CT scans before (left) and after (right) treatment with FOLFIRI.
Outcome and follow-up
The tumour responded to the treatment, with reduction in the size of the tumour present in the chest wall. The patient increased weight and improved in ECOG performance status from 3 to 0.
In 2013 upon presentation of the case at the Medical Oncology Group of Australia’s annual scientific meeting, the patient had continued response to therapy, 346 days after tumour profiling.
- Dean A, Zeps N. Outcomes of molecular profiling-guided treatment in advanced refractory solid cancers: Follow-up and update on a single-centre experience. Poster presented at MOGA Annual Scientific Meeting, Melbourne, 1-2 August 2013. [Full text]
- Allolio B, Fassnacht M. Adrenocortical carcinoma: Clinical update. J Clin Endocrinol Metab. 2006;91(6):2027-37. [Full text]
- Wajchenberg BL, Albergaria Pereira MA, Medonca BB, et al. Adrenocortical carcinoma: Clinical and laboratory observations. Cancer. 2000; 88(4):711-36. [Full text]
- Peppercorn JM, Smith TJ, Helft PR, et al. American Society of Clinical Oncology Statement: Toward individualized care for patients with advanced cancer. J Clin Oncol. 2011;29(6):755-60. [Full text]
- Von Hoff DD, Stephenson JJ, Rosen P, et al. Pilot study using molecular profiling of patients’ tumors to find potential target treatments for their refractory cancers. J Clin Oncol. 2010;28(33):4877-83. [Full text]
- Dean A, Byrne A, Marinova A, et al. Clinical outcomes of patients with rare and heavily pretreated solid tumors treated according to the results of tumor molecular profiling. Biomed Res Int. 2016;2016:4627214. [Full text]