Published online Feb 10, 2018. doi: 10.5306/wjco.v9.i1.20
Peer-review started: September 7, 2017
First decision: October 30, 2017
Revised: November 5, 2017
Accepted: December 4, 2017
Article in press: December 4, 2017
Published online: February 10, 2018
A 60-year-old female patient with a past medical history significant only for poorly controlled hypertension despite medical therapy presented with a large left adrenal mass incidentally discovered on MRA during workup for renal artery stenosis.
The clinical examination of the patient revealed elevated blood pressure and abdominal pain.
The differential diagnosis includes renal artery stenosis, adrenal phenochromcytoma, adrenocortical carcinoma, hyperaldosteronism, and hyperthyroidism.
Serology values revealed normal urine cortisol [14.1 mcg/24 h (normal range: 3-22)], low adrenocorticotropic hormone (ACTH) [5.1 pg/mL (normal range: 9-50)], normal plasma metanephrines [0.20 nmol/L (normal range < 0.50)] and high aldoesterone [38.8 ng/dL (normal range: 1-16)].
Abdominal MR imaging revealed a heterogeneous, partially necrotic left adrenal mass measuring 5.6 cm × 8.9 cm as well as multiple bilobar enhancing liver metastases.
Histologic and immunologic evaluation of a biopsied hepatic lesion showed metastatic carcinoma positive for inhibin, melan-A, and CKAE1/3 with an immunohistochemical staining profile consistent with metastatic adrenocortical carcinoma.
We present the first reported case of utilizing yttrium-90 microsphere selective internal radiation therapy (SIRT) in combination with first line EDP-M (Etoposide, Doxorubicin, Cisplatin, Mitotane) chemotherapy and debulking surgical primary tumor resection for treatment of metastatic adrenocortical carincoma.
While surgical resection can be curative for early local disease, most patients present with advanced ACC owing to nonspecific symptoms. For those patients, previous reports studied treatment options including systemic chemotherapy and locoregional therapies including radiofrequency ablation and transarterial chemoembolization.
Adrenocortical carcinoma (ACC) is a rare malignancy that constitutes less than 5% of all adrenal incidentalomas and has an estimated annual incidence of 1-2 cases per million people. EDP-M (etoposide, doxorubicin and cisplatin plus mitotane) is first line chemotherapy for advanced adrenocortical carcinoma demonstrating an overall response rate of 49%, a median time to progression of 9.1 mo, and an overall survival of 28.5 mo in prior studies.
This case study presents the successful treatment of metastatic ACC by combining Y-90 locoregional therapy for bilobar liver disease with first line EDP-M chemotherapy and debulking surgery. This strategy allowed for disease management for at least 12 mo following a single outpatient Y-90 treatment for each hepatic lobe rather than the repeated inpatient treatments required previously through TACE with possibly superior results.