Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy. Surgery is the mainstay of treatment, still even after a R0 surgical margins, there is a high risk of recurrence.

A 26-year-old female diagnosed with a large functioning ACC treated with a trimodality approach (surgery, radiotherapy and chemotherapy). Postoperative radiotherapy was proposed regarding the high risk of recurrence. A dose of 50.4 Gy in 28 fractions was delivered to the patient using a volumetric-modulated arc therapy plan. Radiotherapy was safe and well tolerated, and no local recurrence was observed so far (13 months after radiotherapy).

Due to the rarity of ACC and the lack of evidence regarding this entity, therapeutic approach can be challenging. Recent evidence suggests that radiotherapy could have an important role in the therapeutic arsenal.

To determine the clinical effect of post-operative radiotheraphy and systemic chemotherapy for the treatment of salivary duct carcinoma.

Retrospective review.

The medical records of 26 patients treated by surgery with or without radiotheraphy and/or systemic chemotherapy for salivary duct carcinoma were retrospectively reviewed to investigate the role of post-operative adjuvant treatment for the patients' prognosis.

The overall three-year and five-year survival rates were 54 and 48.1 per cent, respectively. There was no correlation with the clinical stage and the patients' prognosis. The overall three-year survival of the patients with or without post-operative radiotheraphy was 64 and 33 per cent, respectively (p = 0.29). The overall three-year survival of the patients with or without post-operative chemotherapy was 53 and 56 per cent, respectively (p = 0.78).

Post-operative adjuvant therapy did not improve the patients' overall prognosis with salivary duct carcinoma. Developing novel treatment modalities may be necessary to improve the prognosis of this aggressive disease.