The role of immune checkpoint inhibitor therapy in advanced adrenocortical carcinoma revisited: review of literature
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REVIEW
The role of immune checkpoint inhibitor therapy in advanced adrenocortical carcinoma revisited: review of literature E. P. Brabo1 · A. B. Moraes2 · L. V. Neto2 Received: 16 April 2020 / Accepted: 21 May 2020 © Italian Society of Endocrinology (SIE) 2020
Abstract Purpose Adrenocortical carcinoma (ACC) is a rare disease with few therapeutic options. There is an urgency of new effective therapeutic options for these patients. The role of immune checkpoint inhibitors (ICI) in advanced ACC patients is still unclear. Methods We conducted a MEDLINE search using the following string: adrenocortical carcinoma and immunotherapy or checkpoint inhibitors. Results We found four case series comprising 10 patients, and four prospective studies totaling 115 patients. The response rate (RR) in the group of 10 patients was 1 complete response, 3 partial response (PR), 4 stable disease (SD), and 2 progressive disease (PD). The median progression-free survival (mPFS) ranged from 2 to 31 months and the median overall survival (mOS) ranged from 4.3 to 31 months. The results in the 115 patients from prospective trials was variable, the PR ranged from 6 to 23%, the SD ranged from 18 to 50% and overall disease control rate ranged from 30 to 64%. The mPFS reported varied from 1.8 to 2.6 months while the mOS varied from 10.6 to 24.9 months. There were five patients with sustained response for more than 24 months. The most common treatment-related adverse event (TRAE) was the increase in liver enzymes. No treatment-related deaths were reported. Better results in terms of RR and survival were observed in studies that used pembrolizumab. No predictive biomarker of response was found up to now. Conclusion ICI, mainly pembrolizumab, is a potential therapeutic option, which is safe and associated with prolonged OS benefit, in selected patients with advanced ACC. Keywords Immune checkpoint inhibitors · Immunotherapy · Adrenocortical carcinoma · Anti-programmed cell death
Introduction Adrenocortical carcinoma (ACC) is a rare disease with few therapeutic options. Surgery for localized disease is the only chance of cure, but even these patients have a high recurrence rate. The disease is very heterogeneous and even in * L. V. Neto [email protected] 1
Oncology Unit and Neuroendocrine Section, Clementino Fraga Filho University Hospital, Federal University of Rio de Janeiro, 255 Professor Rodolpho Paulo Rocco Street, ground floor, University City, Rio de Janeiro, RJ 21941‑913, Brazil
Department of Internal Medicine and Endocrine Unit, Medical School and Clementino Fraga Filho University Hospital, Federal University of Rio de Janeiro, 255 Professor Rodolpho Paulo Rocco Street, 9th floor, University City, Rio de Janeiro, RJ 21941‑913, Brazil
2
the context of metastatic disease some patients may present long survival [1]. Clinical and pathological prognostic factors such as stage, completeness of resection, Weiss Score, Ki67 labeling index, and functioning status are well known [2], but molecular ones are still waiting for clini
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