Abstract
Pheochromocytoma and paraganglioma are neuroendocrine neoplasms, originating in the adrenal medulla and in parasympathetic and sympathetic autonomic nervous system ganglia, respectively. They usually present as localized tumours curable with surgery. However, these tumours may exhibit heterogeneous clinical course, ranging from no/minimal progression to aggressive (progressive/metastatic) behavior. For this setting of patients, current therapies are unsatisfactory. Immune checkpoint inhibitors have shown outstanding results for several types of solid cancers. We therefore aimed to summarize and discuss available data on efficacy and safety of current FDA-approved immune checkpoint inhibitors in patients with pheochromocytoma and paraganglioma. After an extensive search, we found 15 useful data sources (four full-published articles, four supplements of scientific journals, seven ongoing registered clinical trials). The data we detected, even with the limit of the small number of patients treated, make a great expectation on the therapeutic use of immune checkpoint inhibitors. Besides, the newly detected predictors of response will (hopefully) be of great helps in selecting the subset of patients that might benefit the most from this class of drugs. Finally, new trials are in the starting blocks, and they are expected to shed in the next future new light on a therapy, which is considered a milestone in oncology.
Highlights
Pheochromocytoma (PHEO) and paraganglioma (PGL) are neuroendocrine neoplasms (NENs), originating in the adrenal medulla and in parasympathetic and sympathetic autonomic nervous system ganglia, respectively.Most patients with PHEO and PGL present with localized tumours that are curable with surgery
We found four short articles published as supplements of scientific journals
We found 952 Registered Clinical Trials (RCTs), seven of which matched the aim of the study
Summary
Most patients with PHEO and PGL present with localized tumours that are curable with surgery. These tumours exhibit heterogeneous clinical course, ranging from licenses/by/4.0/). No/minimal progression to aggressive (progressive/metastatic) behavior, [1], with high morbidity and mortality rates [2]. For this subgroup of patients, several therapeutic strategies for control of tumour burden have been evaluated, and/or clinical trials are ongoing. Some favorable results have been obtained with somatostatin analogues [7], temozolomide [8], sunitinib [9], and everolimus [10]
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