LCNDG Rapid Review: Sirolimus for malignant perivascular epithelial cell tumours (PEComa)

Source: London Cancer New Drugs Group Area: Evidence > Drug Specific Reviews PEComas are a rare group of tumours with a distinct underlying cell type. The majority of these tumours are benign and removed by surgical excision; however there is a small subset of PEComas that are malignant.   Ideal treatment strategies remain undefined but there could be an emerging role for the use of sirolimus for resected malignant tumours. Published literature in the form of case reports/series suggests that patients (n=11) may achieve some disease control or reduction of tumour burden following treatment with sirolimus. However because of the small numbers of cases reported and the limitations of these data, further investigation in the form of clinical trials is required to define the role of sirolimus with respect to optimal dosing regimen, treatment duration and place in therapy. Given the rarity of these tumours, this approach may not be feasible.
Source: NeLM - Drug Specific Reviews - Category: Drugs & Pharmacology Source Type: news