Neoadjuvant everolimus in renal angiomyolipoma with or without tuberous sclerosis complex: Results from a multicenter, retrospective study

Cancer Med. 2024 Sep;13(17):e70181. doi: 10.1002/cam4.70181.

Abstract

Objectives: To assess the efficacy and safety of preoperative neoadjuvant everolimus in renal angiomyolipomas (AML) patients with or without Tuberous Sclerosis Complex (TSC).

Materials and methods: This multi-institutional retrospective study enrolled renal AML patients who underwent partial nephrectomy (PN) or total nephrectomy after receiving at least 1 month of pre-operative everolimus. Imaging evaluations were collected before and after treatment, along with demographic, surgical, and follow-up information. The primary outcome was tumor volume reduction of ≥25%, with additional outcomes including recurrence, perioperative outcomes, renal function, and safety.

Results: From January 2015 to July 2022, 68 renal AML patients were studied-41 with TSC and 27 without. During everolimus treatment, 61.0% (25/41) of TSC patients and 44.4% (12/27) of non-TSC patients achieved tumor reduction of ≥25%. Additionally, 41.5% (17/41) of TSC patients and 18.5% (5/27) of non-TSC patients achieved a ≥ 50% reduction. Three TSC patients and 1 non-TSC patient discontinued treatment due to side-effects. Most patients (92.7% TSC, 85.2% non-TSC) underwent PN. After everolimus treatment, the necessary total nephrectomy decreased to 41.2% (7/17) from baseline. Postoperatively, 1 grade 3 and 3 grade 2 complications occurred, with no grade 4 or 5 complications. After a median follow-up of 24 months, only 1 TSC patient recurred with a diameter >3 cm. Retrospective nature is the major limitation of this study.

Conclusion: Everolimus was effective and well-tolerated in neoadjuvant treatment for renal AML, especially in TSC patients. This neoadjuvant combination strategy of everolimus and PN could effectively controls recurrence and preserves renal function.

Keywords: adverse events; everolimus; neoadjuvant; renal angiomyolipomas; treatment response; tuberous sclerosis complex.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Aged
  • Angiomyolipoma* / drug therapy
  • Angiomyolipoma* / pathology
  • Everolimus* / administration & dosage
  • Everolimus* / adverse effects
  • Everolimus* / therapeutic use
  • Female
  • Humans
  • Kidney Neoplasms* / drug therapy
  • Kidney Neoplasms* / pathology
  • Kidney Neoplasms* / surgery
  • Male
  • Middle Aged
  • Neoadjuvant Therapy* / methods
  • Nephrectomy*
  • Retrospective Studies
  • Treatment Outcome
  • Tuberous Sclerosis* / complications
  • Tuberous Sclerosis* / drug therapy

Substances

  • Everolimus