# Activity of sirolimus in progressive epithelioid haemangioendothelioma (Italian Rare Cancer Network)

Source: https://onco.cc/key-papers/paper-sirolimus-ehe-stacchiotti-cancer-2021/  
OnCo record `paper-sirolimus-ehe-stacchiotti-cancer-2021` (Key paper). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

In patients with progressing epithelioid haemangioendothelioma, the mTOR inhibitor sirolimus stabilised the disease in most, with a median progression-free survival of about a year, but did not help patients who had developed serosal effusions.

## Summary

Retrospective case series of 38 patients with progressive epithelioid haemangioendothelioma treated with sirolimus within the Italian Rare Cancer Network.

Best response was partial response in 11 percent and stable disease in 71 percent, with median progression-free survival of 13 months and median overall survival of 19 months in patients with serosal effusions against not reached in those without.

## Fields

- Kind: Key paper
- Last checked: 2026-09-17
- Journal: Cancer
- Year: 2021
- DOI: 10.1002/cncr.33247
- Authors: Stacchiotti S, Simeone N, Lo Vullo S, et al.
- Findings: Partial response 11 percent; stable disease 71 percent; median progression-free survival 13 months.; Poor outcome with serosal effusions.
- What it means: Sirolimus is the first-choice systemic therapy for progressing EHE, ideally started before pleural or peritoneal effusions develop.
- Caveats: Retrospective series without a comparator.

## Sources

- Cancer 2021: https://doi.org/10.1002/cncr.33247
- PubMed: https://pubmed.ncbi.nlm.nih.gov/33107985/

## Connected records

- cancers: [Epithelioid haemangioendothelioma](https://onco.cc/cancers/epithelioid-haemangioendothelioma/)
- journals: [Cancer](https://onco.cc/journals/cancer-wiley/)

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