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Cancers & treatments·Sequencing

Perivascular epithelioid cell tumour (PEComa): lines of therapy

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3 standard-of-care settings across 3 lines and 1 biomarker subgroup. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.

LineAll comers
Early / localised1
Advanced, first line1
Other settings1

Early / localised

SubgroupSettingApproachProducts and trialsEvidence
All comersLocalisedComplete resection; surveillance for tumours with malignant features; no proven adjuvant therapy.
Limb-salvage surgery and endoprosthetic reconstructionActive surveillance
63

Advanced, first line

SubgroupSettingApproachProducts and trialsEvidence
All comersAdvanced malignant PEComaNab-sirolimus (AMPECT; FDA approved 2021); oral sirolimus, everolimus or temsirolimus as alternatives; check TSC status.
Sirolimus protein-bound particlesAMPECTEverolimusTemsirolimusmTOR
91

Other settings

SubgroupSettingApproachProducts and trialsEvidence
All comersAfter mTOR inhibitorAnthracycline- or gemcitabine-based chemotherapy has modest activity; trials of mTOR-based combinations; PRECISION 1 for TSC-altered tumours.
DoxorubicinGemcitabinePhase 2 Basket Trial of Nab-sirolimus in Patients With Malignant Solid Tumors With Pathogenic Alterations in TSC1/TSC2 Genes (PRECISION 1)
90

Lines and subgroups are parsed from the setting text of each standard-of-care row and can misclassify an unusual phrasing; the row’s own setting is always shown. Guideline chips reflect the NCCN category and ESMO-MCBS grade recorded on the cancer page, checked on its stated date. Not medical advice.