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Living with cancer·Standard of care

Perivascular epithelioid cell tumour: the decisions you may face

Cancer page →Second-opinion finder →

3 treatment settings, 3 with more than one named option. Each section lays out the options the standard of care names, what each is for, the trials behind them with their recorded results, the side effects and cautions on record, and questions to ask. Built from the cancer page's standard-of-care rows; nothing here is advice for your case.

2 options

Complete resection; surveillance for tumours with malignant features; no proven adjuvant therapy.

The options, in plain words
Limb-salvage surgery and endoprosthetic reconstructionStandard of care

Removing a bone or soft-tissue sarcoma while keeping the arm or leg, rebuilding with metal implants, bone grafts or growing prostheses in children.

  • Preserves function without compromising survival
  • Custom implants for pelvis and spine
Active surveillanceStandard of care

For low-risk prostate cancer, monitoring with PSA, MRI, and repeat biopsy instead of treating, because most such cancers never cause harm.

  • Avoids incontinence and erectile dysfunction of treatment in men who would never be harmed
  • Level-1 evidence of safety (ProtecT)
The evidence behind it

No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.

The main trade-offs on record
Limb-salvage surgery and endoprosthetic reconstruction
  • Implant infection and mechanical failure over decades
  • Requires specialist sarcoma centres
Active surveillance
  • Anxiety and adherence
  • Repeat biopsies
  • Under-used outside high-income countries
Questions to ask about this decision
  1. Between Limb-salvage surgery and endoprosthetic reconstruction and Active surveillance, which do you recommend for me, and what about my case would make you choose differently?
    Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
  2. What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
    Why: The aim shapes how much side effect and disruption is worth accepting.
  3. What happens if I delay, or decline this step for now? Is the decision reversible?
    Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
  4. For my situation (localised), which of the standard options do you recommend and why?
    Why: Guideline options include: Complete resection; surveillance for tumours with malignant features; no proven adjuvant therapy.

Add these to your appointment list, or take the full question set for this cancer.

Advanced, first line

Advanced malignant PEComa

3 optionsCompare side by side →

Nab-sirolimus (AMPECT; FDA approved 2021); oral sirolimus, everolimus or temsirolimus as alternatives; check TSC status.

The options, in plain words
Sirolimus protein-bound particlesApprovedAlbumin-bound mTOR inhibitor (intravenous)

Fyarro is an infusion of the transplant drug sirolimus packaged in albumin particles. It is the first approved treatment for malignant PEComa, a rare soft-tissue tumour driven by loss of the TSC genes.

EverolimusApprovedSmall-molecule mTOR inhibitor

An mTOR-blocking pill that doubled progression-free time with exemestane in 2012 and is now paired with the oral SERD giredestrant.

TemsirolimusApprovedIntravenous mTOR inhibitor (rapamycin analogue)

A weekly infusion that was the first drug to extend survival in poor-risk kidney cancer (2007), and in 2024 the first targeted drug to improve outcomes in childhood rhabdomyosarcoma.

Also referenced:mTOR
The evidence behind it
The main trade-offs on record
Everolimusall recorded rates →
  • Avoid grapefruit. Live vaccines are contraindicated.
  • 7.5 mg (mild), 5 mg (moderate), 2.5 mg (severe).
Questions to ask about this decision
  1. Between Sirolimus protein-bound particles, Everolimus and Temsirolimus, which do you recommend for me, and what about my case would make you choose differently?
    Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
  2. What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
    Why: The aim shapes how much side effect and disruption is worth accepting.
  3. How closely do I match the people in AMPECT, and does that change what the results mean for me?
    Why: Trial populations are selected; age, fitness, prior treatment and biomarkers all affect how far results carry.
  4. What happens if I delay, or decline this step for now? Is the decision reversible?
    Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
  5. For my situation (advanced malignant pecoma), which of the standard options do you recommend and why?
    Why: Guideline options include: Nab-sirolimus (AMPECT; FDA approved 2021); oral sirolimus, everolimus or temsirolimus as alternatives; check TSC status.
  6. Am I a candidate for Sirolimus protein-bound particles, Everolimus, Temsirolimus, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
  7. How do the results of AMPECT apply to someone like me?
    Why: Trial populations differ from individual patients; ask how closely you match.

Add these to your appointment list, or take the full question set for this cancer.

Other settings

After mTOR inhibitor

2 optionsCompare side by side →

Anthracycline- or gemcitabine-based chemotherapy has modest activity; trials of mTOR-based combinations; PRECISION 1 for TSC-altered tumours.

The options, in plain words
DoxorubicinApprovedCytotoxic chemotherapy (anthracycline)

The red chemotherapy drug from a soil bacterium that is still the backbone of treatment for sarcoma, lymphoma and breast cancer, limited by cumulative heart damage.

GemcitabineApprovedNucleoside analogue (deoxycytidine)

A versatile chemotherapy used in pancreatic, bladder, lung, ovarian, breast and biliary cancers, in nasopharyngeal cancer, and as a bladder instillation.

The evidence behind it
The main trade-offs on record
Doxorubicinall recorded rates →
  • Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
  • Reduce by 50% for bilirubin 20-50 µmol/L and 75% for 50-85 µmol/L.
Questions to ask about this decision
  1. Between Doxorubicin and Gemcitabine, which do you recommend for me, and what about my case would make you choose differently?
    Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
  2. What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
    Why: The aim shapes how much side effect and disruption is worth accepting.
  3. How closely do I match the people in Phase 2 Basket Trial of Nab-sirolimus in Patients With Malignant Solid Tumors With Pathogenic Alterations in TSC1/TSC2 Genes (PRECISION 1), and does that change what the results mean for me?
    Why: Trial populations are selected; age, fitness, prior treatment and biomarkers all affect how far results carry.
  4. What happens if I delay, or decline this step for now? Is the decision reversible?
    Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
  5. For my situation (after mtor inhibitor), which of the standard options do you recommend and why?
    Why: Guideline options include: Anthracycline- or gemcitabine-based chemotherapy has modest activity; trials of mTOR-based combinations; PRECISION 1 for TSC-altered tumours.
  6. Am I a candidate for Doxorubicin, Gemcitabine, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
  7. How do the results of Phase 2 Basket Trial of Nab-sirolimus in Patients With Malignant Solid Tumors With Pathogenic Alterations in TSC1/TSC2 Genes (PRECISION 1) apply to someone like me?
    Why: Trial populations differ from individual patients; ask how closely you match.

Add these to your appointment list, or take the full question set for this cancer.

How to read this page. Options and results come from OnCo records with their sources; the settings are the standard-of-care rows on the cancer page, and the lines of therapy are on the sequencing grid. Where a setting names one path, the choice is usually about timing, trials and where to be treated: see expert centres. OnCo is orientation, not medical advice.