Desmoplastic small round cell tumour: the decisions you may face

Cancer page →Second-opinion finder →

1 treatment setting, 1 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.

Other settings

All cases

8 optionsCompare side by side →

Ewing-type multi-agent chemotherapy, complete cytoreductive surgery with or without hyperthermic intraperitoneal chemotherapy, radiotherapy in some centres; pazopanib, sunitinib or mTOR inhibitors on relapse; trials recommended.

The options, in plain words

VincristineApprovedVinca alkaloid (microtubule inhibitor)

A plant-derived chemotherapy from the Madagascar periwinkle that has been in almost every childhood leukaemia and lymphoma regimen since the 1960s.

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.

CyclophosphamideApprovedAlkylating agent (oxazaphosphorine prodrug)

Cyclophosphamide is an alkylating chemotherapy that damages DNA in dividing cells. It is part of CHOP for lymphoma, AC for breast cancer and VAC for childhood sarcomas, clears lymphocytes before CAR-T and prevents graft-versus-host disease after transplant; bladder bleeding, infertility and secondary leukaemia are its harms.

IfosfamideApprovedCytotoxic chemotherapy (alkylating agent)

Ifosfamide is an alkylating chemotherapy partnered with doxorubicin in sarcoma and with etoposide in Ewing sarcoma, given with a bladder-protecting drug.

EtoposideApprovedTopoisomerase II inhibitor (podophyllotoxin derivative)

Etoposide is a chemotherapy from the mayapple plant, essential to curing testicular cancer (BEP), treating small-cell lung cancer, lymphomas, childhood sarcomas and leukaemias, and used in transplant conditioning.

HIPEC / PIPAC (intraperitoneal chemotherapy)Established

Washing the abdominal cavity with heated chemotherapy during surgery to kill microscopic peritoneal deposits.

High local concentration

PazopanibApprovedSmall-molecule kinase inhibitor (VEGFR/PDGFR/KIT)

Pazopanib is the only multi-kinase inhibitor approved for soft-tissue sarcoma (excluding fat-derived tumours), used after chemotherapy fails.

SunitinibApprovedSmall-molecule multi-kinase inhibitor (VEGFR, PDGFR, KIT)

Sunitinib is an anti-angiogenic pill approved for pancreatic neuroendocrine tumours, kidney cancer and GIST.

Also referenced:Sarcomas (soft tissue, bone, GIST)

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

Vincristine all recorded rates →

Fatal if given intrathecally: label all syringes.

Doxorubicin all 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.

Etoposide all recorded rates →

Reduce to 75% for CrCl 15-50.

HIPEC / PIPAC (intraperitoneal chemotherapy)

Morbidity, centre expertise
Mixed trial results

Pazopanib all recorded rates →

Take on an empty stomach (1 hour before or 2 hours after food).
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
200 mg daily in moderate impairment; avoid in severe.

Sunitinib all recorded rates →

Avoid grapefruit.
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.

Questions to ask about this decision

Between Vincristine, Doxorubicin, Cyclophosphamide and the other options, 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.
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.
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.
For my situation (all cases), which of the standard options do you recommend and why?
Why: Guideline options include: Ewing-type multi-agent chemotherapy, complete cytoreductive surgery with or without hyperthermic intraperitoneal chemotherapy, radiotherapy in some centres; pazopanib, sunitinib or mTOR inhibitors on relapse; trials recommended.
Am I a candidate for Vincristine, Doxorubicin, Cyclophosphamide or related drugs, and what side effects should I expect?
Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

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.

Living with cancer·Standard of care