# Uterine carcinosarcoma

Source: https://onco.cc/cancers/uterine-carcinosarcoma/  
OnCo record `uterine-carcinosarcoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Uterine carcinosarcoma is a two-faced cancer with a carcinoma part and a sarcoma-like part that both come from the same faulty epithelial cell. It is treated as a high-grade endometrial cancer, with surgery, carboplatin-paclitaxel and often radiotherapy, and its frequent HER2 expression is opening a route to antibody-drug conjugates.

## Summary

Carcinosarcoma contains both a carcinomatous component, usually serous or high-grade endometrioid, and a sarcomatous component, which may resemble fibrosarcoma, leiomyosarcoma or heterologous tissue such as cartilage or skeletal muscle. Sequencing shows that both components share the same TP53, PIK3CA, FBXW7 and PPP2R1A mutations, so the tumour is a carcinoma that has undergone epithelial-to-mesenchymal transition rather than a true sarcoma, and it is classified and staged as an endometrial carcinoma. Almost all are p53-abnormal; a minority are mismatch-repair deficient and a few POLE-ultramutated, and HER2 is expressed or amplified in a substantial minority. Patients are older than average, tamoxifen exposure and prior pelvic radiotherapy are risk factors, and the tumour often presents as a polypoid mass protruding through the cervix.

Surgery with hysterectomy, salpingo-oophorectomy, nodal assessment and omental sampling is followed by chemotherapy for almost every stage. Ifosfamide-paclitaxel had been the standard after GOG-0161, but GOG-0261 showed carboplatin-paclitaxel non-inferior and less toxic, and it is now the regimen of choice. Radiotherapy improves local control and is added for pelvic-confined disease with risk factors, and the ESGO/ESTRO/ESP guideline treats carcinosarcoma as p53-abnormal high-risk disease for adjuvant decisions. RUBY included carcinosarcoma among its histologies, so dostarlimab with chemotherapy is an option in advanced disease, with the largest benefit in the mismatch-repair-deficient minority.

HER2 is the most promising target. The Japanese STATICE trial gave trastuzumab deruxtecan to HER2-expressing carcinosarcoma and reported responses in about half of patients, and DESTINY-PanTumor02 included carcinosarcoma in its endometrial cohort, which underpins the tumour-agnostic approval for HER2 3+ tumours. Trials of WEE1 and ATR inhibitors exploit the p53-null cell cycle, and PARP inhibition is being tested on the same homologous recombination logic as in ovarian cancer. Rarity keeps carcinosarcoma out of most dedicated randomised trials, so its evidence base is borrowed from serous endometrial cancer.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: Malignant mixed Mullerian tumour; MMMT; Endometrial carcinosarcoma
- Tags: subtype-page
- Group: gynaecologic
- Burden: About five percent of uterine cancers but a far larger share of deaths; most patients are older women, half present with disease beyond the uterus, and even stage I tumours recur in a third of cases.
- Subtypes: Homologous carcinosarcoma (sarcoma element resembles uterine tissue); Heterologous carcinosarcoma (cartilage, bone or skeletal muscle elements); Serous-type carcinoma component (most common, p53-abnormal); HER2-expressing carcinosarcoma (trastuzumab deruxtecan); Mismatch-repair-deficient carcinosarcoma (minority, immunotherapy-responsive); Stage I carcinosarcoma (adjuvant chemotherapy with or without radiotherapy)
- Biomarkers: p53 immunohistochemistry (abnormal in most); HER2 immunohistochemistry and in situ hybridisation; MMR immunohistochemistry (deficient in a minority); POLE (rare, favourable); Percentage of sarcomatous component and heterologous elements; CA-125

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/uterine-carcinosarcoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/uterine-carcinosarcoma/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/uterine-carcinosarcoma/#what-it-is [6 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/uterine-carcinosarcoma/#finding-it [6 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/uterine-carcinosarcoma/#treating-it [3 settings, 3 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/uterine-carcinosarcoma/#evidence [4 trials, 3 key papers, 5 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/uterine-carcinosarcoma/#science [15 targets]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/uterine-carcinosarcoma/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/uterine-carcinosarcoma/#living-with-it [16 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/uterine-carcinosarcoma/coming/ [7 medicines, 3 trials, 1 idea, 3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/uterine-carcinosarcoma/data/ [50 connected records]

## Standard of care

- Surgery: Hysterectomy with bilateral salpingo-oophorectomy, sentinel node mapping or lymphadenectomy, and omental sampling. ([Hysterectomy](https://onco.cc/terms/hysterectomy/), [Sentinel lymph node biopsy](https://onco.cc/technologies/sentinel-node/), [Robotic & minimally invasive surgery](https://onco.cc/technologies/robotic-surgery/))
- Adjuvant, all stages: Carboplatin-paclitaxel (GOG-0261), with pelvic radiotherapy and vaginal brachytherapy for stage I to III disease with risk factors; ifosfamide-paclitaxel is the older alternative. ([Carboplatin](https://onco.cc/drugs/carboplatin/), [Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Brachytherapy](https://onco.cc/technologies/brachytherapy/), [PORTEC-3](https://onco.cc/trials/portec-3/))
- Advanced or recurrent: Carboplatin-paclitaxel with dostarlimab (RUBY included carcinosarcoma); trastuzumab deruxtecan for HER2-expressing disease; lenvatinib-pembrolizumab after platinum. ([Dostarlimab](https://onco.cc/drugs/dostarlimab/), [RUBY / ENGOT-EN6 / GOG-3031](https://onco.cc/trials/ruby/), [Trastuzumab deruxtecan](https://onco.cc/drugs/trastuzumab-deruxtecan/), [DESTINY-PanTumor02](https://onco.cc/trials/destiny-pantumor02/), [HER2](https://onco.cc/targets/her2/), [Lenvatinib](https://onco.cc/drugs/lenvatinib/), [Pembrolizumab](https://onco.cc/drugs/pembrolizumab/))

## State of the art

- Genomics reclassified carcinosarcoma from sarcoma to a metaplastic carcinoma, so it follows endometrial cancer guidelines.
- GOG-0261 replaced ifosfamide-paclitaxel with carboplatin-paclitaxel.
- HER2-directed antibody-drug conjugates are the first targeted therapy to show activity.

## Open problems

- No dedicated randomised trials for a tumour that behaves worse than the serous cancers it is grouped with.
- Whether the sarcomatous component needs different drugs.
- High relapse rate after apparently complete treatment of stage I disease.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Uterine_carcinosarcoma
- Wikipedia: https://en.wikipedia.org/wiki/Uterine_carcinosarcoma

## Connected records

- cancers: [Advanced or recurrent endometrial cancer](https://onco.cc/cancers/advanced-recurrent-endometrial-cancer/), [Endometrial cancer](https://onco.cc/cancers/endometrial/), [Endometrial cancer with no specific molecular profile](https://onco.cc/cancers/endometrial-nsmp/), [Mismatch-repair-deficient endometrial cancer](https://onco.cc/cancers/endometrial-mmr-deficient/), [p53-abnormal endometrial cancer, including uterine serous carcinoma](https://onco.cc/cancers/endometrial-p53-abnormal/), [POLE-ultramutated endometrial cancer](https://onco.cc/cancers/endometrial-pole-ultramutated/)
- key papers: [DESTINY-PanTumor02: trastuzumab deruxtecan in HER2-expressing solid tumours](https://onco.cc/key-papers/paper-destiny-pantumor02-jco-2024/), [GOG-0261: paclitaxel-carboplatin versus paclitaxel-ifosfamide in uterine carcinosarcoma](https://onco.cc/key-papers/paper-gog-0261-carcinosarcoma-powell-jco-2022/), [RUBY: dostarlimab with chemotherapy for advanced or recurrent endometrial cancer](https://onco.cc/key-papers/paper-ruby-nejm-2023/)
- drugs: [Carboplatin](https://onco.cc/drugs/carboplatin/), [Dostarlimab](https://onco.cc/drugs/dostarlimab/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [Lenvatinib](https://onco.cc/drugs/lenvatinib/), [Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/), [Pembrolizumab](https://onco.cc/drugs/pembrolizumab/), [Trastuzumab deruxtecan](https://onco.cc/drugs/trastuzumab-deruxtecan/)
- targets: [ATR](https://onco.cc/targets/atr/), [BCLAF1](https://onco.cc/targets/bclaf1/), [FAM135B](https://onco.cc/targets/fam135b/), [HER2](https://onco.cc/targets/her2/), [HIP1](https://onco.cc/targets/hip1/), [SPOP](https://onco.cc/targets/spop/), [TP53](https://onco.cc/targets/tp53/), [WEE1](https://onco.cc/targets/wee1/), [WNT1](https://onco.cc/targets/wnt1/), [ZBTB7B](https://onco.cc/targets/zbtb7b/)
- ideas: [HER2 ADCs as standard for HER2-positive serous endometrial cancer](https://onco.cc/ideas/idea-her2-adc-serous-endometrial/)
- terms: [Hysterectomy](https://onco.cc/terms/hysterectomy/), [POLE ultramutation (POLEmut)](https://onco.cc/terms/pole-ultramutation/)
- technologies: [Brachytherapy](https://onco.cc/technologies/brachytherapy/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Robotic & minimally invasive surgery](https://onco.cc/technologies/robotic-surgery/), [Sentinel lymph node biopsy](https://onco.cc/technologies/sentinel-node/)
- trials: [DESTINY-PanTumor02](https://onco.cc/trials/destiny-pantumor02/), [PORTEC-3](https://onco.cc/trials/portec-3/), [RUBY / ENGOT-EN6 / GOG-3031](https://onco.cc/trials/ruby/), [Ziv-aflibercept in Treating Patients With Locally Advanced, Unresectable, or Metastatic Gynecologic Soft Tissue Sarcoma](https://onco.cc/trials/nct00390234/)

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JSON: https://onco.cc/api/v1/entities/uterine-carcinosarcoma.json