Sarcoma of the corpus uteri

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22Specialists8Treatment centers

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Overview

Sarcoma of the corpus uteri is a rare and aggressive malignant tumor arising from the mesenchymal (connective) tissues of the uterine body. Unlike the more common endometrial carcinomas, which originate from the glandular lining of the uterus, uterine sarcomas develop from the smooth muscle, stromal, or other supportive tissues of the uterine wall. The major subtypes include uterine leiomyosarcoma (arising from smooth muscle), endometrial stromal sarcoma (arising from the uterine stroma), and undifferentiated uterine sarcoma. These tumors collectively account for a small percentage of all uterine malignancies but carry a disproportionately poor prognosis compared to endometrial carcinomas. Key symptoms include abnormal uterine bleeding (the most common presenting symptom), a rapidly enlarging pelvic mass, pelvic pain or pressure, and occasionally vaginal discharge. Some patients may present with symptoms related to metastatic disease, as uterine sarcomas have a tendency for early hematogenous spread, particularly to the lungs. The disease primarily affects the reproductive system but can metastasize to the lungs, liver, bones, and peritoneal cavity. Treatment typically involves surgical intervention, with total hysterectomy and bilateral salpingo-oophorectomy being the standard primary approach. The role of lymph node dissection varies by subtype. Adjuvant therapy options include chemotherapy (commonly doxorubicin-based regimens or gemcitabine-docetaxel combinations, particularly for leiomyosarcoma) and radiation therapy, though their benefit in improving overall survival remains debated and is often subtype-dependent. Hormonal therapy may be considered for low-grade endometrial stromal sarcomas, which frequently express hormone receptors. Prognosis depends heavily on the histological subtype, tumor stage at diagnosis, and the completeness of surgical resection. Overall, uterine sarcomas remain challenging to treat, and clinical trials investigating targeted therapies and immunotherapy are ongoing.

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

FDA & Trial Timeline

7 events
Nov 2025A Prospective Validation Study of Radiomics in the Differential Diagnosis of Uterine Leiomyoma and Uterine Sarcoma

Tongji Hospital

TrialNOT YET RECRUITING
Jan 2025Radiomics-Based Non-Invasive MRI Differentiation of Uterine Sarcomas and Fibroids

Tongji Hospital

TrialENROLLING BY INVITATION
Nov 2022Construction of a Model for the Differential Diagnosis of SArcoma/myoma Based on the RAdiomics Features: Single-center Observational Study

IRCCS Azienda Ospedaliero-Universitaria di Bologna

TrialRECRUITING
Aug 2022A Study of Niraparib in People With Soft Tissue Sarcoma Who Have Changes in Their Tumor DNA

Memorial Sloan Kettering Cancer Center — PHASE2

TrialACTIVE NOT RECRUITING
Sep 2021Diagnostic Performances of Preoperative Echo-guided Uterine Biopsy in the Management of Suspect Uterine Sarcoma Tumors (BIOPSAR)

Institut Bergonié — NA

TrialACTIVE NOT RECRUITING
Mar 2020Ultrasound Evaluation of the Myometrium Using the MUSA Terminology Comparison With Histology

Universitaire Ziekenhuizen KU Leuven

TrialRECRUITING
Jan 2020Pelvic Floor Dysfunction and Quality of Life in Uterine Cancer Survivors

M.D. Anderson Cancer Center

TrialACTIVE NOT RECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Sarcoma of the corpus uteri.

View clinical trials →

No actively recruiting trials found for Sarcoma of the corpus uteri at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Sarcoma of the corpus uteri community →

Specialists

22 foundView all specialists →
MM
Margaret von Mehren
Specialist
PI on 4 active trials1 Sarcoma of the corpus uteri publication
MH
Martee Hensley
NEW YORK, NY
Specialist
PI on 4 active trials4 Sarcoma of the corpus uteri publications
RP
Robert Maki, MD, PhD
PHILADELPHIA, PA
Specialist
PI on 8 active trials
RM
Robert Maki
PHILADELPHIA, PA
Specialist
PI on 1 active trial6 Sarcoma of the corpus uteri publications
GM
Gregory P. Sutton, MD
Specialist
PI on 3 active trials1 Sarcoma of the corpus uteri publication
LL
Lilie L Lin
HOUSTON, TX
Specialist
PI on 4 active trials209 Sarcoma of the corpus uteri publications
SM
Shivaani Kummar, MD
PORTLAND, OR
Specialist
PI on 5 active trials
MM
Margaret von Mehren, MD
Specialist
PI on 7 active trials
MM
Mary L. Keohan, MD
NEW YORK, NY
Specialist
PI on 1 active trial
WP
William Petty
Specialist
PI on 1 active trial
SM
Scot C. Remick, MD
SCARBOROUGH, ME
Specialist
PI on 9 active trials
DK
David Kushner
Specialist
PI on 2 active trials4 Sarcoma of the corpus uteri publications
FB
Floor Backes
Specialist
PI on 1 active trial1 Sarcoma of the corpus uteri publication
DF
Debra Barton, RN, PhD, AOCN, FAAN
Specialist
PI on 4 active trials
RM
Robert DeBernardo, MD
TRUMBULL, CT
Specialist
PI on 1 active trial
FM
Francesca Ciccarone, M.D.
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Sarcoma of the corpus uteri.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Sarcoma of the corpus uteri

Disease timeline:

New recruiting trial: Ultrasound Evaluation of the Myometrium Using the MUSA Terminology Comparison With Histology

A new clinical trial is recruiting patients for Sarcoma of the corpus uteri

New recruiting trial: Construction of a Model for the Differential Diagnosis of SArcoma/myoma Based on the RAdiomics Features: Single-center Observational Study

A new clinical trial is recruiting patients for Sarcoma of the corpus uteri

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Common questions about Sarcoma of the corpus uteri

What is Sarcoma of the corpus uteri?

Sarcoma of the corpus uteri is a rare and aggressive malignant tumor arising from the mesenchymal (connective) tissues of the uterine body. Unlike the more common endometrial carcinomas, which originate from the glandular lining of the uterus, uterine sarcomas develop from the smooth muscle, stromal, or other supportive tissues of the uterine wall. The major subtypes include uterine leiomyosarcoma (arising from smooth muscle), endometrial stromal sarcoma (arising from the uterine stroma), and undifferentiated uterine sarcoma. These tumors collectively account for a small percentage of all uter

How is Sarcoma of the corpus uteri inherited?

Sarcoma of the corpus uteri follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Sarcoma of the corpus uteri typically begin?

Typical onset of Sarcoma of the corpus uteri is adult. Age of onset can vary across affected individuals.

Which specialists treat Sarcoma of the corpus uteri?

22 specialists and care centers treating Sarcoma of the corpus uteri are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.