Malignant mixed epithelial and mesenchymal tumor of corpus uteri

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Overview

Malignant mixed epithelial and mesenchymal tumor of the corpus uteri, also known as malignant mixed Müllerian tumor (MMMT) or uterine carcinosarcoma, is a rare and aggressive neoplasm arising in the body of the uterus. This tumor is characterized by the presence of both malignant epithelial (carcinomatous) and malignant mesenchymal (sarcomatous) components within the same mass. The carcinomatous component may include endometrioid, serous, or clear cell patterns, while the sarcomatous component can be homologous (resembling normal uterine tissues such as endometrial stroma or smooth muscle) or heterologous (containing elements foreign to the uterus such as cartilage, bone, or skeletal muscle). The disease primarily affects the female reproductive system, specifically the uterine corpus, and can spread to the pelvic and abdominal organs, lymph nodes, and distant sites. Patients typically present with abnormal or postmenopausal vaginal bleeding, pelvic pain, and a rapidly enlarging uterus. A mass may protrude through the cervical os. The disease predominantly affects postmenopausal women, with a median age of diagnosis in the sixth to seventh decade of life. Risk factors may include prior pelvic radiation, obesity, and exogenous estrogen use. Diagnosis is established through histopathological examination of tissue obtained by biopsy or hysterectomy. Treatment generally involves total abdominal hysterectomy with bilateral salpingo-oophorectomy, often accompanied by pelvic and para-aortic lymph node dissection for surgical staging. Adjuvant therapy may include chemotherapy (commonly platinum-based regimens combined with ifosfamide or paclitaxel) and/or radiation therapy, depending on the stage and extent of disease. Despite multimodal treatment, the prognosis remains poor, with high rates of recurrence and metastasis. Ongoing clinical trials continue to explore novel therapeutic approaches, including targeted therapies and immunotherapy, to improve outcomes for patients with this challenging malignancy.

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Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Late onset

Begins later in life, typically after age 50

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Malignant mixed epithelial and mesenchymal tumor of corpus uteri.

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No actively recruiting trials found for Malignant mixed epithelial and mesenchymal tumor of corpus uteri at this time.

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Specialists

1 foundView all specialists →

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

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Common questions about Malignant mixed epithelial and mesenchymal tumor of corpus uteri

What is Malignant mixed epithelial and mesenchymal tumor of corpus uteri?

Malignant mixed epithelial and mesenchymal tumor of the corpus uteri, also known as malignant mixed Müllerian tumor (MMMT) or uterine carcinosarcoma, is a rare and aggressive neoplasm arising in the body of the uterus. This tumor is characterized by the presence of both malignant epithelial (carcinomatous) and malignant mesenchymal (sarcomatous) components within the same mass. The carcinomatous component may include endometrioid, serous, or clear cell patterns, while the sarcomatous component can be homologous (resembling normal uterine tissues such as endometrial stroma or smooth muscle) or

How is Malignant mixed epithelial and mesenchymal tumor of corpus uteri inherited?

Malignant mixed epithelial and mesenchymal tumor of corpus uteri follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Malignant mixed epithelial and mesenchymal tumor of corpus uteri typically begin?

Typical onset of Malignant mixed epithelial and mesenchymal tumor of corpus uteri is late onset. Age of onset can vary across affected individuals.

Which specialists treat Malignant mixed epithelial and mesenchymal tumor of corpus uteri?

1 specialists and care centers treating Malignant mixed epithelial and mesenchymal tumor of corpus uteri are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.