Adenosarcoma of the corpus uteri

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Overview

Adenosarcoma of the corpus uteri, also known as Müllerian adenosarcoma of the uterus, is a rare mixed tumor of the uterine body composed of benign or atypical glandular (epithelial) elements combined with a malignant mesenchymal (stromal) component. This neoplasm primarily affects the uterine corpus and most commonly presents in postmenopausal women, although it can occur at any adult age. The tumor typically arises from the endometrium and may protrude into the uterine cavity as a polypoid mass. Key symptoms include abnormal uterine bleeding (most commonly postmenopausal bleeding), pelvic pain, and an enlarging pelvic or uterine mass. Some patients may present with tissue protruding through the cervical os. The tumor is generally considered low-grade malignant, but a subset of cases demonstrates sarcomatous overgrowth, which is associated with a significantly worse prognosis including higher rates of recurrence and metastasis. The primary treatment for adenosarcoma of the corpus uteri is surgical, typically involving total hysterectomy with bilateral salpingo-oophorectomy. The role of adjuvant therapy, including radiation therapy and chemotherapy, remains debated and is generally reserved for cases with high-risk features such as sarcomatous overgrowth, deep myometrial invasion, or advanced-stage disease. Recurrences tend to be local, occurring in the pelvis or vagina, though distant metastases can occur, particularly in cases with sarcomatous overgrowth. Long-term follow-up is recommended due to the risk of late recurrence. Prior pelvic radiation and long-term tamoxifen use have been identified as potential risk factors in some cases. Given its rarity, management is often guided by institutional experience and case series rather than large randomized trials.

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 ↗

Treatments

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

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

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No specialists are currently listed for Adenosarcoma of the corpus uteri.

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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

Financial Resources

1 resources

Makena

AMAG Pharma USA, Inc.

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Caregiver Resources

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Common questions about Adenosarcoma of the corpus uteri

What is Adenosarcoma of the corpus uteri?

Adenosarcoma of the corpus uteri, also known as Müllerian adenosarcoma of the uterus, is a rare mixed tumor of the uterine body composed of benign or atypical glandular (epithelial) elements combined with a malignant mesenchymal (stromal) component. This neoplasm primarily affects the uterine corpus and most commonly presents in postmenopausal women, although it can occur at any adult age. The tumor typically arises from the endometrium and may protrude into the uterine cavity as a polypoid mass. Key symptoms include abnormal uterine bleeding (most commonly postmenopausal bleeding), pelvic pai

How is Adenosarcoma of the corpus uteri inherited?

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

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

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

What treatment and support options exist for Adenosarcoma of the corpus uteri?

1 patient support program are currently tracked on UniteRare for Adenosarcoma of the corpus uteri. See the treatments and support programs sections for copay assistance, eligibility, and contact details.