Adenosarcoma of the cervix uteri

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Overview

Adenosarcoma of the cervix uteri is an extremely rare malignant mixed tumor (also known as Müllerian adenosarcoma of the cervix) that arises in the uterine cervix. It is a biphasic neoplasm composed of a benign or atypical epithelial (glandular) component combined with a malignant mesenchymal (stromal/sarcomatous) component. This tumor primarily affects the female reproductive system, specifically the endocervical region, and can present as a polypoid mass protruding from the cervical os. Key symptoms include abnormal vaginal bleeding, pelvic pain or pressure, vaginal discharge, and the presence of a cervical mass that may be detected on pelvic examination. Some patients may be asymptomatic, with the tumor discovered incidentally during routine gynecological evaluation. Adenosarcoma of the cervix uteri is far less common than its uterine corpus counterpart, which itself is already a rare neoplasm. The tumor can occur across a wide age range but is most frequently diagnosed in adult and postmenopausal women. A variant with sarcomatous overgrowth carries a more aggressive clinical course and a higher risk of recurrence and metastasis. The primary treatment is surgical, typically involving hysterectomy with or without bilateral salpingo-oophorectomy, depending on the patient's age, disease extent, and reproductive wishes. In cases of localized cervical disease, more conservative surgical approaches such as wide local excision or cervical conization may be considered, particularly in younger patients desiring fertility preservation. The role of adjuvant chemotherapy and radiation therapy remains uncertain due to the rarity of the disease, though they may be considered in cases with high-risk features such as sarcomatous overgrowth, deep myometrial invasion, or advanced stage at diagnosis. Long-term follow-up is essential due to the risk of local recurrence.

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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 cervix uteri.

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

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Specialists

1 foundView all specialists →
NB
Nicolò Bizzarri
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 Adenosarcoma of the cervix uteri.

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Common questions about Adenosarcoma of the cervix uteri

What is Adenosarcoma of the cervix uteri?

Adenosarcoma of the cervix uteri is an extremely rare malignant mixed tumor (also known as Müllerian adenosarcoma of the cervix) that arises in the uterine cervix. It is a biphasic neoplasm composed of a benign or atypical epithelial (glandular) component combined with a malignant mesenchymal (stromal/sarcomatous) component. This tumor primarily affects the female reproductive system, specifically the endocervical region, and can present as a polypoid mass protruding from the cervical os. Key symptoms include abnormal vaginal bleeding, pelvic pain or pressure, vaginal discharge, and the presen

How is Adenosarcoma of the cervix uteri inherited?

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

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

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

Which specialists treat Adenosarcoma of the cervix uteri?

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