Adenoid basal carcinoma of the cervix uteri

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ORPHA:213828C53.0C53.1C53.8
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Overview

Adenoid basal carcinoma of the cervix uteri (also known as adenoid basal cell carcinoma of the cervix or adenoid basal epithelioma) is an extremely rare, low-grade malignant neoplasm arising from the uterine cervix. It is classified among the rare carcinomas of the cervix and is characterized by nests and cords of small, uniform basaloid cells that infiltrate the cervical stroma, often with a pattern resembling basal cell carcinoma of the skin. The tumor typically arises from the reserve cells of the cervical epithelium and predominantly affects postmenopausal women, most commonly in the sixth to seventh decades of life. It is strongly associated with high-risk human papillomavirus (HPV) infection, particularly HPV type 16. Clinically, adenoid basal carcinoma of the cervix is often asymptomatic and is frequently discovered incidentally during evaluation of abnormal cervical cytology (Pap smear) or in hysterectomy specimens removed for other indications. When symptoms are present, they may include abnormal vaginal bleeding, postcoital bleeding, or vaginal discharge. The tumor affects the female reproductive system, specifically the cervix uteri, including the endocervix (C53.0), exocervix (C53.1), and overlapping regions (C53.8). Importantly, this neoplasm has an indolent clinical course and an excellent prognosis, with metastasis being exceedingly rare in its pure form. Treatment typically involves surgical excision, most commonly simple hysterectomy, which is generally curative. Conservative management may be considered in select cases. Because of its rarity, there are no standardized chemotherapy or radiation protocols specific to this tumor. However, it is important to distinguish pure adenoid basal carcinoma from mixed tumors that contain components of more aggressive histologic types (such as adenoid cystic carcinoma or squamous cell carcinoma), as these mixed variants may carry a worse prognosis and require more aggressive treatment. Long-term follow-up is recommended, though recurrence in pure adenoid basal carcinoma is uncommon.

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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 Adenoid basal carcinoma of the cervix uteri.

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No actively recruiting trials found for Adenoid basal carcinoma of the cervix uteri at this time.

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No specialists are currently listed for Adenoid basal carcinoma of the cervix 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

Travel Grants

No travel grants are currently matched to Adenoid basal carcinoma of the cervix uteri.

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Community

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Latest news about Adenoid basal carcinoma of the cervix uteri

Disease timeline:

New recruiting trial: Tissue Sample Collection From Patients With Head and Neck Cancer and From Healthy Participants

A new clinical trial is recruiting patients for Adenoid basal carcinoma of the cervix uteri

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Common questions about Adenoid basal carcinoma of the cervix uteri

What is Adenoid basal carcinoma of the cervix uteri?

Adenoid basal carcinoma of the cervix uteri (also known as adenoid basal cell carcinoma of the cervix or adenoid basal epithelioma) is an extremely rare, low-grade malignant neoplasm arising from the uterine cervix. It is classified among the rare carcinomas of the cervix and is characterized by nests and cords of small, uniform basaloid cells that infiltrate the cervical stroma, often with a pattern resembling basal cell carcinoma of the skin. The tumor typically arises from the reserve cells of the cervical epithelium and predominantly affects postmenopausal women, most commonly in the sixth

How is Adenoid basal carcinoma of the cervix uteri inherited?

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

At what age does Adenoid basal carcinoma of the cervix uteri typically begin?

Typical onset of Adenoid basal carcinoma of the cervix uteri is late onset. Age of onset can vary across affected individuals.