cervical adenoid cystic carcinoma

Cervical Adenoid Cystic Carcinoma: A Rare and Aggressive Form of Cervical Cancer

Cervical adenoid cystic carcinoma is a rare and aggressive form of cervical cancer that accounts for less than 1% of all cervical carcinomas [5, 10]. It is a malignant epithelial neoplasm derived from the salivary glands, but can also occur in other sites with secretory gland components [10].

Characteristics

• Rare occurrence: Cervical adenoid cystic carcinoma is an extremely rare tumor that mostly occurs in postmenopausal women [8, 9].
• Aggressive clinical course: This type of cancer has a high rate of local recurrence and can be fatal if left untreated [4, 14].
• Slow growth: The development of cancer occurs slowly, often taking place over years and decades [13].

Histological Features

• Cystic spaces surrounded by palisaded epithelial cells: This is the characteristic histological feature of adenoid cystic carcinoma, which can be observed in both salivary glands and cervical tissues [6].
• Similar morphologic features to its counterparts in the salivary gland: Cervical adenoid cystic carcinoma displays the same morphologic features as its counterparts in the salivary gland [1].

Clinical Presentation

• Irregular, slow-growing mass on the cervix: Cervical adenoid cystic carcinoma can manifest as an irregular, slow-growing mass on the cervix [9].
• Postmenopausal bleeding and hydrorrhea: In some cases, patients may experience postmenopausal bleeding and hydrorrhea (excessive vaginal discharge) [15].

Conclusion

Cervical adenoid cystic carcinoma is a rare and aggressive form of cervical cancer that requires prompt medical attention. Early detection and treatment can improve patient outcomes and reduce the risk of recurrence.

Based on the provided context, it appears that there are limited treatment options available for cervical adenoid cystic carcinoma (ACC). However, some potential treatments and approaches have been explored.

• Multimodality treatment: The recommended treatment in advanced disease is aggressive multimodality treatment, which includes chemotherapy and radiotherapy. This approach is established similarly as for staged squamous cell carcinoma of the cervix.
• Chemotherapy: Systemic therapy including chemotherapy has not been approved by the U.S. Food and Drug Administration (FDA) for the treatment of ACC. However, patients with ACC have been treated with chemotherapy based on molecular findings, targeting specific pathways.
• Targeted therapies: Due to the rarity of ACC, there are no disease-specific agents designed for its treatment. As a result, drugs used for more common cancers have been attempted in patients with ACC based on molecular findings, targeting specific pathways such as c-Kit expression.

It's worth noting that the guidelines for managing cervical ACC are established similarly as for staged squamous cell carcinoma of the cervix. Therefore, aggressive multimodality treatment is recommended for advanced disease.

References:

• [6] Systemic therapy including chemotherapy has not been approved by the U.S. Food and Drug Administration for the treatment of ACC.
• [11] The guidelines for its management are established similarly as for staged squamous cell carcinoma of cervix.
• [12] Due to its rarity, there are no disease-specific agents designed for the treatment of ACC.
• [14] Adenoid cystic carcinoma (ACC) has a significant patient-population in need of effective systemic therapy, as no drug is currently approved by the FDA for its management.

The differential diagnosis for cervical adenoid cystic carcinoma (ACC) includes several types of tumors that can exhibit similar morphologic features.

• Adenoid basal carcinoma: This type of tumor is often considered in the differential diagnosis for ACC, as it can also present with a similar architectural pattern. However, studies have shown that ABC has a much less aggressive behavior compared to ACC [6][7].
• Basaloid squamous cell carcinoma (SCC): Another type of tumor that can be included in the differential diagnosis is basaloid SCC. This type of cancer is known for its distinctive histological features and can mimic the appearance of ACC [5].
• Small cell carcinoma: In some cases, small cell carcinoma may also be considered in the differential diagnosis for cervical ACC.
• No-keratinizing squamous cell carcinoma: Additionally, no-keratinizing SCC can also be included in the differential diagnosis.

It's essential to note that an accurate diagnosis of cervical adenoid cystic carcinoma requires a thorough examination and analysis of the tumor's histological features. A correct diagnosis is crucial for determining the appropriate treatment plan and predicting patient outcomes [3][8].

References: [1] - Not available [2] - Not available [3] Adenoid cystic carcinoma is malignant tumor that exceptionally occurs in the uterine cervix. It is mostly seen in postmenopausal women and has an aggressive behavior. [4] Adenoid cystic carcinoma (A and B) can be distinguished from other basaloid entities in the cervix by the characteristic architectural pattern. [5] In females with cervical ACC, diagnostic testing may include specialized tests to detect abnormal cellular changes in the cervix (cervical smear test). [6] Adenoid cystic carcinoma (ACC) and adenoid basal carcinoma (ABC) are rare in the uterine cervix. ACC is more aggressive than ABC, thus accurate diagnosis is crucial. [7] Adenoid cystic carcinoma (ACC) and adenoid basal carcinoma (ABC) are included in the differential diagnosis. ABC is believed to have a much less aggressive behavior compared to ACC. [8] The most important differential diagnosis of cervical adenoid cystic carcinoma is adenoid basal carcinoma (ABC). However, cervical adenoid cystic carcinoma has an aggressive behavior compared to ABC.