Adenoid Cystic Carcinoma of the Bartholin Gland - An Overview

Introduction

The occurrence of cancer in the primary Bartholin gland is rare and accounts for approximately 0.001 percent of all female genital tract carcinomas and 0.1 to 7 percent of vulvar carcinoma cases. Bartholin's gland is linked to several histological forms of malignant tumors, such as transitional cell carcinoma, squamous cell carcinoma, adenocarcinoma, adenoid cystic carcinoma (ACC), and adenosquamous cell carcinoma. ACC is a rare variant of the cancer, and its characteristics are slow growth and a tendency toward local recurrence, which can eventually result in distant metastases.

Bartholin gland tumors can be difficult to distinguish from other lesions since they frequently have vague clinical characteristics. As a result, these tumors may initially be misdiagnosed as other Bartholin gland lesions, which could lead to a delayed diagnosis and later stages of advanced disease. Delays in diagnosis can make it more challenging to manage these tumors efficiently and raise the possibility of complications, including metastasis and recurrence, which can affect treatment outcomes and prognosis.

What Is Bartholin Gland?

The Bartholin gland (BG) is impalpable (cannot be palpated or felt by touch). It is situated bilaterally in the base of the labia minora and is pea-sized. It contributes to vaginal lubrication during sexual intercourse along with other glands, and hence its removal does not have an impact on vaginal lubrication. Numerous lesions can affect the BG, such as lymphoma, mesenchymal lesions, tumors that are benign and tumor-like lesions, lesions that are premalignant and malignancies, and inflammatory lesions, among which cysts and abscesses are the most common.

Bartholin gland cancers include a variety of histological subtypes, with squamous cell carcinoma and adenocarcinoma being the most common. However, there are other less common varieties, such as adenoid cystic carcinoma (ACC), which has been reported to be an uncommon kind of genital cancer.

What Are the Clinical Manifestations of ACCBG?

It might be challenging to diagnose adenoid cystic carcinoma (ACC) of the Bartholin's gland because of its wide range of vague signs and symptoms. The most common symptoms reported by patients are discomfort, burning sensation, or palpable mass in the affected location, with painful nodules being the most common. These signs and symptoms frequently coincide with abscesses or cysts in Bartholin's duct, which could result in a delayed or incorrect diagnosis. The difficulties in diagnosing ACC are made more difficult by its clinical similarities to other benign or malignant growths. It is further complicated by symptoms including bleeding, pruritus (itching), local inflammation, dyspareunia (painful intercourse), and the existence of a painless lump close to the Bartholin's gland location.

Due to the overlapping nature of the symptoms, it is still difficult to accurately identify and differentiate ACC from cysts or tumors. Therefore, a thorough evaluation is required for a prompt diagnosis and suitable therapy.

What Are the Diagnostic Criteria of ACCBG?

Adenoid cystic carcinoma (ACC) was first identified in 1853 as a subtype of adenocarcinoma. ACC is a cancer that is commonly found in the skin, breast, and salivary glands. Though ACC typically appears in the cervix of the female vaginal canal, instances within Bartholin's gland are comparatively uncommon, but there are reported incidences of the same. The mean age of occurrence in women is above 40 years. However, the age range varies from 25 to 80 years.

The following criteria must be met to establish the diagnostic criteria for primary carcinoma in Bartholin's glands:

• The tumor must be located within the Bartholin's gland area.

• There must be a visible transition from normal to neoplastic epithelium.

• No tumor should be found in other anatomical sites.

It is crucial to remember that situations in which no noticeable shift from average to the neoplastic epithelium can still be classified as primary carcinomas of Bartholin's gland as long as the tumor completely replaces the gland. There is no skin involvement or ulceration.

What Are the Histological Features of ACCBG?

• According to histological analysis, most cases of adenoid cystic carcinoma of the Bartholin's gland (ACCBG) display a typical 'cribriform' pattern, which may be seen using the stain hematoxylin and eosin (H and E).

• Interlacing cords (anastomosing) of cells surrounded by acellular gaps filled with hyaline and mucin is the physical manifestation of a cribriform pattern. The tiny, basaloid type of tumor cells have regular nuclei and little cytoplasm. Interestingly, these cells frequently exhibit a broad invasion into the skeletal and perineural muscles. ACCBGCs appear to originate from the myoepithelial cells found in the Bartholin's gland and hence differ from the other cancers of the Bartholin gland.

What Are the Metastatic Patterns of ACCBG Tumors?

ACCBG is a slowly growing tumor that is locally invasive, showcasing distinct lymphatic and perineural invasion that causes the symptoms and leads to local recurrence. The survival rates after five years are 71 percent to 100 percent, and after ten years, are 59 percent to 100 percent, respectively.

A prolonged time without illness may eventually lead to distant metastases through hematogenous dissemination (spreads through the bloodstream). The lung and bone are the most often metastasized locations. The kidney, brain, and liver are the less frequently metastatic sites.

What Is the Treatment Approach for Adenoid Cystic Carcinoma of the Bartholin Gland (ACCBG)?

For these sporadic tumors, there are no established treatment protocols. Currently, surgery is the mainstay of treatment.

• Surgery:

  • Hemivulvectomy (removal of half of the vagina), wide local excisions, simple vulvectomy (removal of the entire vulva), and radical vulvectomy (partial removal of the vulva with deep tissue) with or without inguinal or femoral lymphadenectomy (dissection of the lymph node) are among the surgical options that have been documented.

  • The ideal surgical strategy has yet to be established.

  • Some suggest radical vulvectomy as the surgery of choice since it removes the cancerous tissue and margins and hence prevents the recurrence of cancer. In contrast, others offer a conservative surgical approach as the best surgical plan to avoid delays in chemotherapy and radiation therapy.

  • Surgery might not be a treatment choice for a few cases.

• Adjuvant Radiation Therapy - Adjuvant radiation is a postoperative treatment sometimes used by physicians for adenoid cystic carcinoma of the Bartholin gland (ACCBG) to ensure that any cancer cells left are eliminated. Experts, however, have yet to agree on the appropriate dosage, the size of the target area, or the overall amount of radiation required. If, following surgery, it is discovered that there are still cancer cells around the margins of the resected tissue (positive margins), then this additional radiation is strongly advised.

• Chemotherapy - Chemotherapeutic strategies comprise a variety of regimens that include medications such as 5-fluorouracil, Methotrexate, Doxorubicin, Paclitaxel, Cyclophosphamide, and Cisplatin. These chemotherapy regimens are usually used in conjunction with radiation therapy or after adjuvant radiotherapy and surgery in the event of recurrence of adenoid cystic carcinoma of the Bartholin gland. However, there is less evidence for chemotherapy in ACCBG than adjuvant radiotherapy.

• Immunotherapy - Immunotherapy as a new treatment modal is gradually being established with excellent results.

Conclusion

A rare form of vulvar carcinoma is called adenoid cystic carcinoma. The most typical sign is a painful nodule, often ignored as a benign condition. Therefore, if a woman over 40 years old has a lump close to her Bartholin glands, she should be evaluated for the risk of cancer. This uncommon tumor frequently presents significant challenges for diagnosis and treatment.