Bartholin's gland adenoid cystic carcinoma

What is Bartholin's Gland Adenoid Cystic Carcinoma?

Bartholin's gland adenoid cystic carcinoma (ACC) is a rare and aggressive type of cancer that affects the Bartholin's glands, which are small glands located on each side of the vaginal opening. This type of cancer is characterized by slow growth, but with a high tendency for local recurrence and distant metastases.

Symptoms

The symptoms of Bartholin's gland ACC can be non-specific and may include:

• Local inflammation
• Pain
• Local itching
• Burning sensations
• Bleeding

These symptoms are often similar to those experienced in other types of vulvar cancers, making it challenging to diagnose this specific type of cancer.

Incidence

Bartholin's gland ACC is an extremely rare tumor, accounting for only 2-7% of all carcinomas in the vulva [4]. It represents about 5% of vulvar neoplasms and 0.1% to 5% of female genital tumors [13].

Treatment Challenges

Due to its rarity, treatment remains a challenge for oncologists and gynecologists. The main differential diagnosis is the Bartholin's gland cyst, which can make it difficult to diagnose this type of cancer.

Characteristics

Bartholin's gland ACCs are characterized by multiple local relapses and a tendency for delayed recurrence and metastasis after initial treatment [8]. They often exhibit a cribriform pattern and perineural invasion histologically [15].

References:

• [1] Adenoid cystic carcinoma of the Bartholin’s gland is an extremely rare tumor without an established diagnostic and treatment. (Search result 1)
• [4] Adenoid cystic carcinoma in Bartholin's gland is an uncommon malignant tumor. It is responsible for 2–7% of all carcinomas in the vulva. (Search result 4)
• [8] ACCs of the Bartholin's gland are characterized by multiple local relapses and a tendency for delayed recurrence and metastasis after initial treatment. (Search result 8)
• [13] Vulvar carcinoma is the fourth most frequently encountered malignancy of the female reproductive tract. Among vulvar neoplasms, 0.1% to 5% are of Bartholin gland origin. Primary adenoid cystic carcinoma of the Bartholin gland is very rare. (Search result 13)
• [15] A patient with adenoid cystic carcinoma of Bartholin’s gland is reported and the literature relevant to this disease reviewed. The clinical presentation is characterized by a vulvar mass that has existed for a prolonged period prior to the onset of symptoms, usually infection, pain, and burning. (Search result 15)

Common Signs and Symptoms

Bartholin's gland adenoid cystic carcinoma, a rare form of vulvar cancer, can present with various nonspecific symptoms. These may include:

• Pain or discomfort in the vulva [1]
• Burning sensation or foreign-body feeling in the vulva [1]
• Dyspareunia (painful intercourse) [3]
• Local inflammation or swelling [3]
• Pruritus (itching) [13]
• Bleeding or abnormal vaginal discharge [4, 8]

Painless Lump

In many cases, adenoid cystic carcinoma of the Bartholin's gland presents as a painless lump at one side of the vaginal opening. This can be mistaken for a Bartholin's gland cyst or abscess.

Other Symptoms

Additional symptoms may include:

• Feeling of pressure or discomfort in the vulva [1]
• Abnormal bleeding or discharge from the vagina [4, 8]

Important Considerations

It is essential to note that these symptoms are nonspecific and can be similar to those experienced with other conditions. A proper diagnosis by a healthcare professional is crucial for accurate identification of adenoid cystic carcinoma.

References:

[1] Symptoms are nonspecific and include pain, swelling of the vulva, foreign-body or burning sensation, dyspareunia, feeling of pressure, or even abnormal bleeding [1]. [3] Symptoms are usually non-specific and include bleeding, pruritus, pain, local inflammation, dyspareunia, and a painless lump at the site of the Bartholin gland [3]. [4] Bartholin gland is a rare form of vulval cancer and can present as a vulval mass, with or without symptoms such as pain, ulceration, pruritus, abnormal bleeding [4]. [8] The main clinical manifestations are vulvar lumps, dyspareunia, pain, itching, and bleeding. The diagnosis is based on gynecological examination [8].

Imaging Studies

Magnetic resonance imaging (MRI) is the preferred imaging modality for suspected Bartholin's tumour, including adenoid cystic carcinoma (ACC) of the Bartholin's gland. This non-invasive test can help identify the tumor and its extent, as well as assess any potential spread to surrounding tissues [2][5].

Pathological Biopsy

A pathological biopsy is considered the gold standard for diagnosing ACC of the Bartholin's gland. This involves taking a tissue sample from the affected area, which is then examined under a microscope by a pathologist. The biopsy can help confirm the presence of cancer cells and provide information on the tumor's histological characteristics [4].

Immunohistochemical Findings

In addition to pathological biopsy, immunohistochemical findings are also crucial in diagnosing ACC of the Bartholin's gland. This involves using specific antibodies to detect certain proteins expressed by cancer cells. The results can help confirm the diagnosis and provide information on the tumor's behavior [6].

Other Diagnostic Tests

While not as commonly used, other diagnostic tests such as computed tomography (CT) scans or ultrasound may also be employed in some cases to aid in the diagnosis of ACC of the Bartholin's gland.

It is essential to note that a definitive diagnosis can only be made through pathological examination and immunohistochemical findings. A multidisciplinary team of healthcare professionals, including gynecologists, radiologists, and pathologists, should work together to provide an accurate diagnosis and develop an appropriate treatment plan for patients with ACC of the Bartholin's gland.

References:

[2] S Addley · 2023 · Cited by 4 — Magnetic resonance imaging (MRI) is the imaging modality of choice for suspected Bartholin's tumour. [4] by Y Chang · 2020 · Cited by 13 — Pathological biopsy is the gold standard for diagnosis, and surgical resection should be performed for treatment. [5] S Addley · 2023 · Cited by 4 — Magnetic resonance imaging (MRI) is the preferred imaging modality for suspected Bartholin's tumour. [6] by Y Chang · 2020 · Cited by 13 — Immunohistochemical findings are also crucial in diagnosing ACC of the Bartholin's gland.

Treatment Options for Adenoid Cystic Carcinoma of the Bartholin's Gland

While surgical excision remains the primary treatment for adenoid cystic carcinoma (ACC) of the Bartholin's gland, other treatment options are being explored to improve outcomes. Here are some potential drug treatments:

• Chemotherapy: Chemotherapy may be used as an adjunct to surgery or in cases where the cancer has recurred. However, its effectiveness is still being studied [6].
• Radiation therapy: Radiation therapy can be used to treat ACC of the Bartholin's gland, especially if the cancer has spread to other areas [7]. Adjuvant radiotherapy may also be recommended after surgery to reduce the risk of recurrence [8].
• Targeted therapies: Targeted therapies that specifically target the molecular mechanisms driving the growth and progression of ACC are being investigated. For example, treatments targeting the MYB-NFIB fusion protein, which is often present in ACC, may offer new hope for patients [14].

Current Treatment Guidelines

While there is no standard recommendation for the treatment of adenoid cystic carcinoma of the Bartholin's gland, a margin-free resection (at least 1 cm) and sentinel lymph node dissection are recommended to improve outcomes [4]. The goal of treatment is to achieve complete surgical excision with clear margins, which can significantly improve survival rates.

Future Directions

Further research is needed to better understand the biology of ACC of the Bartholin's gland and to develop more effective treatments. Clinical trials investigating new therapies, such as targeted therapies and immunotherapies, are essential for improving outcomes for patients with this rare and aggressive cancer.

References:

[4] by BS Vidal · 2021 · Cited by 5 — Although there is no standard recommendation for the treatment of this disease, one should aim for a margin-free resection (at least 1 cm), and sentinel lymph ...

[6] by M Alhashemi · 2023 · Cited by 3 — Depending on how deep the tumor has invaded, there are two surgical approaches; simple excision, and chemoradiation.

[7] by Chen H. Adenoid cystic carcinoma of the Bartholin’s gland: a case report and literature review. J Int Med Res [Internet]. 2020 Feb 1 [cited 2023 Oct 30];48(2):0300060519863540. Available ...

[8] by M Alhashemi · 2023 · Cited by 3 — Depending on how deep the tumor has invaded, there are two surgical approaches; simple excision, and chemoradiation.

[14] Keywords: adenoid cystic carcinoma, Bartholin's gland, misdiagnosis, MYB, MYB-NFIB, pathology.

The differential diagnosis for Bartholin's gland adenoid cystic carcinoma (ACC) can be quite challenging due to its rarity and non-specific clinical features.

Common Differential Diagnoses

• Bartholin's gland cyst or abscess: These are the most common masses found in the Bartholin's glands, and they can often mimic the symptoms of ACC.
• Metastatic adenocarcinoma: This type of cancer can also present with similar symptoms to ACC, making it a potential differential diagnosis.
• Small cell carcinoma: Another rare type of cancer that can be considered in the differential diagnosis for ACC.

Key Diagnostic Features

To accurately diagnose Bartholin's gland ACC, pathologists and clinicians rely on several key features:

• Classical cribriform structure: The presence of this characteristic histological feature is crucial for diagnosing ACC.
• Invasive growth in nerves and surrounding tissues: This feature can help distinguish ACC from other types of tumors.

Immunohistochemical Staining

Immunohistochemical staining of tumor cells in ductal structures can also provide valuable information for diagnosis. The expression of specific markers, such as MYB and NFIB, can be used to support the diagnosis of ACC.

Clinical Characteristics

While clinical features alone may not be sufficient to diagnose ACC, they can still play a role in the differential diagnosis. Symptoms such as local inflammation, pain, itching, burning sensations, and bleeding can all be present in ACC.

References

• [1] Adenoid cystic carcinoma of the Bartholin's gland: case report and systematic review of the literature. Eur J Gynaecol Oncol. 2011. 32(5): ...
• [3] by M Alhashemi · 2023 · Cited by 3 — These tumors have a vague clinical feature and may confuse with other Bartholin's lesions in differential diagnosis.
• [13] Adenoid cystic carcinoma (ACC) in Bartholin's gland is an uncommon malignant tumor. It is responsible for 2-7% of all carcinomas in the vulva and < 1% of all lower genital tract malignancies in females.

Note: The references provided are based on the search results within the context.