A case of primary clear cell adenocarcinoma of Bartholin's gland

Authors


* Dr K. C.-K. Lim, Department of Obstetrics and Gynaecology, University Hospital of Wales, Heath Park, Cardiff CF14 4XW, South Wales, UK.

Primary carcinoma of Bartholin's gland is rare. The incidence has been reported as 0.02–0.1 per 100,000 woman-years1. Primary adenocarcinoma of the Bartholin's gland occurs in 1% of all genital malignancies and 2–7% of all vulvar carcinomas2, and just over 200 cases of cancers of Bartholin's gland have been reported3. The criteria for the diagnosis of tumours of Bartholin's gland have been inconsistent. As is the tendency with rare neoplasms, their biologic behaviour is poorly understood. Recommendations for treatment, such as extensive vulvar surgery and inguinal and pelvic lymphadenectomy, are based on limited clinical experience. Some authors have recommended primary radiation therapy and chemotherapy, as it may prevent incontinence4.

Primary adenocarcinoma of Bartholin's gland is a slow-growing tumour characterised by a marked tendency for perineural and local invasion, which is associated with the itching and burning sensations that many women feel before a mass becomes palpable at physical examination5. Primary clear cell adenocarcinoma of Bartholin's gland has hitherto been unreported, and as there are clinical implications with this rare diagnosis, we felt it important to present this case.

Firstly, it is necessary to exclude primary clear cell carcinoma elsewhere, especially in the ovary or kidney. Secondly, the natural history and prognosis of this disease is not known, and so treatment is uncertain.

Case report

A 46 year old woman attended our outpatient clinic complaining of a painless lump in the left side of her vulva for approximately eight months. It had not increased in size during that time but it had prevented her from having intercourse and she had an occasional burning sensation in area. She was otherwise well, apart from rheumatoid arthritis, which she had had for five years. On examination, the inguinal there was a large, indurated, partly cystic, partly solid enlarged left Bartholin's gland, measuring 4–5 cm in diameter. It was not tender. The inguinal lymph nodes were not enlarged. The immediate clinical suspicion was that of a carcinoma, and she was admitted for a wide local excision of the lesion.

This was carried out one week later. A cystic mass was enucleated, containing necrotic material as well as fluid.

At gross pathologic examination, the specimen was an irregular piece of membranous tissue measuring 3 × 1 × 0.4 cm with multiple fragments of grey-coloured material. Microscopically, there was a solid piece of membranous tissue with stratified squamous epithelium, consistent with its origin from the vulva. The other fragments were clumps of adenocarcinoma with a distinct although variable clear cell, hobnail cell or mesonephroid pattern (Figs 1–3). The conclusion was that this was a clear cell adenocarcinoma that could be arising primarily from the Bartholin's gland, or be secondary tumour from another site.

Figure 1.

Clear cell carcinoma of Bartholin's gland, showing papillary architecture peripherally and glandular pattern centrally (haematoxylin and eosin ×25).

Figure 2.

Clear cell carcinoma of Bartholin's gland, showing typical features of a clear cell carcinoma (haematoxylin and eosin ×200).

Figure 3.

Clear cell carcinoma of Bartholin's gland, showing clear cells and ‘hobnail cells’, characteristic of mesonephroid tumours (haematoxylin and eosin ×200).

An ultrasound scan of her abdomen and pelvis showed a normal liver, pancreas, spleen and kidneys, as well as a normal pelvis. Her chest X-ray was also normal. Her serum CA125 concentration was 18 u/L (normal <30 u/L) and her Cyfer 21-1 concentration was 1.2 u/L (normal <3 u/L). Two weeks later, bilateral inguinal lymphadenectomy was carried out. The vulval incision had healed completely. Histologic examination of the lymph nodes showed no abnormality. Nine months later, she had no evidence of recurrence.

Discussion

Carcinoma of Bartholin's gland is rare. We believe that this may be the first reported case of primary clear cell adenocarcinoma of Bartholin's gland. The age of presentation of adenocarcinoma is 50–62 years6; a proportion occurs in premenopausal women. The tumour usually presents as a nodule deep in the posterior part of the labium majus, or with perineal pain. In about one-quarter of women, the first manifestation is an abscess of Bartholin's gland that fails to resolve following treatment.

Many tumours may arise in Bartholin's gland. The criteria for a diagnosis of a primary tumour of Bartholin's gland are that the neoplasm must arise at the site of Bartholin's gland, be consistent histologically with a primary neoplasm of Bartholin's gland and is not be metastatic7. This is already an adaptation of the stricter criteria of Honan published in 18978. Our case report fulfils the first and the third criteria, but as the gland was completely replaced by the tumour there were no normal glandular elements to confirm the second criterion, however there are reports that normal gland elements may not remain in large tumours9.

Adenocarcinoma accounts for approximately 40% of Bartholin's gland carcinomas; other types include squamous cell carcinoma (40%), adenoid cystic carcinoma (15%) and transitional cell carcinoma (less than 5%) and poorly differentiated adenocarcinomas10.

The tumours are typically solid, deeply infiltrative and occupy the site of the gland, occasionally obscuring its presence. They are well circumscribed but not encapsulated, yellowish or grey-white in colour and, although generally solid, may show areas of cystic change, necrosis or haemorrhage. Adenocarcinomas of the Bartholin's gland usually are non-specific in type, but mucinous and papillary types have been described7. The differential diagnosis of adenocarcinoma of Bartholin's gland includes adenocarcinoma of a skin appendage and metastatic adenocarcinoma. These tumours typically do not involve the gland and the tumour type may not be consistent with a primary tumour of Bartholin's gland. Adenoid cystic carcinomas arising in Bartholin's gland are similar to those occurring in salivary glands, the upper respiratory tract and skin. They are composed of uniform, small cells arranged in cords and nests with a cribriform pattern. Adenosquamous carcinomas of Bartholin's gland contain a mixture of squamous cells identified by keratin formation and intracellular bridges, and glandular cells that typically contain mucin.

Because these tumours are very rare, primary treatment for carcinoma of Bartholin's gland is based on limited experience. It may include wide excision down deep to the fascia; radical hemivulvectomy, or radical total vulvectomy. Ipsilateral or bilateral inguinal lymphadenectomy is necessary, regardless of the type of primary excision9,11,12. Adjuvant radiation therapy to the vulva and regional lymph nodes has also been advocated13. Some authors have suggested combined radiation therapy and chemotherapy, especially in with metastasis to the inguinal lymph nodes and in women where inguinal lymphadenectomy has not been carried out4. Primary therapy for adenoid cystic carcinoma of the Bartholin's gland should be radical vulvectomy and inguinal lymphadenectomy, rather than wide local excision5.

Metastases to the inguinal lymph nodes occurs in one-third to one-half of women, and 18% of these women have metastases to the external and common iliac lymph nodes3. If there are no metastases to the inguinal lymph nodes, there is actually no risk of metastases to the iliac lymph nodes.

The overall 5-year survival of women with carcinoma of Bartholin's gland is approximately 50% when there is no metastases to the inguinal lymph nodes, but decreases to 18% when two or more inguinal lymph nodes are involved. Survival is better with adenoid cystic carcinoma than with other forms of carcinoma of the Bartholin's gland14. Adenosquamous carcinomas has a poorer prognosis than squamous cell carcinoma, which is related to the higher frequency of lymph node metastasis.

Primary clear cell adenocarcinoma of Bartholin's gland has not been reported before. We are therefore uncertain of the natural history of this disease and its treatment. It seems logical to perform a wide local excision of the primary tumour and bilateral inguinal lymphadenectomy.

The woman gave her permission for her case to be reported.

Ancillary