FIGO, International Federation of Gynecology and Obstetrics; NED, no evidence of disease; F/U, follow-up. a Median follow-up in each study was at least 5 years. bTotal >100% because some patients with recurrence remained NED. From references 46, 200, 201, 205.
Signs and Symptoms The most common initial symptom of Bartholin gland carcinoma is a vulvar mass or perineal pain. About 10% of patients have a history of inﬂammation of the Bartholin gland, and malignancies may be mistaken for benign cysts or abscesses. Delay of diagnosis is common, particularly for premenopausal patients. The differential diagnosis of any pararectovaginal neoplasm should include cloacogenic carcinoma and secondary neoplasm (199).
Treatment Traditionally, treatment was radical vulvectomy with bilateral groin and pelvic node dissection (201). There seems to be no indication for dissection of the pelvic nodes in the absence of positive groin nodes, and good results were reported with hemivulvectomy or radical local excision for the primary tumor (199). Because these lesions are deep in the vulva, extensive dissection is required in the ischiorectal fossa; surgical margins are often close. Postoperative radiation to the vulva decreased the likelihood of local recurrence from 27% (6 of 22 patients) to 7% (1 of 14 patients) (199). If the ipsilateral groin nodes are positive, bilateral groin and pelvic irradiation may decrease regional recurrence. If the tumor is fixed to the inferior pubic ramus or involves adjacent structures, such as the anal sphincter or rectum, preoperative radiation and chemotherapy is preferable to avoid ultraradical surgery. A recent report of 10 consecutive patients with primary Bartholin gland carcinoma suggests that treatment with radiation or chemoradiation using teletherapy combined with a boost to the primary site or regional nodes and/or interstitial brachytherapy may offer an effective alternative to surgery with 3-and 5-year survival rates of 72% and 66%, respectively (202).
Prognosis Because of the deep location of the gland, disease tends to be more advanced than squamous carcinomas at the time of diagnosis but, stage for stage, the prognosis is similar. Five-year disease-free survival rates by stage are summarized in Table 38.9.
Adenoid Cystic Carcinoma of the Bartholin Gland
The adenoid cystic variety accounts for 15% of Bartholin gland carcinomas. A review of 62 cases reported in the literature demonstrates that adenoid cystic carcinoma of the Bartholin gland is a slow growing tumor characterized by perineural infiltration and a marked propensity for local relapse preceding distant recurrences by years. It is less likely to metastasize to lymph nodes and carries a somewhat better prognosis (Fig. 38.8) (203– 205). The slowly progressive nature of these tumors and the tendency for late recurrences is reﬂected in the disparity between progression-free interval and overall survival (204).
Adenocarcinomas of the vulva usually arise in a Bartholin gland or occur in association with Paget disease. They may arise rarely from the skin appendages, paraurethral glands, minor vestibular glands, aberrant breast tissue, endometriosis, or a misplaced cloacal remnant (206).
Figure 38.8 Adenoid cystic tumor of the Bartholin gland. Basaloid cells form cribriform, sievelike spaces containing mucinous material. The hyaline stroma is another distinct feature of this tumor.