Recurrent Squamous Cell Carcinoma of the Bartholin's Duct Treated with en Bloc Resection

Gynecologic Oncology 75, 282–284 (1999) Article ID gyno.1999.5464, available online at http://www.idealibrary.com on

CASE REPORT Recurrent Squamous Cell Carcinoma of the Bartholin’s Duct Treated with en Bloc Resection 1,2 Lawrence R. Nycum, M.D., John H. Farley, M.D., Mark E. Reed, M.D., and Robert R. Taylor, M.D. Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, Walter Reed Army Medical Center, 6900 Georgia Avenue NW, Washington, DC 20307-5001 Received September 29, 1998

CASE REPORT

Bartholin’s gland carcinomas are a rare entity. A case of a recurrent Bartholin’s gland carcinoma is described. These neoplasms have a myriad of treatment options for primary therapy but there is a paucity of information regarding treatment for a lethal recurrence. The patient’s primary therapy consisted of an initial wide local excision followed by radiation therapy with chemosensitization. She was disease-free for 2 years before her recurrence. A novel treatment approach incorporating a mulitdisciplinary en bloc radical surgery is described. The patient is alive and well without evidence of disease at 22 months. Key Words: Bartholin’s gland carcinoma; en bloc resection; chemotherapy; radiation; surgery; recurrence.

INTRODUCTION

BP is a 39-year-old white female who was first diagnosed with a squamous cell carcinoma of the BG duct in 1994. The patient had a past medical history remarkable for tubal ligation and pilonidal cystectectomy. Her obstetric and gynecologic history was notable for three spontaneous vaginal deliveries without incident and no abnormal PAP smears, STDs, PID, or menstrual abnormalities. She had a 30-pack year smoking history and used alcohol socially. She presented to a General Surgical service for a fluctuant vulvar mass thought to represent a pararectal abscess that was incised and drained. The patient returned 1 month later complaining of persistent left vaginal/rectal sidewall pain. She was then referred to Gynecologic Oncology. A physical evaluation including a pelvic exam revealed an irregular, firm mass along the left vaginal sidewall with a 1-cm nodule fixed to the left pubic ramus and a tumor plaque along the medial aspect of the ischiorectal fossa. A CT scan was then ordered which revealed a 2 cm 3 3 cm left perineal mass with poorly defined borders infiltrating into the fatty tissue. She subsequently underwent a wide local excision of this mass with resection of the entire tumor except a small amount overlying the rectum. The final pathology revealed a squamous cell carcinoma and she was assigned as a stage IV Bartholins gland cancer secondary to involvement of the bone. Postoperatively, she received teletherapy to a total of 5500 cGy in conjunction with 5-FU and Mitamycin for chemosensitization. The patient tolerated this regimen without incident and was without evidence of disease for over 2 years. She presented in June 1996 with complaint of a dull persistent pain and a 1 cm 3 2 cm fullness in her left vaginal introitus wall. A needle biopsy was performed of the mass that revealed fibrous tissue and muscle. A Pap smear obtained at the same time was normal. The patient was asked to return for reexamination at 2 months and was noted to have an increase in the size of the mass to 2 cm 3 3 cm with increased pain. She

Carcinoma of the Bartholin’s gland is a rare lesion accounting for only 3% of all vulvar cancers. Caspar Bartholin, the Danish anatomist, described the glands that bear his name in 1675 in Exercitationes Miscellanea Varii Argumenti in Primis Anatomic [1]. Nearly 200 years later, the first described malignancy of the BG was in 1864 by Kolb [2]. The primary histologic types are adenocarcinoma and squamous cell carcinoma, which account for 80% of malignancies of the BG. The remaining histologic subtypes include adenoid cystic (15%), adenosquamous, and transitional cell carcinomas (5%) [3, 4]. The unusualness of this lesion has led to a dirth of information in the literature, particularly regarding a recurrence of the ductal portion of the gland. This also shows that there are no standard treatment options available in these recurrent cases. We present a novel multidisciplinary approach to the treatment of a recurrent Bartholin’s gland duct carcinoma. 1 This paper was presented at The Society of Air Force Clinical Surgeons meeting in San Antonio, Texas on April 7, 1998. 2 The views expressed herein are those of the authors and do not reflect the official policy or opinion of the Department of Defense or the United States Army, Navy, or Air Force.

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underwent an excisional biopsy of this area that revealed squamous cell carcinoma in the deep submucosal tissue. A metastatic workup was obtained which included CT scans of the chest, abdomen, and pelvis, an MRI of the abdomen and pelvis, and a bone scan. The CT and bone scan failed to reveal any evidence of metastatic disease and the soft tissue mass was revealed to be 3 cm 3 2.5 cm in size. The MRI was notable for an abnormally high signal in the adjacent left obturator internus muscle and pubic rami, which was suspicious for an invasive component of the tumor. The Orthopedic Oncology service was consulted for possible bony involvement of the pubic rami and ischium. Her case was reviewed at our Tumor Planning Conference with a consensus recommendation of a multidisciplinary salvage en bloc resection. The Plastic Surgical service was consulted for myocutaneous graft placement in the ischiorectal fossa and vagina. Recommendations for and alternatives for the patient were reviewed and included vaccine protocols, chemotherapy, and pain service consultation. The patient was taken to the operating room where she underwent an awake standing prep, was placed sterilely in the modified lithotomy position, and successfully underwent general endotracheal anesthesia. She was then again sterilely prepped and draped. A standard left inguinal/femoral lymph node resection was performed with frozen section of the lymph nodes obtained. This returned as no evidence of metastatic disease and this incision was closed with the placement of a Jackson-Pratt vacuum drainage system. A separate incision was then made approximately 1 cm above the left inguinal ligament extending from the anterior superior iliac spine to the pubic symphysis. Using a retroperitoneal approach, access was gained to the external iliac vessels. The ureter was identified and the infundibulopelvic ligament was ligated. We then dissected the lymph node bearing tissues off the external iliac vessels, the internal iliac vessels, and the obturator space. These were sent for frozen section and returned without evidence of metastatic disease. The intraperitoneal cavity was entered and the small bowel was packed cephalad with the aid of a Bookwalter retractor. The paravesical, pararectal, rectovaginal and retrorectal spaces were then sequentially isolated, developed, and found to have no evidence of disease. The ureters were identified bilaterally and a window was made in the mesentery of the rectosigmoid as it entered the pelvis and then transected with a GIA stapler. The rectosigmoid mesentery was then sequentially clamped, cut, and ligated into the sacral hollow. For the perineal phase, an incision was made around the lesion with 2-cm margins extending laterally and inferiorly to the pararectal space, distally below the rectum, and then circumferentially to the medial portion of the right and left labia majora (Fig. 1). The dissection was carried deep to the endopelvic fascia at all times. The rectum was dissected off of the coccyx and sacrum and laterally the labia was dissected inferior to the lateral portion of the right endopelvic fascia. The Orthopedic Oncology service then divided the left adductor

FIGURE 1

muscles from their origins on the pubic rami and resected a portion of the left pubic symphysis and left pubic rami. The bladder and urethra were also dissected free from the paravesical space and mobilized medially. Following this, the retrorectal space was entered from the peritoneal side and connected to the perineal side. A hemivaginectomy was performed encompassing the left lateral and inferior one-third of the vagina with the incision carrying greater than 2 cm around the left lateral labial lesion. The specimen was then removed en bloc containing the anus to the sigmoid, the portion of the pubic symphysis and pubic rami, the distal vagina including the left BG lesion with its 2-cm margins, and all the subcutaneous tissues. Additional biopsies were taken from the tissues along the coccyx and sacrum and sent for frozen section and these were all negative for metastatic disease. The uterus and proximal vagina were retained. The Plastic Surgical service then assisted in developing a right gracilis myocutaneous flap, which was rotated into the previously irradiated posterior pelvis to form a portion of the posterior and lateral vagina. The left portion of the paravesical endopelvic fascia was then sutured to Cooper’s ligament to support the urethra. Additionally, the uterus was approximated to the sacral promontory and retained its position at the vaginal apex to aid in preventing an enterocele and giving substance to the pelvic floor. The remaining portion of the descending colon was then mobilized and a colostomy was made. After copious irrigation, JacksonPratt drains were placed, the abdominal fascia was closed with a No. 2 nylon suture, and the skin was closed with surgical staples. The incisions were dressed, the colostomy was matured, and the patient was then transported to the Surgical Intensive Care Unit. Postoperatively, the patient did well and was transferred to the Ward on day 1. She continued to do well until postop day 10 when the left side of the wound broke down in the previously radiated area. The wound required debridement and oral

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antibiotics were added. On postop days 19 and 20 she went to the OR for high-volume pulse irrigation. The patient returned to the OR on postop day 26 and had a right vertical rectus abdominis myocutaneous flap placed into the wound and a split thickness skin graft was placed into the lateral portion of the vagina. The patient was discharged from the hospital on postop day 40 and went back to work approximately 3 months later. She developed a stricture of her colostomy 9 months out and had a revision performed. She currently is alive and well, ambulating without assistance at 27 months postop. DISCUSSION This case describes a multidisciplinary surgical approach to a recurrent BG tumor. These tumors, particularly recurrent, are rare and management recommendations have been based on limited information. There is also often confusion regarding the appropriate way to make a diagnosis of a BG primary. The diagnosis of a BG primary has been based on differing criteria over the years. Honan’s criteria as cited by Bernstein et al. [5] have been used in prior reports. The first criterion is that the tumor must be in the correct anatomic position. The second criterion states that the tumor must be located deep in the labium majus. The third criterion states that the overlying skin must be intact. The fourth criterion was that the presence of normal gland must be demonstrated. This criteria set has undergone critical scrutiny by other authors, who stated that the second criterion was redundant and the third not applicable to large ulcerated tumors. They also state that the fourth criterion is not diagnostic because one may be observing normal glandular tissue adjacent to a deeply invasive vulvar neoplasm or that a very large BG neoplasm might obliterate residual normal gland. Subsequently, it was felt that Honan’s criteria were unrealistic. Chamlian and Taylor [1] at the Armed Forces Institute of Pathology used the following as their criteria to accept a tumor as being a BG primary: (i) areas of apparent transition from normal elements to neoplastic ones, (ii) tumors involving the BG were histologically compatible with origin from BG, and (iii) there was no evidence of a primary tumor elsewhere. Copeland et al. [6] note that a definite diagnosis of a BG neoplasm can be made only when an area of transition from normal gland or duct tissue to neoplastic tissue is identified. A uniform theme runs through each of the above in that one needs to demonstrate a transition from normal to neoplastic tissue to render a diagnosis of a BG primary. This case also serves to illustrate that a BG carcinoma may initially be misdiagnosed as a cyst or an inflammatory process. Subsequently, much time may be lost prior to establishing a diagnosis and initiating definitive therapy. If necrotic material is drained or induration noted during incision and drainage of a BG abscess or a marsupialization procedure, a biopsy should be obtained [3].

It appears that there are a myriad of treatment options for BG carcinoma primaries that may include singly or in combination: wide local excision, hemivulvectomy, radical vulvectomy with possible radiation, radiation with chemosensitization, or radiation alone. The 5-year survival rates with primary therapy vary from 33 to 84% depending on which author you cite [6 – 8]. However, there appears to be a paucity of data in the literature regarding the management of BG carcinomas that recur and their survivorship. Copeland noted in 1986 [6] in his review of 36 patients over a 30-year period that there was an approximately 25% recurrence rate at M.D. Anderson. These patients were treated with cyclophosphamide, radiation, or a combination of excision and radiation or radical surgery and radiation. In those patients without histologically confirmed nodal metastasis, 3 of 6 (50%) lived greater than 5 years and another patient was alive and well with no evidence of disease at 4 years. Those who were alive had had an excision with vulvar radiation. Due to death from intercurrent disease, it is difficult to interpret an average length of survival in this patient population. Our case is unique in that the patient had received primary therapy in the form of a wide local excision followed by loco-regional irradiation and then recurred. The use of chemotherapy in recurrent BG carcinoma has shown no benefit and additional radiation therapy was contraindicated. We subsequently developed a novel surgical approach to this disease presentation. We found that doing an en bloc resection of the recurrent tumor in which all potentially affected tissues, including the pubic bone, are removed is an excellent mode of local control in the absence of metastatic disease. It also serves to illustrate the need to assemble a proper multidisciplinary surgical team to effect the best outcome for the patient. REFERENCES 1. Chamlian DL, Taylor HB: Primary carcinoma of Bartholin’s gland. Obstet Gynecol 39:489 – 494, 1972 2. Kolb JM: Pathologische Anatomieder Wieblichen Sexualorgane, Vienna, 1864 3. Hoskins WJ: Principles and Practice of Gynecologic Oncology, Philadelphia, PA, Lippincott-Raven, 1997 4. Copeland LJ, Sneige N, Gershenson DM, Saul PB, Stringer CA, Seski JC: Adenoid cystic carcinoma of the Bartholin gland. Obstet Gynecol 67:115– 120, 1986 5. Bernstein SG, Voet RL, Lifshitz S, et al.: Adenoid cystic carcinoma of the Bartholin’s gland: Case report and review of the literature. Obstet Gynecol 147:385, 1983 6. Copeland LJ, Sneige N, Gershenson DM, McGuffee VB, Abdul-Karim F: Bartholin gland carcinoma. Obstet Gynecol 67:794 – 801, 1986 7. Miller B, Morris M, Levenback C, Burke TW, Gershenson DW: Pelvic exenteration for primary and recurrent vulvar cancer. Gynecol Oncol 58:202–205, 1995 8. Hopkins MP, Morely GW: Pelvic exenteration for the treatment of vulvar cancer. Cancer 70:2835–2838, 1992