GYNECOLOGIC

ONCOLOGY

38, 138-140(19%)

CASE REPORT Metastatic Basal Cell Carcinoma of the Vulva SUSAN E. WINKELMANN, Department

of Obstetrics

M.D., AND ALFRED S. LLORENS, M.D.

and Gynecology,

University

of Missouri,

Columbia,

Missouri

65212

Received September28. 1989 Basal cell carcinoma of the vulva is rare and was initially thought to be nonmetastasizing; however, there are four reports in the literature of metastasisto regional lymph nodes. We present the case report of a fifth patient with well-documented metastasizing basal cell carcinoma of the vulva with a brief discussion on clinical presentation, treatment, and positive response to preoperative radiation which could make radical surgery unneces=rY- 0 1990 Academic Press, Inc. INTRODUCTION

Basal cell carcinoma is the most common malignant skin cancer, accounting for approximately 65 to 75% of malignancies of the skin [1,2]; however, involvement of the vulva is rare, accounting for approximately 2 to 3% of all vulvar malignancies [3]. In addition to being very rare, basal cell carcinoma of the vulva was initially thought to be nonmetastasizing [4,5], until 1975 when Jimenez et al. presented the first well-documented case with metastasis [7]. There have been three more well-documented cases of vulvar basal cell carcinoma with metastases since the report by Jimenez, et al., for a total of four cases [7lo]. I now present a fifth case which we encountered at our institution. CASE REPORT

A 71-year-old white female, G3P3&, status post total abdominal hysterectomy and bilateral salpingo-oophorectomy in 1957 for uterine leiomyoma, had noticed “something growing” on her vulva for approximately 5 years. Two years prior to admission, she had begun to notice vaginal spotting. Approximately 2 weeks prior to admission she noticed a vaginal odor and constipation. She presented to her local physician with these complaints and a large vulvar lesion was noted, with biopsies revealing basal cell carcinoma (Fig. 1). Shortly there-

after, she was referred to the University of Missouri and exam revealed a 6 x 15-cm fungating lesion on her right labium majus and minus. There was an area of ulceration and necrosis to the depth of the symphysis pubis. There was induration involving the urethral meatus, levator ani, and lower one-third of the rectovaginal septum. The rectal mucosa was smooth. Firm mobile inguinal nodes were palpable bilaterally (N 1). She had a negative chest X ray, intravenous pyelogram, mammogram, and flexible sigmoidoscopy. Cystoscopy revealed tumor involvement of the distal urethra at the meatus. Because of the extensive involvement, the patient received 50 Gy of external-beam radiation therapy through a 10 x IO-cm field to the vulva followed by two boosts of 5 Gy to the symphysis pubis and perineal body. Postradiation she had complete resolution of her tumor. Approximately 6 weeks later, she underwent a radical vulvectomy and proctectomy with removal of the anus with transverse colostomy and superficial and deep inguinal lymph node dissection. She had an uncomplicated postoperative course and was discharged on the eighth postoperative day with a well-functioning colostomy and intact inguinal incisions. Pathologic examination of the surgical specimen revealed no tumor in the vulvar skin; eight of eight left groin nodes were negative, and one of six right inguinal nodes were positive for metastatic basal cell carcinoma (Fig 2). DISCUSSION

This patient had a tumor which was massive and locally invasive. The four cases previously reported in the literature with lymph node metastases also appeared as large, locally invasive lesions. Jimenez et al. reported a case of metastatic basal cell carcinoma which measured 5 x 5 x 2.5 cm on the labia with a left inguinal mass 138

0090-8258BO $1.50 Copyright 0 19% by Academic Press, Inc. All rights of reproduction in any form reserved.

CASE REPORT

139

FIG. 1. Vulvar biopsy revealing basal cell carcinoma with a morpheic pattern. Courtesy of Sanford Sharp, M.D., Department of Anatomic Pathology, University of Missouri School of Medicine, Columbia.

FIG. 2. Right inguinal lymph node biopsy revealing metastatk basal cell carcinoma. Courtesy of Sanford Sharp, M.D., Department of Anatomic Pathology, University of Missouri School of Medicine. Columbia.

measuring 7 x 4 x 3.5 cm which grossly appeared to be matted lymph nodes. Histologically, metastatic basal cell carcinoma was present in both the superficial and deep left inguinal lymph nodes [8]. Sworn et al. described an ulcerated hard mass on the mons pubis extending down the prepuce of the clitoris with the ulcer measuring 8 x 4.5 cm. Five lymph nodes in the fat adjacent to the ulcer were positive for tumor, with no tumor intervening between the lesion and the lymph nodes [lo]. Hoffman et al. described a 6 x 4-cm mobile exophytic tumor involving the left labium majus and minus, extending to the perineal body, and approaching the anal skin. Radical vulvectomy and bilateral inguinal lymphadenectomy revealed basal cell carcinoma with 2 of 12 left superficial inguinal nodes positive for basal cell carcinoma [S]. Perrone et al. described another case of metastasizing basal cell carcinoma of the vulva. This lesion was described as large, fungating, cavitating, nodular, indur-

ated, destroying the urethra and clitoris, and deeply infiltrating the lateral wall of the vagina [9]. Perrone et al. also found several features that distinguished their case of metastasizing basal cell carcinoma from the nonmetastasizing type: vaginal bleeding; advanced clinical stage; invasion of subcutaneous fat, urethra, and vagina; tumor thickness greater than 1 cm; and a morphealike growth pattern [9]. Similarly, our case demonstrated all of these features. Of the four cases previously reported, one underwent wide local excision and the other three underwent radical vulvectomy and bilateral inguinal lymphadenectomy, with one of these receiving postoperative radiation therapy to the pelvis and inguinal areas [7-101. It was thought for many years that metastases did not occur in the pure form of basal cell carcinoma of the vulva; therefore, it was felt that the lesion could be cured by wide local excision [3]. This is still true for most basal cell carcinomas of the vulva. However, some large and invasive lesions may require radical surgery. Indeed,

140

WINKELMANN

Peronne et al. felt that possibly the best indication for regional lymph node dissection was a basal cell tumor that was large and deeply invasive 193. There is little information available in the literature on the use of radiotherapy or chemotherapy in the treatment of primary or metastatic basal cell of the vulva. Safai and Good reported in 1977 that treatment of metastatic basal cell carcinoma of the skin with radiation resulted in only temporary improvement [ 1I]. In a review of management of basal cell carcinomas of the skin, however, Goldberg and Rubin report cure rates of 94 to 98% with radiation therapy in appropriately selected cases [ 121. Because of the extensive involvement of the tumor in our patient, we did not consider her to be an operable candidate at the time of her initial presentation. We therefore chose preoperative radiation to attempt tumor reduction so that radical surgery could then be considered. This patient did have an excellent response to the radiation with no gross tumor seen prior to her surgery, and indeed, the histologic report showed no tumor in the surgical specimen. In conclusion, because the rare basal cell tumors of the vulva that do metastasize all appear to be large and locally invasive, and because of the good response obtained in this case, we propose that preoperative radiation should be considered in similar future cases for tumor shrinkage prior to radical surgery. If no tumor can be identified after radiation, perhaps surgery may not be necessary to improve the overall prognosis.

ANDLLORENS

REFERENCES 1. Pillsbury, (1956).

D. M.

2. Conway, H. IL (1956).

Tumors

W. B. Saunders, Philadelphia

Dermatology,

offhe

skin,

Charles C Thomas, Springfield,

3. Schueller, E. F. Basal cell cancer of the vulva, Gynecol. 93, 199-208 (1965).

Amer.

J. Obstet.

4. Palladino, V. S., Duffy, J. L., and Bures, G. J. Basal cell carcinoma of the vulva, Cancer 24, 460-470 (1969). 5. Breen, J. L., Neubecker, R. D., Greenwald, E., and Gregori, C. A. Basal cell carcinoma of the vulva, Obstet. Gynecol. 46, 122129 (1975). 6. Deleted in proof. 7. Jimenez, H. T., Fenoglio, C. M., and Richart, R. M. Vulvar basal cell carcinoma with metastasis: A case report, Amer. J. Obster. Gynecol. 121, 285-286 (1975). 8. Hoffman, S., Roberts, W. S., and Ruffolo, E. H. Basal cell carcinoma of the vulva with inguinal lymph node metastases, Gynecol. Oncol. 29, 113-l 19 (1988). 9. Perrone, T., Twiggs, L. B., Adcock, L. L., and Dehner, L. P. Vulvar basal cell carcinoma: An infrequently metastasizing neoplasm, In?. J. Gynecol. Pafhol. 6, 152-165 (1987). 10. Sworn, M. J., Hammond, G. T., and Buchanan, R. Metastatic basal cell carcinoma of the vulva case report, hit. J. Obstet. Gynaecol. 86, 332-334 (1979). II. Safai, B., and Good, R. A. Basal cell carcinoma with metastasis, Arch. Pathol. Lab. Med. 101, 327-331 (1977). 12. Goldberg, L. H., and Rubin, H. A. Management of basal cell carcinoma. Which option is best? Postgrad. Med. 85, 57-63 (1989).

Metastatic basal cell carcinoma of the vulva.

Basal cell carcinoma of the vulva is rare and was initially thought to be nonmetastasizing; however, there are four reports in the literature of metas...
1MB Sizes 0 Downloads 0 Views