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An unusual presentation of metastatic squamous cell carcinoma of the vulva
GYNECOLOGIC ONCOLOGY
39, 21%220(1990)
CASE REPORT An Unusual Presentation of Metastatic Squamous Cell Carcinoma of the Vulva D. MICHAEL STEELE, M.D.,* Departments
of *Surgery,
tPathology,
LINDA R. HEGSTRAND, M.D. ,t THOMAS M, JULIAN, M.D.,+ AND F. KRISTIAN STORM, M.D.“*
and SObstetrics and Gynecology, University of Wisconsin School Cancer Center, 600 Highland Avenue, Madison, Wisconsin 53792
of Medicine and Comprehensive
Received May 10, 1990
Recurrent squamous cell cancer of the vulva metastasized via regional lymphatics. Hematogenous spread is late and unusual. A patient with metastatic diseasepresented with soft tissue mass in her thigh musculature. We believe this to be the first reported case of noncontiguous skeletal metastasis for this tumor. Q 1990 Academic Press, Inc. INTRODUCTION Recurrent squamous cell cancer of the vulva is a slowly progressive tumor that advances locally and then spreads via regional lymphatics. Hematogenous spread is late and uncommon. An elderly woman presented to our institution for treatment of a large, mixed, cystic and solid mass, characteristics of a primary soft tissue tumor, in her left thigh muscles. Biopsy of this mass showed metastatic vulvar carcinoma. This is the first reported case of noncontiguous, muscle metastasis of this tumor presenting as a soft tissue mass of unknown etiology. CASE REPORT
Mrs. A is a 68-year-old nulliparous woman of Italian extraction who initially presented with a 3-week history of vaginal bleeding, foul-smelling discharge, and a lump in her vulva. Examination showed a large, fungating, necrotic tumor replacing the patient’s entire vulva and extending above the clitoris. The tumor extended to but did not involve the urethra. Bilateral groin examination ’ To whom correspondence should be addressed.
showed 3-cm firm, fixed inguinal nodes. Biopsy was consistent with poorly differentiated squamous cell cancer, T3 N3 MO, or stage III disease. The patient underwent external-beam radiation therapy to 70 Gy delivered in 35 fractions over a 67-day period. Included were 50 Gy to the genitalia and both inguinal regions, a 20-Gy bolus to the vulva, and lo-Gy boluses to both inguinal regions. Two weeks after completion of radiotherapy she was noted to have persistence in the left labium majorum and underwent partial vulvectomy followed by a radium implant. Two and one-half months postoperatively she had a local recurrence in the posteromedial aspect of her vulvectomy scar, in the anterior portion of the pubic region, and in the left vulva. She developed progressive, intermittent pain and swelling in her left posterior thigh above the knee. An g-cm nontender solid/cystic lesion unassociated with erythema or warmth was found in her thigh. Aspiration of this mass produced chocolate-colored, heme-positive fluid. Cultures and gram strains were negative. Cytology was suspicious but not diagnostic for malignancy. A CT scan showed a large, irregular, thick-walled mass on the posterior aspect of the left thigh just above the knee in the belly of the hamstring muscle (Fig. la). The popliteal fossa was uninvolved (Fig. lb). The mass enlarged to 16 cm in diameter. The patient was referred to the University of Wisconsin for evaluation and treatment of a suspected primary soft tissue tumor. A core needle biopsy was performed showing well-differentiated metastatic squamous cell cancer consistent with the primary vulvar tumor (Fig. 2).
218 OO!N-8258/!30 $1.50 Copyright 0 I!390by AcademicPress,Inc. All rights of reproductionin any form reserved.
CASE REPORT
219
FIG. 1. I !xtremity CT scan showing (a) mixed cystic-solid mass within posterior thigh musculature and (b) uninvolved popliteal fossa.
DISCUSSION Cancer of the vulva accounts for 3% of gynecologic malignancies [l]. With the continued aging of the population this percentage is likely to increase. These tumors are squamous cell neoplasms which grow slowly and progress from dysplasia to carcinoma in situ, invasive carcinoma, and metastatic disease [2]. Regional, metastatic spread of these tumors is via the superficial inguinal lymph nodes, the ipsilateral deep inguinal lymph nodes, and the ipsilateral deep pelvic lymph nodes in a sequential manner [l-6]. The importance of the draining lymphatics and their role in the natural history and surgical cure of vulvar malignancy were first noted by Taussig [lo], and then by Way and Mennigan [ 1l-131, revolutionizing the treatment of this disease. More recently,
Plentl and Friedman have thoroughly described the lymphatic drainage of the vulva [14]. Hematogenous spread is late and unusual [2,7-91. The sites of distal spread vary and occur in 8-12% of patients [2,7-91. In the Mayo Clinic experience 50% of patients with distal recurrence had multiple sites of recurrence, including metastases to the lung, extragenital skin, bone, and intraabdominal sites in three patients each; to the liver in two patients; and to the heart and central nervous system in one patient each [15]. Hacker et al. reported two patients with negative inguinal nodes who developed pulmonary metastases, one patient with two positive inguinal nodes who developed pulmonary metastases, and eight patients with three or more positive groin nodes who died with multiple sites of distal recurrence. Of these last eight patients, three developed
220
STEELE
ET AL
been associated with other maliiancies. Although the second primaries are usually located within the genital tract, associated cancers have included leukemia and neoplasms of the skin, urinary bladder, colon and rectum, breast, stomach, parotid gland, pancreas, lung, and oropharynx [5,9,10,16,17]. The mechanism for skeletal muscle metastasis in vulvar cancer is not readily apparent. REFERENCES I, Ho&ins,
W. D., Perez, C., and Young, R. C. Gyneculogi~ m&g(S. T. &Vita. S. nancies, in Principles und practice qfoncology
Mellmau, and S. A. Rosenberg, Eds.), 3rd ed., J.B. Lippincott, Philadelphia
(1989).
2. Krupp, P. J. Invasive tumors of vulva: Clinical features and management, in Gynec&gic oncology (M. Coppleson, Ed.), Churchill Livingstone, New York, pp. 329-338 (1481). 3. Figge, D. C., Tamini, H. K., and Greer, B. E. Lymphatic spread in carcinoma of the vulva, Amer. J. Ubstet. Gynecof. 152, 387394 (19851. 4. Krupp. P. J., and Bohm, J. W. Lymph giand metastases in invasive squamous cell cancer of the vulva, Amer. J. i&let. liynecof. 30, 943-452 (1978). 5. Krupp, P. J., Lee, F. Y., Batson, H. W., AIlen, P. M., and Collins, J. H. Carcinoma of the vulva, Gynccol. Untoi. 1, 345-362 (1973). 6. Hacker, N. F., Berek, J. S., Legasse, L. D., Leuchter, R. S., and Moore, J. G. Management of regional lymph nodes and their prognostic influence in vulvar cancer, Obs!er. Gynecoi. Cl, 408-412 {1983j. 7. Monaghan, J. M., and Mammond, I, G. Pelvic node dissection in the treatment of vulvar cancer-It is necessary? &-it. J. Obstet. Gynaecol.
F1G. 2. Tumor histopathology consistent with metastatic squamous cell carcinoma of vulvar or&~ H&E, x 3 LO.
pulmonary metastases, two developed skin metastases, and one developed hepatic metastases [6]. Other authors also list the lung, liver, bone, SupraclavicuIar lymph nodes, and extragenital skin as sites of distant metastases
~1,U~l.
91, 270-274 (1984).
8. Krain, L. S. Carcinoma of the vulva in California 1942%I%Y: The California Tumor Registry Experience, Oncology 28, 110-l 16 (1973). 9. Japcze, H., Gargia-Bunnvel, R., and Woodruff, J. D. Primary vulvar neoplasia: A r-e&w of in situ and invasive carcinoma 193572, Qbsrer. Gynecal. 49, 404-411 (1977). 10, Taussig, F, J. Cancer of the vulva: An aualysis of 155cases, Amer. J. Obstet. Gynecnl. 40, 764 (1940), 11 Way, S. Malignant disease uf the female g~nitul tract, Churchill, London (1951). 12 Way, S., and Mennigan, M. The late results of extended radical vulvectomy for carcinoma of the vulva, J. O&et. Gynaecoi. Briif. Commonw. 73, 594-611 (1966). 13 Way, S. The anatomy of the lymphatic drainage of the vulva and its influence on the radical operation for carcinoma, Ann. R. Coli. Surg. Engl. 3, 187 (1948). 14. Plentl, A. A., and Friedman, E. A. Lymphatic system ofthefimale genitalia: The morphologic basis of oncologic diagnosis and therupy. Saunders, Philadelphia, 168-180 (1973). 15, Podratz, K. C., Symmonds, R. E., and Taylor, W. F. Carcinoma of the vulva: Analysis of treatment hlures, Amer. 3. Obstet. Gy-
A search of the English literature reveals no prior cases metastasic to skeletal muscle. Our patient was initially diagnosed as having a Baker’s cyst. After serial aspirations the mass persisted, increasing the suspicion of malignancy. Recurrence in the patient’s groin made retrograde lymphatic metastasis to the popliteal lymph nodes a possibility, but CT scan localized the mass to the belly of one of the hamstring muscles with no evidence af skin or subcutaneous tissue involvement (Fig. la). The popliteal fossa, in particular, appeared free of need. 143, 340-3S1 (1982). disease (Fig. lb). 16. Podratz, K. C., Symmonds, R. E., Taylor, W, F., and Williams, Since the occurrence of metastatic vulvar cancer in T. J. Carcinoma of the vulva: Analysis of treatment and survival, skeletal muscle had not been described previously, the Ubster. Gynecol. 61, 43-74 (1983). mass was thought to be a soft tissue sarcoma, not sur- 17. Green, T. H.. Ulfelder, i-l., and Meigs, J. V. Epiderrnoid carciDrisine since sauamous cell cancer of the vulva has lone noma of vulva, Amer. J. Gynecul. 75, 834-847 (1958). L
the
Obstst.
39, 21%220(1990)
CASE REPORT An Unusual Presentation of Metastatic Squamous Cell Carcinoma of the Vulva D. MICHAEL STEELE, M.D.,* Departments
of *Surgery,
tPathology,
LINDA R. HEGSTRAND, M.D. ,t THOMAS M, JULIAN, M.D.,+ AND F. KRISTIAN STORM, M.D.“*
and SObstetrics and Gynecology, University of Wisconsin School Cancer Center, 600 Highland Avenue, Madison, Wisconsin 53792
of Medicine and Comprehensive
Received May 10, 1990
Recurrent squamous cell cancer of the vulva metastasized via regional lymphatics. Hematogenous spread is late and unusual. A patient with metastatic diseasepresented with soft tissue mass in her thigh musculature. We believe this to be the first reported case of noncontiguous skeletal metastasis for this tumor. Q 1990 Academic Press, Inc. INTRODUCTION Recurrent squamous cell cancer of the vulva is a slowly progressive tumor that advances locally and then spreads via regional lymphatics. Hematogenous spread is late and uncommon. An elderly woman presented to our institution for treatment of a large, mixed, cystic and solid mass, characteristics of a primary soft tissue tumor, in her left thigh muscles. Biopsy of this mass showed metastatic vulvar carcinoma. This is the first reported case of noncontiguous, muscle metastasis of this tumor presenting as a soft tissue mass of unknown etiology. CASE REPORT
Mrs. A is a 68-year-old nulliparous woman of Italian extraction who initially presented with a 3-week history of vaginal bleeding, foul-smelling discharge, and a lump in her vulva. Examination showed a large, fungating, necrotic tumor replacing the patient’s entire vulva and extending above the clitoris. The tumor extended to but did not involve the urethra. Bilateral groin examination ’ To whom correspondence should be addressed.
showed 3-cm firm, fixed inguinal nodes. Biopsy was consistent with poorly differentiated squamous cell cancer, T3 N3 MO, or stage III disease. The patient underwent external-beam radiation therapy to 70 Gy delivered in 35 fractions over a 67-day period. Included were 50 Gy to the genitalia and both inguinal regions, a 20-Gy bolus to the vulva, and lo-Gy boluses to both inguinal regions. Two weeks after completion of radiotherapy she was noted to have persistence in the left labium majorum and underwent partial vulvectomy followed by a radium implant. Two and one-half months postoperatively she had a local recurrence in the posteromedial aspect of her vulvectomy scar, in the anterior portion of the pubic region, and in the left vulva. She developed progressive, intermittent pain and swelling in her left posterior thigh above the knee. An g-cm nontender solid/cystic lesion unassociated with erythema or warmth was found in her thigh. Aspiration of this mass produced chocolate-colored, heme-positive fluid. Cultures and gram strains were negative. Cytology was suspicious but not diagnostic for malignancy. A CT scan showed a large, irregular, thick-walled mass on the posterior aspect of the left thigh just above the knee in the belly of the hamstring muscle (Fig. la). The popliteal fossa was uninvolved (Fig. lb). The mass enlarged to 16 cm in diameter. The patient was referred to the University of Wisconsin for evaluation and treatment of a suspected primary soft tissue tumor. A core needle biopsy was performed showing well-differentiated metastatic squamous cell cancer consistent with the primary vulvar tumor (Fig. 2).
218 OO!N-8258/!30 $1.50 Copyright 0 I!390by AcademicPress,Inc. All rights of reproductionin any form reserved.
CASE REPORT
219
FIG. 1. I !xtremity CT scan showing (a) mixed cystic-solid mass within posterior thigh musculature and (b) uninvolved popliteal fossa.
DISCUSSION Cancer of the vulva accounts for 3% of gynecologic malignancies [l]. With the continued aging of the population this percentage is likely to increase. These tumors are squamous cell neoplasms which grow slowly and progress from dysplasia to carcinoma in situ, invasive carcinoma, and metastatic disease [2]. Regional, metastatic spread of these tumors is via the superficial inguinal lymph nodes, the ipsilateral deep inguinal lymph nodes, and the ipsilateral deep pelvic lymph nodes in a sequential manner [l-6]. The importance of the draining lymphatics and their role in the natural history and surgical cure of vulvar malignancy were first noted by Taussig [lo], and then by Way and Mennigan [ 1l-131, revolutionizing the treatment of this disease. More recently,
Plentl and Friedman have thoroughly described the lymphatic drainage of the vulva [14]. Hematogenous spread is late and unusual [2,7-91. The sites of distal spread vary and occur in 8-12% of patients [2,7-91. In the Mayo Clinic experience 50% of patients with distal recurrence had multiple sites of recurrence, including metastases to the lung, extragenital skin, bone, and intraabdominal sites in three patients each; to the liver in two patients; and to the heart and central nervous system in one patient each [15]. Hacker et al. reported two patients with negative inguinal nodes who developed pulmonary metastases, one patient with two positive inguinal nodes who developed pulmonary metastases, and eight patients with three or more positive groin nodes who died with multiple sites of distal recurrence. Of these last eight patients, three developed
220
STEELE
ET AL
been associated with other maliiancies. Although the second primaries are usually located within the genital tract, associated cancers have included leukemia and neoplasms of the skin, urinary bladder, colon and rectum, breast, stomach, parotid gland, pancreas, lung, and oropharynx [5,9,10,16,17]. The mechanism for skeletal muscle metastasis in vulvar cancer is not readily apparent. REFERENCES I, Ho&ins,
W. D., Perez, C., and Young, R. C. Gyneculogi~ m&g(S. T. &Vita. S. nancies, in Principles und practice qfoncology
Mellmau, and S. A. Rosenberg, Eds.), 3rd ed., J.B. Lippincott, Philadelphia
(1989).
2. Krupp, P. J. Invasive tumors of vulva: Clinical features and management, in Gynec&gic oncology (M. Coppleson, Ed.), Churchill Livingstone, New York, pp. 329-338 (1481). 3. Figge, D. C., Tamini, H. K., and Greer, B. E. Lymphatic spread in carcinoma of the vulva, Amer. J. Ubstet. Gynecof. 152, 387394 (19851. 4. Krupp. P. J., and Bohm, J. W. Lymph giand metastases in invasive squamous cell cancer of the vulva, Amer. J. i&let. liynecof. 30, 943-452 (1978). 5. Krupp, P. J., Lee, F. Y., Batson, H. W., AIlen, P. M., and Collins, J. H. Carcinoma of the vulva, Gynccol. Untoi. 1, 345-362 (1973). 6. Hacker, N. F., Berek, J. S., Legasse, L. D., Leuchter, R. S., and Moore, J. G. Management of regional lymph nodes and their prognostic influence in vulvar cancer, Obs!er. Gynecoi. Cl, 408-412 {1983j. 7. Monaghan, J. M., and Mammond, I, G. Pelvic node dissection in the treatment of vulvar cancer-It is necessary? &-it. J. Obstet. Gynaecol.
F1G. 2. Tumor histopathology consistent with metastatic squamous cell carcinoma of vulvar or&~ H&E, x 3 LO.
pulmonary metastases, two developed skin metastases, and one developed hepatic metastases [6]. Other authors also list the lung, liver, bone, SupraclavicuIar lymph nodes, and extragenital skin as sites of distant metastases
~1,U~l.
91, 270-274 (1984).
8. Krain, L. S. Carcinoma of the vulva in California 1942%I%Y: The California Tumor Registry Experience, Oncology 28, 110-l 16 (1973). 9. Japcze, H., Gargia-Bunnvel, R., and Woodruff, J. D. Primary vulvar neoplasia: A r-e&w of in situ and invasive carcinoma 193572, Qbsrer. Gynecal. 49, 404-411 (1977). 10, Taussig, F, J. Cancer of the vulva: An aualysis of 155cases, Amer. J. Obstet. Gynecnl. 40, 764 (1940), 11 Way, S. Malignant disease uf the female g~nitul tract, Churchill, London (1951). 12 Way, S., and Mennigan, M. The late results of extended radical vulvectomy for carcinoma of the vulva, J. O&et. Gynaecoi. Briif. Commonw. 73, 594-611 (1966). 13 Way, S. The anatomy of the lymphatic drainage of the vulva and its influence on the radical operation for carcinoma, Ann. R. Coli. Surg. Engl. 3, 187 (1948). 14. Plentl, A. A., and Friedman, E. A. Lymphatic system ofthefimale genitalia: The morphologic basis of oncologic diagnosis and therupy. Saunders, Philadelphia, 168-180 (1973). 15, Podratz, K. C., Symmonds, R. E., and Taylor, W. F. Carcinoma of the vulva: Analysis of treatment hlures, Amer. 3. Obstet. Gy-
A search of the English literature reveals no prior cases metastasic to skeletal muscle. Our patient was initially diagnosed as having a Baker’s cyst. After serial aspirations the mass persisted, increasing the suspicion of malignancy. Recurrence in the patient’s groin made retrograde lymphatic metastasis to the popliteal lymph nodes a possibility, but CT scan localized the mass to the belly of one of the hamstring muscles with no evidence af skin or subcutaneous tissue involvement (Fig. la). The popliteal fossa, in particular, appeared free of need. 143, 340-3S1 (1982). disease (Fig. lb). 16. Podratz, K. C., Symmonds, R. E., Taylor, W, F., and Williams, Since the occurrence of metastatic vulvar cancer in T. J. Carcinoma of the vulva: Analysis of treatment and survival, skeletal muscle had not been described previously, the Ubster. Gynecol. 61, 43-74 (1983). mass was thought to be a soft tissue sarcoma, not sur- 17. Green, T. H.. Ulfelder, i-l., and Meigs, J. V. Epiderrnoid carciDrisine since sauamous cell cancer of the vulva has lone noma of vulva, Amer. J. Gynecul. 75, 834-847 (1958). L
the
Obstst.