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Vulvar dermatofibrosarcoma protuberans
GYNECOLOGIC
ONCOLOGY
30, 149-152 (1988)
Vulvar Dermatofibrosarcoma Protuberans’ DANNY
R. BARNHILL, M.D.,* RALPH BOLING, D.O., WILEY NOBLES, M.D., LIDA CROOKS, M.D., AND THOMAS BURKE, M.D.
Department of Obstetrics and Gynecology and the Department of Pathology, Brooke Army Medical Center, Ft. Sam Houston, Texas 78234, and *Gynecologic Oncology Service, Department of Obstetrics and Gynecology, Walter Reed Army Medical Center, Washington, D.C. 20307 Received May 19, 1987 Dermatofibrosarcoma protuberans of the vulva is an extremely rare neoplasm. This lowgrade sarcoma of the dermis clinically appears to be encapsulated but microscopically has tumor projections well away from the central nodules. Wide local excision is the recommended treatment for dermatofibrosarcoma protuberans; however, a more radical surgical approach may be needed to obtain clear surgical margins. The following case study presents the fifth reported patient with this unusual neoplasm on the vulva. 0 1988 Academic Press. Inc.
INTRODUCTION
Dermatofibrosarcoma protuberans is a rare, low-grade sarcoma of the dermis. It is a slowly growing, fibrous tumor that is locally aggressive, frequently recurrent, and rarely metastatic [l-3]. It characteristically appears as a multinodular, firm, well-circumscribed mass laying just beneath the epidermis [l]. The tumor is usually nontender; however, those with more rapid growth may cause mild pain. Ulceration of the overlying skin can occur with fast growing tumors. These neoplasms may develop in any cutaneous surface although they have not been reported on the palms or soles [3]. The treatment is wide local excision [l-51. While surgical therapy is usually curative, deaths due to this tumor have been reported [5]. A review of the literature reveals only four reported casesof dermatofibrosarcoma protuberans occurring on the vulva [2,3,6,7]. The following case report presents an additional patient with this unusual vulvar neoplasm. CASE REPORT
The patient is a 45year-old G3P2A1Caucasian female who underwent a total abdominal hysterectomy at age 34 for recurrent cervical dysplasia. On routine ’ The assertions and opinions contained herein are those of the authors and are not to be construed as official or as representing the views of the Department of Defense or the Department of the Army. 149 0090~8258/88$1.50 Copyright 0 1988 by Academic Press, Inc. All rights of reproduction in any form reserved.
150
BARNHILL
ET AL.
FIG. 1. Photomicrograph showing dermatofibrosarcoma protuberans infiltrating the subcutaneous tissue and surrounding individual fat cells (X 30.8).
examination at age 42, a 1 x l-cm, firm, nontender, well-circumscribed cutaneous nodula was found in the anterior right vulva lateral to the clitoris. The nodule was excised, and, on histologic examination, it was identified as a dermatofibroma. No further therapy was administered. At age 45, the patient noted a similar nodule in the same location, and 6 months later she presented for evaluation. Excisional biopsy of this cutaneous nodule was performed. Histologic examination revealed dermatofibrosarcoma protuberans with all surgical margins involved. Review of microscopic sections from the original tumor showed that it was also consistent with dermatofibrosarcoma protuberans, and had been incompletely excised. Microscopically, the dermis and subcutaneous tissue were infiltrated by the neoplasm (Fig. 1). The cells were spindle shaped, and generally arranged in a storiform or woven mat configuration (Fig. 2). Mitotic figures were rare, and microscopic projections of the tumor extended well away from the central nodules. The patient was referred to the Gynecologic Oncology Service where her examination revealed two residual 1 x l-cm nodules in the anterior right vulva, 1.5 cm lateral to the clitoris. The remainder of the physical examination was normal. There were no clinically suspicious inguinal lymph nodes, and an abdominopelvic CT scan showed no lymphadenopathy. A wide surgical excision was performed with 2-cm margins except in the area of the clitoris where the margin was 1.5cm. Microscopic examination of the excised tissue showed extension
CASE
REPORTS
151
FIG. 2. Photomicrograph demonstrating the typical storiform appearance of dermatofibrosarcoma protuberans (X 77).
of the tumor to the superior and medial margins. Six weeks later, the patient underwent a radical hemivulvectomy with removal of the clitoris. The margins of resection were free of tumor. Her postoperative course was uncomplicated. She is clinically free of disease 12 months after this surgery. DISCUSSION
While dermatofibrosarcoma protuberans clinically appears to be a well-circumscribed nodular tumor, this is usually not the case. Condensation of the connective tissue at the edge of the nodule gives the false impression of encapsulation [5]. Fine microscopic projections of the tumor may actually extend well beyond the apparent margins. Histologically, this low-grade sarcoma of the dermis is composed of spindle-shaped cells with an apparent ability to make collagen. There is often a relatively uninvolved zone immediately beneath the atrophic epidermis. The usual histologic pattern is one of interwoven fibrocellular fascicles comprised of uniform spindle-shaped cells and collagen. At points of intersection of the fascicles, there may be an acellular collagenous focus from which the fascicles appear to radiate. Myxoid areas may be prominent. The mitotic rate is usually very low with the finding of zero to four mitoses per 10 power fields
[51. Complete surgical excision is the treatment for dermatofibrosarcoma protuberans. With the clinical appearance of encapsulation and the microscopic finding of
152
BARNHILL
ET AL.
tumor projections well away from the central nodules, it is understandable that complete excision may be difficult, and recurrences may be common. Patients who have developed lymph node metastases and who later died generally had persistent or recurrent tumors [5]. Surgical margins of at least 3 cm from the palpable tumor edge have been recommended [3-51; however, margins of this size may be difficult to obtain on the vulva. Of the four previously reported patients with vulvar dermatofibrosarcoma protuberans, three were treated with wide local excision, and one underwent a radical vulvectomy [2,3,6,7]. Three of the patients had been followed for 6 months, and one for 20 years. All were clinically free of disease at the time the reports were published. While conservative vulvar surgery may be attempted in order to obtain a good cosmetic result or to preserve structures like the clitoris, it is important that the surgical margins be critically examined. In the event that margins are involved, a more radical procedure is indicated. It is also important that gynecologists and pathologists are aware that dermatofibrosarcoma protuberans may occur on the vulva since prompt diagnosis and complete excision appear to be essential to the successful treatment of this uncommon tumor. REFERENCES 1. Shapiro, L., and Brownstein, M. Dermatofibrosarcoma protuberans, in Cancer of the SkinBiology, Diagnosis, Management (R. Andrade, S. L. Gumport, G. L. Popkin, and T. D. Rees, Eds.), Saunders, Philadelphia, pp. 1069-1078 (1976). 2. Sohan, M. Dermatofibrosarcoma protuberans of the vulva, Bn’t. J. Obstet. Gynaecol. 88, 203205 (1981). 3. Bock, J., Andreasson, B., Thorn, A., and Holck, S. Dermatofibrosarcoma protuberans of the vulva, Gynecol. Oncol. 20, 129-135 (1985).
4. Roses, D., Valensi, Q., La Trenta, G., and Harris, M. Surgical treatment of dermatofibrosarcoma protuberans, Surg. Gynecol. Obstet. 162, 449-452 (1986). 5. McPeak, C., Cruz, T., and Nicastri, A. Dermatofibrosarcoma protuberans: An analysis of 86 cases-five with metastasis, Ann. Surg. 166, 803-816 (1967). 6. Agress, R., Figge, D., Tamimi, H., and Greer, B. Dermatofibrosarcoma protuberans of the vulva, Gynecol. Oncol. 16, 288-291 (1983). 7. Davos, I., and Abell, M. Soft tissue sarcomas of vulva, Gynecol. Oncol. 4, 70-86 (1976).
ONCOLOGY
30, 149-152 (1988)
Vulvar Dermatofibrosarcoma Protuberans’ DANNY
R. BARNHILL, M.D.,* RALPH BOLING, D.O., WILEY NOBLES, M.D., LIDA CROOKS, M.D., AND THOMAS BURKE, M.D.
Department of Obstetrics and Gynecology and the Department of Pathology, Brooke Army Medical Center, Ft. Sam Houston, Texas 78234, and *Gynecologic Oncology Service, Department of Obstetrics and Gynecology, Walter Reed Army Medical Center, Washington, D.C. 20307 Received May 19, 1987 Dermatofibrosarcoma protuberans of the vulva is an extremely rare neoplasm. This lowgrade sarcoma of the dermis clinically appears to be encapsulated but microscopically has tumor projections well away from the central nodules. Wide local excision is the recommended treatment for dermatofibrosarcoma protuberans; however, a more radical surgical approach may be needed to obtain clear surgical margins. The following case study presents the fifth reported patient with this unusual neoplasm on the vulva. 0 1988 Academic Press. Inc.
INTRODUCTION
Dermatofibrosarcoma protuberans is a rare, low-grade sarcoma of the dermis. It is a slowly growing, fibrous tumor that is locally aggressive, frequently recurrent, and rarely metastatic [l-3]. It characteristically appears as a multinodular, firm, well-circumscribed mass laying just beneath the epidermis [l]. The tumor is usually nontender; however, those with more rapid growth may cause mild pain. Ulceration of the overlying skin can occur with fast growing tumors. These neoplasms may develop in any cutaneous surface although they have not been reported on the palms or soles [3]. The treatment is wide local excision [l-51. While surgical therapy is usually curative, deaths due to this tumor have been reported [5]. A review of the literature reveals only four reported casesof dermatofibrosarcoma protuberans occurring on the vulva [2,3,6,7]. The following case report presents an additional patient with this unusual vulvar neoplasm. CASE REPORT
The patient is a 45year-old G3P2A1Caucasian female who underwent a total abdominal hysterectomy at age 34 for recurrent cervical dysplasia. On routine ’ The assertions and opinions contained herein are those of the authors and are not to be construed as official or as representing the views of the Department of Defense or the Department of the Army. 149 0090~8258/88$1.50 Copyright 0 1988 by Academic Press, Inc. All rights of reproduction in any form reserved.
150
BARNHILL
ET AL.
FIG. 1. Photomicrograph showing dermatofibrosarcoma protuberans infiltrating the subcutaneous tissue and surrounding individual fat cells (X 30.8).
examination at age 42, a 1 x l-cm, firm, nontender, well-circumscribed cutaneous nodula was found in the anterior right vulva lateral to the clitoris. The nodule was excised, and, on histologic examination, it was identified as a dermatofibroma. No further therapy was administered. At age 45, the patient noted a similar nodule in the same location, and 6 months later she presented for evaluation. Excisional biopsy of this cutaneous nodule was performed. Histologic examination revealed dermatofibrosarcoma protuberans with all surgical margins involved. Review of microscopic sections from the original tumor showed that it was also consistent with dermatofibrosarcoma protuberans, and had been incompletely excised. Microscopically, the dermis and subcutaneous tissue were infiltrated by the neoplasm (Fig. 1). The cells were spindle shaped, and generally arranged in a storiform or woven mat configuration (Fig. 2). Mitotic figures were rare, and microscopic projections of the tumor extended well away from the central nodules. The patient was referred to the Gynecologic Oncology Service where her examination revealed two residual 1 x l-cm nodules in the anterior right vulva, 1.5 cm lateral to the clitoris. The remainder of the physical examination was normal. There were no clinically suspicious inguinal lymph nodes, and an abdominopelvic CT scan showed no lymphadenopathy. A wide surgical excision was performed with 2-cm margins except in the area of the clitoris where the margin was 1.5cm. Microscopic examination of the excised tissue showed extension
CASE
REPORTS
151
FIG. 2. Photomicrograph demonstrating the typical storiform appearance of dermatofibrosarcoma protuberans (X 77).
of the tumor to the superior and medial margins. Six weeks later, the patient underwent a radical hemivulvectomy with removal of the clitoris. The margins of resection were free of tumor. Her postoperative course was uncomplicated. She is clinically free of disease 12 months after this surgery. DISCUSSION
While dermatofibrosarcoma protuberans clinically appears to be a well-circumscribed nodular tumor, this is usually not the case. Condensation of the connective tissue at the edge of the nodule gives the false impression of encapsulation [5]. Fine microscopic projections of the tumor may actually extend well beyond the apparent margins. Histologically, this low-grade sarcoma of the dermis is composed of spindle-shaped cells with an apparent ability to make collagen. There is often a relatively uninvolved zone immediately beneath the atrophic epidermis. The usual histologic pattern is one of interwoven fibrocellular fascicles comprised of uniform spindle-shaped cells and collagen. At points of intersection of the fascicles, there may be an acellular collagenous focus from which the fascicles appear to radiate. Myxoid areas may be prominent. The mitotic rate is usually very low with the finding of zero to four mitoses per 10 power fields
[51. Complete surgical excision is the treatment for dermatofibrosarcoma protuberans. With the clinical appearance of encapsulation and the microscopic finding of
152
BARNHILL
ET AL.
tumor projections well away from the central nodules, it is understandable that complete excision may be difficult, and recurrences may be common. Patients who have developed lymph node metastases and who later died generally had persistent or recurrent tumors [5]. Surgical margins of at least 3 cm from the palpable tumor edge have been recommended [3-51; however, margins of this size may be difficult to obtain on the vulva. Of the four previously reported patients with vulvar dermatofibrosarcoma protuberans, three were treated with wide local excision, and one underwent a radical vulvectomy [2,3,6,7]. Three of the patients had been followed for 6 months, and one for 20 years. All were clinically free of disease at the time the reports were published. While conservative vulvar surgery may be attempted in order to obtain a good cosmetic result or to preserve structures like the clitoris, it is important that the surgical margins be critically examined. In the event that margins are involved, a more radical procedure is indicated. It is also important that gynecologists and pathologists are aware that dermatofibrosarcoma protuberans may occur on the vulva since prompt diagnosis and complete excision appear to be essential to the successful treatment of this uncommon tumor. REFERENCES 1. Shapiro, L., and Brownstein, M. Dermatofibrosarcoma protuberans, in Cancer of the SkinBiology, Diagnosis, Management (R. Andrade, S. L. Gumport, G. L. Popkin, and T. D. Rees, Eds.), Saunders, Philadelphia, pp. 1069-1078 (1976). 2. Sohan, M. Dermatofibrosarcoma protuberans of the vulva, Bn’t. J. Obstet. Gynaecol. 88, 203205 (1981). 3. Bock, J., Andreasson, B., Thorn, A., and Holck, S. Dermatofibrosarcoma protuberans of the vulva, Gynecol. Oncol. 20, 129-135 (1985).
4. Roses, D., Valensi, Q., La Trenta, G., and Harris, M. Surgical treatment of dermatofibrosarcoma protuberans, Surg. Gynecol. Obstet. 162, 449-452 (1986). 5. McPeak, C., Cruz, T., and Nicastri, A. Dermatofibrosarcoma protuberans: An analysis of 86 cases-five with metastasis, Ann. Surg. 166, 803-816 (1967). 6. Agress, R., Figge, D., Tamimi, H., and Greer, B. Dermatofibrosarcoma protuberans of the vulva, Gynecol. Oncol. 16, 288-291 (1983). 7. Davos, I., and Abell, M. Soft tissue sarcomas of vulva, Gynecol. Oncol. 4, 70-86 (1976).