Soft tissue sarcoma of the vulva: A clinical study

Abstract

The purpose of this study is to report clinical aspects and treatment results of patients seen at Johns Hopkins. A search of the tumor registry of the Sidney Kimmel Comprehensive Cancer Center found 453 patients with malignancies of the vulva registered between 1977 and 1997. Patient and tumor characteristics, treatment methods, and follow-up were obtained from charts. Seven patients were identified with sarcoma of the vulva. Of these, one was removed from analysis due to histology. Three patients had leiomyosarcoma, two had fibrosarcoma, and one had epithelioid sarcoma. The mean age was 41. Mean time to diagnosis was 6 months. All but one of the tumors was located on the labia majora. Median tumor size was 3.5 cm. Surgery varied from wide local excision to radical vulvectomy with inguinal lymph node dissection. Surgical margins were microscopically negative in five of the six cases. Two patients had received adjuvant external beam radiation. One of them had a tumor greater than 5 cm and close surgical margins and the other had high-grade tumor, which recurred after previous surgery. Mean follow-up was 127.8 months. There have been no recurrences to date.

Primary epithelioid sarcoma of the vulva in pregnancy

“…Excision should include 2 cm from the tumor margin. On the other hand, some authors prefer radical vulvectomy to local excision [9,[12][13][14][15]. Because of the aggressive biology, poor outcome, and resistance to radiation and chemotherapy of ES, radical resection including inguinal lymph node dissection is suggested as appropriate treatment, except for early stage disease.…”

“…Ross et al suggested that radiation therapy had some benefits for the tumor greater than 5 cm [10]. Ulutin et al stated that definitive surgery with adjuvant radiation therapy provided excellent tumor control in low-grade sarcomas [14]. However, radiation therapy can be recommended for cases with high-grade tumor or those with margin involvement or lymph node metastasis.…”

“…Epithelioid sarcoma (ES) is extremely rare. Since Piver et al first reported a case with ES in the vulva [5], 20 cases of vulvar ES have been published in the English literature [6][7][8][9][10][11][12][13][14][15]. One was diagnosed during pregnancy [13].…”

Old and new perspectives in the management of high-risk, locally advanced or recurrent, and metastatic vulvar cancer

“…Surgery for vulvar sarcoma ranges from wide local excision to radical vulvectomy and inguinal-femoral lymphadenectomy [11,36]. Similarly, endodermal sinus tumours of the vulva, that appear to be more fatal than those arising in other sites, are surgically treated by wide local excision or modified radical vulvectomy and ipsilateral groin dissection [13,14].…”

“…Squamous cell carcinoma accounts for approximately 85-90% of vulvar cancers [4,5], melanoma for 2.4-5% [4][5][6], and Bartholin gland carcinoma for 1-3% [4,7,8]. Very rare tumours are represented by basal cell carcinoma [9], invasive Paget's disease or intraepithelial Paget's disease with underlying adenocarcinoma [10], sarcoma [11] and metastatic tumours of the vulva [12], whereas endodermal sinus tumour [13,14] and primary breast carcinoma developing within breast ectopic tissue [15] are exceptional. Verrucous carcinoma is a low aggressive variant of squamous cell carcinoma with very low propensity for lymphatic spread [16].…”