A squamous cell carcinoma with sarcomatoid features of the vulva is an extremely rare malignancy of the female genital tract. This type of tumor is known to grow rapidly and associated with poorer clinical outcomes than those of squamous cell carcinoma of the vulva.
A 43-year-old woman presented to our institute with a 4-month history of an aggravated vulvar mass. A radical local excision, bilateral inguinal lymph node dissection and laparoscopic assisted vaginal hysterectomy were performed. The FIGO stage of the vulvar cancer was stage II (T(2)N(0)M(0)) and the pathologic finding was consistent with a poorly differentiated squamous cell carcinoma with extensive sarcomatoid features. No further treatment was given and there was no clinical evidence of recurrence during the 2 years of follow-up.
A squamous cell carcinoma with sarcomatoid features of the vulva is a tumor with aggressive biological behavior. To date, there have been only 15 cases of this disease reported in the literature. So, a collection and close study of these cases would be extremely useful in singling out and identifying the best treatment possible for this type of tumor.
"Spindle cell carcinomas consisting of spindle cells alone have been reported only incidentally in vulvar carcinoma, and it is thought to be an aggressive tumour type that occurs in 1–5% of all VSCC patients (Steeper et al, 1983; Santeusanio et al, 1991; Cockayne et al, 1998; Cooper et al, 2002; Santos-Briz et al, 2002; Choi et al, 2006; Petrillo et al, 2011; Terada, 2011). Some VSCCs show a specific pattern of invasion in which there is infiltration of the stroma by single cells or cords of tumour cells adjacent to the 'conventional' squamous cell carcinoma cells. "
[Show abstract][Hide abstract] ABSTRACT: Background:
Vulvar cancer is the fourth most common gynaecological malignancy, with an annual incidence of 2 out of 100 000 women. Although most cases of early stage vulvar cancer have a good prognosis, recurrence and rapid tumour progression can occur. We investigated the prevalence of spindle cell morphology in vulvar cancer and its association with survival.
This retrospective cohort study included 108 patients with primary vulvar squamous cell carcinoma who were treated at the Leiden University Medical Center during 2000–2009. Paraffin-embedded tissue was examined for the presence of spindle cell morphology. Survival and histology data were compared between cases with spindle and without spindle cell morphology.
Twenty-two (20%) tumours showed spindle cells infiltrating the stromal tissue. All spindle cell tumours were human papillomavirus (HPV) negative. Spindle cell morphology was strongly associated with poor prognosis and with a high risk of lymph node involvement at the time of diagnosis (relative risk 2.26 (95% CI 1.47–3.47)). Five-year disease-specific survival was lower in patients with vs without spindle cell morphology (45.2% vs 79.7%, respectively; P=0.00057).
Vulvar spindle cell morphology occurs frequently and seems to develop through the non-HPV pathway. It is associated with a worse prognosis than conventional vulvar squamous cell carcinoma.
British Journal of Cancer 09/2013; 109(8). DOI:10.1038/bjc.2013.563 · 4.84 Impact Factor
"Management of this kind of lesions includes a multidisciplinary approach involving the gynecologist, plastic surgeon, dermatologist, and histopathologist. If the tumor is suitable of surgery, a surgical excision with wide margins of 3 to 5 cm of normal skin should be performed . Our patient presented a fast-growing cutaneous lesion, so the diagnosis was relatively early. "
[Show abstract][Hide abstract] ABSTRACT: Type 1 neurofibromatosis (NF1) is a dominantly inherited neurologic disorder that affects primarily the skin, bones, and peripheral nervous system. It may be associated with a variety of clinical manifestations including cafe-au-lait spots, skinfold freckling, Lisch nodules, and visceral neurofibromas. Individuals affected by NF1 harbor an increased risk for both benign and malignant tumors. Malignant transformation is usually observed in the form of neurosarcoma. Rarely, NF1 affects the genital tract, and isolated vulvar localization is extremely rare. Here is reported a rare case of a solitary neurosarcoma of the vulva in a 43-year-old woman affected by NF1 syndrome treated with surgical excision. The purpose of this case is to underline the possibility of association between NF1 and genital tract sarcoma and to suggest an accurate evaluation of rapid growth vulvar mass in this setting.
" up, SSCC recur rapidly and metastasize to regions such as the peritoneum, kidney, and subcutaneous tissue. Most cases of SSCC of the female genitalia have been reported to arise from the cervix and vulva. There are currently 16 cases of cervical and vulvar SSCC reported in the English literature to date. (Tae-Wook Kong et. al., 2010 and Dong-Seok Choi et. al., 2006), with our current case of vulvar SSCC making up the 17 th case."
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