Prevalence and incidence of vulvar cancer in developing nations tend to have a relatively high as compared to that of the developed nations . Squamous cell carcinoma is the most common histology of vulvar cancer. Other less common histological subtypes are extramammary Paget’s disease, melanoma, Bartholin’s gland tumors, adenocarcinoma, and basal cell carcinoma . About two-thirds (65%) of our patients presented in advanced stage (FIGO stage III–IV). This figure corresponds to the same range as in the previously published literature .
Surgical management should be individualized. Even though the majority of the patient had undergone wide local excision with primary repair in our study, the margins had never been compromised and the oncological outcome had always been taken as a priority. The psychosexual sequelae and surgical morbidities associated with vulvar surgery and groin nodal dissection have driven treatment approaches to the more conservative ones. Only 10 patients were followed up beyond 6 months. The reason for poor follow-up could be due to poor patient compliance, as most of the patients were from low or modest socioeconomic status, uneducated and negligence. Clinical and histological nodal positivity were seen in 8 and 13 patients respectively. Among these, six patients developed either loco-regional (n = 3) or distant metastasis (n = 3) in a follow-up period. The present study suggests that the stage at presentation and lymph node positivity have poor prognostic values. Ipsilateral lymph node dissection is indicated for unilateral lesions, not crossing midline, and either negative ipsilateral nodes, or with positive lymphadenopathy with vulvar lesion smaller than 2 cm [8,9,10]. Also, the depth of invasion (DOI) was reported in only 2 patients histopathological reports. They had more than 6 mm DOI, along with few positive groin lymph nodes. The strict adherence to FIGO staging is important for disease prognostication and treatment outcome [11, 12].
In our study, nodal positivity was solely the most important bad predictive and prognostic factor; nevertheless, the final tumor stage, histology, the degree of differentiation, depth of invasion and lymphovascular invasion (LVI) also decide the survival outcome in literature [13,14,15]. The reason for the minimal inguinal and pelvic lymphadenectomy wounds morbidity could be due to Ray’s ‘River flow’ incision (two parallel curvilinear incisions) [5, 6] technique for ilioinguinal dissection. This may be contrary to the author Siller et al. , who had reported a major wound breakdown rate of 15–30%. In our study, adjuvant radiation was given based on lymph node metastasis, close surgical margin, size, and depth of the primary tumor. Out of 17 eligible patients for adjuvant treatment, only 11 patients had received it. The potential reasons for not getting treatment to the remaining six patients were defaulted follow-up and non-willingness due to logistic issues. Neoadjuvant radiotherapy/chemotherapy was not frequently practiced in our institutional setting that is why only one patient was treated with neoadjuvant chemoradiotherapy followed by surgery. However, the recent trend is shifting toward conservative surgery with the combined use of preoperative radiotherapy or chemo-radiotherapy [17,18,19,20]. The 5 years disease-free survival is 66%, which is comparable to the studies published by Sharma DN. et al. , Singh N. et al. , Rajshree D K. et al. , and Meelapkij P. et al. . There are no large randomized controlled trials or meta-analysis because of the rarity of the disease. So, treatment guidelines are based on small retrospective individual center-based studies in the literature.
The majority of the patients present in the advanced stage in developing countries due to social stigma, low to middle socioeconomic status, low literacy rate, logistic issues, poor screening program, and insufficient awareness about the disease. Public awareness of warning symptoms of vulvar malignancy may help in early detection and cure. There is no current evidence for a specific screening of vulvar cancer. However, self-examination in women with lichen sclerosis advised for early detection of vulvar neoplasm . Also, any patients with suspicious signs (e.g., pigmented lesions, irregular ulcers) or symptoms (e.g., chronic vulvar pruritus) should be early evaluated with skin biopsy . Further research is warranted with large multicentric prospective randomized controlled trials to establish the definite screening guidelines, treatment protocols and survival outcome data for this rare gynecological malignancy in low-middle income countries.