ORIGINAL ARTICLE |
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Year : 2019 | Volume : 2 | Issue : 2 | Page : 25-27 | DOI : https://doi.org/10.46319/RJMAHS.2019.v02i02.007 |
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Clinical and oncology work-up including CT scan abdomen, MRI pelvis, cystoscopy, pap smear (negative for intra epithelial lesion- Figure 2) and a biopsy revealed a differential diagnosis of a poorly differentiated, non-keratinising squamous cell carcinoma or a malignant melanoma. On immunohistochemistry, the tumor was positive for HMB45 and S100 confirming the diagnosis of primary vaginal malignant melanoma. (Figure 3).
She was then referred to a Radiotherapy unit where after proper assessment of her general condition she was planned for concomitant chemoradiation.
Discussion
Primary vaginal malignant melanoma was first reported in 1887 with peak age of incidence between 35 and 70 years of age, and about 73% of cases occurring in women older than 60 years.
They present with foul smelling vaginal discharge, pelvic pain, pelvic mass, vaginal bleeding, and inguinal lymph node enlargement. The cells of origin are melanocytes which are located in the vaginal mucosa. The commonest location is lower one-third of the vagina and antero-lateral aspect without involving the vulva. Around twenty percent of mucosal melanomas have multiple foci, and only about 10%–23% are amelanotic melanoma.[4] There is an increased risk of recurrence, metastases to distant sites and poor prognosis.
Various treatment options are available with no clear consensus on the standard approach. The 1st line of management is resection of the tumor surgically with improved survival benefits.[5] It can range from wide local excision along with Sentinel lymph node dissection (SLND) or Elective lymph node dissection (ELND), Complete Vaginectomy or Radical extirpation with en bloc removal of the involved pelvic organs. However, research till date shows that in reality there exists no much difference in the survival rates among patients who were treated with radical surgery and those patients who had conservative surgery like wide local excision. Controversies still remain unsettled on the role of Elective lymph node dissection. SLND remains popular as it gives important data on prognosis and staging with less morbidity.
Radiotherapy is indicated in patients with surgically unresectable lesions or as Adjuvant therapy in cases of histopathologically proven positive tumor margins or positive lymph nodes.
Adjuvant chemotherapy is indicated in such high-risk cases. The chemotherapeutic agents include dacarbazine, temozolomide and platinum based compounds, nitrosourea and taxanes either as monotherapy or combination therapy have been tried with limited success. The rate of response is 11%–22% and the median survival is 5.6–11 months.[6]
Role of monoclonal antibodies (MABs): Ipilimumab is a Cytoxic T- lymphocytic-associated protein - 4 has demonstrated remarkable promise in patients with unresectable/metastatic melanoma. Nivolumab is a monoclonal antibody to programmed cell death receptor – 1 protein (PD-1). It blocks the interaction between PD-1 and its legends PDL-1 and PDL-2. It is indicated for unresectable/metastatic melanoma.
Conclusion
Primary vaginal malignant melanoma is a rare and aggressive condition which affects women of sixth and seventh decade. Prognosis depends on the clinical stage at presentation and the tumor size. Primary efficient modality of treatment is surgery which can significantly improve survival. Following surgery, adjuvant therapy in the form of radiation, chemotherapy, immunotherapy and targeted therapy can be useful in preventing recurrence. Survival largely depends on lymph nodal and metastases to distant sites after the initial resection of tumor. As it's a rare tumor, there is a need to formulate a standard therapeutic regime.
Financial support and sponsorship: Nil
Conflict of interest: Nil
References
1. Creasman WT, Philips JL, Menck HR,. The National Cancer Data Base report on cancer of the vagina. Cancer. 1998; 83(5):1033-40.
2. Pride GL, Schultz AE, Chuprevich TW. Primary invasive carcinoma of the vagina. Obstet Gynecol. 1979; 53:218- 25.
3. Sheets EE, Berman ML, Hrountas CE, et al. Surgically treated, early stageneuroendocrine small-cell cervical carcinoma. Obstet Gynecol 1988;7:10–14.
4. Miner TJ, Delgado R, Zeisler J, Busam K, Alektiar K, Barakat R, et al. Primary vaginal melanoma: A critical analysis of therapy. Ann Surg Oncol 2004;11:34-9.
5. Frumovitz M, Etchepareborda M, Sun CC, Soliman PT, Eifel PJ, Levenback CF, et al. Primary malignant melanoma of the vagina. Obstet Gynecol 2010; 116: 1358-65.
6. Lin LT, Liu CB, Chen SN, Chiang AJ, Liou WS, Yu KJ, et al. Primary malignant melanoma of the vagina with repeated local recurrences and brain metastasis. J Chin Med Assoc 2011;74: 376-9.
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