జర్నల్ ఆఫ్ ఆంకాలజీ మెడిసిన్ & ప్రాక్టీస్

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcomas. It was described for the first time by Weber in 1854. RMS is recognized in approx. 90% of patients fewer than 20 years of age. The disease in adults is extremely rare.
Materials and methods: A retrospective analysis was performed on 5 cases of RMS of the female genital tract. These 5 cases of the disease in women aged 20 and 87 years of age treated in a Warsaw hospital from 1991 to 2013. In all women the diagnosis was made on the basis of histopathological examination of tissues after excision of changes in the vulva and vagina. Tumors were classified according to the Intergroup Rhabdomyosarcoma Study (IRS) staging. All patients were treated with multimodality treatment: surgery, chemotherapy and/or ratiotherapy.
Results: Of the five patients with RMS, three originated from the vagina one from vulvae and one from Bartholin’s glands. Two out of 5 patients presented with distant metastasis and death after 9 and 4 years. Tree patients alive for 3, 10, 11 years without recurrence or active disease a median duration was of 7.7 years.
Conclusion: RMS in adults have poor prognosis as compared to childhood RMS. Adult RMS should therefore be treated aggressively with multidisciplinary approach comprising of surgery, radiation, and chemotherapy to achieve cure and prolonged survival. The prognosis of embryonal rhabdomyosarcoma of female genital tract is good. The standard treatment for adults RMS consisting the multimodal therapy with radical surgery and chemotherapy and radiotherapy.

Medulloepithelioma is a rare congenital neuroepithelial tumor commonly arising from the non-pigmented ciliary body epithelium and rarely from iris, retina or the optic nerve. It occurs in patient under 10 years. It is a rare neuroepithelial tumor and is the second most frequent intraocular tumour in children after retinoblastoma. Unlike cases reported in the literature in which the tumour recurs rapidly, recurrence occurred in our case five years later.