Title: Rhabdomyosarcoma of the vagina in a 30-month-old girl: Refractory to conventional chemotherapy
Authors: Faisal R GURU, Shah Saqib Ahmad, Aiffa Aiman, Javvid Muzammil, Gul Muhammad
DOI: https://dx.doi.org/10.18535/jmscr/v8i8.04
Abstract
Introduction
Gynecologic tumors in children are rare and represent <5% of all pediatrics neoplasms(1). Rhabdomyosarcoma (RMS) are malignant mesenchymal tumors originating from myogenic progenitor cells. They represent the most common soft tissue tumor in childhood. The head and neck region are the most affected tumor sites, followed by the genitourinary tract(2). Four major histologic subtypes of RMS are identified, Embryonal, Alveolar, Botryoid, Pleomorphic/ undifferentiated (2,3). Botryoid variant is a type of embryonal RMS arising within the wall of the bladder or vagina, can also occur in the cervix. This tumor is seen almost exclusively in infants, it is characterized by ‘grape-like’ appearance caused by polypoid mass arising in submucosal tissue(2,4). Embryonal RMS of the vagina is a variant which represent an extreme rare and aggressive form associated with poor prognosis(2)
Case Report
An 30-month-female, full term, presented with history of vaginal swelling at the age of 28 months. A red small mass protrude through the introitus was noticed at the age of 28 months by the mother initially; there was also difficult micturition without change in urine’s color or odor. No history of medication use by mother during pregnancy. Family history was unremarkable
The baby appeared well, vitals stable.Abdomen was distended with no palpable inguinal lymph node, no hepato-splenomegaly. Pelvic examination revealed a rounded red mass 5 × 5 cm, protruding through the vaginal introitus, normal labia minora and majora. Digital rectal exam: palpable non-fixed mass pushing on the rectum. (Figure1) Other examinations were within normal.
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