Imelda Ghofar, Bethseba B Pulinggomang,Harjoedi Adji Tjahjono,Susanto Nugroho,Sony Wicaksono
Dr Saiful Anwar General Hospital Malang
Background : Background: Rhabdomyosarcoma is the most common soft-tissue sarcoma in children and rhabdomyosarcomas constitute about 50% of soft tissue sarcomas. This tumor accounts for 10% to 15% of solid malignant tumors and 6% of all malignancies in children under 15 years of age.
Case Presentation Summary : Case Report: A 3 – years – old girl had a history of clitoromegaly for 1 year. No history of pain, itching, discharge nor bleeding from the mass, and had been increasing in size over 7 months. No mass in other part of her body. On general physical examinations was normal, and her nutritional status was good. Laboratory findings were normal (17-OH Progesterone 0.48ng/mL, progesterone <0.21 ng/mL, testosteron <2.5 ng/dL). Pelvic ultrasound revealed no abnormalities, and present a solid hypervascular mass in supraclitoris. Mass biopsy revealed malignant small rounded cell tumor mostly embryonal rhabdomyosarcoma. A pelvic MRI examination after biopsy revealed there is no recidive mass in supraclitoris. She underwent an excision of the skin surrounding the clitoris with wide skin edges and complete removal of the mass. Then she recieved brachytheraphy for rhabdomyosarcoma, with dose 36 Gy in 12 fraction (3 Gy/fraction delivered twice a day). However in the follow up, the progression of the vulvar tissues showed sensitive to radiation and no complication.
Learning Points/Discussion : Summary: Clitoral tumors was very rare case and should be considered in the differential diagnosis especially with normal hormonal studies for exclusion of adrenogenital syndromes. Systemic control is achieved by chemotherapy even for patients with localized disease to eradicate the micrometastasis. Local control is accomplished by complete surgical excision and/or local irradiation. The treatment for our patient was surgical excision with wide skin edges and complete removal the mass, and followed by brachytherapy low-dose. In the follow-up her vulvar tissues are very sensitive to radiation.