Rhabdomyosarcoma is a malignant neoplasm of striate muscle occours commonly in children. They have predilection in soft tissue of head and neck(I), tongue, soft palate(2), cheek, pharynx, orbit, parotidian space, temporal space(3). Rhabdomyosarcoma also seen in adults(4). Tumor is poor prognosis with local recurrence and distant metastasis. The presented case is a more than one year old girl (DOB: 9/1999) suffered from a tumour of left Pteygoidian space 3x4 cm dimension, painless, bone erosion on Xray view. She was operated the first time for biopsic diagnosis and also for surgical treatment on 2000/11/25. Histological diagnosis: Embryonal rhabdomyosarcoma with anaplastic plemorphic of strap fusiform cells in combination with hyperplasic strands of fibrous tissue. She was treated with chemotherapy against malignant tumour and followed - up periodically. On 2002/01/30 she was operated again to remove radically suspect tissue, and continued to treat with chemotherapy. At present (9.2012), after 12 years of treatment and follow up, no sign of recurrence, the patient growths up normally and in good health; all examens: clinic, radiographic (CT scanner, MRI, Panorex...), biochemical showed in normal limit. Conclusion: After long term treatment and follow up nearly twelve years, a malignant rhabdomyosarcoma on a little girl was well treated owing to early and precise diagnosis, and in combination of surgery and chemotherapy.
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