We report four cases of embryonal rhabdomyosarcoma treated successfully with multimodality therapy which included surgery, chemotherapy and radiotherapy. Our first case was a 2 year old who admitted with the chief complaint of swelling of the upper lip. Biopsy was taken, which showed embryonal rhabdomyosarcoma. It was followed by a CT scan (face + neck), MRI face, USG abdomen and bone scan of the patient. Pre operatively chemotherapy was given. After completion of chemotherapy regimen, wide local excision of the lesion along with a reverse fan flap and bilateral modified radical neck dissection type 3 was done. This was followed by post operative chemotherapy and radiotherapy. There was no recurrence at 6 months post operatively. The second patient was 6 years old with similar complaints, after undertaking all the investigations as in the previous case, patient was given neoadjuvant chemotherapy following which wide local excision of the lesion along with reconstruction with the help of bilateral nasolabial flap was done. This was followed by post operative chemotherapy. No recurrence was present 6 months post operatively. The third patient was a 17 year old male presenting with complaints of left sided nasal obstruction and left nasal swelling. Examination revealed left sided nasal mass which on biopsy turned out to be embryonal rhabdomyosarcoma. The patient underwent surgery followed by chemoradiation. At 6 months the patient had no evidence of recurrence. The fourth patient was 16-year-old male who presented with complaints of right sided nasal swelling and nasal obstruction. Radiological investigation showed the tumor involving the right sinonasal cavity with extension to anterior cranial fossa and with neck nodes. Patient was given concurrent chemoradiation. Patient was followed up for 6 months when the patient presented with recurrence of disease with distant metastasis. Patient is currently on palliative chemotherapy. To summarise, embryonal rhabdomyosarcoma in young children when treated aggressively with surgery and chemotherapy has better prognosis than with chemo-radiation alone.
Keywords: Embryonal rhabdomyosarcoma; Rhabdomyosarcoma; Sinonasal tumours.
© Association of Otolaryngologists of India 2019.