Primary alveolar rhabdomyosarcoma: a horrendous presentation with miraculous remission.
نویسنده
چکیده
To cite: Dhull AK. BMJ Case Reports Published online: 10 December 2012 doi:10.1136/bcr-2012007657 DESCRIPTION Alveolar rhabdomyosarcoma constitutes approximately 1% of all paediatric tumours. Primary alveolar rhabdomyosarcoma of lip is a very rare pathological entity. Here is presented a case of primary alveolar rhabdomyosarcoma of upper lip treated with palliative external radiation therapy followed by combination chemotherapy. A 5-year-old girl presented with painful swelling of upper lip of 3 months duration, which progressed rapidly. There was no history of other swelling. There was no history of any malignancy in the family. Local examination revealed an ulceroproliferative growth of 10×8 cm size present over the upper lip involving bilateral nostrils and was locally advanced, causing difficulty in swallowing and respiration (figures 1 and 2). Histopathological examination confirmed the diagnosis of alveolar rhabdomyosarcoma. The poor general condition of the patient as well as obstructive symptoms prompted us to treat the patient with palliative intention. The patient received palliative external radiation therapy and showed partial regression of the swelling after radiotherapy. The patient was further planned for intravenous combination chemotherapy with vincristine 1 mg, actinomycin 0.5 mg and cyclophosphamide 400 mg 3 weekly for six cycles and after 6 months of the initial presentation, the patient was in complete remission as is evident from the serial photographs (figures 3–5). Malignancy at this rare site is uncommon and clinical management presents considerable challenges. Intensive multimodality approach should be tested, incorporating surgery, dose-intensive combination chemotherapy and radiation therapy. Future challenges include the
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عنوان ژورنال:
- BMJ case reports
دوره 2012 شماره
صفحات -
تاریخ انتشار 2012