Oral rhabdomyosarcoma-embryonal subtype in an adult: A rarity
A 36-year old male patient was referred to our service for the evaluation of a painless swelling on his face that had appeared one and a half months earlier, with rapid evolution. During extra-oral examination, an extensive swelling on the right side of the face was observed, with un-defined limits. The skin overlying the swelling appeared shiny and stretched with ulceration []. The swelling was associated with difficulty in mouth opening and mastication. Medical history was non-contributory to the present complaint.\nOn intra-oral examination, brownish-black colored mass was verified involving the right buccal mucosa, with necrotic changes and sloughing. The mass extended anteriorly from the right mandibular first premolar region to the retromolar region posteriorly with involvement of the posterior-lateral aspect of hard palate []. On palpation, it was fibrous in consistency and non-tender. Ulceration was observed over the mass due to impingement of the teeth. No palpable cervical lymphadenopathy was present.\nOrthopantomograph revealed no changes in the adjacent bone. Computerized tomography revealed a large expansile lesion with respect to right gingivo-buccal space showing slight erosion of the maxilla []. Further to rule out distinct metastasis, chest radiography and ultrasonography of the abdomen were performed, that revealed no pathology.\nIn view of these findings, a diagnostic possibility of soft tissue sarcoma was hypothesized with differential diagnosis of Fibrosarcoma, Extra-osseous Ewing's sarcoma and Rhabdomyosarcoma. An incisional biopsy of the mass was performed and the histopathology of the specimen disclosed embryonal rhabdomyosarcoma [Figure and ].\nWithin a period of 10 days after the incisional biopsy, there was considerable increase in the size of extra-oral swelling along with an enormous increase in the intra-oral soft tissue mass involving the entire right buccal mucosa and hard palate [Figure and ]. Patient was referred to an oncology centre (Madras Medical College Tamil Nadu, India) and was treated with chemotherapeutic drugs (vincristine, etoposide and ifosfamide) over a period of 32 weeks [According to Inter-group Rhabdomyosarcoma Study (IRS)-IV protocol]. Adjuvant external beam radiotherapy with a dose of 50.40 cGy in 28 fractions was given for a period of a month. Partial recovery occurred during the treatment's initial phase [] but with tapering of chemotherapeutic doses, there was sudden flare up in the tumor size. After that unfortunately, we lost communication with the patient for further treatment and follow up.