Abstract

Rhabdomyosarcoma is a rare oral soft tissue malignant tumor with pathologic features that may influence the clinical behavior, treatment, and prognosis of the lesion. We report the case of a 13-year-old female patient presenting with an asymptomatic polypoid swelling in the left jugal mucosa measuring approximately 2.5 cm in diameter and of 3 months evolution. The presumptive diagnosis was fibrous hyperplasia. An excisional biopsy was carried out. Pathologic analysis revealed proliferation of predominantly ovoid cells, with eosinophilic cytoplasm and pleomorphic nuclei, arranged in the subepithelial cambium layer. The mucosal surface presented a papillary–verrucous appearance. Immunohistochemical analysis revealed intense positivity for desmin, myogenin, and ki67. The diagnosis was embryonal rhabdomyosarcoma (botryoid variant). The patient underwent complementary chemotherapy and radiotherapy, with no evidence of recurrence or metastatic disease at the 4-month follow-up. The clinical, histopathologic, immunohistochemical, and therapeutic aspects of botryoid rhabdomyosarcoma will be discussed.

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