Embryonal Rhabdomyosarcoma (Botryoid Subtype) Affecting the Buccal Mucosa

Rhabdomyosarcoma (RMS) is a rare oral malignant soft tissue tumor whose pathological features may influence the clinical behavior, treatment and prognosis of the lesion. We report a case of a 13-year-old female patient, presenting an asymptomatic polypoid swelling in the left buccal mucosa that was...

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Veröffentlicht in:Head & neck pathology (Totowa, N.J.) N.J.), 2019-12, Vol.13 (4), p.671-676
Hauptverfasser: Silva Cunha, John Lennon, De Assis Almeida Lima-Júnior, Francisco, Gonçalves Júnior, Wilson Déda, De Santana Santos, Thiago, Da Silveira, Ericka Janine Dantas, De Sousa, Silvia Ferreira, De Albuquerque-Júnior, Ricardo Luiz Cavalcanti
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container_title Head & neck pathology (Totowa, N.J.)
container_volume 13
creator Silva Cunha, John Lennon
De Assis Almeida Lima-Júnior, Francisco
Gonçalves Júnior, Wilson Déda
De Santana Santos, Thiago
Da Silveira, Ericka Janine Dantas
De Sousa, Silvia Ferreira
De Albuquerque-Júnior, Ricardo Luiz Cavalcanti
description Rhabdomyosarcoma (RMS) is a rare oral malignant soft tissue tumor whose pathological features may influence the clinical behavior, treatment and prognosis of the lesion. We report a case of a 13-year-old female patient, presenting an asymptomatic polypoid swelling in the left buccal mucosa that was approximately 2.5 cm in diameter and 3 months evolution. The presumptive diagnosis was fibrous hyperplasia and an excisional biopsy was carried out. Pathologic analysis revealed proliferation of predominantly ovoid cells, with eosinophilic cytoplasm and pleomorphic nuclei, arranged in subepithelial cambium layer. The mucosal surface presented a papillary–verrucous appearance. Immunohistochemical analysis revealed intense positivity for desmin, myogenin and Ki-67. The diagnosis was of embryonal RMS (botryoid variant). The patient was subjected to complementary chemotherapy and radiotherapy, with no evidence of recurrence or metastatic disease after 12 months follow-up. A discussion on the clinical, histopathological, immunohistochemical and therapeutic aspects of botryoid RMS will be provided.
doi_str_mv 10.1007/s12105-018-0957-8
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source MEDLINE; EZB-FREE-00999 freely available EZB journals; SpringerNature Journals; PubMed Central
subjects Adolescent
Dentistry
Female
Humans
Medicine
Medicine & Public Health
Mouth Mucosa - pathology
Mouth Neoplasms - pathology
Oral and Maxillofacial Surgery
Otorhinolaryngology
Pathology
Rhabdomyosarcoma, Embryonal - pathology
Sine qua non Clinicopathologic Correlation
title Embryonal Rhabdomyosarcoma (Botryoid Subtype) Affecting the Buccal Mucosa
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