Rhabdomyosarcoma of the Oral Cavity: A Case Report (original) (raw)
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Rhabdomyosarcoma of the Oral Tissues - two new cases and literature review
Rhabdomyosarcoma (RMS) is a malignant soft tissue neoplasm consisting of cells derived from the primitive mesenchyme that exhibit a profound tendency to myogenesis. About 35% of RMS arises in the head and neck, being classified as parameningeal and non-orbital non-parameningeal forms. Parameningeal tumors carry the worst prognosis. The use of contemporary, multi-agent chemotherapy, radiotherapy, and surgery has made treatment of the disseminated disease possible, and has significantly improved overall survival from 25% in 1970 to 70% in 1991. Here, we present the management of two cases of orofacial RMS in adolescents: an 18-year-old, white female that had a 9-month history of a nodule in the left buccal mucosa, and a 19-year-old, white male who had been aware of a nodule in the left, posterior maxillary ridge with progressive growth for 4 months. Before final diagnosis, both cases were previously treated as inflammatory lesions. Their clinicopathological aspects, treatment, and poor survival as a consequence of delays in diagnosis are discussed.
Oral Rhabdomyosarcoma: A review
Journal of Clinical and Experimental Dentistry, 2012
Rhabdomyosarcoma (RMS) is a rare malignant soft tissue neoplasm comprised of cells derived from the primitive mesen¬chyme. About 35% of RMS arises in the head and neck, are are classified as parameningeal and non-parameningeal forms. These are the most common soft tissue sarcoma of the children, adolescents and young adults. Their etiopathogenesis and its molecular relevance have been emphasized. The first line of treatment is radical excision and this is usually supplemented by radiotherapy. It is believed that adjunct combination chemotherapy may greatly improve the prognosis. Inadequately treated tumours grow in an infiltrative manner and recur in a high percentage of cases. Bone does not constitute an effective barrier to the growth of the tumour and bone invasion is a frequent finding in head and neck rhabdomyosarcomas.
Orofacial rhabdomyosarcoma: report of a case and review of the literature
Medicina oral, patología oral y cirugía bucal, 2008
Rhabdomyosarcomas (RMS) are among the most common soft-tissue tumors in children. These tumors are derived from mesenchymal tissue with a tendency toward myogenic differentiation that probably originates from immature and highly invasive satellite cells associated with the embryogenesis of skeletal muscle. Some of these tumors are associated with high rates of recurrence and metastasis. The diagnosis is made by microscopic analysis and auxiliary techniques such as immunohistochemistry, electron microscopy, cytogenetic analysis, and molecular biology. We report here a case of orofacial RMS in a 4- year-old child and provide an updated review of the literature, focusing mainly on the clinicopathological aspects, diagnosis and treatment of RMS of the head and neck.
Oral rhabdomyosarcoma-embryonal subtype in an adult: A rarity
Journal of Natural Science, Biology and Medicine, 2014
Rhabdomyosarcoma is a malignant tumor composed of neoplastic mesenchymal cells, with varying degrees of striated muscle cell differentiation. With most cases occurring in children younger than 10 years, it is remarkably rare in adults. Further in adults, the typical pediatric rhabdomyosarcoma variants (embryonal and alveolar sub-types) occur less frequently and exhibit predilection for viscera followed by the head and neck region. A rare case of embryonal rhabdomyosarcoma arising from the buccal mucosa in a 36-year old male patient is herewith reported. Recognition of the correct diagnosis and histological sub-type is of critical importance in the therapy of this disease, since the treatment is not uniform in the literature because of the rarity of this neoplasm in the adult population.
Rhabdomyosarcoma of the Maxillary Gingiva
Journal of Periodontology, 2007
Rhabdomyosarcoma is a malignant skeletal muscle neoplasm. The tumor is much more common in children, and the most frequent site is head and neck region. Since this tumor is less frequent than other neoplasms in oral cavity, the clinicians sometimes ignore it, working the patients up. Rhabdomyosarcoma is a high-grade malignancy with poor prognosis. Considering the aggressive behavior and various clinical or histopathologic presentations of the tumor, early diagnosis has a significant impact on the treatment outcome and prognosis of the patients. We highlight the importance of combining the clinical, radiographic, and histopathologic examination to obtain a definitive diagnosis in sarcomas of the head and neck region, especially rhabdomyosarcoma. A case of rhabdomyosarcoma of the maxillary gingiva is presented in a 32-year-old woman in which the primary incisional biopsy was erroneously interpreted as an inflammatory process and consequently, the accurate diagnosis postponed for about 10 months.
Oral Oncology, 2002
Rhabdomyosarcoma (RMS) is an aggressive malignant skeletal muscle neoplasm arising from embryonal mesenchyme. It accounts for over 50% of all pediatric soft tissue sarcomas. The head and neck region is the most common site for this tumor in children. Neonatal presentation of this tumor is rare. We present the management of one neonatal case and three additional cases of orofacial RMS in children under the age of 7 years. All four patients were seen in the department of oral and maxillofacial surgery at Children's Hospital and Regional Medical Center (CHRMC) in Seattle between 1992–2000. Three of the four cases were alveolar RMS and one was botryoid sub-type of embryonal RMS. Three patients were treated with a combination of surgery, chemotherapy and radiation, while the patient with botryoid RMS was treated with surgery and chemotherapy only. The patient with congenital RMS died at 2.5 years of age due to recurrent metastatic disease. The other three patients are alive without evidence of recurrent with a mean follow up was 5.5 years (range 2.5–8.5 years). We discuss the current management, diagnosis, biological behavior, histopathology, prognosis and survival of head and neck RMS in neonates and young children.