Rhabdomyosarcoma (RMS) is the most common malignant soft tissue tumor. RMS derives from primitive mesenchymal tissues that exhibit a tendency toward myogenic differentiation and probably originate from satellite cells associated with the embryogenesis of skeletal muscle. There are reports of cases arising in oral tissues, which correspond to 10 to 12% of all head and neck RMS. Here we report a case of challenging diagnose of a pleomorphic spindle-cell RMS of the oral cavity. In June 2012, a 24-year-old male patient was referred to the Department of Oral Medicine and Pathology by his dentist to extract tooth 2.8. On history taking, the patient described a persistent left cheek abscess caused by malposition of 2.8 and non-responsive to 2-month amoxicillin (2 g/die) therapy. He also referred absence of pain, pyretic state and pus/blood discharge during this period. Objective examination revealed slight extraoral asymmetry and intraoral exophityc, hard, immovable cheek proliferation, swelling and redness, associated to ecstasy of Stenone duct. No lymphoadenopathy was recognized. No signs of disease were detected on x-rays. An incisional biopsy was immediately performed. The pathologist diagnosed a “fibromatous lesion” although she stated the small dimension of the specimen could be misleading. An excisional biopsy was scheduled 15 days later and revealed the extension of the lesion deep in the muscular tissue and its pedunculated nature. The pathologist reported a “myofibroblastic inflammatory tumor”, positive for desmin, vimentin, CD65 and ki67 but urged on further consultation with another pathologist. Conclusive diagnose after consultation was “spindle-cells/sclerosant multifocal rhabdomyosarcoma”. Seven days after, PET, TC and MRI denied the presence of residual tumor cells or of metastasis. Although the patient was recommended to perform adiuvant RT/CT, he refused further interventions. Presently, patient is still free of disease.

Rhabdomyosarcoma of the oral cavity in a 24-year-old male patient.

GOBBO, MARGHERITA;OTTAVIANI, GIULIA;RUPEL, KATIA;DI LENARDA, Roberto;BIASOTTO, MATTEO
2013

Abstract

Rhabdomyosarcoma (RMS) is the most common malignant soft tissue tumor. RMS derives from primitive mesenchymal tissues that exhibit a tendency toward myogenic differentiation and probably originate from satellite cells associated with the embryogenesis of skeletal muscle. There are reports of cases arising in oral tissues, which correspond to 10 to 12% of all head and neck RMS. Here we report a case of challenging diagnose of a pleomorphic spindle-cell RMS of the oral cavity. In June 2012, a 24-year-old male patient was referred to the Department of Oral Medicine and Pathology by his dentist to extract tooth 2.8. On history taking, the patient described a persistent left cheek abscess caused by malposition of 2.8 and non-responsive to 2-month amoxicillin (2 g/die) therapy. He also referred absence of pain, pyretic state and pus/blood discharge during this period. Objective examination revealed slight extraoral asymmetry and intraoral exophityc, hard, immovable cheek proliferation, swelling and redness, associated to ecstasy of Stenone duct. No lymphoadenopathy was recognized. No signs of disease were detected on x-rays. An incisional biopsy was immediately performed. The pathologist diagnosed a “fibromatous lesion” although she stated the small dimension of the specimen could be misleading. An excisional biopsy was scheduled 15 days later and revealed the extension of the lesion deep in the muscular tissue and its pedunculated nature. The pathologist reported a “myofibroblastic inflammatory tumor”, positive for desmin, vimentin, CD65 and ki67 but urged on further consultation with another pathologist. Conclusive diagnose after consultation was “spindle-cells/sclerosant multifocal rhabdomyosarcoma”. Seven days after, PET, TC and MRI denied the presence of residual tumor cells or of metastasis. Although the patient was recommended to perform adiuvant RT/CT, he refused further interventions. Presently, patient is still free of disease.
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11368/2758165
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