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Gazzetta Medica Italiana Archivio per le Scienze Mediche 2017 October;176(10):545-9

DOI: 10.23736/S0393-3660.16.03427-6


lingua: Inglese

Primitive neuroectodermal tumor/Ewing’s sarcoma: a case report and literature review

Izabela S. ALVES 1, Luiz G. BERRIEL 2, Rafael T. ALVES 2 , Mateus O. POTRATZ 2, Marcele B. PINTO 2, Camila F. OLIVEIRA 2, Aurenivea C. CAZZOTTO 2, Wesley V. MOURA 2

1 Hospital Universitário Cassiano Antonio Moraes, Santa Cecilia, Vitória, Brazil; 2 Hospital Santa Casa de Misericória de Vitória, Vila Rubim, Vitória, Brazil


Primitive neuroectodermal tumor (PNET)/ Ewing’s sarcoma is rare, with most cases occurring in children and young adults. Because of the scarcity of data, treatment options remain few. This report describes the case of an 18-year old male patient with initial tetraparesis progressing to tetraplegia. In October 2014, magnetic resonance imaging of the cervical spine showed a large mass extending from C2 to T1, displacing and compressing the spinal cord, with signs of edema and myopathy. The C3 vertebral body and part of the structures of the right posterior arch were destroyed. An incisional biopsy performed that same month revealed a poorly differentiated neoplasm. Immunohistochemical analysis presented positivity for FLI1 and CD34, a result compatible with PNET/ Ewing’s sarcoma. In November, chemotherapy was initiated for a proposed 18 cycles, alternating between a VAC regimen (vincristine, doxorubicin, and cyclophosphamide) and a combination of ifosfamide and etoposide. The patient responded well to treatment according to imaging tests and clinical examinations. He recovered movement, but muscle strength remained at grade 3. As resection of the lesion remained impossible after six cycles of chemotherapy, radiotherapy of the cervical spine was performed. After 17 cycles of chemotherapy, the patient is currently healthy, clinical improvement is expressive, and he is able to move all four limbs.

KEY WORDS: Neuroectodermal tumors, primitive - Sarcoma, Ewing - Drug therapy - Radiotherapy

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