CASE REPORT
O Sarcoma de Ewing (SE) afeta a base do crânio em 1-6% dos casos. O envolvimento da órbita, do nervo óptico e do cérebro pode impedir a ressecção cirúrgica completa sem morbidade grave. A quimioterapia neoadjuvante faz parte do cuidado padrão no SE extracraniano. Exames de imagem sugerindo um diagnóstico diferente, mais comum ou sob necessidade urgente de descompressão de estruturas neurais são comumente as razões pelas quais a quimioterapia neoadjuvante não é usada em SE da base de crânio. Apresentamos um caso de um paciente de 3 anos com SE da base de crânio onde, apesar do rápido declínio visual, a quimioterapia neoadjuvante demonstrou ser uma ferramenta valiosa para melhora dos sintomas e redução de morbidade cirúrgica sem comprometer a cirurgia radical.
Ewing’s Sarcoma (ES) affects skull base in 1-6% of the cases. Involvement of orbit, optic nerve and the brain may preclude complete surgical resection without severe morbidity. Neo-adjuvant chemotherapy is part of the standard care in extracranial ES. Imaging suggesting a different, more common diagnosis or urgent need to decompression of neural structures are commonly reasons by which neoadjuvant chemotherapy is not used in skull base ES. We present a case of a 3-year-old patient presenting a skull base ES where in spite of the fast progressing visual decline, neoadjuvant chemotherapy had demonstrated to be a valuable tool to symptoms improvement and reduce surgical morbidity without compromising radical surgery.
1. Bricha M, Jroundi L, Boujida N, El Hassani MR, Chakir N, Jiddane M. Sarcome d’Ewing primitif de la voûte du crâne. J Radiol. 2007;88(12):1899-901. http://dx.doi.org/10.1016/S0221-0363(07)78370-1.
2. Lin JK, Liang J. Sinonasal ewing sarcoma: a case report and literature review. Perm J. 2018;22(2):17-086. http://dx.doi.org/10.7812/TPP/17-086. PMid:29616903.
3. Yeshvanth SK, Ninan K, Bhandary SK, Lakshinarayana KPH, Shetty JK, Makannavar JH. Rare case of extraskeletal Ewings sarcoma of the sinonasal tract. J Cancer Res Ther. 2012;8(1):142-4. http://dx.doi.org/10.4103/0973-1482.95197. PMid:22531536.
4. Howarth KL, Khodaei I, Karkanevatos A, Clarke RW. A sinonasal primary Ewing’s sarcoma. Int J Pediatr Otorhinolaryngol. 2004;68(2):221-4. http://dx.doi.org/10.1016/j.ijporl.2003.09.011. PMid:14725990.
5. Martin E, Senders JT, Ter Wengel PV, Smith TR, Broekman MLD. Treatment and survival of osteosarcoma and Ewing sarcoma of the skull: a SEER database analysis. Acta Neurochir (Wien). 2019;161(2):317-25. http://dx.doi.org/10.1007/s00701-018-3754-y. PMid:30578430.
6. Desai KI, Nadkarni TD, Goel A, Muzumdar DP, Naresh KN, Nair CN. Primary Ewing’s sarcoma of the cranium. Neurosurgery. 2000;46(1):62-8. http://dx.doi.org/10.1093/neurosurgery/46.1.62. PMid:10626936.
7. Steinbok P, Flodmark O, Norman MG, Chan KW, Fryer CJ. Primary Ewing’s sarcoma of the base of the skull. Neurosurgery. 1986;19(1):104-7. http://dx.doi.org/10.1227/00006123-198607000-00016. PMid:3748329.
8. Souheil J, Skander K, Sawssen D, et al. Ewing sarcomas of the sinonasal tract and maxillary bone. Egyptian J Ear Nose Throat Allied Sci. 2016;17(3):147-53. http://dx.doi.org/10.1016/j.ejenta.2016.08.001.
9. Vaccani JP, Forte V, de Jong AL, Taylor G. Ewing’s sarcoma of the head and neck in children. Int J Pediatr Otorhinolaryngol. 1999;48(3):209-16. http://dx.doi.org/10.1016/S0165-5876(99)00030-0. PMid:10402117.
10. Craft A, Cotterill S, Malcolm A, et al. Ifosfamide-containing chemotherapy in Ewing’s sarcoma: The Second United Kingdom Children’s Cancer Study Group and the Medical Research Council Ewing’s Tumor Study. J Clin Oncol. 1998;16(11):3628-33. http://dx.doi. org/10.1200/JCO.1998.16.11.3628. PMid:9817284.
11. Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med. 2003;348(8):694-701. http://dx.doi.org/10.1056/NEJMoa020890. PMid:12594313.
12. Balamuth NJ, Womer RB. Ewing’s sarcoma. Lancet Oncol. 2010;11(2):184-92. http://dx.doi.org/10.1016/S1470-2045(09)70286-4. PMid:20152770.
13. Cugati G, Singh M, Pande A, Symss NP, Chakravarthy VM, Ramamurthi R. Isolated skull base primary Ewing’s sarcoma: an extremely rare location. J Cancer Res Ther. 2013;9(4):741-2. http://dx.doi.org/10.4103/0973-1482.126479. PMid:24518733.
1 MD, Neurosurgeon, Skull Base Division, Neurological Institute of Curitiba, Curitiba, PR, Brazil.
2 MD, Department of Skull Base Surgery, Clínica Bio Bio; Department of Neurological Surgery, Hospital Clínico Regional de Concepcion, Concepcion, Chile.
3 MD, MR, Resident in Neurosurgery, Neurological Institute of Curitiba, Curitiba, PR, Brazil.
4 MD, ENT, Skull Base Division, Neurological Institute of Curitiba, Curitiba, PR, Brazil.
5 MD, PhD, Chairman of Neurosurgery, Neurosurgeon, Neurological Institute of Curitiba, Curitiba, PR, Brazil.
Received June 21, 2022
Accepted July 10, 2022
