Tumor de Ewing: Diagnóstico Diferencial e Bases para o Tratamento Oncológico e Cirúrgico

Alves, Debora Yumi Yoshimura Orlandin; Rotondo, Italo Gerardo; Freitas, Matheus Trindade Bruxelas de; Narazaki, Douglas Kenji

doi:10.1590/S1808-185120252404298086

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REVIEW ARTICLE: TUMOR/INFECTION • Coluna/Columna. 24 (4) • 2025 • https://doi.org/10.1590/S1808-185120252404298086 linkcopiar

Tumor de Ewing: Diagnóstico Diferencial e Bases para o Tratamento Oncológico e Cirúrgico

Autoria SCIMAGO INSTITUTIONS RANKINGS

O sarcoma de Ewing (SE) é um tumor ósseo primário altamente agressivo que apresenta desafios diagnósticos e terapêuticos significativos quando afeta a coluna vertebral. Esta revisão sintetiza a literatura atual sobre o SE vertebral, abrangendo sua epidemiologia, manifestações clínicas, métodos diagnósticos, diagnósticos diferenciais e estratégias de tratamento multimodal. O foco é para cirurgiões de coluna, destacando o equilíbrio entre o controle oncológico e a preservação neurológica. O reconhecimento precoce e uma abordagem multidisciplinar rigorosa são cruciais para otimizar a eficácia oncológica e os resultados neurológicos. A quimioterapia sistêmica é fundamental, enquanto a ressecção cirúrgica é vital para o controle local, descompressão neurológica e estabilidade espinhal. A ressecção em bloco, quando viável, mostra vantagens no controle local e na sobrevida livre de doença. A radioterapia é um tratamento local adjuvante ou primário importante. Esta revisão esclarece o manejo do SE espinhal, enfatizando o cuidado especializado e integrado para melhorar o prognóstico e a qualidade de vida do paciente. Nível de Evidência III; Artigo de Revisão.

Descritores:
Sarcoma de Ewing; Neoplasias da Coluna Vertebral; Coluna Vertebral, Terapia Combinada

Differential Diagnoses	Clinical Features	Radiological Features and Diagnosis
Osteosarcoma	The most common primary malignant bone tumor. Spinal involvement is less frequent than in long bones	Mixed lytic and blastic pattern (osteoid matrix). Periosteal reaction (“sunburst” or “Codman’s triangle”).¹,³
Primary Spinal Lymphoma	Systemic B symptoms (fever, night sweats, weight loss), similar to ES. More common in older adults than in children.	Diffuse marrow infiltration and lytic lesions. Less overt cortical bone destruction and a more homogeneous soft tissue. Definitive differentiation relies on histopathology and immunohistochemistry (CD45, CD20).¹
Metastatic Neuroblastoma	The most common extracranial solid tumor in children. Metastatic lesions to the spine are typically lytic and can cause back pain and neurological deficits	Differentiation often relies on the identification of the primary tumor, elevated urinary catecholamines (VMA, HVA), and specific immunohistochemical markers (e.g., synaptophysin, chromogranin) and molecular genetics (e.g., MYCN amplification) on biopsy, contrasting with the EWSR1 fusion characteristic of ES.³
Eosinophilic Granuloma	Can cause pain and even neurological symptoms due to vertebral collapse, but have a more favorable course and lack the systemic aggression of ES	On radiographs, purely lytic, well-defined lesions. Common benign cause of “flat vertebra” (diffuse collapse of a vertebral body). MRI may show diffuse marrow signal changes without the large enhancing soft tissue component seen in ES.⁷
Aneurysmal Bone Cyst	Benign lesion that commonly affects the spine in children and adolescents. Pain, spinal deformity, and neurological symptoms due to mass effect.	Expansile, osteolytic lesion with multiple fluid-fluid levels on MRI, reflecting blood products within cystic loculations, a pathognomonic feature not seen in ES.⁷
Osteoblastoma	Benign bone-forming tumor, typically affecting the posterior elements of the spine in children and young adults. Back pain is common.	Well-circumscribed, often with a sclerotic rim on radiographs and CT, and may have a central nidus. Tend to be less aggressive and infiltrative than ES. The presence of osteoid matrix on biopsy is benign, unlike the malignant osteoid of osteosarcoma.¹
Spondylodiscitis and Vertebral Osteomyelitis	Back pain is often accompanied by fever and elevated inflammatory markers (ESR, CRP). The presence of acute inflammatory markers and, sometimes, positive blood cultures or disc aspirates, aids in diagnosis	On MRI, spondylodiscitis typically shows narrowing and enhancement of the disc space, along with T2 hyperintensity and enhancement of adjacent vertebral endplates. (Figure 3) While ES can cause diffuse marrow signal changes, disc space involvement is rare in ES, and the soft tissue mass in ES is usually more prominent and less centered on the disc.³
Spinal Tuberculosis (Pott’s Disease)	Remains a significant differential in endemic areas. Slow, progressive destruction of the vertebral bodies (often anteriorly), with relative sparing of the posterior elements initially, leading to severe kyphotic deformity (Figure 3). Systemic symptoms (night sweats, weight loss)	diffuse marrow signal changes, extensive paraspinal abscesses (cold abscesses), and involvement of multiple contiguous vertebral bodies and intervening discs. This multifocal, multi-level involvement with significant disc pathology is less typical of ES, which usually affects a single or limited number of vertebrae.³

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