SMARCA4-deficient thoracic tumor with vascular differentiation requiring superior vena cava reconstruction: a case report

SMARCA4-deficient tumors are rare, highly aggressive malignancies characterized by inactivation of the SMARCA4 gene, most frequently observed in male smokers. We report an unusual case of a SMARCA4-deficient tumor with vascular differentiation in a 54-year-old non-smoking Asian female with a right paratracheal mass invading the superior vena cava (SVC). Final pathology confirmed a SMARCA4-deficient neoplasm with strong vascular markers on immunohistochemical staining. The patient underwent staged surgical management, including en bloc resection of the mass and SVC with complex vascular reconstruction using a bifurcated graft. This case illustrates the diagnostic complexity of SMARCA4-deficient tumors and highlights an unusual clinical and pathological presentation.