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Context:  Adrenocortical cancer (ACC) is rare but frequently fatal malignancy. Tumor extension into the inferior vena cava signifies an advanced stage (stage III) of the disease and is frequently associated with high risk of recurrence and short-term survival.    Objective:  To present the surgical and medical management of an unusual case of ACC with IVC invasion up to the right atrium. He has the longest reported tumor-free survival of such a situation. We also reviewed and summarized the literature of similar cases.    Patient:  We present a 15-year old boy who presented with an 11 cm ACC extending into the IVC up to the right atrium and causing the Budd Chiari syndrome. He had complete surgical excision under cardiopulmonary bypass of a large ACC followed by Mitotane adjunctive therapy for 5 years. He is alive and free of any clinical or radiological signs of recurrence 98 months after surgery. This is the longest tumor-free survival reported in the literature of similar cases.    Conclusion:  Significant invasion of the IVC up to the right atrium by ACC should not preclude surgery with the intent of complete resection. Cardiopulmonary bypass significantly aids this surgical procedure and Mitotane therapy should be instituted postoperatively. Long-term free-survival is possible in such a situation.    Lessons:  our patient and the literature review strongly suggest that complete surgical extirpation is the primary choice for patients with ACC invading the IVC, including those reaching the right atrium.

Long-term recurrence-free survival of adrenocortical cancer extending into the inferior vena cava and right atrium