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Adrenocortical carcinoma (ACC) is a rare aggressive tumor. Renal vein and inferior vena cava (IVC) thrombi have been found as uncommon presentations of ACC; however, the implementation of comprehensive therapy has remained controversial in such cases. We report a case of a 46-year-old woman with a large ACC associated with the invasion of tumor to IVC confirmed by imaging and immunohistochemistry examinations. The patient was treated successfully using aggressive surgery, including adrenalectomy and thrombectomy adjunct to an adrenocorticolytic agent. However, she died of metastasis complications at 3-month follow-up period. ACC is a rare malignancy, mostly presenting in advanced stages with poor prognosis. Implementing aggressive surgical therapy might be effective for the management of such cases; however, the short survival duration in our case underscores the need for defining the …