THU620 Metastatic Adrenocortical Carcinoma Arising From A Testicular Rest Tumor

Disclosure: L.E. Chozet: None. J.M. Bruder: None. R. Flores: None. S. Hackman: None. K. highes: None. L. Vasquez: None. G. Le: None. T. cortez: None. M.A. Escobar Vasco: None. Background: Congenital adrenal hyperplasia (CAH) involves a range of autosomal recessive disorders characterized by abnormalities in the adrenal biosynthesis pathway. Testicular adrenal rest tumors (TARTs) are a complication mostly seen in male patients with congenital adrenal hyperplasia which can lead to infertility. These tumors are known to have benign characteristics. We present a unique case of a male patient with CAH diagnosed with metastatic adrenocortical carcinoma arising from a TART. Clinical Case: A 40-year-old man with poorly controlled congenital adrenal hyperplasia from 21-hydroxylase deficiency and bilateral testicular adrenal rest tumors presented with syncope and worsening left testicular pain for 2 months. Scrotal ultrasound showed enlarged bilateral scrotal masses (left bigger than right). Initial evaluation for his syncopal event included CTA chest which showed an incidental finding of innumerable bilateral solid pulmonary nodules. 17-hydroxyprogesterone levels were elevated at 5273 ng/dL (normal less than 138 ng/dL). Additional biochemical workup of the testicular mass was unremarkable. The patient underwent left radical orchiectomy with pathology showing high-grade carcinoma with adrenocortical differentiation. A right upper lobe lung biopsy was done which demonstrated metastatic carcinoma with adrenocortical differentiation, morphologically similar to the testicular tumor. The initial patient presentation of progressively worsening left testicular pain in the setting of testicular adrenal rest tumors (TARTs) led to the diagnosis of adrenal carcinoma. Conclusion: This case presents a unique presentation of adrenal carcinoma in a patient with congenital adrenal hyperplasia arising in the setting of testicular adrenal rest tumors (TARTs). This case demonstrates a unique presentation of adrenal carcinoma, as malignant degeneration has not been described in patients with TARTs. It also highlights the importance of close monitoring even if these tumors are thought to be of benign origin. Presentation: Thursday, June 15, 2023