FRI222 Large Non-functional Adrenocortical Carcinoma In A Patient With History Of Occupational Radiation Exposure

Abstract

Abstract Disclosure: A. Makawi: None. G. Elshimy: None. N. Martinez de Andino: None. A. Bolduc: None. Introduction: Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.1-2.0 cases/per million per year. Up to 70% of tumors present with biochemically apparent adrenocortical hormone production. Diagnosis requires investigation of clinical, biological, and imaging features with measurement of steroid hormones key for evaluation. Anderson’s syndrome is commonly seen. Research has shown ACC is driven by several molecular changes that include the inactivation of tumor suppressor genes and activation of different oncogenes, DNA mutations, and epigenetic changes. The widely used staging of ACC is by the European Network of Study of Adrenal Tumors (ENSAT) as it is correlated with clinical outcomes. Here we present a case of a non-secretory ACC noted for 10 years with growth up to 7.5 cm in a patient with history of occupational radiation exposure. Case Presentation: Patient is a 57-year-old white male with history of colon polyps, basal and squamous cell carcinoma, and hypertension diagnosed 10 years prior, on amlodipine 5 mg and lisinopril 20 mg daily who presented to adrenal clinic for evaluation of a 7cm left adrenal mass that had grown from 1.8cm 10 years prior, to 5.7cm 7 years later to 7.5cm at time of surgery. He had symptoms of fatigue and occasional lightheadedness with no other signs or symptoms of Cushing's or pheochromocytoma. Family history noncontributory. He has a history of occupational exposure to radiation working at a nuclear reactor. Workup for adrenal incidentaloma included assays of serum normetanephrine which was increased at 1.2 nmol/L (reference <0.9 nmol/L) with normal metanephrine less than 0.20 nmol/L, normal DHEA-S of 52 mcg/dL, normal ACTH of 16 pg/mL and negative 1mg dexamethasone suppression test with serum cortisol suppressed to 0.78 mcg/dL. CT scan adrenal protocol showed a heterogeneous 7.7x6.6 cm left adrenal mass with 30 HU, with an absolute adrenal washout of 39% and relative washout of 31.4%. He then underwent left adrenalectomy on 12/16/2022. The tumor measured 7.5cm with pathology of low-grade ACC with staging of pT3. Margins were negative for carcinoma but microscopic angioinvasion and capsular invasion were noted. Postoperatively, the patient had resolution of his symptoms with marked improvement in BP. We continue to follow him in clinic for postoperative management. He has been referred for genetic testing with results pending. Discussion: The prognosis of ACC has been believed to be poor due to the low prevalence and sample size of ACC patients, but recent studies have shown that ACC presentation, survival, and prognosis are heterogeneous. Only 20-30% of ACC are nonfunctional. It is important to note that only 30% of ACC cases are curable at early stages, further impacting the poor prognosis. The goal of this report is to bring further attention to the timely assessment and personalized management of ACC given the evolving knowledge base. Presentation: Friday, June 16, 2023