A 66-year-old female with newly diagnosed diabetes mellitus type 1 (DMT1) and hypertension was evaluated for severe hypokalemia on routine labs. Associated symptoms included generalized fatigue, muscle cramps, bilateral lower extremity edema. Physical exam was remarkable for moon face, bibasilar crackles, and lower extremity edema. Initial workup showed sodium of 151 mEq/L, potassium of 1.7 mEq/L, bicarbonate of 42 mg/dL, creatinine of 1.38 mg/dL. Bilateral pleural effusions and innumerable round lung masses were seen on chest radiograph. CT imaging revealed innumerable soft tissue pulmonary nodules suspicious for hematogenous metastases and a heterogeneous mass in the left adrenal gland with few calcifications, highly suspicious for ACC. CT guided lung biopsy was not diagnostic, but ultrasound guided fine needle aspiration of a liver lesion was positive for metastatic malignancy.
Serum ACTH, AM cortisol, Dexa suppression test, DHEA-S, and 11-deoxycortisol values were consistent with a cortisol secreting tumor. Review of a 2012 CT abdomen and pelvis revealed a heterogenous fat containing mass in the left adrenal gland, highly suggestive of an adrenal myelolipoma (AML). Unfortunately, follow-up imaging was not done although the patient’s niece had died from metastatic adrenal cancer at age 39. Ultimately, the patient was diagnosed with metastatic ACC associated with new onset DMT1 and enrolled for hospice care since she was not deemed a surgical candidate.
Discussion:
Adrenocortical carcinoma (ACC) is a rare malignancy that usually presents with type 2 diabetes mellitus. ACC is a rare malignancy with poor prognosis that occurs in 1-2/million, mainly in females during the 4-5th decade of life with evidence of adrenal steroid hormone excess. In our patient, the incidental left sided AML found on CT abdomen 3 years prior could have coexisted with an underlying adrenocortical tumor or represented a malignant transformation not found on time due to lack of follow-up.
Furthermore, the patient’s family history of ACC might have indicated a familial form although genetic evaluation was not pursued. The negative antibody panel was compatible with idiopathic diabetes, which is the first case described of new onset DMT1 in a patient with ACC. Overall, there have only been 4 cases reported of ACC and AML coexistence according to literature. Thus, physicians should be aware of the fundamental importance of close follow-up imaging of AML and the possibility of their coexistence.
Conclusions:
This case demonstrates the interesting association between DMT1 and ACC. It is paramount that physicians be aware of the existence of such an association.