Adrenocortical Carcinoma: A Challenging Diagnosis

Adrenocortical carcinoma (ACC) is a rare malignancy with aggressive behaviour and a poor prognosis. Patients can present with adrenal hormonal excess or with nonspecific symptoms driven by the presence of an abdominal mass or metastatic disease. Many are completely asymptomatic and diagnosed incidentally. ACC can cause considerable morbidity and mortality, mostly due to its ability to invade surrounding tissues, produce hormones, and spread to distant organs. The authors describe a case of a 62-year-old woman who presented with subacute dorso-lumbar pain. A computed tomography (CT) scan revealed osteolytic lesions in her dorsal spine and sacrum, suggesting metastatic disease. Later on, she presented with hypercortisolism and refractory hypokalemia, so an abdominal and pelvis CT was performed, which showed a suspicious mass in the right adrenocortical gland. A CT-guided adrenal biopsy confirmed ACC. Unfortunately, our patient's clinical status rapidly deteriorated, resulting in her death only a few weeks later. ACC is often found at an advanced stage and with distant metastases, most commonly in the liver, lungs, lymph nodes, and bone. The overall prognosis of ACC is generally poor, but it varies depending on the extent of the disease. Multiple factors have been shown to be relevant in the prognostic classification, such as tumor stage, cell proliferation markers, and resection status. Currently, the only curative treatment is complete surgical resection. Adjuvant therapies have often been shown to decrease recurrence rates or as an alternative in patients with advanced disease. Many studies have been conducted to better understand the molecular basis of ACC, thus enabling the classification into molecular subtypes, but more studies are necessary to identify targets amenable to pharmaceutical intervention. With this case report, we want to emphasize that the diagnosis of ACC is not always obvious. Although metastases are infrequent, their presence is by far the strongest indicator of poor prognosis. All patients with proven or suspected ACC benefit from multidisciplinary monitoring, preferably at a specialized center.