Adrenocortical Carcinoma

This 14 x 7 cm mass arising from the adrenal cortex represents an adrenocortical carcinoma. The somewhat irregular external surface in the left photo shows a yellow-tan color. The cut surface in the right photo is hemorrhagic, of a yellow-pink-tan color, and shows minor cystic change. These are rare neoplasms which can be quite large and efface the adrenal gland, as in this case. Microscopically, the cells in adrenocortical carcinoma vary from well-differentiated cells difficult to distinguish from normal calls or cells of an adenoma to bizarre, obviously malignant cells. In the latter case, a metastatic lesion must be excluded.

Clinically, these tumors can cause Cushing syndrome or Conn syndrome or they can be nonfunctional. They account for 5-10% of cases of Cushing syndrome. Certain syndromes predispose to adrenocortical carcinoma, e.g. Li-Fraumeni, Beckwith-widemann, and Gardner syndromes. This tumor often invades the adrenal vein and vena cava. It also spreads via lymphatics and can spread hematogenously, especially to the lungs. The prognosis is poor with average survival time being about two years.

In this case obvious anaplasia is indicative of a malignant lesion. In cases with more “benign” histological appearance, the differentiation between adenoma and carcinoma can be difficult unless clinical metastases have been demonstrated. Features suggestive of malignancy include size (>500 grams), areas of calcification and necrosis, and gross lobulation.

Most patients with adrenocortical carcinoma present with advanced (stage IV) disease. Malignancy is almost 3X more common in females than in males. With the increased use of CT scans of the abdomen in various clinical circumstances, clinicians not infrequently are faced with the task of differentiating “incidentalomas” from adrenocortical carcinomas. One approach is to consider that lesions larger than 6 cm should be removed and that lesions less than 3 cm can be followed if the lesion is nonfunctional. However, this is controversial and leaves one in a quandary as to what should be done with lesions between 3 and 6 cm. In children even the smaller lesions carry a significant risk of malignancy.

Contributed by Dr. Ronald DeLellis