The myxoid variant of adrenocortical carcinoma is a rare neoplasm described
previously in only two case reports. Because of the rarity of these lesion
s, the presence of myxoid changes in adrenal cortical neoplasms usually rai
ses the possibility of malignancy. We studied the histopathologic features
of 14 cases of myxoid adrenocortical neoplasms, including six adenomas and
eight carcinomas. All patients with adenomas with sufficient follow-up (n =
5) were alive with no recurrence of their tumors or evidence of metastatic
disease. Four patients with carcinomas died of their disease, two were ali
ve with metastatic disease, and one was alive with no evidence of recurrenc
e or metastatic disease. Histologically, the 14 tumors varied in their myxo
id composition, ranging from 10% to 95%. The myxoid foci stained positively
with Alcian blue and were usually negative with periodic acid-Schiff and m
ucicarmine stains. As a group, the immunophenotype of the lesions was typic
al of other adrenal cortical neoplasms, with positive immunostaining for vi
mentin, synaptophysin, and alpha-inhibin. One tumor was focally positive fo
r keratin. Myxoid adrenal cortical neoplasms should be included in the diff
erential diagnosis of myxoid retroperitoneal neoplasms. Myxoid changes in a
drenal cortical neoplasms may be present in both adenomas and carcinomas, a
nd the usual clinical and histopathologic features for adrenocortical neopl
asms should be used to diagnose these neoplasms.