The clinical and pathologic featurfs of “non-hormonal” adrenocortical tumors.Report of twenty new cases and review of the literature

Abstract

The clinicopathologic features of 178 patients with “non‐hormonal” or “nonfunctioning” adrenocortical tumors are presented. Such tumors usually affect patients in the 5th, 6th, and 7th decades and occur in males twice as often as females. The prognosis is poor, most patients succumbing during the 1st year after diagnosis. The most helpful radiographic diagnostic test is the intravenous pyelogram, although arteriography and pre‐sacral air insuffiation may also aid in the diagnosis. Lymphangiography was found to be of value in assessing the lymph nodes during treatment and at subsequent followup examination. No reliable, definite indication of the functional or metastatic potential of these tumors can be made from histopathologic examination; all should be viewed as malignant. The tumors are capable of forming precursor steroids without hormonal activity. Hence they are not in fact “non‐functioning.” Their treatment should be primarily surgical, but postoperative radiotherapy should be administered if there is residual tumor. All chemotherapeutic agents are relatively ineffective but some form of combination chemotherapy may be beneficial.

A Survey on Adrenal Incidentaloma in Italy1

“…These findings do not agree with those observed in previous series of silent carcinomas, where men outnumbered women and elderly patients were predominantly affected (43,44). Adrenal cancer presented at younger ages without a gender predominance, whereas cortical adenoma was more frequently found in older patients and in women.…”

Adrenocortical carcinoma with delayed cutaneous metastasis

“…This hypothesis was supported by one study which found patients with anaplastic ACC had a median survival of 5 months, compared with a 40‐month survival in patients with more differentiated tumors 3 . Other studies have further confirmed that nuclear grade alters the patient’s prognosis 10 . Moreover, two recent retrospective studies that analyzed large cohorts with ACC, showed that tumor grade, in addition to the presence metastatic disease at the time of diagnosis and overall tumor burden were the most important prognostic indicators in determining survival 7,23 …”

“…ACC is a rare malignancy that in general has a dismal prognosis. Typically, ACCs are classified as functional or non‐functional; however, all ACC secrete hormones, albeit some hormones are functionally inactive and fail to elicit clinical symptoms 10 . Approximately 95% of children and 50–79% of adult patients have biologically active tumors.…”

Successful long‐term disease‐free survival following multimodal treatments in a patient with a repeatedly recurrent refractory adrenal cortical carcinoma

“…Carcinoma of the adrenal cortex is an extremely rare malignancy. Although complete surgical excision is the only effective form of therapy, many patients develop recurrences despite an apparently curative resection, with between 30 and 50% of patients having metastatic disease at the time of diagnosis 3–5 . The prognosis of patients with refractory or metastatic adrenal cortical carcinoma is thought to be very poor, although a few reports have indicated that aggressive treatments could lead to prolonged survival in patients with metastatic adrenal tumors of the slow‐growing type 1,2 .…”