A clinical and pathological study of adrenocortical carcinoma. Therapeutic implications

Abstract

Twenty-one patients with adrenocortical carcinoma (ACC) diagnosed at three hospitals over a ten-year period were reviewed for clinical and pathologic features that might have therapeutic implications.Depending upon the extent of cellular pleomorphism, ACC could be defined as anaplastic or differentiated. Anaplastic ACC occurred more often in male patients, produced more frequent cutaneous metastases ( P = 0.01), and was associated with a lack of clinical or laboratory evidence of hormone production ( P = 0.01). In contrast, differentiated ACC usually occurred in women and produced clinical or laboratory evidence of hormonal excess. Median survival time of patients with anaplastic ACC was only five months, while median survival time of those with differentiated ACC was 40 months ( P = 0.005). Patients with differentiated ACC survived for long periods, even with metastatic disease. Three of 5 such patients had objective responses to o,p'-DDD (Mitotane) therapy. The histopathology of ACC is an important prognostic factor and should be considered in the design of future therapeutic trials.Cancer 45:2880-2883, 1980. DRENOCORTICAL CARCINOMA (ACC) is a rareA disease and patients are few, even at major cancer center^.^ Clinical experience is therefore relatively limited. Prior studies of the morphology of ACC have focused on the differences between adenomas and carcinomas or the relationship between tumor histopathology and overproduction of h o r m o n e~.~J -'~ Much of this prior work revealed no association between histology or hormone production and clinical behavior.3,4,6,11-14 H owever, our experience with 5 patients with ACC suggested that histopathology and biochemical behavior might be prognostically important. Therefore, 16 additional cases were retrospectively studied. Materials and MethodsEvery patient with ACC seen at each of three hospitals during the period from 1969 through 1978 was reviewed.

Impact of standardised reporting in adrenocortical carcinoma: a single centre clinicopathological review

“…Tumour hyperfunctionality occurred in the majority of cases and manifested most commonly as Cushing syndrome. We did not find a relationship between tumour functionality and outcome, which despite the suggestions of some authors21 22 is consistent with the findings of most studies to date (see review 8). …”

Programmed death ligand-1 expression in adrenocortical carcinoma: an exploratory biomarker study

“…However, different clinical courses have been described among patients into the same tumor stage. Some retrospective analyses have reported that functional tumors may be associated with worse prognosis [30]. In addition, few studies have established histological or molecular markers, such as Ki67 index or TP53 mutations, as predictors of poor prognosis and its value still needs to be confirmed [31].…”

Treatment of adrenocortical carcinoma: Contemporary outcomes

“…In addition to symptoms from hormone excess, a palpable abdominal mass is the most common finding of adrenocortical carcinoma, with abdominal pain, weight loss, and fever also occurring frequently [8]. In the 1980s, Hogan et al [9] and Karakousis et al [10] suggested that functional tumors exhibited better prognosis than nonfunctional tumors. Functional status of tumors may alter time to diagnosis because nonfunctional tumors may not present until they reach large size and produce secondary symptoms from mass effect or metastatic disease [5].…”