Adrenal Cortical Carcinoma

Abstract

A review of 28 cases of adrenal cortical carcinoma provides some insights into the natural history of this disease and suggests modifications in current techniques of diagnosis and management. Our patients ranged in age from 1 to 69 years but it is notable that all of our male patients were more than 39 years old. In addition the men tended to have high stage tumors that were nonfunctional. Female patients, on the other hand, were more evenly distributed in terms of stage, function and age at diagnosis. Staging was extremely important and is discussed in some detail. A dismayingly large number of patients were found to have stage 3 or 4 tumor at initial diagnosis, suggesting that aggressive and rapid evaluation and a high index of suspicion are important to optimize survival. Function is helpful but non-function is common and should not dissuade one from the diagnosis. Excretory urography with tomography followed by angiography frequently will provide the diagnosis. Endocrinologic evaluation should be done with alacrity. Survival with high stages of disease is poor and lengthy evaluation should not be allowed to delay prompt and, hopefully, curative surgery. Surgical resection is the key to cure; chemotherapy and radiotherapy provided relatively poor results in our hands although mitotane has shown a 34 to 54 per cent response rate.

Scintigraphic patterns of adrenocortical carcinoma: morpho-functional correlates

“…Nonfunctioning tumors were defined as adrenal masses in the absence of any clinical sign of adrenal hyperfunction and in the absence of abnormally elevated adrenal hormone levels, as previously reported (8). Staging of the disease was carried out according to the MacFarlaneSullivan classification (10,11). Diagnosis of malignancy was confirmed by histological examination following the criteria reported by Weiss et al (12) (Weiss score, see Table 1), which define malignancy as the presence of three or more histological findings, including high nuclear grade (13), mitotic rate greater than 5 per 50 high-power fields, atypical mitotic figures, eosinophilic tumor cell cytoplasm ($75% of tumor cells), diffuse architecture ($33% of tumor), necrosis, venous invasion, sinusoidal invasion, and capsular invasion.…”

Clinical and molecular aspects of a pediatric metachronous adrenocortical tumor

“…Among various clinical parameters that have been shown to impact adrenocortical carcinoma prognosis, tumor staging has been demonstrated as one of the most important (13). The staging system for adrenocortical carcinomas depends upon tumor size, nodal involve-ment, invasion of adjacent organs, and presence of distant metastasis (9). However, it is important to know that is not uncommon for patients with small tumors to experience relapses.…”

Tumores adrenocorticais na criança: da abordagem clínica à avaliação molecular

“…Duas escalas de estadiamento tumoral têm sido utilizadas para a classificação dos TAC pediátricos, a Escala de Macfarlane modificado por Sullivan (6,14), que utiliza tamanho tumoral (< ou > 5 cm) e a Escala de Sandrini modificada, que utiliza volume tumoral (< ou > 200 cm 3 ) (1). A distinção na criança entre adenomas e carcinomas pela classificação histopatológica é difícil.…”