Adrenal cortical carcinoma

The adrenal cortex is subdivided into three areas based upon the hormones that are produced: Zona Glomerulosa, Zona Fasciculata, and Zona Reticularis providing mineralocorticoids, glucocoriticoids, and androgens respectively. Carcinomas of the adrenal gland are rare with an annual occurrence of 2 cases per million. In the United States this equates to be approximately 250 cases per year. Most tumors are sporadic, but they may occur within the tumor spectrum of Li Fraumani syndrome or MEN1. [1,3]
Adrenal Cortical Carcinoma can be divided into 2 broad categories that occur with equal frequency: Functional and Non-functional. For functional carcinomas, the zone affected will produce an abundance of hormones which may include cortisol (30%), androgens (20%), estrogens (10%), aldosterone (2%), or multiple hormones (35%) [2]. This leads to a variety of presenting conditions including hyperaldosteronism, Cushing’s, and virilisation syndromes. Women who have a functional tumor causing a virilization develop hirustism, amenorrhea, as well as increased muscle mass, deepened voice, and temporal balding. This leads to an earlier diagnosis in women and children but is problematic in men and leads to difficultly in recognizing a potential carcinoma. As a result men with androgen secreting tumors usually presents with advance disease. Less frequently, one may develop an estrogen secreting tumor. Gynecomastia, impotence, and testicular atrophy arise when estrogen is elevated in men. Women with these tumors develop irregular menses or dysfunctional uterine bleeding. Children with either androgen or estrogen secreting tumors develop precocious puberty with either male or female characteristics predominating. [3]
Non-functional carcinomas have later onset of symptoms which are derived from mass effect and hence found at advance stages. This includes abdominal discomfort or pain, indigestion, or site specific symptoms depending on the location of metastatic disease. [1,3]
Early diagnosis is pivotal as cancer of the adrenal cortex has a poor prognosis. The high rate of mortality is due to diagnosis at advance stages of disease; 70% of patients present with stage 3 or 4 [4]. With recent advancement in medical care, 5 year survival has increased from 30% to 60% between 1983 and 2001[1]. In addition to serum chemistries and hormone levels, CT and MRI scans have proven to be essential in detecting malignancy. Findings suggestive of malignancy on CT include tumors >4-6 cm, irregular margins, tumor calcifications, hemorrhage into lymphatics, and enhanced CT attenuation values greater than 10 HU. MRI findings indicative of cancer include bright signal intensity on T2 weighted images, tumor enhancement, and slow washout following gadolinium injection. Since Adrenal cortical carcinoma spreads by direct invasion, hematogenously, and through lymphatics, all confirmed cases should be followed by CT of chest, abdomen and pelvis. Common areas of metastasis include lung, liver, bones, and regional lymphatics. [2,3,4]