History
Unfortunately, most patients with adrenocortical carcinoma (AC) present with advanced disease that is characterized by multiple abdominal or extra-abdominal metastatic masses (stage IV disease); therefore, distinguishing potential AC from adrenal incidentalomas is crucial (and controversial).
Nonfunctional variants
These hormonally silent tumors account for approximately 40% of patients with AC. Nonfunctional variants of AC tend to be more common in older patients and appear to progress more rapidly than functional tumors do. Although in some cases, they are found incidentally, during either examination or radiologic imaging, nonfunctional ACs typically present with any of the following:
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Fever
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Weight loss
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Abdominal pain and tenderness
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Back pain
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Abdominal fullness
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Symptoms related to metastases
Hormonally active variants
The hormonally active variants of AC constitute approximately 60% of cases. Approximately 30-40% of adult patients with these present with the typical features of Cushing syndrome, while 20-30% present with virilization syndromes.
More than 80% present of pediatric patients, however, present with virilization syndromes. Isolated Cushing syndrome is much less common, occurring in approximately 6% of pediatric cases. Virilization (in girls) or precocious puberty (in boys) is the most common endocrine presentation of a functional AC.
Hirsutism, facial acne, oligo/amenorrhea, and increased libido all are possible presenting symptoms. Feminization as a presentation of AC is quite rare. Other modes of presentation include profound weakness, hypertension, and/or ileus from hypokalemia related to hyperaldosteronism and hypoglycemia.
Physical Examination
Physical findings almost always include a palpable mass in the abdomen; the mass is hard and nonmovable. [6]
Virilization
Findings in males include premature puberty with enlargement of the penis and scrotum, pubic hair, acne, and deepening voice.
Findings in females include premature appearance of pubic and axillary hair, clitoral hypertrophy, acne, deepening voice, premature increase in growth velocity, lack of appropriate breast development, and lack of menarche.
Cushing syndrome
Signs of Cushing syndrome include a round face, a double chin, buffalo-hump fat distribution, generalized obesity, failure of growth velocity, and hypertension. [7]
Feminization
In rare cases, feminization may occur. Findings in male patients include gynecomastia and hypertension; findings in female patients include precocious sexual development and hypertension.
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A 68-year-old woman with a large right upper quadrant primary adrenocortical carcinoma with curvilinear calcification. Low-attenuation regions anteriorly are consistent with necrosis.
