Adrenal Carcinoma Clinical Presentation

Updated: Jan 18, 2017
  • Author: Bagi RP Jana, MD; Chief Editor: Neetu Radhakrishnan, MD  more...
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Presentation

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:

  • Fever
  • Weight loss
  • Abdominal pain and tenderness
  • Back pain
  • Abdominal fullness
  • 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.

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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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