Although the condition is exceedingly rare, tumor-caused hirsutism does occur and today, Richard S. Legro, MD of Penn State University College of Medicine, discussed the ways in which clinicians can best identify them in their patients.
Hirsutism: Differential Diagnosis
• Congenital adrenal hyperplasia
• Cushing’s Syndrome
• Androgen-producing tumor (ovary or adrenal)
• Exogenous sex steroids
• Simple obesity
• Severely insulin resistant states
Dr. Legro cautions that idiopathic hirsutism is a debatable differential diagnosis because it is a very rare disorder.
Studies demonstrate that tumor-caused hirsutism is exceedingly rare, representing approximately 0.2 percent of cases, even among hyperandrogenic women. However, findings that are suggestive of a tumor include increasing age, a defined onset of symptoms, and a relatively rapid onset of symptoms.
When evaluating your patient for a tumor, you “should establish cause, using the history and physical, laboratory ultrasound, and radiologic studies, as needed,” in order to exclude 21-OH deficient NCAH, androgen-secreting tumors, syndromes of severe insulin resistance, Cushing’s Disease, acromegaly, and drug-induced hirsutism.
In the presence of symptoms and elevated T levels, imaging—transvaginal ultrasound and an adrenal CT and MRI—is required.
Circulating Androgens and their Source
There is no clear cutoff for a circulating androgen level that identifies a woman with a tumor. The standard measures (T > 200 ng/dL; DHEAS > 8000 mcg/mL) do not offer much positive predictive value.
Dr. Legro states unequivocally that suppression tests are not useful in the diagnosis of tumors because the tumors themselves may be suppressing ACTH secretion.
Dr. Legro summarizes his presentation thusly:
1) The history and physical are very useful for diagnosing tumors.
2) Markedly elevated serum androgen levels are suggestive, but not diagnostic of a tumor.
3) There is no clear cutoff for diagnosing tumors.
4) Imaging can diagnose most tumors.
5) The role of selective venous catheterization is limited.
6) Definitive treatment is surgical removal with follow-up to confirm normalization.