Tiny pituitary tumor causes severe Cushing’s in young man: Case report
Doctors take unusual treatment approach after ruling out surgery
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An exceptionally small pituitary tumor caused severe Cushing’s disease in a young man, leading doctors to take an unusual treatment approach after determining the disease-causing microadenoma would be too difficult to remove surgically, according to a case report from Iraq.
Instead of surgery — the standard treatment for Cushing’s — the man underwent Gamma Knife radiosurgery, a noninvasive procedure that delivers highly focused radiation therapy to the tumor. When his levels of the cortisol hormone remained high five months later, doctors added the antifungal medication ketoconazole, which brought them back into the normal range within four weeks.
“This case highlights that very small adenomas can cause severe disease and underscores the delayed response to [Gamma Knife radiosurgery], necessitating early adjunctive medical therapy to control [high cortisol levels] and reduce [disease complications],” researchers wrote.
The study, “An unusually small pituitary microadenoma causing severe Cushing disease in a male patient: lessons from early Radiosurgical management,” was published in Oxford Medical Case Reports.
Antifungal medication blocks key enzymes
Cushing’s disease is caused by tumors in the pituitary gland, located at the base of the brain. These tumors release excess adrenocorticotropic hormone (ACTH), which stimulates the adrenal glands atop the kidneys to produce cortisol. Persistently high cortisol levels can lead to a range of symptoms such as weight gain, diabetes, high blood pressure, fatigue, and muscle weakness.
Transsphenoidal adenomectomy, a minimally invasive surgery to remove the tumor through the nose, is usually the treatment of choice. When surgery isn’t possible or isn’t successful, however, Gamma Knife radiosurgery can offer an alternative treatment method. Because its effects often take months or even years to fully develop, medications that lower cortisol production may be used in the meantime.
One such medication is the antifungal agent ketoconazole. The drug, which is approved for Cushing’s in Europe and used off-label for the same indication in the U.S., works by blocking several enzymes involved in the production of steroid hormones, including cortisol.
Doctors opt for radiation treatment, antifungal medication
In this report, a team of researchers described the case of a 27-year-old man whose cortisol levels remained abnormally high for months after Gamma Knife treatment. He eventually required additional therapy with ketoconazole.
The young man sought medical attention after two years of progressively worsening symptoms. His face became increasingly round and flushed, he gained about 15 kilograms (33 pounds) over one year, and his abdomen became enlarged.
He also developed wide reddish-purple stretch marks across his abdomen and flanks, along with severe fatigue during physical activity, excessive sweating (especially at night), daytime sleepiness, headaches, intermittent blurred vision, low mood, and reduced libido. About a year before his Cushing’s diagnosis, he had been diagnosed with high blood pressure, diabetes, and high cholesterol.
Blood tests confirmed Cushing’s disease, showing abnormally high levels of both ACTH and cortisol. A high-resolution MRI scan then revealed the cause: a pituitary microadenoma measuring just 1.2 millimeters (0.05 inches).
Persistent ACTH elevation despite normalized cortisol levels demonstrates the intricate feedback disruption in treated Cushing disease.
Because the tumor was so small, neurosurgeons determined it would be too difficult to accurately locate and remove during transsphenoidal adenomectomy. Instead, they opted for Gamma Knife radiosurgery.
The treatment, however, did not produce an immediate response. Five months later, the man’s cortisol and ACTH levels remained elevated, and he continued to experience fatigue and weight gain. Thus, doctors prescribed ketoconazole. Four weeks after starting ketoconazole, his cortisol levels returned to the normal range, although ACTH levels remained elevated.
“Persistent ACTH elevation despite normalized cortisol levels demonstrates the intricate feedback disruption in treated Cushing disease,” the researchers wrote. “Ongoing surveillance and multidisciplinary care remain essential to optimise outcomes, manage [co-occurring diseases], and monitor for therapy-related adverse effects.”
According to the researchers, the case highlights several important lessons. First, even extremely small pituitary tumors can cause severe Cushing’s disease. Second, while Gamma Knife radiosurgery may be an option when surgery isn’t feasible, it often takes months or even years to achieve hormonal remission, making close monitoring essential. Finally, medications such as ketoconazole may help control excess cortisol during that waiting period, reducing the risk of complications from prolonged high cortisol levels.
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