Rare case of spontaneous remission in Cushing’s reported in girl, 14
Remission follows rare occurrence of condition known as pituitary apoplexy
The rare case of a 14-year-old girl who experienced spontaneous remission of Cushing’s disease after pituitary tumor apoplexy has been reported by researchers in South America.
Pituitary apoplexy, or PA, is a rare and potentially life-threatening condition caused by bleeding or infarction (cell death due to impaired blood flow) in the pituitary gland, commonly due to a tumor.
“Spontaneous remission of this disease is very uncommon, but when it occurs, it is mainly due to PA,” the researchers wrote, noting that this case had other “unique aspects,” including the early age of symptom onset in the teen.
“It is imperative for clinicians to be aware of this possible outcome in patients with CD [Cushing’s disease],” the team wrote.
The case was described in “Spontaneous Cushing’s Disease Remission Induced by Pituitary Apoplexy,” published in Cureus.
Teenager diagnosed with Cushing’s after two years of symptoms
Cushing’s disease is marked by high levels of the cortisol hormone due to a tumor, usually benign, in the brain’s pituitary gland. This tumor results in the production of greater than normal amounts of ACTH, which stimulates the adrenal glands, located atop the kidneys, to produce cortisol.
As such, surgery to remove the pituitary tumor is the first-line treatment of Cushing’s disease.
Now, researchers in South America described the case of a teenage girl whose Cushing’s disease was spontaneously resolved after pituitary apoplexy.
Spontaneous remission in CD due to apoplexy has been rarely reported in the past; hence, our case is an important addition to the scant literature on this unusual phenomenon.
Due to bleeding or loss of blood flow in the pituitary gland, pituitary apoplexy — a rare event — can cause sudden headache, visual symptoms, and altered mental status.
“Spontaneous remission in CD due to apoplexy has been rarely reported in the past; hence, our case is an important addition to the scant literature on this unusual phenomenon,” the researchers wrote.
The girl had a two-year history of weight gain, growth impairment, high blood pressure, and type 2 diabetes. as well as depression. A physical examination revealed she had a moon face, acne, excess body hair, fat buildup in the upper back and abdomen, and skin stretch marks. All these signs and symptoms were suggestive of Cushing’s disease.
Given the high suspicion of Cushing’s, hormonal analyses were performed, which indicated the girl had higher than normal levels of cortisol and ACTH. Another diagnostic test, called dexamethasone suppression test, confirmed that the girl had Cushing’s disease.
A brain MRI indicated the presence of a small pituitary tumor, and the girl was referred for surgery to remove it.
About one month later, while awaiting surgery, she sought treatment at the emergency department for a sudden, severe headache accompanied by vomiting and double vision. A CT scan revealed no structural abnormalities, and the girl’s symptoms resolved with intravenous, or into-the-vein, pain medication.
Severely low cortisol levels found weeks after spontaneous remission
Weeks after this episode, the girl was reevaluated at the emergency department due to abdominal and muscle pain, vomiting, generalized weakness, low blood pressure, abnormally fast heart rate, and low blood sugar levels.
She was suspected to have acute adrenal insufficiency, which was confirmed by severely low cortisol levels. She started on treatment with hydrocortisone 50 mg every six hours, leading to the complete resolution of symptoms within three days.
The girl was discharged on maintenance therapy with oral hydrocortisone, at a dose of 20 mg in the morning and 10 mg at night.
The patient was followed for 10 months after the event, and showed persistent clinical and hormonal disease remission.
However, given that several cases of Cushing’s recurrence “have been described after remission … due to AP,” the researchers noted that longer-term follow-up “is required to detect both [disease] reactivation and hypopituitarism,” or decreased function of the pituitary gland.
In their report, the team also highlighted three unique, and rare aspects of this case: the patient’s young age at disease onset, the spontaneous disease remission due to pituitary apoplexy, and the occurrence of PA in a small pituitary tumor.
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