Home Radiation Aggressive tumor in Cushing’s disease managed by radiotherapy, chemo

Aggressive tumor in Cushing’s disease managed by radiotherapy, chemo


An aggressive tumor in the pituitary gland of the brain, which caused severe Cushing’s disease, has been successfully treated with a series of radiotherapy and chemotherapy with temozolomide, as described in a recent case report.

The study, “ACTH-secreting pituitary carcinoma: rare and lasting response to concomitant chemotherapy and re-irradiation with a review of the literature. Re-irradiation of recurrent pituitary carcinoma, ”Was published in the journal Advances in radiation oncology.

Cushing’s syndrome is a disease characterized by high levels of the stress hormone cortisol. In many cases, it is caused by a tumor in the pituitary gland, which produces greater amounts of the cortisol-controlling adrenocorticotropic hormone (ACTH), and known as Cushing’s disease.

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The majority of pituitary gland tumors, called adenomas, are benign. In rare cases, pituitary adenomas become aggressive, spread (metastasize), and respond poorly to treatment. These atypical adenomas are called pituitary carcinomas and, in some cases, result from ACTH-producing adenomas.

Doctors from the Medical College of Wisconsin described the case of a 53-year-old woman with severe Cushing’s disease due to an ACTH-producing pituitary carcinoma.

The woman presented to the hospital with complaints of blurred vision, swelling of the face and extremities, and weight gain over the two weeks.

An eye exam revealed that she had a type of partial blindness where peripheral vision is lost, and she underwent an MRI. The exam revealed a large mass in an area of ​​the brain called the suprasellar cistern, which is a fluid-filled space between the top of the pituitary gland and the bottom of the hypothalamus – an area of ​​the brain that regulates body temperature, sleep-wake cycles, and appetite, among other functions.

Further tests showed that she had elevated levels of ACTH and cortisol which, with imaging evidence, confirmed Cushing’s disease caused by a large pituitary adenoma producing ACTH.

She was subjected to tumor reduction – a procedure in which surgeons try to remove as much of the tumor as possible – followed by radiation therapy two months later.

After three years of remission, her symptoms worsened and were related to high cortisol levels. She received cabergoline followed by pasireotide, but failed to improve. Cabergoline, sold under the brand name Dostinex, and pasireotide, sold under the brand name Signifor, are drugs that work by decreasing the release of ACTH and ultimately cortisol.

Imaging revealed that the tumor had returned and had spread, which was consistent with a diagnosis of pituitary carcinoma. She had a second surgery to remove the tumor, but her cortisol levels remained high.

The woman’s health declined rapidly over the next two months, with weakness and worsening of Cushing’s symptoms.

She enrolled in a Phase 3 trial testing Isturisa (osilodrostat), a drug approved for certain patients with Cushing’s disease for whom brain surgery is either ineffective or not an option. But she was unable to tolerate it and was taken off the drug.

Follow-up MRIs showed signs of disease progression. The woman was referred for radiation therapy and chemotherapy with temozolomide (marketed as Temodar in the United States and as Temodal in Europe and Canada) for 42 days.

Over the next two years, her ACTH and cortisol levels steadily declined and her symptoms improved significantly. His cortisol levels, surprisingly, have fallen below normal.

After chemotherapy and radiation therapy, his strength and walking improved, as well as his vision.

A year and a half later, surveillance scans showed a marked decrease in residual tumor compared to previous scans.

Six months later, a new nodule was detected in the clivus, a bony part of the skull located at the base of the skull. A biopsy confirmed it was a pituitary carcinoma and she received radiation therapy.

At the time of intercourse, the woman was in clinical remission with a stable tumor appearance.

“Here we describe a unique and complete improvement in severe Cushing’s disease due to ACTH-secreting pituitary carcinoma … after reirradiation with concomitant temozolomide,” the investigators wrote.

The results support this regimen for “patients with ACTH secretion [pituitary carcinoma] that fail standard surgical and medical procedures, ”they added.