Introduction: Primary pituitary sarcoma causally unrelated to radiotherapy, but an increased risk of second brain tumors continues beyond 20 and 30 year after treatment.
We report an acromegalic patient with intrasellar giant adenoma invased suprasellar and nasal cavity during follow-up after radiotherapy.
Case: A 39-year-old woman had transsphenoidal and transcranial operation in 2002 and 2003 respectively. She had also conventional external beam radiotherapy after surgery. Thereafter, she treated with octreotide LAR 30 mg/month at the same time. Cabergoline had added one year later because of resistance of the therapy. She applied to outpatient clinic with severe headache and nevralgia on her face. Huge sellar mass invased suprasellar region and cavrnous sinus was found in sellar MRI. Sellar mass was bigger three times compared to previous MRI scan which was performed only three months ago. Growth hormone and IGF-I levels were markedly elevated. She underwent hypophyseal surgery immediately. Pathological examination and immunohistochemical stains revealed undifferentiated pleomorphic sarcoma. Though the surgery was performed, sellar mass regrew through the nasal cavity, clinical signs were progressively worsen and she died within two months.
Conclusion: In an acromegalic patient, persistently raised levels of growth hormone may associated with increased risk of second brain tumors in patients with pituitary adenoma treated with surgery and radiotherapy. An increased risk of second brain tumors usually appear more than 5 years after radiotherapy, in our case sarcomateous transformation was seen only two years later. It might be related either radiotherapy or high levels of GH or both.