Introduction: Atypical pituitary adenomas are often characterized by rapid growth and massive invasion of the surrounding structures. Usually, they are associated with poor prognosis and high recurrence rate due to resistance to conventional therapies. When surgery and radiation are ineffective, alternative therapies remain the last line treatment. Lately, a novel chemotherapy with capecitabine and temozolomide was proposed as a highly effective and extending the life of the patient procedure in refractory corticotroph pituitary tumors.
Aim: Aim of study was to present an effectiveness of CAPTEM in atypical corticotropinoma in a patient with Nelsons syndrome.
Case report: 56-year-old male with Cushings disease established 10 years earlier. During the first MRI 10×9×8 mm pituitary adenoma was detected. Initially the patient underwent three subtotal selective transsphenoidal adenomectomies (2005, 2007, 2009). A postoperative pathologic exploration revealed a densely granulated corticotroph cell adenoma with MIB-1 index >10% and MGMT (−). Due to a re-growth of the tumor, the patient underwent consecutive stereotactic radiotherapy (9Gy) and received cabergoline for 19 months. Afterwards, because of intractable complications of hypercortisolism, as well as previous treatment failures, a total bilateral adrenalectomy was performed as a lifesaving procedure. Subsequently, the patient developed Nelsons syndrome with intense skin hyperpigmentation and aggressive pituitary tumor progression. In 2011 transcranial neurosurgery and tomotherapy (45Gy) were applied. Due to next re-growth of the tumor with its expansion to cavernous sinuses and suprasellar region with a compression of the optic chiasm, and exhaustion of all conventional therapeutic options, the patient was admitted to our department for qualification to TMZ. Two cycles of TMZ (150 mg/m2) for 5 days were applied and because of the progression of eye damage CAPTEM was introduced. After four cycles hormonal and imaging stabilizations are observed.
Conclusion: CAPTEM can be an effective treatment option in atypical adenomas in Nelsons syndrome.
28 - 31 May 2016
European Society of Endocrinology