Temozolomide-Induced Shrinkage of Invasive Pituitary Adenoma in Patient with Nelson’s Syndrome: A Case Report and Review of the Literature

Abstract

Introduction. Invasive tumours in Nelson's syndrome need aggressive therapy. Recent reports have documented the efficacy of temozolomide (TMZ) in the treatment of adenomas resistant to conventional management. Objective. The review of the literature concerning TMZ treatment of atypical corticotroph adenomas and a case study of 56-year-old woman who developed Nelson's syndrome. Treatment Proceeding. The patient with Cushing's disease underwent transsphenoidal adenomectomy followed by a 27-month-long period of remission. Due to a regrowth of the tumor, she underwent two reoperations followed by stereotactic radiotherapy. Because of treatment failures, bilateral adrenalectomy was performed. Then she developed Nelson's syndrome. A fourth transsphenoidal adenomectomy was performed, but there was a rapid recurrence. Five months later, she underwent a right frontotemporal craniotomy. Due to a rapid regrowth of the tumour, the patient did not receive gamma-knife therapy and was treated with cabergoline and somatostatin analogue for some time. Only TMZ therapy resulted in marked clinical, biochemical, and radiological improvement. To date, this is the first case of invasive corticotroph adenoma in Nelson's syndrome treated with temozolomide in Poland. Conclusion. In our opinion, temozolomide can be an effective treatment option of invasive adenomas in Nelson's syndrome.

Temozolomide therapy for aggressive pituitary Crooke’s cells corticotropinoma causing Cushing’s Disease: A case report with literature review

“…After six courses of standard TMZ therapy tumour regression was observed, and nine courses resulted in a total radiological tumour shrinkage and hormonal normalisation [18]. In another case of invasive corticotroph adenoma (a patient with Nelson Syndrome after bilateral adrenalectomy) resistant to standard therapy treated with TMZ, tumour volume reduction was about 20% after three cycles with TMZ with a decrease in ACTH level [19]. The patient received nine cycles of TMZ without the progression of the disease and with a stable volume of the tumour.…”

Długotrwała całkowita remisja guza kortykotropowego z komórek Crooke’a po leczeniu temozolomidem

“…According to many authors [5,[9][10][11][12][13][14][15], temozolomide is an efficacious, accessible, easy to use, and well-tolerated medication of low toxicity that can be used for treatment of life-threatening pituitary tumours that are refractory to standard treatment modalities. The general effectiveness of TMZ therapy is approximately 55% for aggressive adenomas and 58% for pituitary carcinomas [9].…”

“…Based on heretofore publicated reports, Bruno et al [9] and Li et al [12] suggest, that TMZ may be a salvage therapy mainly in atypical prolactinomas (73% response rate) and atypical corticotropinomas (60% response rate). Since 2004, TMZ therapy has been used in approximately 130 patients with atypical adenoma or carcinoma of the pituitary with varying results [5,[9][10][11][13][14][15]. Experience with TMZ treatment in patients with Crooke's cell tumours is limited.…”

“…Atypical pituitary corticotropinomas, which account for approximately 1/3 of tumours treated with TMZ [9,11,14], including Crooke's tumours, seem to respond particularly well to this type of treatment. TMZ therapy resulted in stabilising the disease (total remission, partial remission, or lack of progression) in more than 60% of patients [9,11,14].…”

“…TMZ therapy resulted in stabilising the disease (total remission, partial remission, or lack of progression) in more than 60% of patients [9,11,14].…”