Temozolomide: Anti-tumor effect on giant, invasive and resistant pediatric prolactinoma

Chentli, F.; Yaker, Fetta; Azzoug, S.; Belhimer, F.

doi:10.4103/2230-8210.122657

Cited by 5 publications

(1 citation statement)

References 4 publications

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“…There is now considerable experience with temozolomide in pituitary adenomas in adults (9). Four pediatric patients treated with temozolomide for a pituitary tumor (10–12) were previously described, whereas reports of two more patients with childhood-onset disease and temozolomide treatment in adulthood (13, 14) were published. Temozolomide was initiated in the current case because of aggressive tumor behavior showing rapid enlargement after surgery, with the aim of controlling tumor growth and sensitizing it to radiotherapy (15).…”

Section: Discussionmentioning

confidence: 99%

Surgery, Octreotide, Temozolomide, Bevacizumab, Radiotherapy, and Pegvisomant Treatment of an AIP Mutation‒Positive Child

Dutta

Reddy

Rai

et al. 2019

The Journal of Clinical Endocrinology &Amp; Metabolism

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Context Inactivating germline mutations in the aryl hydrocarbon receptor interacting protein (AIP) gene are linked to pituitary adenoma predisposition. Here, we present the youngest known patient with AIP-related pituitary adenoma. Case Description The patient presented at the age of 4 years with pituitary apoplexy and left ptosis with severe visual loss following a 1-year history of abdominal pain, headaches, and rapid growth. His IGF-1 level was 5× the upper limit of normal, and his random GH level was 1200 ng/mL. MRI showed a 43 × 24 × 35‒mm adenoma with suprasellar extension invading the left cavernous sinus (Knosp grade 4). After transsphenoidal surgery, histology showed a grade 2A sparsely granulated somatotropinoma with negative O6-methylguanine-DNA methyltransferase and positive vascular endothelial growth factor staining. Genetic testing identified a heterozygous germline nonsense AIP mutation (p.Arg81Ter). Exome sequencing of the tumor revealed that it had lost the entire maternal chromosome-11, rendering it hemizygous for chromosome-11 and therefore lacking functional copies of AIP in the tumor. He was started on octreotide, but because the tumor rapidly regrew and IGF-1 levels were unchanged, temozolomide was initiated, and intensity-modulated radiotherapy was administered 5 months after surgery. Two months later, bevacizumab was added, resulting in excellent tumor response. Although these treatments stabilized tumor growth over 4 years, IGF-1 was normalized only after pegvisomant treatment, although access to this medication was intermittent. At 3.5 years of follow-up, gamma knife treatment was administered, and pegvisomant dose increase was indicated. Conclusion Multimodal treatment with surgery, long-acting octreotide, radiotherapy, temozolomide, bevacizumab, and pegvisomant can control genetically driven, aggressive, childhood-onset somatotropinomas.

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