Guidelines Summary

Guidelines published in 2017 by the European Society of Endocrinology on the management of aggressive pituitary tumors and carcinomas include the following^[45]:

A recommendation that all pituitary tumors undergo histopathologic analysis, including, at a minimum, immunodetection of pituitary hormones and Ki-67 proliferative index evaluation; the p53 immunodetection and the mitotic count should be evaluated at least when the Ki-67 index is 3% or greater
A recommendation that radiotherapy be used in patients with clinically relevant tumor growth despite surgery in nonfunctioning tumors or surgery and standard medical treatment in functioning tumors
A suggestion that adjuvant radiotherapy be considered in the setting of a clinically relevant invasive tumor remnant with pathologic markers (Ki-67 index; mitotic count; p53 immunodetection) strongly indicating aggressive behavior
A recommendation that temozolomide monotherapy be used as first-line chemotherapy for aggressive pituitary tumors and pituitary carcinomas, following documented tumor growth
A recommendation that the first evaluation of treatment response occur after 3 cycles and that if radiologic progression is demonstrated, temozolomide treatment be stopped
A recommendation that the standard dosing regimen—150-200 mg/m ² for 5 consecutive days every 28 days—be used
A suggestion that in patients with rapid tumor growth in whom maximal doses of radiotherapy have not been reached, combination temozolomide with radiotherapy (Stupp protocol) be used
A suggestion that in patients responding to first-line temozolomide, as assessed after 3 cycles, treatment be continued for at least 6 months in total, with consideration for longer duration if continued therapeutic benefit is observed
A suggestion that patients who develop a recurrence following response to temozolomide treatment undergo a second trial of 3 cycles of temozolomide
A recommendation, in the follow-up of an aggressive pituitary tumor, that imaging (magnetic resonance imaging [MRI] in most instances) be performed every 3-12 months, as guided by prior tumor growth rate and/or location (proximity to vital structures)

Medication

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