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European guideline addresses treatment of aggressive pituitary tumors, carcinomas
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Patients with aggressive pituitary tumors and carcinomas should be treated by a multidisciplinary team and receive full endocrine laboratory evaluation and histopathologic analyses for tumor classification, according to recommendations included in new a clinical practice guideline released by the European Society of Endocrinology.
The guideline further identifies temozolomide monotherapy as first-line chemotherapy for these patients.
“Early identification of aggressive pituitary tumors is challenging, but is of major clinical importance as they are associated with an increased morbidity and mortality even in the absence of metastases,” the researchers wrote. “Despite numerous studies and advances in prognostic classification, no pathological marker has been shown as yet to reliably predict pituitary tumor behavior. This guideline proposes a definition of an aggressive pituitary tumor and provides recommendations for current management.”
The guideline recommends that, when necessary, surgery should be performed by a neurosurgeon with extensive experience following discussions between the patient and surgeon regarding repeat surgery before considering other treatment options.
Radiotherapy should be performed in patients with clinically relevant tumor growth despite surgery or surgery with standard medical treatment. Adjuvant radiotherapy should be used for clinically relevant invasive tumor remnant with pathologic markers, and discussions should take place between the patient and an expert radiation oncologist to address different radiotherapeutic options.
To control tumor growth, the guideline recommends standard medical treatment with maximally tolerated doses.
The guideline also recommends use of the oral chemotherapy drug temozolomide as first-line treatment and evaluation of treatment success after three cycles. Treatment with temozolomide should be continued for 6 months in patients who respond to the first three cycles.
Follow-up with an MRI, in most cases, and full endocrine evaluation should be performed every 3 to 12 months, according to the guideline.
“The publication of the [European Society of Endocrinology] clinical practice guidelines on aggressive pituitary tumors and carcinomas will hopefully improve identification and treatment of these rare tumors,” the researchers wrote. “Future efforts, similar to other European networks working on rare endocrine tumors, should combine the efforts of researchers and clinicians to establish an international register for this rare disorder. Our most recent experience in compiling the European survey on aggressive pituitary tumors highlights many clinicians who are interested in developing such an international clinical register.” – by Amber Cox
Disclosures: Some of the authors report financial ties with Ipsen, Pfizer and Novartis. Please see the guidelines for all other authors’ relevant financial disclosures.
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