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Pituitary carcinoma

What's new

The European Society of Endocrinology (ESE) has published an updated guideline for the management of aggressive pituitary tumors and pituitary carcinomas. Recommended treatments include surgery, radiotherapy for tumor control as well as in the adjuvant setting, and medical therapy with standard endocrine agents (somatostatin receptor ligands and/or dopamine agonists) for functioning tumors, first-line chemotherapy with temozolomide (150-200 mg/m² for 5 days every 28 days), and a trial of immune checkpoint inhibitors for cases of rapid tumor progression following temozolomide therapy. .

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of pituitary carcinoma are prepared by our editorial team based on guidelines from the Acromegaly Consensus Conference (ACC 2018), the European Society of Endocrinology (ESE 2018), and the Endocrine Society (ES 2011).
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Screening and diagnosis

Diagnosis: as per ESE 2018 guidelines, suspect an aggressive pituitary tumor in patients with a radiologically invasive tumor and unusually rapid tumor growth rate, or clinically relevant tumor growth despite optimal standard therapies (surgery, radiotherapy, and conventional medical treatments).
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Diagnostic investigations

Brain imaging: as per ESE 2018 guidelines, obtain imaging (MRI in most instances) for quantification of tumor dimensions, invasion, and growth.
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  • Imaging for staging

  • Laboratory testing

  • Genetic testing

Diagnostic procedures

Histopathology
As per ESE 2018 guidelines:
Obtain histopathological analysis, including a minimum immunodetection of pituitary hormones and Ki-67 proliferative index evaluation, in all patients with pituitary tumors. Obtain at least p53 immunodetection and mitotic count assessment when the Ki-67 index is ≥ 3%.
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Interpret histopathological results in the clinical context of the individual patient.
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Medical management

General principles
As per ESE 2018 guidelines:
Discuss patients with aggressive pituitary tumors and carcinomas in a multidisciplinary expert team meeting (including endocrinologists, neurosurgeons, pituitary pathologists, neuroradiologists, radiation oncologists, and medical oncologists).
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Offer standard medical treatment with maximally tolerated doses in order to control tumor growth, as per current guidelines.
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  • Temozolomide

  • Immune checkpoint inhibitors

Therapeutic procedures

Radiotherapy: as per ESE 2018 guidelines, offer radiotherapy in patients with clinically relevant tumor growth despite surgery in nonfunctioning tumors or surgery and standard medical treatment in functioning tumors.
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  • Locoregional therapies

Surgical interventions

Indications for surgery
As per ESE 2018 guidelines:
Perform surgery by a neurosurgeon with extensive experience in pituitary surgery.
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Discuss repeat surgery with an expert neurosurgeon before considering other treatment options.
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Follow-up and surveillance

Assessment of treatment response: as per ESE 2018 guidelines, obtain careful clinical observation for potential adverse effects and monitor hematological parameters and LFTs during treatment.
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  • Follow-up