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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 European Society of Endocrinology (ESE 2025), the Acromegaly Consensus Conference (ACC 2018), and the Endocrine Society (ES 2011).
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Screening and diagnosis

Diagnosis: as per ESE 2025 guidelines, suspect aggressive pituitary tumor in patients with an invasive tumor and either unusually rapid tumor progression or clinically relevant tumor progression despite optimal standard therapies, including surgery, radiotherapy, and conventional medical treatments.
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Diagnostic investigations

Brain imaging
As per ESE 2025 guidelines:
Obtain imaging, preferably MRI, to quantify tumor dimensions, define invasion, and establish progression. Consider reporting tumor progression following a new treatment according to the RECIST 1.1.
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Consider obtaining radiological re-evaluation within 3-6 months in patients with suspicion of having an aggressive pituitary tumor based on clinical, radiological, and pathological features.
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  • Imaging for staging

  • Laboratory testing

  • Genetic testing

Diagnostic procedures

Histopathology: as per ESE 2025 guidelines, obtain immunohistochemical staining for pituitary hormones, assess proliferation with mitotic count, and use Ki67/MIB1 index and p53 immunostains for the histopathological diagnosis of pituitary tumors. Obtain immunostaining for pituitary-specific transcription factors in non-functioning tumors when staining for pituitary hormones is negative, and exclude metastasis from other tumors or other tumors of the sellar region when these factors are negative.
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Medical management

Hormonal therapy: as per ESE 2025 guidelines, offer standard medical treatment with somatostatin receptor ligand and/or dopamine agonist in functioning pituitary tumors using maximally tolerated doses to control tumor growth and hormone excess.
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  • Temozolomide

  • Immune checkpoint inhibitors

Therapeutic procedures

Radiotherapy: as per ESE 2025 guidelines, offer radiotherapy to improve tumor control in patients with clinically relevant tumor progression despite surgery and standard medical treatment.
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  • Locoregional therapies

Surgical interventions

Indications for surgery
As per ESE 2025 guidelines:
Perform surgery by an expert 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 2025 guidelines:
Obtain monitoring of hematological parameters and LFTs, and conduct careful clinical observation for potential adverse effects during treatment.
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Obtain the first evaluation of temozolomide treatment response after 3 cycles.
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  • Follow-up