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Pituitary carcinoma

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 2018), the Acromegaly Consensus Conference (ACC 2018), and the Endocrine Society (ES 2011).
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Guidelines

1.Screening and diagnosis

Diagnosis: 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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2.Diagnostic investigations

Brain imaging: 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

3.Diagnostic procedures

Histopathology
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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4.Medical management

General principles
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

  • Other cytotoxic therapies

5.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

6.Surgical interventions

Indications for surgery
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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7.Follow-up and surveillance

Assessment of treatment response: obtain careful clinical observation for potential adverse effects and monitor hematological parameters and liver function tests during treatment.
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  • Serial imaging and laboratory assessment

  • Management of recurrence