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Thymic neuroendocrine tumors

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of thymic neuroendocrine tumors are prepared by our editorial team based on guidelines from the European Society of Medical Oncology (ESMO 2021). ...
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Classification and risk stratification

Prognostic classification
As per ESMO 2021 guidelines:
Use the WHO and pTNM classifications as the basis of the prognostic classification.
B
Take into account specific prognostic factors including tumor growth rate and the presence of functioning syndromes in advanced-stage thymic carcinoids.
B
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Diagnostic investigations

Biomarker analysis: as per ESMO 2021 guidelines, obtain Ki-67/MIB-1,
B
TTF-1, and p53/RB1 biomarker analyses in selected patients for differential diagnosis or site of origin orientation.
B

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  • Imaging for staging

Medical management

General principles
As per ESMO 2021 guidelines:
Manage patients with TNETs by expert multidisciplinary teams in centers grouped within national or regional networks for care and research optimization,
B
and ensure a multidisciplinary standardized approach to management.
B
Consider controlling the presenting functioning syndrome before any invasive therapeutic intervention.
B

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  • Management of local/locoregional disease

  • Management of advanced/metastatic disease

  • Management of carcinoid syndrome

  • Palliative care

Follow-up and surveillance

Follow-up: as per ESMO 2021 guidelines, focus on tumor and functioning syndrome evaluations as well as evaluations of long-term toxicity and specific conditions, including diffuse pulmonary neuroendocrine cell hyperplasia or MEN1, during follow-up of patients with TNETs.
B
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