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Thymic neuroendocrine tumors

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).
1

Guidelines

1.Classification and risk stratification

Prognostic classification
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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2.Diagnostic investigations

Biomarker analysis: 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

3.Medical management

General principles
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

4.Follow-up and surveillance

Follow-up: focus on tumor and functioning syndrome evaluations as well as evaluations of long-term toxicity and specific conditions, including diffuse pulmonary neuroendocrine cell hyperplasia orMEN1 , during follow-up of patients with TNETs.
B
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