Guideline sources The following summarized guidelines for the evaluation and management of thymic neuroendocrine tumors (TNET) are prepared by our editorial team based on guidelines from the European Society of Medical Oncology (ESMO 2021). 1 Calculator Calculator Eastern Cooperative Oncology G Karnofsky performance status s 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 https://web.pathway.md/diseases/rechotlBAJnerRWkJ 1/3 6/24/23, 4:06 PM Thymic neuroendocrine tumors Pathway 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 Imaging for staging: obtain contrast-enhanced cross-sectional conventional imaging, including liver late arterial phase and positron emission tomography-CT with 68-galium-labeled somatostatin analogs, for TNM evaluation. B 3. Medical management General principles: Manage patients with thymic NETs 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 Management of local/locoregional disease: perform thymectomy by median sternotomy and/or thoracotomy and lymph node dissection in patients with thymic NETs. B Show 2 more Management of advanced/metastatic disease: guide the decision-making for therapeutic management based on prognosis. B Show 7 more Management of carcinoid syndrome: offer somatostatin analogs as first-line therapy for carcinoid syndrome and for other functional syndromes, such as acromegaly or hypercalcemia. B Administer IV short-acting somatostatin analogs for perioperative treatment. B Show 2 more Palliative care: Consider offering management in specialized palliative care units for pain treatment, psychosocial support, and rehabilitation in patients with disseminated disease. C Consider performing palliative surgery or locoregional procedures (radiofrequency ablation, cryoablation, endobronchial treatment) of the primary tumor in cases of advanced disease at risk of local events or refractory carcinoid syndrome. C 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 or MEN1, during follow-up of patients with thymic NETs. B Show 2 more https://web.pathway.md/diseases/rechotlBAJnerRWkJ 2/3 6/24/23, 4:06 PM Thymic neuroendocrine tumors Pathway References 1. E Baudin, M Caplin, R Garcia-Carbonero et al. Lung and thymic carcinoids: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2021 Apr;32 4 439 451. Open https://web.pathway.md/diseases/rechotlBAJnerRWkJ 3/3