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Thymic neuroendocrine tumors are rare, representing less than 5% of all thymic cancers, and are biologically distinct in histology and behavior. Conclusive treatment guidelines are limited due to a lack of randomized clinical trials. Current literature, consisting mainly of case reports and series, suggests that complete surgical resection combined with adjuvant radiation therapy offers the greatest survival benefit for patients with thymic neuroendocrine tumors (NET). Adjuvant chemotherapy may also delay the onset of distant metastasis. Prognosis is generally poor, particularly in high-grade tumors, as these patients are prone to early metastasis. Here we present the case of a 62-year-old woman who achieved a durable response after undergoing an aggressive multimodal treatment approach for a primary thymic NET that initially presented as chronic cough and mild dyspnea on exertion.
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Syed et al. (2017) studied this question.
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