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Abstract：
Neuroendocrine tumors (NETs) are a rare spectrum of neoplasms that are characterized by neuroendocrine and neural differentiation. The treatment can be challenging in view of the heterogeneity in differentiation and behavior. Primary renal origin NETs are rare and only a few cases have been reported in the literature. There is limited knowledge on their presentation and response to various lines of treatment. We report a case of a patient with a metastatic renal NET from a rare histological subtype of large cell neuroendocrine carcinoma, known to cause aggressive disease with poor prognosis. A multimodality treatment approach was followed. In spite of surgical management and second-line chemotherapy, the disease progressed. The patient subsequently received peptide receptor radionuclide therapy (PRRNT) using lutetium-177 DOTATATE, following which the patient demonstrated a remarkable clinical and radiological response and is stable to date. In a rare tumor with poor prognosis, the relevance of theranostics and the efficacy of targeted therapies like PRRNT are noteworthy.
摘要：
神经内分泌肿瘤（NETs）是一种罕见的肿瘤，其特征是神经内分泌和神经分化。鉴于分化和行为的异质性，治疗可能具有挑战性。原发性肾源性NETs很少见，文献中仅报道了少数病例。关于他们的介绍和对各种治疗方法的反应的知识有限。我们报告了一例来自罕见的大细胞神经内分泌癌组织学亚型的转移性肾NET患者，已知引起侵袭性疾病，预后不良。遵循多模式治疗方法。尽管有手术治疗和二线化疗，疾病进展。患者随后接受肽受体放射性核素治疗（PRRNT），使用Lutium-177DOTATATE，随后，患者表现出明显的临床和放射学反应，并且迄今为止保持稳定。在一个罕见的肿瘤预后差，治疗药物的相关性和PRRNT等靶向治疗的疗效值得注意.
