Medical Oncology Department, Gastrointestinal and Endocrine Tumor Unit, Vall d'Hebron University Hospital and Vall d'Hebron Institute of Oncology (VHIO), Barcelona, Spain.
Clinical Pharmacology Department, Vall d'Hebron University Hospital, Barcelona, Spain.
Front Endocrinol (Lausanne). 2021 Nov 11;12:731631. doi: 10.3389/fendo.2021.731631. eCollection 2021.
Nelson's syndrome is considered a severe side effect that can occur after a total bilateral adrenalectomy in patients with Cushing's disease. It usually presents with clinical manifestations of an enlarging pituitary tumor including visual and cranial nerve alterations, and if not treated, can cause death through local brain compression or invasion. The first therapeutic option is surgery but in extreme cases of inaccessible or resistant aggressive pituitary tumors; the off-label use of chemotherapy with capecitabine and temozolomide can be considered. However, the use of this treatment is controversial due to adverse events, lack of complete response, and inability to predict results. We present the case of a 48-year-old man diagnosed with Nelson's syndrome with prolonged partial response and significant clinical benefit to treatment with capecitabine and temozolomide.
纳尔逊综合征被认为是库欣病患者行双侧肾上腺全切除术后的一种严重的副作用。它通常表现为垂体瘤增大的临床表现，包括视觉和颅神经改变，如果不治疗，可能会因局部脑压迫或侵犯而导致死亡。第一种治疗选择是手术，但在无法手术或耐药性侵袭性垂体瘤的极端情况下，可以考虑使用卡培他滨和替莫唑胺的化疗。然而，由于不良反应、不完全缓解和无法预测结果，该治疗方法存在争议。我们报告了一例 48 岁男性，诊断为纳尔逊综合征，卡培他滨和替莫唑胺治疗后出现长时间部分缓解和显著临床获益。
