Department of Neurosurgery, Lenox Hill Hospital, Zucker School of Medicine at Hofstra/Northwell, New York, New York.
Department of Radiology, Lenox Hill Hospital, Zucker School of Medicine at Hofstra/Northwell, New York, New York.
Oper Neurosurg (Hagerstown). 2018 Dec 1;15(6):100-109. doi: 10.1093/ons/opy028.
Olfactory neuroblastoma, also known as esthesioneuroblastoma (ENB), is a malignant neoplasm with an unpredictable behavior. Currently, the widely accepted treatment is inductive chemotherapy, with or without surgery, followed by radiotherapy. Since data on genetics and molecular alterations of ENB are lacking, there is no standard molecularly targeted therapy. However, ENB commonly expresses the somatostatin receptor (SSTR) that is also expressed by neuroendocrine tumors. Peptide receptor radionuclide therapy (PRRT) using radiolabeled somatostatin analogues, such as 177Lu-octreotate, is an effective treatment for the latter. We present the complex neuroradiological and neuropathological changes associated with 177Lu-octreotate treatment of a patient with a highly treatment-resistant ENB.
A 60-yr-old male presented with an ENB that recurred after chemotherapy, surgery, stereotactic radiosurgery, and immunotherapy. Pathology revealed a Hyams grade 3 ENB and the tumor had metastasized to lymph nodes. Tumor SSTR expression was seen on 68Ga-octreotate positron emission tomography (PET)/computed tomography (CT), suggesting that PRRT may be an option. He received 4 cycles of 177Lu-octreotate over 6 mo, with a partial response of all lesions and symptomatic improvement. Four months after the last PRRT cycle, 2 of the lesions rapidly relapsed and were successfully resected. Three months later, 68Ga-octreotate PET/CT and magnetic resonance imaging indicate no progression of the disease.
We describe imaging changes associated with 177Lu-octreotate PRRT of relapsing ENB. To our knowledge, this is the first report describing neuropathological changes associated with this treatment. PRRT is a promising therapeutic option to improve the disease control, and potentially, the survival of patients with refractory ENB.
嗅神经母细胞瘤，又称嗅神经上皮瘤（ENB），是一种行为不可预测的恶性肿瘤。目前，广泛接受的治疗方法是诱导化疗，辅以手术，然后进行放疗。由于缺乏 ENB 的遗传学和分子改变数据，因此没有标准的分子靶向治疗方法。然而，ENB 通常表达生长抑素受体（SSTR），神经内分泌肿瘤也表达该受体。使用放射性标记的生长抑素类似物（如 177Lu-奥曲肽）的肽受体放射性核素治疗（PRRT）是治疗后者的有效方法。我们介绍了一名高度耐药性 ENB 患者接受 177Lu-奥曲肽治疗后出现的复杂神经放射学和神经病理学变化。
一名 60 岁男性因化疗、手术、立体定向放射外科和免疫治疗后复发的 ENB 就诊。病理显示 Hyams 分级 3 级 ENB，肿瘤已转移至淋巴结。68Ga-奥曲肽正电子发射断层扫描（PET）/计算机断层扫描（CT）显示肿瘤 SSTR 表达，提示 PRRT 可能是一种选择。他在 6 个月内接受了 4 个周期的 177Lu-奥曲肽治疗，所有病变均有部分缓解且症状改善。最后一次 PRRT 治疗周期后 4 个月，2 个病变迅速复发并成功切除。3 个月后，68Ga-奥曲肽 PET/CT 和磁共振成像显示疾病无进展。
我们描述了与复发 ENB 的 177Lu-奥曲肽 PRRT 相关的影像学变化。据我们所知，这是第一份描述该治疗相关神经病理学变化的报告。PRRT 是改善疾病控制的一种有前途的治疗选择，可能提高难治性 ENB 患者的生存率。
