Korra Himabindu, Gandi Joseph Benjamin, Nanuvala Prathyusha, Ardha Aarathi
Department of Radiotherapy, MNJ Institute of Oncology & Regional Cancer Centre, Osmania Medical College, Hyderabad, Telangana, India.
South Asian J Cancer. 2022 Aug 16;11(4):336-339. doi: 10.1055/s-0041-1739181. eCollection 2022 Oct.
Olfactory neuroblastoma is a rare epithelial malignancy arising from the odorant receptors in the nasal mucosa or along the cribriform plate of the ethmoid bone. Clinical presentation includes nasal stuffiness, local pain, epistaxis, anosmia, visual impairment, proptosis, headache, and seizures. Radiologic imaging with CT or MRI, an ophthalmic evaluation, and histopathologic confirmation with immunohistochemistry are parts of the initial diagnostic workup. Although surgery, chemotherapy, and radiation have an equally important role in the management, earlier stages may preferably be treated with surgery or radiotherapy and the later stages with a multimodality approach. We conducted a retrospective review of 13 patients diagnosed with olfactory neuroblastoma, registered at Mehdi Nawaz Jung Regional Cancer Center over a decade (2010-2019). We analyzed the age and sex distribution, performance status at presentation, clinical symptomatology, and the Kadish stage. In addition, the therapeutic aspects of patients were studied. The most common presentation noted was nasal stuffiness, followed by epistaxis and proptosis. The majority of patients had good performance status at presentation. Ten patients presented with a Kadish stage C, while the remaining patients presented with Kadish stage B. Cervical nodal metastasis was seen in three patients, four patients received multimodality treatment with neoadjuvant chemoradiotherapy followed by surgery, two patients received neoadjuvant chemotherapy followed by radiation, two patients received only surgery, and one patient received surgery followed by adjuvant radiation. Conformal radiation techniques were used to deliver doses as high as 50 to 66 Gy in 2 Gy per fraction. Two patients presented with distant metastasis during follow-up, one with bone metastasis, and the other with retroperitoneal nodal metastasis; they received palliative chemotherapy and conformal radiation to the primary site. This study concludes that neoadjuvant chemotherapy followed by radiation gives the best outcomes. It has been observed that in multi-modality treatment, radiotherapy played a significant role in improving overall survival and better outcomes. Multidisciplinary discussions provide a better sequencing of management.
嗅神经母细胞瘤是一种罕见的上皮性恶性肿瘤，起源于鼻黏膜或沿筛骨筛板的嗅觉受体。临床表现包括鼻塞、局部疼痛、鼻出血、嗅觉丧失、视力障碍、眼球突出、头痛和癫痫发作。CT或MRI影像学检查、眼科评估以及免疫组化的组织病理学确诊是初始诊断检查的一部分。尽管手术、化疗和放疗在治疗中具有同等重要的作用，但早期阶段可能更适合手术或放疗，晚期阶段则采用多模式方法治疗。
我们对在迈赫迪·纳瓦兹·荣格地区癌症中心登记的13例诊断为嗅神经母细胞瘤的患者进行了回顾性研究，这些患者登记时间超过十年（2010 - 2019年）。我们分析了年龄和性别分布、就诊时的身体状况、临床症状以及卡迪什分期。此外，还研究了患者的治疗情况。
最常见的表现是鼻塞，其次是鼻出血和眼球突出。大多数患者就诊时身体状况良好。10例患者表现为卡迪什C期，其余患者表现为卡迪什B期。3例患者出现颈部淋巴结转移，4例患者接受新辅助放化疗后手术的多模式治疗，2例患者接受新辅助化疗后放疗，2例患者仅接受手术，1例患者接受手术及辅助放疗。采用适形放疗技术，每次分割剂量为2 Gy，总剂量高达50至66 Gy。2例患者在随访期间出现远处转移，1例为骨转移，另1例为腹膜后淋巴结转移；他们接受了姑息化疗和原发部位的适形放疗。
本研究得出结论，新辅助化疗后放疗效果最佳。据观察，在多模式治疗中，放疗在提高总生存率和改善预后方面发挥了重要作用。多学科讨论能提供更好的治疗顺序安排。
