Reddy Y Mounika, Padmanabhan Sourabh, Babu A Shobhan, Swarna B Sai
Department of Otorhinolaryngology, Head and Neck Surgery, Gandhi Medical College and Hospital, Secunderabad, India.
Indian J Otolaryngol Head Neck Surg. 2023 Sep;75(3):2177-2184. doi: 10.1007/s12070-023-03840-z. Epub 2023 May 6.
The main purpose of this study is to understand the characteristics and management of sinonasal small round blue cell tumors and also to emphasise the role of immunohistochemistry in their diagnosis and on the outcomes after endoscopic/open excision in these patients. This is a retrospective study conducted at a tertiary care referral centre in India which included 38 patients with sino nasal for a period of 5 years. All the patients were evaluated clinically and radiologically. All cases were confirmed diagnostically with histopathological examination and immunohistochemistry following surgical excision either by endoscopic or open approach. Some of the cases underwent post operative radiotherapy. In our study, among 176 cases diagnosed with Sino nasal malignancies, 38 (21.6%) cases were diagnosed with sinonasal small round blue cell tumors with male to female ratio 1.4:1. Most common histopathological type among all the sinonasal small round blue cell tumors that presented to us was esthesioneuroblastoma i.e., 8 (21%) patients followed by pituitary macroadenoma in 7(8.4%) patients. Other types are undifferentiated squamous cell carcinoma 10(13.1%), craniopharyngioma 8(10.5%), lymphoma 3(7.9%), synovial/spindle cell sarcoma, malignant melanoma and adenocarcinoma 1(2.6%) each. Schwannoma, rhabdomyosarcoma, neuroendocrine carcinoma and neurofibroma 2 (5.2%) each. : Sinonasal small round blue cell tumors are extremely rare tumours. Histopathological diagnosis with immunohistochemistry is characteristic of various tumors and is conclusive for diagnosis. Knowledge of these tumor entity is essential as early diagnosis helps in further management in preventing spread to vital structures and improving outcome. Most of the tumors have a multimodality treatment approach which includes surgical excision, radiotherapy and chemotherapy.
本研究的主要目的是了解鼻窦小圆形蓝细胞肿瘤的特征及治疗方法，同时强调免疫组织化学在其诊断以及这些患者经内镜/开放切除术后预后评估中的作用。这是一项在印度一家三级医疗转诊中心进行的回顾性研究，研究对象为38例鼻窦疾病患者，研究时长为5年。所有患者均接受了临床和放射学评估。所有病例在手术切除（内镜或开放手术）后，均通过组织病理学检查和免疫组织化学进行确诊。部分病例接受了术后放疗。在我们的研究中，在176例被诊断为鼻窦恶性肿瘤的病例中，38例（21.6%）被诊断为鼻窦小圆形蓝细胞肿瘤，男女比例为1.4:1。在我们所诊治的所有鼻窦小圆形蓝细胞肿瘤中，最常见的组织病理学类型是嗅神经母细胞瘤，即8例（21%）患者，其次是垂体大腺瘤，有7例（8.4%）患者。其他类型包括未分化鳞状细胞癌10例（13.1%）、颅咽管瘤8例（10.5%）、淋巴瘤3例（7.9%）、滑膜/梭形细胞肉瘤、恶性黑色素瘤和腺癌各1例（2.6%）。神经鞘瘤、横纹肌肉瘤、神经内分泌癌和神经纤维瘤各2例（5.2%）。鼻窦小圆形蓝细胞肿瘤是极其罕见的肿瘤。通过免疫组织化学进行组织病理学诊断是各种肿瘤的特征，且对诊断具有决定性意义。了解这些肿瘤实体至关重要，因为早期诊断有助于进一步治疗，防止肿瘤扩散至重要结构并改善预后。大多数肿瘤采用多模式治疗方法，包括手术切除、放疗和化疗。
