Abstract：
BACKGROUND: Small round cell tumors (SRCTs) are a group of malignant neoplasms with minimal or no differentiation, characterized by the presence of round cells with high nuclear-cytoplasmic ratio. Although SRCTs can occur in any part of the body, involvement of central nervous system (CNS) is uncommon.
OBJECTIVE: We aimed to study the clinicopathological spectrum of cranial SRCT diagnosed in our institute over a period of four years (2016-2019).
METHODS: A retrospective review of medical records (2016-2019) with a morphological diagnosis of cranial SRCT was made. Both intra-axial and extra-axial tumors were included. A total of 60 cases were retrieved, and the clinical and histopathological features were studied. Special cytochemical staining and immunohistochemistry were performed, where needed.
RESULTS: The mean age at presentation was 18.4 years (range, 1-60 years), with a male-to-female ratio of 2.5:1. The most common site was posterior fossa of brain (n = 28, 47%), followed by dorso-lumbar spine (n = 9, 15%). The most common type of tumor was medulloblastoma (n = 29, 48.3%), followed by Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumor (pPNET) (n = 11, 18.3%), non-Hodgkin lymphoma (NHL) (n = 9, 15%), neuroblastoma (n = 3, 5%), and CNS embryonal tumor, NOS (n = 2, 3.3%). One case each of atypical teratoid rhabdoid tumor (ATRT), rhabdomyosarcoma, pineoblastoma, melanoma, rhabdomyosarcoma, and undifferentiated pleomorphic sarcoma was also documented.
CONCLUSIONS: SRCTs have a variable age of presentation. Their incidence in CNS is low as compared to other organ systems. On light microscopy, the histopathology of these lesions is overlapping, posing a great diagnostic dilemma for the pathologist. The use of ancillary techniques like immunohistochemistry helps in arriving at the correct diagnosis. Treatment strategy and tumor prognosis also vary along the entire spectrum of SRCT, thus making exact characterization essential for proper management.
OBJECTIVE: We aimed to study the clinicopathological spectrum of cranial SRCT diagnosed in our institute over a period of four years (2016-2019).
METHODS: A retrospective review of medical records (2016-2019) with a morphological diagnosis of cranial SRCT was made. Both intra-axial and extra-axial tumors were included. A total of 60 cases were retrieved, and the clinical and histopathological features were studied. Special cytochemical staining and immunohistochemistry were performed, where needed.
RESULTS: The mean age at presentation was 18.4 years (range, 1-60 years), with a male-to-female ratio of 2.5:1. The most common site was posterior fossa of brain (n = 28, 47%), followed by dorso-lumbar spine (n = 9, 15%). The most common type of tumor was medulloblastoma (n = 29, 48.3%), followed by Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumor (pPNET) (n = 11, 18.3%), non-Hodgkin lymphoma (NHL) (n = 9, 15%), neuroblastoma (n = 3, 5%), and CNS embryonal tumor, NOS (n = 2, 3.3%). One case each of atypical teratoid rhabdoid tumor (ATRT), rhabdomyosarcoma, pineoblastoma, melanoma, rhabdomyosarcoma, and undifferentiated pleomorphic sarcoma was also documented.
CONCLUSIONS: SRCTs have a variable age of presentation. Their incidence in CNS is low as compared to other organ systems. On light microscopy, the histopathology of these lesions is overlapping, posing a great diagnostic dilemma for the pathologist. The use of ancillary techniques like immunohistochemistry helps in arriving at the correct diagnosis. Treatment strategy and tumor prognosis also vary along the entire spectrum of SRCT, thus making exact characterization essential for proper management.
摘要：
背景：小圆细胞肿瘤（SRCT）是一组具有最小分化或无分化的恶性肿瘤，其特征是存在具有高核质比的圆形细胞。虽然SRCT可以发生在身体的任何部位，中枢神经系统（CNS）的受累并不常见。
目的：我们旨在研究在我们研究所诊断的颅骨SRCT的临床病理特征，为期四年（2016-2019）。
方法：对头颅SRCT形态学诊断的病历（2016-2019年）进行回顾性回顾。包括轴内和轴外肿瘤。共检索到60例,并对其临床和组织病理学特征进行了研究。进行特殊的细胞化学染色和免疫组织化学，需要的地方。
结果：演示时的平均年龄为18.4岁（范围，1-60岁),男女比例为2.5:1。最常见的部位是大脑后颅窝（n=28，47%），其次是背腰椎（n=9，15％）。最常见的肿瘤类型是髓母细胞瘤（n=29，48.3％），其次是尤文肉瘤（ES）/外周原始神经外胚层肿瘤（pPNET）（n=11，18.3%），非霍奇金淋巴瘤（NHL）（n=9，15％），神经母细胞瘤（n=3，5%），和中枢神经系统胚胎性肿瘤，NOS(n=2,3.3%)。非典型畸胎瘤样横纹肌样瘤(ATRT)各1例,横纹肌肉瘤,松果体母细胞瘤,黑色素瘤，横纹肌肉瘤,和未分化的多形性肉瘤也被记录。
结论：SRCT具有不同的呈现年龄。与其他器官系统相比，它们在CNS中的发病率较低。在光学显微镜下，这些病变的组织病理学是重叠的，给病理学家带来了巨大的诊断困境。免疫组织化学等辅助技术的使用有助于得出正确的诊断。治疗策略和肿瘤预后也随着SRCT的整个频谱而变化，因此，准确的表征对于适当的管理至关重要。
目的：我们旨在研究在我们研究所诊断的颅骨SRCT的临床病理特征，为期四年（2016-2019）。
方法：对头颅SRCT形态学诊断的病历（2016-2019年）进行回顾性回顾。包括轴内和轴外肿瘤。共检索到60例,并对其临床和组织病理学特征进行了研究。进行特殊的细胞化学染色和免疫组织化学，需要的地方。
结果：演示时的平均年龄为18.4岁（范围，1-60岁),男女比例为2.5:1。最常见的部位是大脑后颅窝（n=28，47%），其次是背腰椎（n=9，15％）。最常见的肿瘤类型是髓母细胞瘤（n=29，48.3％），其次是尤文肉瘤（ES）/外周原始神经外胚层肿瘤（pPNET）（n=11，18.3%），非霍奇金淋巴瘤（NHL）（n=9，15％），神经母细胞瘤（n=3，5%），和中枢神经系统胚胎性肿瘤，NOS(n=2,3.3%)。非典型畸胎瘤样横纹肌样瘤(ATRT)各1例,横纹肌肉瘤,松果体母细胞瘤,黑色素瘤，横纹肌肉瘤,和未分化的多形性肉瘤也被记录。
结论：SRCT具有不同的呈现年龄。与其他器官系统相比，它们在CNS中的发病率较低。在光学显微镜下，这些病变的组织病理学是重叠的，给病理学家带来了巨大的诊断困境。免疫组织化学等辅助技术的使用有助于得出正确的诊断。治疗策略和肿瘤预后也随着SRCT的整个频谱而变化，因此，准确的表征对于适当的管理至关重要。
