Chadha Prerna, Durga Garima, Sharma Anila, Pasricha Sunil, Premsagar Ishwar C, Mehta Anurag
Department of Laboratory and Transfusion Services, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, Delhi 110015, India.
Department of Neurosurgical Oncology & Spine Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, Delhi 110015, India.
Oncol Lett. 2024 Aug 9;28(4):488. doi: 10.3892/ol.2024.14621. eCollection 2024 Oct.
Solitary fibrous tumor of the central nervous system (CNS) accounts for <1% of all primary CNS tumors. These tumors are typically dura-based and are graded using a three-tiered system based on cellularity, mitotic activity and necrosis. Extracranial metastasis of this disease is rare. The present study reports 3 cases of this rare tumor, retrieved from the hospital archives over a period of 5 years. The hematoxylin and eosin-stained and immunohistochemistry (IHC) slides were reviewed by two pathologists and clinical details were obtained from the hospital records. All 3 cases had tumors located in the cranial cavity. These cases had an outside diagnosis of meningioma, which on re-evaluation at our center (Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India) were found to be solitary fibrous tumor. Of the 3 cases, 1 developed local recurrence and distant metastasis. All cases showed histomorphological features of a spindle cell tumor with positivity for STAT6 by IHC. The genomic NGFI-A-binding protein 2-STAT6 fusion in solitary fibrous tumor leads toSTAT6 nuclear expression on IHC, which confirms the diagnosis and also differentiates it from its close mimics. This case series highlights that histomorphology and IHC are imperative for a correct and timely diagnosis of these tumors, which are commonly misdiagnosed clinically.
中枢神经系统(CNS)孤立性纤维瘤占所有原发性CNS肿瘤的比例不到1%。这些肿瘤通常起源于硬脑膜,并根据细胞密度、有丝分裂活性和坏死情况采用三级系统进行分级。该疾病的颅外转移罕见。本研究报告了5年间从医院档案中检索出的3例这种罕见肿瘤。两名病理学家对苏木精-伊红染色切片和免疫组织化学(IHC)切片进行了复查,并从医院记录中获取了临床细节。所有3例病例的肿瘤均位于颅腔。这些病例在外院被诊断为脑膜瘤,但在我们中心(印度新德里拉吉夫·甘地癌症研究所和研究中心)重新评估后发现是孤立性纤维瘤。3例病例中,1例出现了局部复发和远处转移。所有病例均显示出梭形细胞瘤的组织形态学特征,免疫组织化学检测STAT6呈阳性。孤立性纤维瘤中的基因组NGFI-A结合蛋白2-STAT6融合导致免疫组织化学检测时STAT6核表达阳性,这既证实了诊断,也将其与相似肿瘤区分开来。该病例系列强调,组织形态学和免疫组织化学对于正确及时诊断这些临床上常被误诊的肿瘤至关重要。