Singhal Namit, Agarwal Vinay, Chawla Anu, Tangri Rajiv
Department of Neurosurgery, S S Hospital of Neurosciences, Agra, Uttar Pradesh, India.
Dr. Vinay Agarwal Neurology Clinic, Agra, Uttar Pradesh, India.
J Pediatr Neurosci. 2017 Apr-Jun;12(2):188-191. doi: 10.4103/jpn.JPN_95_16.
Inflammatory myofibroblastic tumor (IMT) is a rare tumor in the central nervous system (CNS), mostly being extracranial. Approximately 100 sporadic cases have been reported in the literature. The rarity of the tumor, its various histopathological characteristics, and its variable aggressive course render it difficult to diagnose and treat. IMT is generally a histological diagnosis which is rarely suspected preoperatively. It mimics other intracranial tumors such as giant cell tumor, hemangiopericytoma, anaplastic meningioma, plasmacytoma, and lymphoma. Rarely, it can present with a clinical picture which mimics a benign infective process, Rosai-Dorfman disease, or an idiopathic hypertrophic pachymeningitis. High index of suspicion is required as total resection of this lesion is mandatory to prevent recurrence. Here, we describe a case of a 10-year-old child which initially presented with clinical features mimicking chronic suppurative otitis media and radiological presentation of a small intracranial abscess. He was initially treated by an ENT surgeon who started him on intravenous antibiotics, but the patient was lost to follow up. He returned after 2 months with a large lesion at the same location. Histological examination revealed multiple spindle cells with plasma cells and lymphocytes scattered among these spindle cells. The spindle cells were immunopositive for smooth muscle actin and negative for epithelial membrane antigen, S100, and CD34.
炎性肌纤维母细胞瘤(IMT)是中枢神经系统(CNS)中的一种罕见肿瘤,大多位于颅外。文献中已报道约100例散发病例。该肿瘤的罕见性、其多样的组织病理学特征以及其可变的侵袭性病程使得其诊断和治疗都很困难。IMT通常是一种组织学诊断,术前很少被怀疑。它可模仿其他颅内肿瘤,如巨细胞瘤、血管外皮细胞瘤、间变性脑膜瘤、浆细胞瘤和淋巴瘤。很少情况下,它可表现出类似良性感染性疾病、罗萨伊-多夫曼病或特发性肥厚性硬脑膜炎的临床表现。由于必须彻底切除该病变以防止复发,因此需要高度怀疑。在此,我们描述一例10岁儿童病例,该患儿最初表现出类似慢性化脓性中耳炎的临床特征以及小颅内脓肿的影像学表现。他最初由一名耳鼻喉科外科医生治疗,该医生给他静脉注射抗生素,但患者失访。2个月后他回来时,同一部位出现了一个大病变。组织学检查显示有多个梭形细胞,浆细胞和淋巴细胞散在于这些梭形细胞之间。梭形细胞平滑肌肌动蛋白免疫阳性,上皮膜抗原S100和CD34免疫阴性。