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Metaplastic Meningioma With Extensive Cartilaginous Transformation : A Diagnostic Dilemma

机译:广泛性软骨转化的化生性脑膜瘤:诊断难题

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Meningiomas are the most common extraaxial central nervous system tumours often discovered in middle to late adult life and especially in women. About 85-90% of meningiomas are benign, 5-10 % are intermediate grade, and 3-5% are malignant. Metaplastic meningioma is a rare subtype of WHO grade I meningioma histologically characterized by the presence of mesenchymal components. The presence of pure and extensive cartilaginous differentiation in meningiomas is extremely rare and remains a diagnostic dilemma. We report a case of this entity in a 52 year old woman and discuss the pathogenesis, the imaging features and the histopathological data. INTRODUCTION Meningiomas are the most common extraaxial central nervous system tumours often discovered in middle to late adult life and especially in women and account approximately for 15-30 % of all intracranial tumours (Uygur Er, Do?a Gürkanlar , Atilla Kazanci et al., 2006; Markus et al., 2006). They are usually benign in about 85-90%, atypical in 5-10 % and malignant in 3-5% with characteristic pathologic and imaging features (Black et al 2007). Most patients in whom the diagnosis is suggestive of meningiomas undergo a surgical resection to relieve neurological symptoms, and the diagnosis sometimes remains difficult despite appropriate histological studies. Metaplastic meningioma is a rare subtype of WHO grade I meningioma histologically characterized by the presence of mesenchymal components, including osseous, lipomatous, myxoid or xanthomatous and cartilaginous tissue. The presence of extensive cartilaginous differentiation in this entity of meningiomas is very uncommon. We discuss herein the diagnostic dilemma in this kind of meningioma. CASE REPORT A 52 year old woman, followed for diabetes type II on nutritional regime, had been admitted in our department for a 10 year history of isolated headaches for which she opted for self medication by analgesics. Due to persistence of the headaches ,despite analgesic therapy, she sought medical help in our department to ascertain the probable underlying cause. On admission, neurological examination was normal (no deficit and no papilledema on ophthalmological exam). A brain CT scan was done , which revealed a left parietal spontaneously hyperdense and heterogeneous lesion with no significant modification following contrast injection and suggestive of dural and bone infiltrations(Fig.1). Cerebral MRI to refine the radiologic diagnosis complemented these data. Axial, sagittal and coronal contrast enhanced T1W MRI showed the same heterogeneous lesion not much modified by Gadolinium injection with probable dural and bone thickening and without brain edema or midline shift (Fig.2).Our initial diagnosis basing on the radiological appearances was parietal convexity meningioma or intracranial tuberculoma. She underwent a left craniotomy centred on the lesion that permitted an “en bloc” resection of this tumour together with the overlying dura (Simpson’s grade I). Intraoperatively, the lesion was yellowish, firm with a very good plain of cleavage, and a very loose dural attachment, leaving a cavity that was in conformity to the shape of the lesion and measuring 4.5 cm × 2.5 cm × 1.5 cm (Fig.3). Extemporaneous histopathological examination was in favour of a cartilaginous tumour. These histological data were accepted with difficulty by our team and we recommended extensive studies in order to properly confirm the diagnosis. Finally, the diagnosis of metaplastic meningioma with extensive cartilaginous transformation was made after HES staining (Fig.4). Postoperative CT and MRI scans revealed no residual tumour (Fig.5). She was discharged from hospital without neurological signs 08 days after surgery and is symptom-free 12 months following surgery.
机译:脑膜瘤是最常见的中轴外神经系统肿瘤,通常在成年中后期到晚期发现,特别是在女性中。脑膜瘤约85-90%为良性,中度为5-10%,恶性为3-5%。化生性脑膜瘤是一种以组织学特征为间质成分的世卫组织I级脑膜瘤的罕见亚型。脑膜瘤中纯净和广泛的软骨分化非常罕见,仍然是诊断上的难题。我们报告了一个在52岁女性中此实体的病例,并讨论了其发病机理,影像学特征和组织病理学数据。引言脑膜瘤是最常见的中轴神经系统肿瘤,通常在成年中晚期发现,尤其是在女性中,约占所有颅内肿瘤的15%至30%(维吾尔族,多阿古尔坎拉尔族,阿蒂利亚·卡赞奇等人, 2006; Markus等人,2006)。它们通常具有约85-90%的良性,非典型性的占5-10%,恶性的占3-5%,具有典型的病理和影像学特征(Black等,2007)。大多数诊断提示脑膜瘤的患者都经过手术切除以缓解神经系统症状,尽管进行了适当的组织学研究,有时诊断仍然很困难。组织增生性脑膜瘤是一种罕见的WHO I型脑膜瘤亚型,其组织学特征是存在间质成分,包括骨组织,脂质体,粘液样或黄瘤和软骨组织。在这种脑膜瘤实体中广泛的软骨分化非常罕见。我们在这里讨论这种脑膜瘤的诊断难题。病例报告一名52岁的妇女因营养状况接受了II型糖尿病的治疗,因其孤立性头痛已有10年的病史,因此选择在我科接受止痛药的自我治疗。由于头痛的持续存在,尽管进行了止痛治疗,她仍在我科寻求医疗帮助,以确定可能的潜在原因。入院时神经系统检查正常(眼科检查无缺陷,无视乳头水肿)。进行了一次脑部CT扫描,发现左壁自发性高密度和异质性病变,对比剂注射后无明显改变,提示有硬脑膜和骨浸润(图1)。脑MRI完善放射学诊断补充了这些数据。轴向,矢状位和冠状位对比增强的T1W MRI显示,相同的异质性病变经injection注射并没有多大程度的改变,可能出现硬脑膜和骨骼增厚,并且没有脑水肿或中线移位(图2)。脑膜瘤或颅内结核瘤。她进行了以病变为中心的左颅骨切开术,允许对该肿瘤连同其上覆硬脑膜进行“整体”切除(辛普森I级)。术中病变为淡黄色,坚硬,具有良好的乳沟平原,并且硬脑膜附着非常疏松,留下了与病变形状相符的腔,尺寸为4.5 cm×2.5 cm×1.5 cm(图3) )。临时的组织病理学检查有利于软骨肿瘤。我们的团队难以接受这些组织学数据,我们建议进行大量研究以正确确诊。最后,HES染色后诊断为具有广泛的软骨转化的化生性脑膜瘤(图4)。术后CT和MRI扫描未发现残留肿瘤(图5)。她在手术后08天出院,没有神经系统症状,并且在手术后12个月无症状。

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