颅内血管母细胞瘤的MRI表现与临床病理对照

目的探讨血管母细胞瘤的MRI影像表现与临床病理特点,提高其诊断准确率。方法回顾性分析18例经手术病理证实的颅内血管母细胞瘤MRI表现和临床病理特点。结果 18例病灶,5例位于右小脑半球,9例位于左小脑半球,3例位于小脑蚓部,1例位于幕上右侧脑室三角区;MRI表现为大囊小结节型9例,囊实型3例,单纯囊型1例,实质型5例。血管母细胞瘤典型MRI表现为大囊小结节,且小结节显著异常强化。大体病理学检查,肿瘤细胞以囊性和实质性两种形态,囊液透明、淡黄色、蛋白含量高,实性部分由丰富的血管和血窦构成;显微镜下见肿瘤由大小不等的血管腔隙和其间的基质细胞构成,在血管内皮细胞核周围细胞区见丰富的网状纤维;临床上该病好发于中青年,男性为主。结论血管母细胞瘤的MRI表现具有一定特征,结合临床较易诊断;对非典型的血管母细胞,则需进行鉴别诊断,MRI增强更有利于对病变的定性诊断。     

脊髓血管母细胞瘤的MRI表现

目的评估脊髓血管母细胞瘤的MRI表现。资料与方法回顾性分析7例经手术病理证实的脊髓血管母细胞瘤的MR／表现。7例均行常规MR扫描,应用T1、T2加权序列作轴面、矢状面扫描,且均应用T1WISE作轴面、矢状面、冠状面增强扫描。结果7例肿瘤在T1WI上呈等信号、低信号或等、低混杂信号,在T2WI上呈高信号或混杂信号,增强后均一或不均一强化。其中5例伴有脊髓空洞,4例可见血管流空效应,2例伴有瘤周水肿。结论脊髓血管母细胞瘤的MRI表现具有特征性。MRI还有利于明确肿瘤的位置和范围,以及外科治疗计划的制定。     

血管母细胞瘤MRI诊断

目的：进一步提高对血管母细胞瘤ＭＲＩ表现的认识。方法：搜集经手术病理证实的血管母细胞瘤４３例,对其ＭＲＩ表现进行回顾性分析。结果：血管母细胞瘤常位于小脑半球及蚓部,其它部位少见。可多发和伴发腹部脏器病变。大囊小结节征是囊型血管母细胞瘤的特征表现,其ＭＲＩ信号在平扫各加权像上均高于脑脊液信号;实质型信号强度在Ｔ２ 加权像上随ＴＥ时间延长而升高,瘤内及周围可见血管流空信号。增强扫描有助于病灶的检出和定性诊断。结论：ＭＲＩ是诊断血管母细胞瘤的有效检查方法,但需与囊型胶质瘤、单纯囊肿、脑膜瘤、ＡＶＭ 等鉴别。     

血管母细胞瘤的MRI诊断

目的:探讨血管母细胞瘤的MRI诊断。方法:回顾性分析经手术病理证实的24例血管母细胞瘤,探讨其MRI表现。结果:23例血管母细胞瘤位于后颅窝,1例位于右顶枕叶。16例表现为大囊小结节型,结节强化明显;8例表现为实质型;20例瘤内或瘤周可见血管流空信号。本组MRI正确诊断22例,准确率为91.7%。结论:MRI是诊断血管母细胞瘤的有效检查方法,但不典型者需与胶质瘤、单纯囊肿、脑膜瘤、动静脉畸形等鉴别。     

颅内不典型血管母细胞瘤的MRI表现

目的:探讨颅内不典型血管母细胞瘤(HB)的MRI特征。方法:回顾性分析经手术及病理证实的21例颅内不典型HB的临床及MRI资料,分析病变的部位、数目、形态、大小、信号特点、瘤周水肿及与周围组织的关系。结果:21例中,单发18例,病灶均位于幕下;多发3例11个病灶,幕下9个,幕上2个。29个病灶中,实性肿块型21个,单纯囊肿型5个,大囊小结节型3个。实性肿块型HB的T_1WI呈等或稍低信号,T_2WI及FLAIR呈等稍高或混杂信号,DWI呈等低或混杂信号;部分瘤内及瘤周见多发异常血管影(9/21);瘤周多为中重度水肿(12/21);11个病灶与脑膜关系密切;增强扫描病灶呈均匀(8/21)或不均匀(13/21)明显强化。5个单纯囊肿型T_1WI呈低信号,T_2WI呈高信号,FLAIR呈低信号,信号均匀,增强扫描囊壁及囊内均未强化。结论:颅内不典型HB具有一定的MRI特征,结合发病部位及年龄,可提高该病术前诊断准确率。     

实质性血管母细胞瘤的MRI诊断

目的探讨中枢神经系统实质性血管母细胞瘤的MRI特点及诊断价值。方法回顾性分析7例经病理证实的实质性血管母细胞瘤的MRI资料。结果 4例肿瘤位于小脑半球,1例位于小脑蚓部,颈髓及胸髓各1例。MRI表现为T1WI呈等、低信号,T2WI呈等、高信号,6例病灶内部和周围见血管流空影,增强后肿瘤呈显著强化;1例CE-MRA示肿瘤周边及内部见不规则血管影;2例髓内肿瘤均继发脊髓空洞或囊肿。结论 MRI对中枢神经系统实质性血管母细胞瘤的诊断及鉴别诊断具有临床价值。     

散发性肾血管母细胞瘤的MRI表现并文献复习

目的 探讨散发性肾血管母细胞瘤的MRI特点。方法 选取我院经手术病理证实的2例肾血管母细胞瘤患者的资料,并复习相关文献进行分析。结果 2例均表现为T1WI等或低信号,T2WI高信号,DWI信号不高;其中1例病灶周围见流空血管,1例增强呈“渐进性强化”。结论 该病的MRI表现,尤其是DWI及强化方式有一定的特征性,对于诊断及鉴别诊断有重要意义。     

血管母细胞瘤的CT、MRI表现

目的:探讨血管母细胞瘤的CT和MRI影像学特征。方法:回顾性分析37例血管母细胞瘤的CT和MRI表现,25例行CT平扫和增强扫描,其中2例行CTA三维重建。14例行MRI检查,其中2例作MRI平扫,12例作MRI平扫加增强,其中1例3D TOF MRA检查。结果:37例共43个病灶。35例单发病灶中,发生于小脑半球者23例,小脑蚓部9例,第四脑室1例,延髓背侧1例,左桥小脑角区1例。其中囊结节型29例,实质型3例,囊实型2例,单纯囊性1例。2例为多发病灶,均位于后颅窝,其中1例有6处病灶,实质型和囊结节型混合存在。CT平扫囊结节型肿瘤壁结节显示率仅为54.6%。增强扫描壁结节及实质型、囊实型肿瘤均明显均匀或不均匀强化;23.1%显示肿瘤周围有增粗迂曲的血管。CTA示肿瘤为富血管的团块。MRI平扫肿瘤壁结节显示率100%。肿瘤周围流空血管显示率达57.9%。与CT比较此两项指标差异有统计学意义。增强扫描示壁结节及实质型、囊实型肿瘤明显均匀或不均匀强化。MRA显示肿瘤区域丰富的紊乱血管。结论:CT、MRI可作为血管母细胞瘤的首选检查方法。     

MRI诊断多发性血管母细胞瘤病一例

ＭＲＩ诊断多发性血管母细胞瘤病一例贾宁阳宋平刚宪桢陶晓峰ＶＨＬ（Ｖｏｎｈｉｐｐｅｌｌｉｎｄａｕ）系多发性血管母细胞瘤病，常伴进行性颅内压增高及一侧小脑功能障碍。现报道１例如下。患者男，２８岁。１５年前视力下降诊断为“视网膜血管瘤”，３年前出现颅内...     

Proton magnetic resonance spectroscopy findings of hemangioblastoma

We report a case of proton magnetic resonance spectroscopy (MRS) of hemangioblastoma in a 56-yearold man with a history of hyperlipidemia who was suffering from an equilibrium disorder. Proton MRS revealed a high mobile lipids (Lip) peak between 0.9 and 1.4 ppm, which was compatible with histologically proven lipids in the tumor. No lactate peak was recognized. The creatine/phosphocreatine peak was low. Choline-containing compounds were increased. The N-acetylaspartate peak was absent, which indicated that the tumor is of nonneurogenic origin. Combined with the absence of the necrotic component on magnetic resonance imaging, this Lip peak on proton MRS could be the characteristic pattern of hemangioblastoma. These unique results of proton MRS can play an important role in the differential diagnosis of intracranial hemangioblastoma. However, further investigations are required to establish the typical characteristics of proton MRS of hemangioblastoma.     

Atypical magnetic resonance imaging findings of craniopharyngioma

Three cases of craniopharyngiomas with atypical MRI findings are reported. The first patient had a nasopharyngeal craniopharyngioma. Its unusual location made diagnosis difficult. The second patient had a massive craniopharyngioma with extensive cystic expansion, involving the anterior, middle and posterior cranial fossae, and extending into the foramen magnum. The tumour of the third patient involved the suprasellar region with a large extension into the third ventricle, and demonstrated a predominantly high signal intensity on all T1-weighted, proton-weighted and T2-weighted images. These patients further stressed the complexity of MRI findings in craniopharyngiomas.     

Hoyeraal-Hreidarsson syndrome: magnetic resonance imaging findings

Hoyeraal-Hreidarsson syndrome (HH) has been defined as a severe variant of dyskeratosis congenita (DKC). We report here a case of a 6-year-old girl with HH who presented with bone marrow hypoplasia, skin pigmentation, nail dystrophy, growth retardation, and bilateral retinal hemorrhage. Brain MRI revealed cerebellar hypoplasia, hypoplasia of the corpus callosum, a small pituitary gland, a small brain stem, and focal long T2 lesions in the thalamus and brain stem. A brain computed tomography scan revealed intracranial calcification as well. To the best of our knowledge, a small pituitary gland and focal long T2 lesions in the thalamus and brain stem have never been reported as a feature of HH.     

Creutzfeldt-Jakob disease: magnetic resonance imaging findings

Rapidly progressive dementia in an adult with findings of bilateral, symmetric high signal intensity on T2-weighted sequences and normal findings on T1-weighted sequences predominantly in the deep grey matter is suggestive of Creutzfeldt-Jakob disease (CJD). The peripheral cortex may be involved, as it was in the present case. The absence of subcortical periventricular white matter high signal intensity suggests that symmetric high signal intensities within the basal ganglia and cortical grey matter are more likely to be due to a degenerative process rather than due to ischaemia, infection or tumour.     

Double-chambered right ventricle: magnetic resonance imaging findings

Primary double-chambered right ventricle is an uncommon congenital anomaly that presents with right ventricular outflow tract obstruction in childhood or adolescence. A search of the literature revealed only a few documented case reports with MRI findings. We present a case with mixed lesions, and with anomalous muscle bundles located both in the middle portion of the right ventricular sinus and at the subinfundibular level.     

Cat scratch disease: magnetic resonance imaging findings

Cat scratch disease is an infectious lymphadenitis frequently occurring in children and adolescents. We present the magnetic resonance imaging findings of two patients with this disease. In both cases, lymphadenopathy was characterized by extensive stranding of the surrounding soft tissues, consistent with the inflammatory nature of this condition. Magnetic resonance imaging can be diagnostic and may obviate the need for invasive means of evaluation in patients suspected of having cat scratch disease.     

Nonketotic hyperglycinemia: diffusion magnetic resonance imaging findings

We report about a boy with nonketotic hyperglycinemia who was studied at 15 days of life with a follow-up examination at age 6 months. Magnetic resonance (MR) imaging revealed progressive atrophy, callosal thinning, and delayed myelination. Glycine peaks were shown by proton MR spectroscopy at 3.56 ppm with a long echo time (TE, 135 milliseconds; TR, 1500 milliseconds). Echo-planar diffusion MR imaging (TR, 5700 milliseconds; TE, 139 milliseconds) at 15 days of life revealed high-signal lesions in the pyramidal tracts, middle cerebellar pedicles, and dentate nuclei on b = 1000 s/mm2 images associated with low apparent diffusion coefficient (ADC) values. By age 6 months, the lesions became more prominent on b = 1000 s/mm2 images with lower ADC values. Diffusion MR imaging findings likely reflected the histopathologic changes of the disease which consisted of spongiosis of the myelinated brain tissue due to myelin vacuolation.     

Canavan disease: diffusion magnetic resonance imaging findings

A 15-month-old boy with Canavan disease is reported in whom a restricted diffusion pattern on diffusion magnetic resonance imaging (MRI) (high signal on b = 1,000 mm2/s images and low apparent diffusion coefficient [ADC] values) was evident in the affected regions of the brain, including the peripheral white matter, globi pallidi, thalami, brainstem, dorsal pons, and dentate nuclei. The ADC values at these regions ranged from 0.42 to 0.56 x 10(-3) mm2/s compared with the normal ADC values from the uninvolved deep frontal white matter (0.68-0.92 x 10(-3) mm2/s). The known histopathologic features in Canavan disease include edematous and gelatinous brain tissue associated with diffuse vacuolization. Considering these and the diffusion MRI findings in this patient, it is likely that existence of a gel (gelatinous) state rather than the usual sol state of water molecules in the affected brain regions accounted for the restricted diffusion pattern in Canavan disease.