Hepatic angiomyolipoma with a giant hemangioma

Hepatic angiomyolipoma is a rare hepatic mesenchymal tumor. We report a case of hepatic angiomyolipoma that was successfully resected along with a giant hemangioma. A 53-year-old Japanese woman was admitted to our hospital for further evaluation of a liver tumor in segment 4. The tumor was detected on positron emission tomography during a health check-up. Abdominal ultrasonography revealed a well-defined mass of mixed echogenicity, 1.5 cm in diameter, in segment 4, and a giant hemangioma of mixed echogenicity, 7 cm in diameter, in segment 7. On enhanced computed tomography, the tumor in segment 4 showed hyperattenuation in the early phase and hypoattenuation in the delayed phase. On magnetic resonance imaging, the tumor in segment 4 showed hypointensity on T1-weighted images, hyperintensity on T2-weighted images, and hyperintensity on diffusion-weighted images. On angiography, the tumor in segment 4 appeared as a circumscribed hypervascular mass in the early phase and a slightly hypovascular mass in the delayed phase. The imaging findings suggested a primary hepatocellular carcinoma. The patient consented to resection of the tumor in segment 4 along with the giant hemangioma in segment 7. These tumors were resected with tumor-free surgical margins by partial resection of segments 4 and 7 of the liver. The cut surface of the resected specimen of segment 4 showed a yellowish tumor consisting of mature adipose tissue. The histopathological diagnoses of the resected specimens were angiomyolipoma in segment 4 and cavernous hemangioma in segment 7. The tumor in segment 4 consisted of mature lipocytes with angiomatous and small lymphocytic components, but no mitotic figures. The tumor showed immunoreactivity to smooth muscle antigen and homatropine methylbromide 45 and no immunoreactivity to AE/E3. The postoperative course was uneventful, and the patient remains well 1 year after the operation.     

血管中心性胶质瘤临床病理学特征及相关文献复习

目的探讨血管中心性胶质瘤(angiocentric glioma,AG)的临床病理学特征、诊断及鉴别诊断。方法收集首都医科大学宣武医院病理科诊断的12例AG,回顾性分析患者的临床资料、病理学特征及预后,并复习相关文献。结果12例患者均有难治性癫痫病史,平均发病年龄9岁(2.8~24岁)。头颅核磁共振(MRI)表现为T1WI低信号,T2WI/FLAIR高信号,未见明显强化。组织学特点为均一的双极细胞,沿血管轴排列形成血管周围假菊形团。AG中GFAP、vimentin均阳性,肿瘤细胞中EMA、D2-40呈胞质弥漫阳性或核旁点灶阳性,EMA和D2-40的阳性率分别为91.7%(11/12)和100%(12/12);NeuN、IDH1 R132H、L1CAM、BRAF V600E和H3K27M均阴性。Ki-67的增殖指数为1%~5%。术后影像学检查示患者肿瘤均完整切除。随访14~110个月,患者均无癫痫发作及肿瘤复发。结论AG是以癫痫发病、生长缓慢的肿瘤,手术切除效果良好。     

Peritumoral brain edema in angiomatous supratentorial meningiomas: an investigation of the vascular endothelial growth factor A pathway

The aim of this work was to study the vascular endothelial growth factor A (VEGF‐A) pathway and peritumoral brain edema (PTBE) through comparison of non‐angiomatous and angiomatous meningiomas. Meningiomas are common intracranial tumors, which often have PTBE. VEGF‐A is an integral part of PTBE formation and angiogenesis, and the capillary‐rich angiomatous meningiomas are known for their PTBE. The VEGF‐A receptor VEGFR‐2 is responsible for the angiogenic effect of VEGF‐A on endothelial cells, which is enhanced by the co‐receptor neuropilin‐1. Forty non‐angiomatous, 22 angiomatous meningiomas, and 10 control tissue samples were collected for the study. Magnetic resonance images were available for 40 non‐angiomatous and 10 angiomatous meningiomas. Tissue sections were immunostained for CD34, MIB‐1, estrogen‐ and progesterone receptors. ELISA, chemiluminescence, and RT‐qPCR were used for VEGF‐A, VEGFR‐2, and neuropilin‐1 protein and mRNA quantification. Angiomatous meningiomas had larger PTBE (695 vs 218 cm³, p = 0.0045) and longer capillary length (3614 vs 605 mm/mm³, p < 0.0001). VEGF‐A mRNA, neuropilin‐1 mRNA, and VEGFR‐2 protein levels were higher in angiomatous meningiomas independently of the capillary length (p < 0.05). Neuropilin‐1 protein levels were lower in angiomatous meningiomas (p < 0.0001). The VEGF‐A pathway and tumor capillary length may be essential for PTBE‐formation in meningiomas. Further investigations of this pathway could lead to earlier therapy and targeted pharmacological treatment options.     

Secretory meningiomas: clinical,radiological and pathological findings in 70 consecutive cases at one institution

Secretory meningioma (SM) is a rare, benign subtype of meningioma. Between January 2005 and December 2010, 70 SMs were operated on at the Department of Neurosurgery, Huashan Hospital, Fudan University. We retrospectively analyzed the clinical data, radiological and immunohistochemical findings, and patient outcome to discuss the specific features of SMs. Cranial base preference, hyper-signal in T2 weighted MR image, “xenon light” gadolinium-diethylenetriamine pentaacetic acid (Gd-DTPA) enhancement were frequently observed in the 70 cases. Non-skull base SMs, which received more complete resection (p<0.01) and had better short-term and long-term outcome, were observed with more severe peritumoral brain edema (PTBE) (p<0.001). In follow-up, only 1 cranial base SM case showed tumor progression. 3 cases died after operation, all with cranial base SMs. As for the 10 cases given Simpson grade 3 or 4 resection who were available at follow-up, 3 died, 5 received gamma-knife therapy, and the other 2 cases received no treatment at all. Only one of the 2 residual SMs without postoperative radiation presented minor progression at a median of 48 months follow-up. In conclusion, cranial base preference, hyper-signal T2 weighted MR image and “xenon light” GD-DTPA enhancement are specific for SMs. Prognosis of SMs is related with operation completeness and surgical risks, rather than the extent of PTBE. Residual SM grows slowly and reacts well to gamma-knife therapy.     

Sequential changes of traumatic vertebral compression fracture on MR imaging.

The purpose of this study was to evaluate the sequential signal intensity changes in post-traumatic vertebral compression fractures of varying ages. Sixty-six patients with 115 post-traumatic vertebral compression fractures underwent MR imaging. The ages of fractures at the time of MR images ranged from 1 day to 6 years. Sequential follow-up MR imagings were obtained in 4 patients for 2 years after initial MR examination. The fracture sites in all 52 fractures with traumatic events less than 3 months prior were hypointense on T1-weighted images and hyperintense on T2-weighted images (type I). A type I fracture could be subdivided into 3 patterns depending on its morphologic appearance: diffuse (type Ia); patchy (type Ib); and bandlike (type Ic). In 12 fractures of 3 to 5 months after trauma, six showed focal hypointensity (type II) in all pulse sequences, and six showed isointensity (type IV). Four of 51 fractures with trauma over 5 months showed focal hyperintensity on T1-weighted images and isointensity on T2-weighted images (type III); and the remaining 47 fractures showed isointensity on all sequences (type IV). In conclusion, MR imaging is useful in predicting the age of known traumatic compression fractures, so familiarity with these sequential MR findings would be helpful in distinguishing benign from malignant fractures.     

血管中心型胶质瘤的研究进展

血管中心型胶质瘤是一种近年来新认识到的致癫痫的脑胶质瘤,目前全世界已报道的病例很少。该肿瘤在临床上主要表现为药物难治性癫痫,组织学特点为形态单一的双极肿瘤细胞以各级血管为中心排列。在影像学上该肿瘤具有T1像皮质呈弥漫性或花边样（rimlike）高密度,T2像呈蒂状（stalklike）延伸至脑室等显著特征。2007年,WHO将其纳入中枢神经系统肿瘤新的分类系统中。     

Miniaturized Handheld Confocal Microscopy Identifies Focal Brain Invasion in a Mouse Model of Aggressive Meningioma

Invasion of the brain parenchyma by a meningioma classified by histological criteria as World Health Organization (WHO) grade I meningioma, implies that the tumor has greater likelihood of recurrence and a biological behavior similar to the more aggressive WHO grade II meningiomas. It is therefore important to detect microscopic foci of brain invasion during surgery in order to maximize the resection and/or adapt imaging follow‐up. In this study, we tested the sensitivity of two handheld confocal imaging devices to detect foci of brain invasion in two types of meningioma mouse models: in a genetically engineered mouse model and in a syngeneic xenograft model. Confocal imaging offered precise images of meningothelial and fibroblastic mouse meningiomas as well as malignant meningiomas, which corresponded exactly to the pathological findings. Imaging showed a sharp definition of the brain‐tumor interface and enabled identification of embedded nerves and vessels. Importantly, in both mouse models used in this study, extension of tumor along Virchow–Robin spaces into adjacent brain was detected by imaging. In conclusion, this novel technique, following validation in clinical trials, may open new possibilities for use in operating rooms to influence both decision making during the surgery and planning for additional treatments.     

Diffusion weighted MR imaging in acute vertebral compression fractures: differentiation between malignant and benign causes

Aim

The primary objective of this study was to evaluate the specificity and sensitivity of diffusion weighted MR imaging (DWI) in the differentiation and characterisation between benign and malignant vertebral compression fractures compared with conventional T1 WI, T2 WI and fat suppressed contrast enhanced T1 WI in the Malaysian population.

Materials and Methods

Thirty five patients with 68 vertebral compression fractures were imaged using the conventional T1 WI, T2 WI, fat suppressed contrast enhanced T1-weighted, and steady state free precession diffusion-weighted (SSFP DWI) sequences on a 1.5 T MR scanner. Signal intensities were analysed qualitatively for all the sequences by comparison to adjacent normal marrow. A quantitative assessment of the signal intensity in the SSFP DWI was also performed.

Results

T1 WI and T2 WI images are of limited diagnostic value because of the variability in signal intensities. Contrast enhanced images had sensitivity and specificity of 93% and 71%, respectively with a negative predictive value (NPV) of 93%. On diffusion-weighted MR imaging, sensitivity was 87% with specificity of 92%. The positive predicative value (PPV) and NPV were both 90%. The quantitative assessment of ratio revealed a statistical significant difference between the benign (0.96) and the malignant (1.73) group of lesion (Mann-Whitney U-test, p=0.0001).

Conclusions

We found that absence of contrast enhancement has a high NPV (90%) while SSFP DWI has both a high PPV (90%) and high NPV (90%) in detecting malignant vertebral compression fractures. Furthermore, in our study the ratio of lesion intensity technique offers an excellent criterion to differentiate between the benign and malignant lesions, and the presence of iso- or hypointensity of the collapsed vertebral bodies is suggestive of a benign lesion while hyperintensity is highly suggestive of malignancy. We also found that using the NLMR showed a statistical significant difference between the malignant and benign groups (p<0.0001) with osteoporotic and malignant lesions have mean values of 0.96 (SD 0.25) and 1.73 (SD 0.4) respectively.     

大鼠种植性CBRH-7919肝癌模型的MRI、数字减影血管造影及病理学表现

背景：大鼠种植性CBRH-7919肝癌模型是肝癌实验性治疗和影像学诊断研究重要的工具,但模型建立后如何对模型进行检测和验证鲜有报道。 目的：通过彩色超声、MRI及肝动脉血管数字减影血管造影检查的方法来检测和验证Wistar大鼠种植性CBRH-7919肝癌模型,并探讨大鼠种植性CBRH-7919肝癌模型的影像学及病理表现。 方法：CBRH-7919大鼠肝癌细胞瘤株接种裸鼠双侧肩胛部皮下,再将生成的肿瘤组织在开腹直视下移植接种于成年Wistar大鼠肝脏内,建立大鼠种植性CBRH-7919肝癌模型。模型建立4周后用彩超检查、筛选建模成功的Wistar大鼠进行MRI检查、肝动脉血管数字减影血管造影及病理学检查。 结果与结论：在建模成功的35只大鼠中,超声检查发现37个结节,MRI检查发现肝脏内共41个结节,较超声增加4个;最小的结节约3 mm,最大约8 mm。MRI扫描显示T1WI表现为低信号,T2WI及脂肪抑制序列表现高信号。肝动脉血管数字减影血管造影均表现由肝动脉供血的富血供结节。光学显微镜下观察可见肿瘤细胞呈巢状或条块状排列,周围伴行多条血管,肿瘤细胞体积大小不一致,核大而浓染,细胞质少,异型性明显。结果表明彩超和核磁共振扫描是大鼠肝癌检查简单而有效的方法,肝动脉血管数字减影血管造影可以了解肿块的供血动脉及其血供情况。通过对Wistar大鼠种植性肝癌模型的MRI及肝动脉血管数字减影血管造影表现能反映肿瘤的病理形态学和生物学行为改变。     

Clonal Origin of Recurrent Meningiomas

Meningiomas are common intracranial and intraspinal tumors. They are treated primarily by surgical resection. Meningioma recurrence following surgery is frequent despite advances in microneurosurgery. However, it is not clear whether recurrent meningiomas, close or distant to the primary resection site, arise from incomplete resection, dissemination of tumor fragments or from independent tumor growth. In order to address the question of clonality in recurring meningiomas, we examined a series of five patients with a total of 14 tumors for X-chromosome inactivation in the tumor tissues. Four patients with a total of 11 meningiomas were informative for polymorphisms either in the PGK or the AR genes. All recurrent meningiomas were found to be clonal with respect to the primary lesions. This finding suggests a common molecular pathogenesis of primary meningioma and subsequent recurrences (p<0.01). In a sixth patient, we analyzed the NF2 gene for mutations in the primary and 5 recurrent meningiomas. All six lesions carried the identical NF2 mutation, strongly indicating a common origin for these tumors. We conclude that recurrent meningiomas usually arise from dissemination of tumor fragments, most likely at the time of the first surgical resection. Our data should alert to the potential of meningioma cells for seeding during surgical procedures.     

微囊型脑膜瘤与脑膜尤文肉瘤/外周原始神经外胚层肿瘤的MRI鉴别诊断

目的:探讨微囊型脑膜瘤与脑膜尤文肉瘤/外周原始神经外胚层肿瘤（pPNET）的MRI征象。方法:回顾性分析经病理证实的14例微囊型脑膜瘤和10例脑膜尤文肉瘤/pPNET患者的临床、影像学及病理资料,结合相关文献分析其MRI差异。结果:微囊型脑膜和脑膜尤文肉瘤/pPNET患者MRI特征在肿瘤形态、T2信号、强化特征、骨侵犯及远处转移方面有统计学意义（P<0.05）;而在T1信号、硬膜尾征和瘤周水肿无统计学意义（P>0.05）。结论:微囊型脑膜瘤和脑膜尤文肉瘤/pPNET影像表现有差异,术前精准鉴别诊断有助于改善临床决策和预测预后。     

NF2 mutations in secretory and other rare variants of meningiomas

The WHO classification defines different histological variants of meningiomas. Mutations of the tumor suppressor gene NF2 on 22q have been described in 30% to 60 % of sporadic meningiomas. However, the vast majority of the meningiomas that have been subject to NF2 analysis belong to the most frequent variants like transitional, fibroblastic and meningothelial meningiomas. Within these subtypes, transitional and fibroblastic meningiomas harbor significantly more NF2 mutations than meningothelial meningiomas, indicating molecular subsets of these tumors. To determine whether rare meningioma variants carry NF2 mutations we analyzed 80 tumors. NF2 mutations were detected in 5 (36%) of 14 psammomatous, 1 (11%) of 9 angiomatous, 2 (22%) of 9 clear cell, 1 (33%) of 3 chordoid and 1 (100%) of 1 papillary meningioma. In the single papillary meningioma, 2 different NF2 mutations were observed. No NF2 mutations were found in 33 secretory, 7 microcystic, 2 lymphoplasmacyte-rich, one rhabdoid and one metaplastic meningioma. In the control group of 25 fibroblastic meningiomas, 11 cases were identified to carry an NF2 mutation.These results support the concept of different molecular subgroups of meningiomas which overlap with histological variants.     

Angiomatous meningioma: a diagnostic dilemma

Angiomatous meningioma accounts for 2.1% of all meningiomas. It has features of a typical benign meningioma with many small or large vascular channels which may predominate over its meningothelial elements. We present here a series of three cases of angiomatous meningioma, which posed diagnostic difficulty to clinicians, radiologists, and pathologists. All the three cases showed a tumor entirely composed of thin-walled vascular channels and cells with bland morphology in the background. The diagnosis was confirmed by immunohistochemistry. We present series of three cases to highlight the histomorphological features of this uncommon variant of meningioma that could help in distinguishing it from hemangioblastoma and hemangiopericytoma.     

MIB-1免疫组化法分析脑膜瘤MRI影像

目的将MIB-l染色指数作为评价脑膜瘤恶性度指标,分析MRI图像特点,建立肿瘤影像与病理性质相关模式.方法统计年龄、性别、肿瘤边缘规则度、静脉窦被侵袭性、肿瘤囊性变、肿瘤体积、肿瘤周围水肿的程度.MIB-l免疫组化染色图像用Graphic、Photoshop4.0、Photographic 4.0及NIH 1.6解析得到相应的MIB指数(Labeling In-dices).结果男性患者、非颅底脑膜瘤、囊性脑膜瘤、显著水肿的脑膜瘤、边缘粗糙的脑膜瘤、侵入静脉窦的脑膜瘤具有较高的增殖能力.T1加权相的低信号和T2加权相的高信号,组织病理高肿瘤细胞密度和内皮型脑膜瘤具有较高的增殖潜能.结论MRI的影像特征是预测脑膜瘤的病理组织类型、增殖潜能和判定患者预后非常好的指标.     

颅外原发下颌骨脑膜瘤临床诊治一例报道并文献复习

目的 探讨颅外原发下颌骨脑膜瘤的临床病理和影像学特征,以及诊断和治疗方法。方法 回顾性分析2017年10月无锡市第二人民医院收治的1例57岁女性颅外原发下颌骨脑膜瘤患者的临床资料。在PubMed、中国知网、万方数据及维普数据库中,以“下颌骨(mandibular)”和“原发性脑膜瘤(primary meningioma)”为关键词,检索截至2017年10 月有关下颌骨原发性脑膜瘤的相关文献,共纳入7篇8例颅外原发下颌骨脑膜瘤的报道,均为英文文献;结合本例诊治过程,总结该病临床及影像学表现、组织学形态、免疫组织化学特征、治疗方法及临床预后特点。结果 本文1例中年女性下颌骨无痛性膨胀性病变,CT为境界清楚的透射影,组织学梭形肿瘤细胞伴有局部旋涡状结构,免疫组织化学瘤细胞上皮膜抗原(EMA)、波形蛋白(Vimentin)支持脑膜瘤的诊断;手术完整切除下颌骨肿瘤,术后随访9个月无复发。结合文献报道的8例共9例颅外原发下颌骨脑膜瘤,男2例、女7例,年龄20~74岁;多以下颌骨局部膨胀为主要临床表现;影像学为下颌骨境界清楚的溶骨性病变,多为透射影,也可透射和非透射混合影像;组织学同颅内脑膜瘤,为纤细梭形瘤细胞束状或旋涡状排列,细胞间丰富胶原,部分细胞多边形上皮样,胞界不清,片状分布,可见砂粒体,细胞无异形;免疫组织化学瘤细胞EMA、Vimentin均阳性,孕激素受体、广谱细胞角质蛋白、肌动蛋白结合蛋白、平滑肌肌动蛋白、结蛋白、S-100蛋白、β-微管蛋白-3、CD34、B淋巴细胞瘤-2和尤因肉瘤标记物均阴性,Ki-67增殖指数1%。治疗以肿块切除及下颌骨切除自体骨片移植为主要手术方式。组织学良性者预后好,手术切除可治愈。结论 颅外原发下颌骨脑膜瘤非常罕见,需结合临床及影像学、病理组织学进行鉴别诊断和诊断。手术完整切除肿块及下颌骨重建可治愈。     

Aspiration biopsy of nodular sarcoidosis of the muscle

The nodular form of muscular sarcoidosis is rare and the condition is often confused with a soft tissue neoplasm. We present a case of nodular muscular sarcoidosis of the right calf in a 52-yr-old woman which was diagnosed by thin-needle aspiration biopsy (TNAB). In this patient, a calf mass was the initial manifestation of sarcoidosis. Contrast-enhanced computed tomographic scans of the calf showed a mass with peripheral ring-like enhancement. T2-weighted magnetic resonance images demonstrated a mass with central hypointensity and marked peripheral hyperintensity. TNAB revealed noncaseating epithelioid cell granulomas in association with multinucleated giant cells. Combined with the radiologic findings, TNAB is considered a useful modality for the diagnosis of nodular muscular sarcoidosis.     

Vascular endothelial growth factor A protein level and gene expression in intracranial meningiomas with brain edema

Meningiomas are the second most common primary intracranial tumors in adults. Although meningiomas are mostly benign, more than 50% of patients with meningioma develop peritumoral brain edema (PTBE), which may be fatal because of increased intracranial pressure. Vascular endothelial growth factor (VEGF) is an endothelial cell-specific mitogen and angiogen. VEGF-A protein, which is identical to vascular permeability factor, is a regulator of angiogenesis. In this study, 101 patients with meningiomas, and possible co-factors to PTBE, such as meningioma subtypes and tumor location, were examined. Forty-three patients had primary, solitary, supratentorial meningiomas with PTBE. In these, correlations in PTBE, edema index, VEGF-A protein, VEGF gene expression, capillary length, and tumor water content were investigated. DNA-branched hybridization was used for measuring VEGF gene expression in tissue homogenates prepared from frozen tissue samples. The method for VEGF-A analysis resembled an ELISA assay, but was based on chemiluminescence. The edema index was positively correlated to VEGF-A protein (p = 0.014) and VEGF gene expression (p < 0.05). The capillary length in the meningiomas was positively correlated to the PTBE (p = 0.038). If VEGF is responsible for the formation of PTBE, the edema may be treated with the anti-VEGF drug Bevacizumab (Avastin), which has been shown to reduce PTBE in patients with glioblastoma multiforme.     

原发性脑淋巴瘤的MRI表现特点与诊断

目的：分析颅内原发性淋巴瘤的MRI表现特点，为临床诊断与治疗提供资料。方法：回顾分析28例经病理证实颅内原发性淋巴瘤的MRI资料，所有病例均行平扫及增强扫描。结果：病灶单发6例，多发22例，分布于幕上14例，幕下4例，幕上幕下同时存在10例，大多位于深部脑白质，病灶多呈圆形或不规则形，T1WI呈低信号，T2WI呈等或高信号，瘤周水肿较轻，增强扫描病灶均呈团块状显著强化。结论：颅内淋巴瘤的MRI表现缺乏特异性，需手术或活检才可作出定性诊断。     

Intraoperative radiation therapy as an adjunctive therapy for huge and highly vascular parasagittal meningiomas

This case presents a 34-year-old man who had a huge parasagittal meningioma. Initial treatment consisted of preoperative external carotid artery embolization and partial tumor resection. During the resection, we found that the tumor invaded the adjacent calvarium, and due to massive hemorrhage, total removal of the tumor was impossible. The patient was treated with intraoperative radiation therapy (IORT) (25 Gy via 16 MeV) as an adjunctive therapy. Eight months after IORT, we were able to remove the tumor completely without surgical difficulties. IORT can be considered an useful adjunctive therapy for the superficially located, huge, and highly vascular meningioma.     

Rhabdoides Meningeom

Of the numerous morphological variants of meningiomas only few, and among these the rhabdoid meningioma, have prognostic importance. Rhabdoid meningiomas were described for the first time in 1998 as an unusual variant with increased proliferative activity. In 2000 they have been included in the revised WHO classification of tumours of the CNS as a subtype of meningiomas with increased risk of recurrence and more aggressive growth, corresponding to WHO grade III. We report the case of a rhabdoid meningioma in a 21-year-old woman presenting as a intracerebral tumour mimicking an oligodendroglioma. The tumour showed features of a meningioma and a rhabdoid morphology with angiomatous components and was considered to be a rhabdoid meningioma. After surgery a small residual tumour remained. The patient received postoperative radiotherapy resulting in regression of the residual tumour in control examinations after 4 and 8 months. Using the presented case we discuss the differential diagnosis and prognostic significance of recognition of a rhabdoid meningioma.