Multicentric astrocytomas of the optic chiasm, brain stem and spinal cord

Autopsy was performed on a 52-year-old man with a 20-year history of neurological symptoms. At autopsy, both a brain stem tumor and a spinal cord tumor were found. These showed the features of pilocytic astrocytoma histologically. A pilocytic astrocytoma was also found in the optic chiasm upon microscopical examination. These three tumors were thought to be multicentric astrocytomas, because there was no continuity among them and no evidence of dissemination or metastasis by any pathway. From a review of the literature, the present case is considered to be an exceedingly rare one because of the multicentric sites of occurrence.     

Multicentric Astrocytomas of the Optic Chiasm, Brain Stem and Spinal Cord

Autopsy was performed on a 52 year old man with a 20-year history of neurological symptoms. At autopsy, both a brain stem tumor and a spinal cord tumor were found. These showed the features of pilocytic astrocytoma histologically. A pilocytic astrocytoma was also found in the optic chiasm upon microscopical examination. These three tumors were thought to be multicentric astrocytomas, because there was no continuity among them and no evidence of dissemination or metastasis by any pathway. From a review of the literature, the present case is considered to be an exceedingly rare one because of the multicentric sites of occurrence. Acta Pathol Jpn 39: 664 669, 1989.     

Tanycytic ependymoma: two case reports and review of the literature

Two cases of tanycytic ependymoma are reported, one in a woman aged 33 years and one in a man aged 36 years. The woman presented with lesions at L2 and L3 in the spinal cord and clinical symptoms suggested spinal cord compression, such as movement and sensory dysfunction. The man had a lesion located in the lateral ventricle and presented with headache, vomiting, and impaired vision. Both of these patients developed gradual increases in the intensity of their symptoms over a few months prior to admission. A spinal cord MRI of the woman showed a well-defined, solid cystic mass measuring 1.0 × 6.5 cm at L2 and L3 of the spinal cord. The mass showed low signal intensity on T2-weighted images in the intervertebral disc with equal signal intensity T1-/T2-weighted images. The signal was uniform and enhanced scanning showed a light strengthening of signal. A brain MRI of the man showed a well-defined mass measuring 4 cm in diameter invading the right ventricle. The mass showed low signal intensity on right T1-weighted images and high signal intensity on T2-weighted images. Histopathologically, the tumors were rich with significant and uniform proliferation of long spindle cells, which were arranged in fasciculate and knitted patterns with bipolar and spindled processes focally forming perivascular pseudorosettes. The cell nuclei showed minor pleomorphism. A few tumor cells centered on the vessels resulted in a nuclear-free zone, which was characteristic of ependymoma. Immunohistochemically, the tumor cells were positive for GFAP, EMA, and CD99, but negative for S-100. A review of the cases reported in the literature shows that tanycytic ependymoma occurs more often in the spinal cord. As it can resemble pilocytic astrocytoma and schwannoma, tanycytic ependymoma should be differentially diagnosed to exclude some of the benign spindle cell tumors of the central nervous system.     

An unusual and misleading form of pilocytic astrocytoma

Pilocytic astrocytoma is histologically characterized by a biphasic pattern. We report a myxoid form of frontocallosal pilocytic astrocytoma in a 13-year-old girl. MRI showed a relatively well-defined tumor with necrosis and ring-like zone of contrast enhancement. Histological examination showed a monophasic tumor composed of piloid cells on a myxoid background corresponding to a pilomyxoid astrocytoma. This unusual form of pilocytic astrocytoma can be mistaken for a high grade infiltrating glioma. Pilomyxoid astrocytoma is more aggressive than classic pilocytic astrocytoma and has to be distinguished from it.     

Clinicopathological and immunohistochemical features of three pilomyxoid astrocytomas: Comparative study with 11 pilocytic astrocytomas

Pilomyxoid astrocytoma, first described by Tihan et al ., was recently included as an established variant of pilocytic astrocytoma in the World Health Organization classification of CNS tumors. Histologically, it much resembles pilocytic astrocytoma, but monomorphic myxoid tumor of pilocytic cells with prominent angiocentric growth pattern without Rosenthal fibers or eosinophilic granular bodies is characteristic of pilomyxoid astrocytoma. Pilomyxoid astrocytoma is thought to be more aggressive with more frequent local recurrence as well as cerebrospinal spread. The authors recently encountered a case of pilomyxoid astrocytoma, therefore the purpose of the present study was undertake a retrospective review of pilocytic astrocytomas previously diagnosed during the past 10 years. Consequently, two of them were found to have histological features suggestive of pilomyxoid astrocytoma and both involved multiple recurrence, suggesting aggressive behavior in comparison to pilocytic astrocytoma. Therefore, knowledge of this entity is essential to surgical pathologists and clinicians for patient management.     

Diffuse cerebrospinal gliomatosis with extensive leptomeningeal spread

A case of diffuse cerebrospinal gliomatosis with extensive leptomeningeal spread is presented. The patient, an 18-year-old girl, was admitted due to progressive weakness and paresthesia of both legs, following rapid neuropsychiatric deterioration. An initial magnetic resonance imaging (MRI) study of the T-spine showed diffuse high signal intensities from T9 to T12 spinal cords on a T2 sagittal image and diffuse cord bulging at T1WI. This suggested an inflammatory lesion such as tuberculosis or fungal meningoencephalitis. A limited autopsy was performed. A microscopic examination revealed multifocal GFAP-positive astrocytic proliferations that were low grade astrocytoma in the cerebral leptomeninges, parietal, occipital and temporal lobes and anaplastic astrocytoma in the spinal cord and spinal leptomeninges. The high proliferative indices of the spinal lesion and aneuploidy correspond to a diagnosis of malignant astrocytoma and a rapid fatal clinical course.     

Spinal adrenal cortical adenoma with oncocytic features: report of the first intramedullary case and review of the literature

Ectopic adrenal cortical neoplasms are extremely rare, and only a few have involved the CNS. We report the first case of an intramedullary oncocytic adrenal cortical neoplasm of the spinal cord with immunohistochemical (IMHC) confirmation. A 27-year-old man presented with progressive lower extremity weakness, spastic paraparesis, decreased reflexes, and hypoesthesia below T10. A spinal myelogram showed cauda equina blockade and obliteration of sacral nerve roots. This prompted emergent surgical intervention. A well-circumscribed, approximately 3 x 2 cm, light brown to tan, intramedullary tumor was identified at the level of the conus medullaris. Histologically, the tumor showed sheets and nests of plump, cytologically bland polygonal cells with abundant eosinophilic cytoplasm. A single mitosis, but no necrosis, was identified. By IMHC, the cells were positive for inhibin, melan-A, and synaptophysin, and negative for GFAP, EMA, cytokeratins, S-100, HMB-45, and chromogranin. Electron microscopy study performed from paraffin-embedded tissues demonstrated abundant mitochondria, and lipid vacuoles. This case confirms the occurrence of adrenal cortical neoplasms in the CNS and is the first report of an intradural, intramedullary adrenal cortical adenoma of the spinal cord, and the first to occur in a male. This tumor should be considered in the differential diagnosis of tumors of the CNS.     

毛细胞黏液样型星形细胞瘤的临床病理学观察

目的探讨毛细胞黏液样型星形细胞瘤的临床病理学特点。方法观察3例毛细胞黏液样型星形细胞瘤的光镜形态,采用EnVision法免疫组织化学标记胶质纤维酸性蛋白（GFAP）、CD34、Ki-67,并结合文献复习分析其生物学行为。结果3例均为女性,年龄分别为9个月、10岁和19岁。1例发生于视交叉,2例发生于第三脑室（近鞍区部位）。镜下由双极性的梭形细胞组成,间质内含有大量的黏液。肿瘤内的血管明显增生,部分区域内瘤细胞显示以血管为中心性生长。3例切片内均未见到经典型毛细胞星形细胞瘤中的双相性形态,也未见Rosenthal纤维和嗜酸性颗粒小体。免疫标记显示,瘤细胞强阳性表达GFAP,瘤细胞增殖指数（Ki-67）〈1％。CD34标记显示清晰瘤内增生的血管。结论毛细胞黏液样型星形细胞瘤是毛细胞星形细胞瘤的一种独特亚型,在组织学上与毛细胞星形细胞瘤有不同之处,临床上较毛细胞星形细胞瘤更具有侵袭性。部分病例也可发生于儿童和青少年。GFAP标记有助于该瘤的诊断和鉴别诊断。     

Recurrent primary leptomeningeal astrocytoma of the lumbosacral spinal cord resembling schwannoma. A case report

Primary leptomeningeal astrocytoma has been considered to be derived from ectopic glial cells in the leptomeninges. A case of recurrent primary leptomeningeal astrocytoma of the lumbosacral spinal cord is described. The original tumor was localized in the extramedullary space at the level of the 12th thoracic vertebra, and was solely composed of interlacing bundles of monomorphous spindle-shaped cells. The tumor was therefore diagnosed as schwannoma. The recurrent tumor removed one year after the first operation showed two distinct histologic patterns with increased nuclear atypia and mitosis. Namely, both schwannoma-like areas and astrocytomatous areas were observed. Silver impregnation clearly demonstrated an alveolar pattern in the schwannoma-like area. Immunohistochemically, the tumor cells revealed a positive reaction for glial fibrillary acidic protein. This case particularly emphasizes the usefulness of silver impregnation in differential diagnosis from schwannoma.     

颈髓髓内室管膜瘤的影像解剖学分型及手术策略

目的　探讨颈髓髓内室管膜瘤的影像解剖学特点并制定相应的手术策略。 方法　用MRI成像技术分析26例颈髓髓内室管膜瘤影像学形态特点及脊髓受压变形情况,将肿瘤进行分型并在电生理实时监测下手术分离切除肿瘤。 结果　按肿瘤形态及其与脊髓之间的解剖学关系分为3种类型：I型：实质伴有空洞型;II型：实质型; III型：囊肿内瘤型。对I型肿瘤从肿瘤极端和空洞交界处或肿瘤中部开始从后正中沟切开脊髓分离切除肿瘤;对Ⅱ型肿瘤从肿瘤中部脊髓后正中沟切开分离切除肿瘤;对III型肿瘤从最近肿瘤结节处从脊髓后正中沟切开,囊内切除肿瘤。术中电生理监测显示体感诱发电位（SEP）波幅下降50% 或者潜伏期延长10%;目标肌群运动诱发电位（MEP）波幅持续下降至20%,应暂停手术。术后所有病例病理报告均为室管膜瘤。术后MRI复查23例肿瘤完全切除,3例肿瘤部分切除。术后症状明显减轻23例,好转2例,加重1例,无死亡病例。 结论　依据术前MRI进行分型并制定相应的手术策略可以达到完全切除肿瘤、减少脊髓损伤的目的。     

RECURRENT PRIMARY LEPTOMENINGEAL ASTROCYTOMA OF THE LUMBOSACRAL SPINAL CORD RESEMBLING SCHWANNOMA. A Case Report

Primary leptomeningeal astrocytoma has been considered to be derived from ectopic glial cells in the leptomeninges. A case of recurrent primary leptomeningeal astrocytoma of the lumbosacral spinal cord is described. The original tumor was localized in the extramedullary space at the level of the 12th thoracic vertebra, and was solely composed of interlacing bundles of monomorphous spindle-shaped cells. The tumor was therefore diagnosed as schwannoma. The recurrent tumor removed one year after the first operation showed two distinct histologic patterns with increased nuclear atypia and mitosis. Namely, both schwannoma-like areas and astrocytomatous areas were observed. Silver impregnation clearly demonstrated an alveolar pattern in the schwannoma-like area. Immunohistochemically, the tumor cells revealed a positive reaction for glial fibrillary acidic protein. This case particularly emphasizes the usefulness of silver impregnation in differential diagnosis from schwannoma.     

Spinal intramedullary cysticercosis: a case report and literature review

Neurocysticercosis, involvement of the central nervous system by taenia solium, is one of the most common parasitic diseases of the CNS. However, spinal involvement by neurocysticercosis is uncommon. Here, we reported a 40-year-old woman with intramedullary cysticercosis in the thoracic spinal cord. MRI revealed two well-defined round intramedullary lesions at T4 and T5 vertebral levels, which were homogeneously hypointense on T1WI and hyperintense on T2WI with peripheral edema. Since the patient had progressive neurological deficits, surgery was performed to decompress the spinal cord. Histopathology examination of the removed lesion proved it was intramedullary cysticercosis. In this report, we also discussed the principles of diagnosis and treatment of intramedullary cysticercosis in combination of literature review.     

May 1999--16 year old male with an unexpected MRI finding.

Four years after resection of a supratentorial pilocytic astrocytoma this 16-year-old boy displayed widespread leptomeningeal seeding. Although the primary tumor lacked contrast enhancement, the multiple metastatic nodules were markedly contrast enhancing. Both the initial and disseminated tumor were consistent with a pilocytic astrocytoma. He was treated with vincristin and carboplatinum and the tumor was stable up to Dec. 1998. Dissemination of low-grade intracranial astrocytoma in children occurs in only 4%. It is not a sign of malignancy.The present case is similar to previously published cases. The prognosis of these patients might be quite favorable when treated with radiotherapy and/or chemotherapy.     

A dumbbell-shaped solitary fibrous tumor of the cervical spinal cord

A 40-year-old Asian female presented with a 2-month history of right shoulder pain and right triceps weakness. MRI revealed an extramedullary, extradural, dumbbell-shaped spinal cord tumor with C6 to C7 iso- and hyperintensity on T1 and T2 weighted imaging, respectively. Histological examination revealed monomorphous spindle cells with a storiform pattern. Immunohistochemistry was positive for CD34, CD99, and negative for EMA, SMA, and S100; solitary fibrous tumor (SFT) was confirmed.     

Pilomyxoid astrocytoma of the pineal region: Cytopathological features and differential diagnostic considerations by intraoperative smear preparation

Pilomyxoid astrocytoma (PMA) is a recently identified type of pilocytic astrocytoma (PA) with shorter progression‐free and overall survival, higher rate of recurrence, and higher risk of leptomeningeal spread compared to pilocytic tumors (WHO grade 2 designation). A case is presented here in which intraoperative imprint smears of a pineal region tumor in a 14‐year‐old girl revealed cytologic monomorphism, elongated cells with bland nuclei embedded in a myxoid background. The tumor cells possessed uniformly round nuclei with a smooth nuclear outline, fine granular chromatin, and small nucleoli. Slender cytoplasmic fibrillary processes and angiocentric arrangement were observed but Rosenthal fibers or eosinophilic granular bodies were absent. A cytologic diagnosis of PMA of the pineal region was suggested by intraoperative smear preparation. Histology and immunohistochemical results confirmed the final diagnosis. This report shows that smear preparation can be trustworthy for the intraoperative diagnosis of PMA, helping to determine the appropriate neurosurgical procedure and therapeutic implications. Diagn. Cytopathol. 2015;43:121–124. © 2014 Wiley Periodicals, Inc.     

December 1996 — Cervicomedullary Tumor

A cervicomedullary tumor in a 22-month-old female with an eight-month history of symptoms showed the histological features of a pilocytic astrocytoma but had many areas of palisading cells identical to those described in polar spongioblastomas. The tumor recurred 18 months later and showed predominately pilocytic features. The case is used to discuss polar spongioblastomas and tumors which can histologically simulate them.     

MRI T2WI量化指标评估胸椎黄韧带骨化症手术预后的价值

文题释义：胸椎黄韧带骨化症：是一种病理性的异位骨化,起病隐匿,其病因及发病机制仍不清楚,部分学者认为与遗传、代谢异常、生活环境及退变等因素相关。胸椎黄韧带骨化症是导致胸椎管狭窄的最常见、最重要原因,由于胸脊髓血供较脆弱,当椎管狭窄时可明显影响脊髓血液循环,造成脊髓功能障碍,主要症状表现为下肢的感觉运动障碍。 受试者工作特征(ROC)曲线：就是以假阳性概率为横轴,真阳性为纵轴所组成的坐标图,和受试者在特定刺激条件下由于采用不同的判断标准得出的不同结果画出的曲线。 背景：MRI对于胸脊髓病变具有较高的灵敏性,能够通过脊髓受压的形态及信号强度变化来评估脊髓受损情况,并且对胸椎管狭窄症患者手术预后有提示作用。 目的：分析MRI T2WI量化指标对胸椎黄韧带骨化症患者手术预后的评估价值,并建立预后不良的预测模型,为其预后评估提供参考。 方法：回顾性分析2010年1月至2019年1月沧州市中心医院87例行胸椎管后壁切除减压术且完成术后随访的胸椎黄韧带骨化症患者的临床与影像学资料。根据术后6个月随访时的JOA评分改善率将患者分为预后优良组(改善率≥50%)和预后不良组(改善率<50%)。比较两组患者年龄、性别、病程、JOA评分、骨化物Sato分型、脊髓矢状径残余率、脊髓横截面积、脊髓高信号分布、脊髓信号强度比值、高信号范围、胸椎局部后凸角、后凸角矫正度数、减压节段数以及脑脊液漏变化。单因素分析有显著性差异的指标。采用受试者工作特征曲线(ROC)进行预后分析,纪录曲线下面积及预测界值。多因素Logistic回归分析确定预后不良的独立危险因素,并建立预测模型。 结果与结论：①预后优良组与预后不良组患者病程、JOA评分、脊髓矢状径残余率、脊髓横截面积、脊髓信号强度比值、高信号范围比较差异有显著性意义(P < 0.05);②ROC曲线分析显示,上述各指标预测手术预后不良的曲线下面积分别为0.670,0.733,0.647,0.715,0.753和0.765,预测界值分别为13个月、4 分、29.8%、0.25 cm²、1.593和13.64 mm。病程与脊髓矢状径残余率预测价值较低(ROC曲线下面积 < 0.7);JOA评分、脊髓横截面积、脊髓信号强度比值与高信号范围具有中等预测价值(ROC曲线下面积 0.7-0.9)。脊髓信号强度比值联合高信号范围预测预后不良的ROC曲线下面积为0.791。③多因素Logistic回归分析显示：JOA评分、脊髓横截面积、脊髓信号强度比值联合高信号范围为预后不良的独立危险因素(P < 0.05),并据此建立预测模型,该模型预测预后不良的曲线下面积为0.890,显著高于JOA评分、脊髓横截面积、脊髓信号强度比值联合高信号范围预测的曲线下面积(P < 0.05)。④结果证实,对胸椎黄韧带骨化症患者而言,在MRI量化指标中,脊髓信号强度比值联合高信号范围具有最高的手术预测价值,JOA评分与MRI T2WI量化指标相结合,可更好地预测术后风险。 ORCID: 0000-0003-3354-1228(申沧海) 中国组织工程研究杂志出版内容重点：人工关节;骨植入物;脊柱;骨折;内固定;数字化骨科;组织工程     

Combination Therapy by Tissue-Specific Suicide Gene and Bevacizumab in Intramedullary Spinal Cord Tumor

PurposeMalignant gliomas are aggressive spinal cord tumors. In this study, we hypothesized that combination therapy using an anti-angiogenic agent, bevacizumab, and hypoxia-inducible glioblastoma-specific suicide gene could reduce tumor growth.Materials and MethodsIn the present study, we evaluated the effect of combination therapy using bevacizumab and pEpo-NI2-SV-TK in reducing the proliferation of C6 cells and tumor growth in the spinal cord. Spinal cord tumor was generated by the injection of C6 cells into the T5 level of the spinal cord. Complexes of branched polyethylenimine (bPEI)/pEpo-NI2-SV-TK were injected into the spinal cord tumor. Bevacizumab was then administered by an intraperitoneal injection at a dose of 7 mg/kg. The anti-cancer effects of combination therapy were analyzed by histological analyses and magnetic resonance imaging (MRI). The Basso, Beattie and Bresnahan scale scores for all of the treatment groups were recorded every other day for 15 days to assess the rat hind-limb strength.ResultsThe complexes of bPEI/pEpo-NI2-SV-TK inhibited the viability of C6 cells in the hypoxia condition at 5 days after treatment with ganciclovir. Bevacizumab was decreased in the cell viability of human umbilical vein endothelial cells. Combination therapy reduced the tumor size by histological analyses and MRI. The combination therapy group showed improved hind-limb function compared to the other groups that were administered pEpo-NI2-SV-TK alone or bevacizumab alone.ConclusionThis study suggests that combination therapy using bevacizumab with the pEpo-NI2-SV-TK therapeutic gene could be useful for increasing its therapeutic benefits for intramedullary spinal cord tumors.     

The noradrenergic innervation of spinal cord blood vessels in old rats

The density and pattern of the sympathetic noradrenergic innervation of the extramedullary and intramedullary blood vessels of the spinal cord was studied in 3-, 12- and 25-month-old male Wistar rats using combined catecholamine histofluorescence and quantitative image analysis techniques. The study of innervation of intramedullary vessels was accomplished in spinal cord-transected rats to avoid the interference of descending spinal monoamine fibres in the observations. No age-related changes in the density of noradrenergic innervation of the anterior spinal artery or of sympathetic fibres associated with spinal cord blood vessels occurred. These results suggest that unlike perivascular noradrenergic nerves supplying the cerebrovascular tree, the sympathetic innervation of spinal cord blood vessels does not undergo age-dependent changes. It cannot be excluded that the lesser vulnerability of the spinal compared to the cerebral vascular tree to certain kinds of age-related diseases, may depend on the unchanged sympathetic trophic regulation of spinal vessels with age.     

Cyclin D1 and MIB-1 immunohistochemistry in pilocytic astrocytomas: A study of 48 cases

Pilocytic astrocytoma is an infrequently encountered, generally low-grade neoplasm. No study has extensively looked at both cyclin Dl and MIB-1 labeling indices in pilocytic astrocytoma and their relation to clinical outcome. This study retrospectively examines the clinicopathologic features of 48 patients with pilocytic astrocytoma including MIB-1 (cell proliferation marker) and cyclin Dl (protein that regulates progression from G1 to S phase of the cell cycle) immunohistochemistry. Of 48 patients (27 females and 21 males; mean age, 12.7 years; age range, 2 to 57 years), 26 initially underwent gross total resection; 17, subtotal resection; four, biopsy alone; in one patient, the extent of tumor resection was unknown. Histological features observed included Rosenthal fibers (83.3%), granular bodies (75%), vascular sclerosis (56.2%), vascular proliferation (56.2%), prominent nuclear pleomorphism (14.6%), necrosis (10.4%), and identifiable mitotic figures (2.1%). MIB-1 labeling indices (n = 45) (positive staining tumor nuclei per 1,000 nuclei evaluated) ranged from 0 to 3.5% (mean, 0.6%); seven tumors had a labeling index greater than 1.0%. Cyclin D1 labeling indices (n = 45) ranged from 0 to 0.8% (mean, 0.1%). Most tumors (N = 29, 66.7%) had no immunostaining. At last known follow-up, 27 patients were alive with no evidence of disease (mean, 49.2 months), 17 patients were alive with evidence of disease (mean, 36.8 months), three died with tumor at 2, 22, and 156 months, and one patient was lost to follow-up. Eight patients had at least one tumor recurrence requiring additional surgery; seven of these patients had an initial subtotal resection. In summary, MIB-1 labeling indices were generally low (mean, 0.6%) and are reflective of the slow growth of the tumors. Cyclin D1 immunostaining does not appear to be significantly increased in pilocytic astrocytoma. Adverse outcome in patients with pilocytic astrocytoma may be related to extent of surgical resection and does not seem to correlate with histology, MIB-1 labeling indices, or cyclin D1 immunoreactivity.