Frontal intraparenchymal schwannoma

Intraparenchymal schwannomas are rare. The usual presentation is of a classical intracranial mass lesion. We report a frontal intraparenchymal schwannoma in a pediatric patient with a history of seizure. An MRI scan revealed a uniformly enhancing tumor with perilesional edema. This type of tumor is rarely found in this location. It is important to recognize and distinguish a schwannoma from other more common frontal brain tumors as the outcome is good and recurrence is rare.     

Manifestations of Pediatric Intracranial Hypertension From the Intracranial Hypertension Registry

ObjectiveThe purpose of this study was to examine the presenting symptoms, demographics, and interventions in pediatric patients enrolled in the Intracranial Hypertension Registry.MethodsWe analyzed confirmed intracranial hypertension patients ≤18 years at the time of initial diagnosis who were enrolled in the registry.ResultsA total of 203 patients met the criteria for inclusion; 142 (70%) were considered primary intracranial hypertension. Females made up 72.5% (103 of 142) and 75.8% (47 of 61) in the primary intracranial hypertension and secondary intracranial hypertension groups, respectively. There were no clinically significant differences in age, body mass index, or opening pressure between the primary intracranial hypertension and secondary intracranial hypertension groups. Symptoms most often reported were headache and blurred vision. Bilateral optic disc edema occurred in 89.3% of primary intracranial hypertension and 78.7% of secondary intracranial hypertension patients. When divided into pre- and postpubertal status, 32.5% of patients were classified prepubertal; 77.3% of these had primary intracranial hypertension. This resulted in a female to male ratio of 1:1.04 for prepubertal and 6:1 for postpubertal primary intracranial hypertension patients. The body mass index was significantly higher in the postpubertal primary intracranial hypertension group (P = 0.0014). There was no significant difference in opening pressure.ConclusionsThe common symptoms of intracranial hypertension, including headache, optic disc edema, and vision changes, occurred with similar frequencies in our cohort to those reported in the literature. In separate subanalyses, we found significantly higher rates of obesity in postpubertal females with primary intracranial hypertension. The female-to-male ratios in the postpubertal primary intracranial hypertension and secondary intracranial hypertension groups were higher than reported in the literature.     

Regulatory peptides in neuronal neoplasms of the central nervous system

Neuronal tumors of CNS were examined immunohistochemically for regulatory peptides. Thirteen ganglion cell neoplasms, one cerebellar ganglioneuroblastoma, one cerebellar neuroblastoma, and four medulloblastomas were studied. Sixteen non-neuronal intracranial neoplasms were examined as controls. Immunoreactive vasoactive intestinal polypeptide (VIP) was observed in seven cases of ganglion cell neoplasm and in the one cerebellar ganglioneuroblastoma. The cerebellar neuroblastoma, all of the medulloblastomas, and all of the non-neuronal intracranial neoplasms were negative. Four additional ganglion cell neoplasms were tested for the presence of neurotensin and somatostatin. Two contained neurotensin. The results suggest that CNS ganglion cell neoplasms share with their extracranial counterparts the production of certain hormonal polypeptides. Since these peptides are presumed to be specific markers for neurons, the immunohistochemical detection of these substances may provide diagnostically useful technique in the diagnosis of such lesions.     

A Malignant Transformation of a Spinal Epidural Mass from Ganglioneuroblastoma to Neuroblastoma

Ganglioneuromas are benign tumors. Surgical excision is the treatment of choice with very good prognosis. However, neuroblastomatous malignant transformation of ganglioneuromas was previously reported. We report a patient with spinal neuroblastoma recurrent from a ganglioneuroblastoma after disease free survival of 13 years. This is one of the rare examples of spinal neuroblastoma and to our knowledge the second case report with malignant transformation from a ganglioneuroblastoma or a ganglioneuroma. The present case is the only report in the literature with further genetic investigations.     

Primary rhabdomyosarcoma of the leptomeninx: Clinical, neuroradiological and pathological aspects

Report of an eight-year-old boy presenting with untreatable focal epilepsy and slowly progressing hemiparesis combined with personality changes. Continuous diagnostic efforts eventually resulted in the diagnosis of a primary intracranial rhabdomyosarcoma several months later. The delay in making the correct diagnosis was due to the fact that the malignant process originated as a diffuse involvement of the leptomeninx, and only in later stages a tumor mass developed. Nosology and histogenesis of primary intracranial rhabdomyosarcomas are discussed.     

Primary intracranial low‐grade fibromyxoid sarcoma with FUS gene rearrangement

Low‐grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma typically presenting as a deep soft tissue mass in the lower extremities or trunk. Only four cases of primary intracranial LGFMS have been reported in the literature, but none with molecular/cytogenetic data. We report here a new bona fide case confirmed by the characteristic FUS gene rearrangement, as shown by fluorescence in situ hybridization analysis, in addition to typical histopathological findings. This would be the first genetically confirmed report of the extremely rare occurrence of intracranial LGFMS.     

Congenital glioblastoma multiforme with abnormal vascularity presenting as intracranial hemorrhage in prenatal ultrasound

Background A rare case of a congenital brain neoplasm with intratumoral massive hemorrhage suggested by prenatal ultrasound examination in a 32-week gestational age male fetus is reported. The child died shortly after birth due to cardiorespiratory insufficiency.Methods Autopsy disclosed a large well-delimited tumor with a sponge-like appearance due to high vascularization, which involved nearly the whole left cerebral hemisphere and led to marked hydrocephalus by secondary aqueductal stenosis. Histological and immunohistochemical examination confirmed the diagnosis of a malignant glioma with features of a glioblastoma multiforme (GBM) matching well with previous findings in primary pediatric GBMs.Findings The present case demonstrates that malignant congenital neoplasms should be considered in the differential diagnosis of fetal intracranial hemorrhage.     

Primary meningeal sarcoma with leiomyoblastic differentiation complicating pregnancy

Intracranial malignant tumors during pregnancy are rare. Primary meningeal sarcoma with leiomyoblastic differentiation during pregnancy has not been reported. We present the case of a 25-year-old woman in the third trimester of pregnancy with a large intracranial tumor destructing the parietal calvaria and invasion of soft tissues. Histological examination revealed primary meningeal sarcoma with leiomyoblastic differentiation. A gross macroscopical resection of the tumor with removal of the infiltrated parietal calvaria was performed after delivery of a healthy baby through caesarean section. A mass on the head rapidly enlarging during pregnancy should be considered for a malignancy of intracranial origin. Early radiological exams as well as tumor resection followed by staging and multimodality treatment should be urgently performed.     

Intracranial presentation of teratocarcinosarcoma

Teratocarcinosarcoma (TCS), an aggressive and extremely rare neoplasm, usually presents as a nasal or paranasal mass. TCS can have intracranial or dural extension from a nasal mass. We found only two instances in the literature that described this lesion as primarily manifesting as an intracranial mass while arising from a primary nasal focus. We describe a patient who had a predominantly frontal-lobe TCS without any symptoms of a primary nasal mass. The gross surgical appearance was a glistening, slimy, firm white mucoid lesion, in contrast to the friable, necrotic and tan-colored lesion usually described. We report a patient with a rare intracranial TCS, and focus on the differential diagnoses of intracranial tumors. Neuropathologists and neurosurgeons should be aware of this mode of presentation for an accurate diagnosis and a well-targeted therapeutic approach.     

Malignant meningioma as a second malignancy after therapy for acute lymphatic leukemia without cranial radiation

Rationale Meningiomas in the pediatric age group are very rare tumors, comprising about 1–4.2% of all primary pediatric intracranial tumors.Case report We present a 17-year-old patient who suffered from an intraventricular malignant meningioma. At the age of 2 years, acute lymphatic leukemia (common ALL [cALL]) was diagnosed and successfully treated with chemotherapy. There was no cranial radiation therapy.In December 2001, 13 years after diagnosis of cALL, he complained of headache, vomiting, and walking difficulties. Magnetic resonance imaging showed an enhancing mass with cystic components in the trigone of the right lateral ventricle. The tumor was removed completely. Histological diagnosis revealed a malignant papillary meningioma. After removal of a recurrent meningioma 16 months later, he received local radiotherapy.Conclusion Pathogenetic mechanisms, treatment options, and prognosis of meningiomas and secondary malignancies of this age group are discussed.     

Variante amelanótica en melanoma cerebral primario: a propósito de un caso

Primary brain melanoma is a very rare tumour (only 0.07% of primary CNS neoplasms) which usually shows with abundant melanin content; whereas hypo/melanotic variants have been scarcely described.We introduce the case of a female patient with headache, left brachial paresis and frontal lobar syndrome. The MRI image showed a right frontal mass with homogeneous contrast uptake. As treatment, a complete surgical resection was performed. Pathology was diagnostic for melanoma, with very low melanin content and a high proliferative index. A thorough extension study was performed to rule out an extracranial primary origin. Due to several intercurrent complications, the patient evolved unfavorably, not being able to receive further treatment.The amelanotic variant of primary intracranial malignant melanomas has not been described in detail previously. We will review the literature, focusing on the particularities of management and diagnosis of this clinical entity.     

儿童第四脑室肿瘤的显微外科治疗

目的 :提高儿童第四脑室肿瘤的手术效果。方法 :回顾分析我院收治的 3 6例儿童第四脑室肿瘤的临床资料 ,术前均行头颅MRI检查 ,采用显微外科手术治疗。 2 0例术中配合脑室外引流。结果 :肿瘤全切除 2 8例 ,次全切除 6例 ,部分切除 2例。 6例术后脑积水 ,1例术后缄默症 ,1例死于颅内感染。临床治愈 2 6例 ,症状改善 8例。结论 :头颅MRI能良好显示这类肿瘤。术前仔细研究影像资料 ,术中选择合适部位分离切除肿瘤 ,显微操作 ,减少出血 ,积极预防术后并发症是提高手术质量的关键     

Infectious intracranial aneurysms in the pediatric population: endovascular treatment with Onyx

OBJECTS: Infectious intracranial aneurysms present a treatment challenge in the pediatric population. Current endovascular strategies and tools have been developed, which make treatment of infectious intracranial aneurysms with liquid embolics safe and effective. This study reviews the use of the liquid embolic Onyx in the treatment of infectious intracranial aneurysms in the pediatric population. MATERIALS AND METHODS: We used an endovascular approach to treat ruptured infectious intracranial aneurysms. We embolized the aneurysms with either Onyx alone or in combination with platinum coils. CONCLUSION: Endovascular therapy with liquid embolics (Onyx) has been shown to be a safe and effectual treatment option in the case of pediatric infectious intracranial aneurysms. The combination of endovascular modalities can also be used to tailor the therapeutic goal of exclusion of infectious aneurysms with good results. Further studies are needed to assess the long-term effectiveness of this approach to pediatric infectious intracranial aneurysms.     

Rhabdoid meningioma: a new subtype of malignant meningioma also apt to occur in children

Case report The case of a 14-year-old girl who presented with a 2-week history of raised intracranial pressure is reported. A left frontal extra-axial tumor was totally removed, whose histopathologic diagnosis was rhabdoid meningioma (RM).Discussion Rhabdoid meningiomas constitute a special malignant phenotype of meningioma that has been recently included in the WHO classification of tumors of the nervous system. Usually, RMs affect middle-aged and elderly individuals. We report the fourth case of a RM occurring in a child to illustrate that the diagnosis of this tumor subtype, given its prognostic implications, must also be considered in pediatric patients     

Traumatic intracranial aneurysms in childhood and adolescence

We report four pediatric traumatic intracranial aneurysms occurring before the age of 10 years. Two of these aneurysms were the results of closed head injury. The remaining two were iatrogenic aneurysms which occurred in unusual circumstances. These four children represent 33% of the pediatric intracranial aneurysms seen at the Children's Hospital of Eastern Ontario from 1974 to 1992. Diagnosis of traumatic intracranial aneurysms requires a high index of suspicion: any head-injured or postoperative child who experiences delayed neurologic deterioration, or who fails to improve as expected following treatment, should promptly undergo diagnostic intracranial imaging. Documented subarachnoid hemorhage, intracerebral or intraventricular hemorrhage, or subdural haematoma in this clinical setting should be further investigated by cerebral angiography to exclude a traumatic aneurysm or other vascular lesion. Traumatic aneurysms typically arise at the skull base or from distal anterior or middle cerebral arteries or branches consequent to direct mural injury or to acceleration-induced shear. Reported traumatic aneurysms account for 14%–39% of all pediatric aneurysms. Iatrogenic aneurysms also occur with unecpected frequency during. childhood and adolescence. Pediatric traumatic cerebral aneurysms may present early or late. Most present early with intracranial hemorrhage. Late presentation occurs infrequently, typically as an aneurysmal mass. Once diagnosed, these aneurysms should be promptly treated by craniotomy employing routine microsurgical techniques, or in some cases, by endovascular detachable balloon techniques. Delay in operative treatment entails significant risks of repeated hemorrhage and death. Outcome in these children is primarily determined by the extent of traumatic cerebral injury and the preoperative clinical status. The latter directly depends upon diagnosis of the aneurysm prior to either initial or repeated hemorrhage.     

儿童中枢神经系统肿瘤（附766例分析）

目的:探讨儿童中枢神经系统肿瘤的发病特点。方法:对华山医院１９８６~１９９８年儿童中枢神经系统肿瘤７６６例,进行回顾性分析。结果:本组占同期中枢神经系统肿瘤病例数的６．１４%。部位:颅内７２５例,其中幕上４９９例,幕下２７６例,椎管内４１例。组织学分类:前三位分别是:星形细胞性肿瘤(１９例,占２６．０%);颅咽管瘤(１１５例,占１５．０%);髓母细胞瘤(１０１例,占１３．２%)。性别:男孩多见。发病涉及各年龄组,病例数随年龄的增长而增加。结论:儿童中枢神经系统肿瘤位于幕上者多于幕下,颅内肿瘤以星形细胞性肿瘤最常见,男孩比女孩好发,为１．５０:１。     

Meningiomas in pediatric patients: report of 2 cases

Meningiomas correspond to 1% - 4% of primary intracranial tumors in pediatric group, with their incidence raising according to age. There is not gender prevalence, in spite of some authors describe a male tendency opposed to female one in adulthood. At present study we describe two cases of pediatric meningiomas reviewing clinical, radiological and histological aspects of these lesions. The authors review also treatment options and prognosis of childhood meningiomas. A two-year-old boy was admitted with seizures. Computerized tomography showed a right parietal lesion, which was totally resected. Histological features were compatible with meningioma. After 17 months the child is doing well, with no deficits or seizures. The second case is a 12-year-old girl, with a headache complain. During investigation, a CT revealed a right frontal lesion. She was operated under a right frontal craniotomy with total tumor resection. Nowadays she is asymptomatic, 20 months after surgery. Despite meningiomas in pediatric group are uncommon; they should be included in differential diagnosis list of expansive intracranial lesions of childhood.     

Intrameningioma metastasis: A case-based literature review

A tumor-to-tumor metastasis inside a meningioma is a rare phenomenon. Malignant neoplasms of the breast and lung are the most common primary tumors. Other sites of origin include prostate, renal and gastric neoplasms. The included case files were retrieved from the medical records of the University Hospital of Crete, Greece. A review of the literature was conducted in March 2020 via PubMed. Relevant search results were few. We report a case of a 66-year-old female, with known Small Cell Lung Cancer, who presented with left-sided hemiparesis. The Magnetic Resonance Imaging scan revealed a right frontal extra-axial mass. The patient underwent a craniotomy and a gross total removal of the tumor. Histological examination of the excised mass revealed metastatic adenocarcinoma deposits inside a meningioma: tumor-to-tumor metastasis. Reviewing the available literature, it has been hypothesized that the following factors play a role in the pathophysiology of this phenomenon: progesterone and estrogen receptors, cell-to-cell adhesion molecules, rich vascularization, favorable metabolic, micro-and immunological environment. Meningiomas seem to be the most common type of intracranial neoplasm to host a metastasis. There is a difference between tumor-to-tumor metastasis and collision tumors. The former implies a recipient role of the host tumor, and the latter refers to a co-localization of two different tumors that grow into one another, both being in the same organ. Tumor-to-tumor brain metastasis is a well-described phenomenon but with unclear pathophysiology. Deeper knowledge could be beneficial for its management.     

Pregnancy and brain tumors

This article discusses the clinical presentation, diagnosis, and treatment of the pregnant patient with an intracranial mass. Common manifestations, pitfalls, and guidelines for management are discussed.     

原发性眶内卵黄囊瘤:病例报告并文献分析

目的 探讨临床少见原发于眶内卵黄囊瘤的病理学特征.并讨论颅内生殖细胞肿瘤的病理学鉴别要点.方法 分析l例原发于左侧眶尖卵黄囊瘤患儿的临床表现.HE和免疫组织化学染色观察颅内卵黄囊瘤的临床病理学特点及免疫表型.结果 患者男性.2岁.表现为左眼上睑下垂并眼球突出.MRI显示颅底肿瘤侵犯左侧后组筛窦、眼眶及翼窝并蝶骨翼板,左侧上颌窦后壁、筛骨及眼眶尖部骨质破坏,与硬膜分界尚清,未突破硬膜向脑实质浸润.术中可见肿瘤位于眶尖区之眶内外,累及左侧海绵窦区,位于硬膜外尚未侵入硬膜下区,无包膜,破坏眶尖区骨质,长人蝶窦和筛窦.肿瘤组织由许多相互交通的间隙形成疏松的网状结构,衬覆胞质透亮的圆形或多边形细胞,核深染、不规则且核仁突出,核分裂象多见;可见Schiller-Duval(S-D)小体结构.为单个圆形或长形乳头状结构,含有单个血管的纤维血管轴心,被覆柱状细胞,乳头占据的间隙衬覆立方、扁平或"鞋钉"样细胞;肿瘤细胞内或细胞间可见嗜伊红且PAS阳性透明小体.肿瘤细胞弥漫表达细胞角蛋白,灶性表达甲胎蛋白、CD99和CD117,但不表达CD30、CD45、胎盘碱性磷酸酶、突触素、嗜铬素A和CD34.连续组织切片HE染色未发现合并其他类型生殖细胞肿瘤.病理诊断左侧眶尖原发性卵黄囊瘤(WHOⅣ级).结论 颅内卵黄囊瘤好发于青少年,多生长于中线结构,具有特殊的组织学构象和免疫组织化学表型,预后不良,诊断时应与其他生殖细胞肿瘤相鉴别,并注意是否同时合并其他生殖细胞肿瘤成分.