Tumeur rhabdoïde du système nerveux central chez une adolescente de 16 ans : à propos d’un cas

Primitive malignant rhabdoid tumors of the central nervous system are rare and have a poor prognosis. Adult and adolescent cases are exceptional. We report the case of a 16-year-old girl who presented an intratumoral hemorrhage in a rhabdoid tumor. She was treated with surgery, followed by intravenous and intrathecal chemotherapy. Despite intensive treatment, she died 5 months after diagnosis. We discuss the different therapeutic options for this patient and review the literature on this kind of tumor.     

Subependymoma of the spinal cord. Case report and review of the literature

Subependymoma is an uncommon, benign and, as a rule, asymptomatic lesion of the central nervous system, only rarely recorded at spinal level. The case reported here brings the total number of spinal subependymomas so far described to 16. The relevant literature is reviewed and the salient features of these tumors are discussed.     

Practical Molecular Pathology and Histopathology of Embryonal Tumors

There have been significant improvements in understanding of embryonal tumors of the central nervous system (CNS) in recent years. These advances are most likely to influence the diagnostic algorithms and methodology currently proposed by the World Health Organization (WHO) classification scheme. Molecular evidence suggests that the tumors presumed to be specific entities within the CNS/primitive neuroectodermal tumors spectrum are likely to be reclassified. All these developments compel reassessing current status and expectations from the upcoming WHO classification efforts. This review provides a synopsis of current developments and a practical algorithm for the work-up of these tumors in practice.     

Practical Neuroimaging of Central Nervous System Tumors for Surgical Pathologists

Imaging has established itself as an irreplaceable component of neuro-oncology, and provided much insight in all aspects of central nervous system (CNS) tumors. Today, similar to some other medical specialties, such as bone and joint disorders, it is an integral part of the diagnosis of CNS tumors. This brief review highlights the critical elements of neuroimaging, especially of MRI, in the study and diagnosis of brain tumors, and considers some of the common entities for the diagnosis, of which a good understanding of imaging characteristics is extremely helpful.     

Urologic tumors presenting as neurosurgical masses

Metastases from urologic neoplasms often occur to the central nervous system or surrounding bony structures. Usually these metastases are discovered after the primary tumor has been identified. However, these tumors may present primarily with only manifestations of their central nervous system metastases. Five cases of different urologic tumors presenting as neuro-surgical masses are presented, along with a brief review of the pertinent literature.     

The different faces of central nervous system metastases

Cerebral metastases are the commonest central nervous system tumors. The MR assessment should include T1-weighted images with and without enhancement and T2/FLAIR images. They usually appear as multiple lesions with nodular or annular enhancement and are surrounded by edema. They are hypervascularized and have no restriction of their diffusion coefficient in their necrotic area and contain lipids on 1H spectroscopy. Metastases can be distinguished from primary tumors by the lack of malignant cell infiltration around the tumor. Stereotactic radiotherapy may temporarily increase tumor volume, although this is not of adverse oncological value. Less commonly, spinal disease may be asymptomatic and should be examined by MR.     

Primary sarcomas of the brain and spinal cord: a study of 18 cases

Primary sarcomas of the central nervous system are exceedingly rare. We reviewed the clinicopathologic features of 18 primary central nervous system sarcomas diagnosed from 1959 through 1999. Median age at diagnosis of the nine female and nine male patients was 28 years (range 3-63 years). Median tumor size was 4 cm (range 1.3-8 cm). Fifteen tumors arose in the cerebrum (83%), two in the cerebellum, and one in the spinal cord. Histopathologically, the most common tumor types included fibrosarcoma (six), malignant fibrous histiocytoma (five), and undifferentiated sarcoma (three). Immunohistochemical and ultrastructural studies supported the histologic diagnosis in 17 and six cases, respectively. All patients had subtotal to gross total tumor resection; 16 also received radiotherapy and/or chemotherapy. Twelve tumors (67%) were high-grade. Follow-up was obtained in all instances (median 2.3 years). Nine patients died of the disease, eight with high-grade tumors. Survival at 5 years for patients with high-grade tumors was 28% compared with 83% for those with low-grade neoplasms (p = 0.03). Primary central nervous system sarcomas most often affect young and middle-aged adults. Most involve the cerebrum and show fibrous, "fibrohistiocytic," or no specific differentiation. The prognosis for high-grade sarcomas seems better than that for glioblastoma multiforme.     

Central neurocytoma. Four patients

We report four cases of central neurocytoma we operated on at our institution, between 1994 and 2002. This uncommon lesion occurred in young patients, was located in the lateral ventricules and was revealed by signs of intracranial hypertension. The tumor was totally removed in three cases and subtotally in one. Immunohistochemistry provided useful information to distinguish neurocytoma from other nervous system tumors. Prognosis was excellent in two patients. One patient developed tumor recurrence seven years after surgery, without any progression on further follow up evaluation. In the last patient, the tumor showed a more aggressive progression requiring the adjunction of radiosurgery.     

Brain tumors in neurofibromatosis

Opinion statement The neurofibromatoses are common neurocutaneous syndromes with multisystem involvement. These disorders place patients at increased risk for the development of malignancies. In particular, there is a predisposition to develop central nervous system and peripheral nervous system neoplasms. Distinct tumor types develop in association with neurofibromatosis type 1 (NF-1) different from those that are typically associated with neurofibromatosis type 2 (NF-2). In general, the tumors associated with NF-1 and NF-2 tend to demonstrate a more indolent course than similar tumors in patients without neurofibromatosis. An exception would be earlier presentation of tumors in each of these disorders. Management decisions are based on multiple factors. These include tumor location, presumed or known histology, and patient symptoms at time of diagnosis or evidence of progression either clinically or as demonstrated by neuroimaging. Once all of these factors have been weighed, therapeutic considerations include expectant observation, surgery, and radiation or chemotherapy. The overall philosophy of treatment is that of attempting to preserve neurologic function for as long as possible, because these are progressive disorders. At times, it may be preferable to keep interventions to a minimum in order to achieve this goal. A multidisciplinary approach is crucial in the care of these patients.     

Endocrine diffuse system--histological and functional aspects interrelated with the tumoral pathology

It was first believed that all these endocrine cells are deriving from the neural crests; in time were discovered more than 40 different types of such cells with different origins and only 6 or 7 are deriving from the neural crests. Serotonin-secreting cells show yellow fluorescence, while those secreting cathecolamines show a green fluorescence, with formaldehyde. The most usual method for the stain of the cells of the endocrine diffuse system is the silver salts impregnation. In the electron microscopy the cells show dense granules, which are modified in appearance in the malignancies developed from such cells. Most of the hormones secreted in the intestine were found also to be hormones secreted in the central nervous system. The border between benign proliferation and malignant tumors arising from these endocrine cells is not well defined. DNES--diffuse neuroendocrine system.     

Torticollis secondary to posterior fossa and cervical spinal cord tumors: report of five cases and literature review

Torticollis is either congenital or acquired in childhood. Acquired torticollis is not a diagnosis but rather a sign of an underlying disorder. The causes of acquired torticollis include ligamentous, muscular, osseous, ocular, psychiatric, and neurologic disorders and may be a symptom of significant abnormalities of the spinal cord and brain, such as spinal syrinx or central nervous system neoplasia. Torticollis is rarely considered to be an initial clinical presentation of posterior fossa and cervical spinal cord tumors. We report five cases of pediatric tumors with torticollis at the onset: an astrocytoma originating from the medulla oblongata, another presumptive astrocytoma of the spinal cord located between C1 and C6 cervical vertebrae (not operated), an ependymoma located throughout the whole cervical spinal cord extending into the bulbomedullary junction, an astrocytoma originating from the bulbus and extending into the posterior fossa, and another case of a eosinophilic granuloma located extradurally through the anterior and posterior portions of the vertebral bodies from C3 to C7 producing the collapse of the sixth cervical vertebra. All five cases were seen in children, aged between 3 and 12 years. All these cases reflect the misinterpretation of this neurological sign and the lack of association with the possibility of spinal or posterior fossa tumor. This delay in the diagnosis of these diseases led to progressive neurological deterioration and to the increase in the tumor size, which made surgical intervention difficult and the prognosis unfavorable. Although torticollis secondary to tumors is rarely seen, it is necessary to be kept in mind in the differential diagnosis.     

Management of metastatic brain tumors

Background: Brain metastases are the most common neurological complication of systemic cancer. They represent a serious cause of morbidity and mortality and a significant challenge for neurosurgeons. They outnumber all other intracranial tumors combined and, with advances in technology and treatment of systemic cancer, are on the increase as cancer patients live longer. Methods: We have reviewed the major factors that influence the occurrences of metastases in the central nervous system: primary cancer, patient age and sex, clinical aspects of presentation, basic diagnostic modalities, diagnostic imaging (computed tomography and magnetic resonance imaging), and treatment considerations. In discussing these different aspects, we emphasize the efficacy of different treatment options, including recent information regarding multiple metastases that broadens the scope of surgical implications. The criteria we present are directed toward considerations made by general surgeons, as well as those made by neurosurgeons. Conclusions: Although radiotherapy remains the main therapeutic modality, surgical excision has increasingly shown advantages in certain settings, as has stereotactic radiosurgery. Chemotherapy is less effective, but its advantages are reviewed, as are the implications of recurrent metastases.     

Tumors of the central nervous system in Taiwan,Republic of China

A series of 1012 surgically removed tumors of the central nervous system from two hospitals in Taiwan were reviewed, tabulated, and compared to series from the United States, Europe, and Asia. Among gliomas, a relatively high incidence of glioblastomas was found in patients less than 20 years old. The percentage of meningiomas in the two hospitals was very high: 20.22% and 26.72%, respectively. Neurilemmomas, as reported from Japan and mainland China, were also more common than in the United States or Europe. Germinomas and teratomas were more frequent than in Western countries, but less so than in Japanese series. Pituitary adenomas constituted 21.8% of cases from the Veterans General Hospital in Taipei, a percentage higher than that in any comparable series.     

True and false cerebral venous malformations. Venous pseudo-angiomas and cavernous hemangiomas

The authors report their classification of venous anomalies and abnormalities within the central nervous system. The so-called venous angiomas are developmental venous anomalies (D.V.A.). The different forms encountered express the extreme variations in the transcerebral venous system. Their symptomatic character depends on the aging changes that the cerebral venous system undergoes or the frequent association of D.V.A. with cavernomas of the central nervous system. The cavernous hemangiomas are true tumors that present a proliferative character. They are localized in the dura or in the extradural space intracranially or at the spinal level. They must be considered as tumors and can be seen at angiography as they present a fine parenchymatous stain. The cavernomas of the central nervous system are true vascular malformations of the venous type; they are locally multiple, but can also be disseminated within the intracranial space. Some familial forms have been reported. They are often associated with D.V.A. They have a high bleeding potential, which does not carry a high morbidity or mortality incidence. However, the natural history of this lesion is poorly known at present. The spinal cord posterior fossa and paraventricular localizations present probably the least favorable localizations. Only the cavernomas deserve to belong to the vascular malformation group. The same comments and observations apply to spinal cord venous lesions.     

Primary intradural extraosseous Ewing sarcoma of the spine: case report and literature review

BACKGROUND AND IMPORTANCE: To report a rare case of spinal intradural extraosseous Ewing sarcoma in an adult and review current literature. Although Ewing sarcoma belongs to the family, the treatment modalities are different, and thus the correct diagnosis is very important despite its rare occurrence. CLINICAL PRESENTATION: A 56-year-old woman presented with nocturnal bilateral buttock and leg pain. Magnetic resonance imaging (MRI) showed an enhancing intradural extramedullary extraosseous tumor at L1. INTERVENTION: A T12-L2 laminectomy was performed to resect the tumor. Immunohistochemical analysis confirmed the diagnosis of Ewing sarcoma. A thorough diagnostic workup did not reveal any bony origin of the tumor. Primary intradural central nervous system Ewing sarcoma is infrequently encountered and shares imaging and histopathological features with central primitive neuroectodermal tumors. Establishment of the right diagnosis is crucial because it mandates a distinct workup and treatment modality different from that for central primitive neuroectodermal tumor. Although osseous Ewing sarcoma predominantly occurs in children and young adults, extraosseous central nervous system Ewing sarcoma is not uncommon in adults and should therefore be considered in the differential diagnosis of extraosseous small blue cell tumors in adult patients.     

Enhanced in vitro uptake and retention of 3H-tetraphenylphosphonium by nervous system tumor cells

Photodynamic therapy is a promising treatment for human brain tumors because of the selective retention of certain compounds by tumor cells. Certain lipophilic cationic compounds, such as tetraphenylphosphonium (TPP), are selectively taken up by a variety of carcinomas. Although preferential retention of TPP has been demonstrated for the breast carcinoma cell line MCF-7, this compound had not been tested previously on cells derived from nervous system tumors. In the present study, tritiated-TPP (3H-TPP) uptake and retention for eight different cell cultures of three histologically different types of nervous system tumors was measured and the data were compared to a positive control (MCF-7) and negative controls (normal African Green monkey kidney epithelium (CV-1) and the normal human fibroblast (WI-38) cell lines). Uptake and retention characteristics could be grouped by specific pathological tumor types, but individual tumor variability was notable. Malignant astrocytoma (grade III/III glioblastoma) and malignant neurofibrosarcoma cells showed preferential uptake and retention of 3H-TPP relative to meningioma cells and normal controls. A clonogenic assay utilizing the cytotoxic lipophilic cationic compound dequalinium showed strong retainers of 3H-TPP to be more susceptible to the effects of dequalinium than weak retainers. These data demonstrate that certain human and experimental animal nervous system tumor cell lines retain lipophilic compounds possessing a delocalized positive charge. Lipophilic cationic compounds may be useful in the intraoperative delineation of tumor margins and in the photodynamic therapy of certain nervous system tumors.     

颅内原发性黑色素瘤的诊断及治疗

目的 探讨颅内原发性黑色素瘤的诊治方法。方法 分析１９６８年以来收治的６例颅内原发性黑色素瘤的临床表现、诊治方法和疗效。结果 临床表现无特异性,ＣＴ及ＭＲＩ均难以明确诊断。肿瘤全切除１例。在碚切除３酌,部分切除１例,活检１例。诊断颅内原发性恶性黑色素瘤３例,脑膜黑色素瘤３例。随访５例,３例恶性黑色瘤患者死亡,２例脑膜黑色素瘤３例,随访５例,３例恶性黑色素瘤鹗２死亡,２例脑膜黑色素瘤１例长期卧床,１     

Solitary fibrous tumors of the meninges

Central nervous system solitary fibrous tumors are a new pathological entity. To our knowledge, only 60 meningeal solitary fibrous tumors both in the spinal cord and in the brain have been described in the literature. The 56 previously reported cases of meningeal solitary fibrous tumors are critically reviewed. In addition, we report four new cases of solitary fibrous tumors of the meninges. There is a slight male prepoderance. Meningeal solitary fibrous tumors show a tendency to arise in the posterior fossa (26%) and spine (25%). The treatment was mainly total surgical excision. Radiotherapy was given only to four patients with tumors involving the cerebral parenchyma. Sporadic cases of recurrence and distant metastasis have been reported. The prognosis of meningeal solitary fibrous tumors is still unknown because the follow-up of the reported cases is short. It is probable that cases of solitary fibrous tumors of the meninges have been misdiagnosed as other tumors in the past. The best management of these tumors seems to be total surgical excision whenever possible. It is important that every new case of meningeal SFT be reported to throw light on this particular tumor and to affirm its status as a clinicopathological entity.     

应该重视特殊类型的胃肿瘤

在临床工作中,经常会遇到一些特殊类型的胃肿瘤,如胃肠间质瘤(GIST)、胃神经内分泌肿瘤(NETs)、原发性胃淋巴瘤(PGL)及特殊类型的胃癌.由于其所具有的特殊的发病机制、组织来源和病理类型,使得疾病本身可供选择的治疗手段相对较少,临床治疗效果有限;同时,其较低的发病率可能容易造成临床医师认识不足.本文通过对此类胃肿瘤进行阐述分析,以提高临床对该类疾病的认识和重视.     

Neurofibromatosis and central nervous system tumors in childhood.

Neurofibromatosis is a multifaceted disease that often results in tumors of the central nervous system. As our understanding of the molecular biology of the disease improves along with better neuroradiology imaging, surgical instrumentation, and adjunctive care, the management schemes for these patients are evolving. This article reviews what is known about neurofibromatosis, common management problems with respect to the central nervous system, and an approach to the handling of these issues.