Optic Pathway Gliomas

Optic pathway gliomas represent less than 1 of all brain tumors, but because three quarters of these tumors occur in the first decade of life, they represent 4 to 6 of all brain tumors seen at major pediatric hospitals.6 Most of these tumors occur within the first 5 years of life. They can arise anywhere from the retroorbital space, along the optic nerve, to the chiasm and hypothalamus, infiltrating these structures diffusely, and then occasionally also spreading along visual pathways...

Neuroimaging

MRI is central to the diagnosis and evaluation of ependymoma. MRI is critical to planning for surgery and radiation therapy and is the primary means for surveillance after treatment or monitoring the response to chemotherapy. Uncommonly, surgery is conducted with only a computed tomography (CT) study and without the benefit of MRI, especially when the tumor arises in the posterior fossa. Lack of preoperative MRI makes it difficult to accurately determine the extent of resection and to plan...

References

Abbruzzese JL, Abbruzzese MC, Lenzi R, et al Analysis of a diagnostic strategy for patients with suspected tumors of unknown origin. J Clin Oncol 13 2094-2103, 1995. 2. Bindal RK, Sawaya R, Leavens ME, et al Surgical treatment of multiple brain metastases. J Neurosurg 79 210-216, 1993. 3. Bohinski RJ, Bejarano PA, Balko G, et al Determination of lung as the primary site of cerebral metastatic adenocarcinomas using monoclonal antibody to thyroid transcription factor-1. J Neu-rooncol 40 227-231,...

Mixed Germ Cell Tumors

Mixed germ cell tumors are composed of various combinations of two or more types of germ cell tumor elements. More than half of the described mixed germ cell tumors show combinations of germinoma and teratoma with or without immature components. The prognosis of mixed germ cell tumors relates to the most malignant element present. According to a report by Sano, germinoma components are found in 79 , ter-atoma components in 63 , yolk sac tumor components in FIGURE 96-3 A, a 3-year-old boy...

Initial Investigations

In the child with back or radicular pain and no identifiable neurologic abnormalities, anteroposterior and lateral radiographs directed to the area of the pain are the initial investigations. Almost 10 of children with back pain and scoliosis will be found to have an underlying pathologic cause, but an intraspinal tumor is only rarely the problem. The majority of these patients with an underlying pathologic cause will have abnormal plain radiographs, most commonly spondylolysis or...

Intraspinal Lipoma

It is imperative to clarify the nomenclature regarding lipo-matous tumors of the spinal cord. This becomes of utmost importance when discussing treatment options and outcomes data. The lipomatous tumors of the spinal cord may be subdivided into three types intradural or intramedullary spinal cord lipomas not associated with spinal dysraphism lipomyelo-meningoceles (often termed lumbosacral lipomas) associated with spinal dysraphism and filum terminale lipomas. Similar to their intracranial...

Schwannoma Pathology

The classic type of schwannoma, also called neurilemoma or neurinoma, is a benign encapsulated peripheral nerve tumor produced by the abnormal proliferation of Schwann cells within a nerve fascicle (Figure 73-3). This neoplasm can originate from cranial, peripheral, or autonomic nerves, usually distal to the oligodendrocyte-Schwann cell junction at the interface of central nervous system (CNS) and PNS. Schwannoma is usually a solitary mass but can also be multiple masses. Their gross appearance...

Info

Brain tumors rank second as the cause of cancer-related deaths in children and young adults younger than the age of 34, and they affect adults of all ages. After cancer of the lung and pancreas, primary malignant brain tumors have the third-highest cancer-related mortality rate in the United States, and they take a disproportionate toll in disability and morbidity.8 The Surveillance, Epidemiology, and End Results (SEER) program estimated that there were 17,400 new cases of brain tumors in the...

Trigeminal Schwannomas

Schwannomas that arise from the intracranial portion of the trigeminal nerve are rare, accounting for 0.8 to 8 of intracranial schwannomas.1 Jefferson6 classified these tumors into three types (1) type A tumors are located mainly in the middle fossa that arise from the gasserian ganglion (2) type B tumors are located predominately in the posterior fossa and arise from the root of the trigeminal nerve and (3) type C tumors are dumbbell-shaped, or hourglass-shaped, tumors with significant...

Diagnostic Evaluation

The most common presenting symptom of sinonasal carcinoma is nasal airway obstruction, which is often unilateral, followed by chronic nasal discharge and epistaxis.90 Table 50-6 summarizes the common presenting features of paranasal sinus malignancies. Signs and symptoms of maxillary sinus tumors have been grouped by regional anatomy into nasal, oral, ocular, facial, and neurologic findings.13 Extension of the neoplasm into the nasal cavity may be seen on anterior rhinoscopy. Oral findings...

Recurrence And Outcome

Most patients with dorsally exophytic tumors treated with radical surgery have long disease-free or progression-free survival despite subtotal resections. The histology is typically low grade. Residual tumor may be followed with serial MRI scans at 3- to 6-month intervals. Disease progression is seen in 25 to 30 of patients.23 Progression is usually at the primary site and is amenable to radiation therapy, chemotherapy, or repeat surgery. If the patient is asymptomatic, continued observation...

Inflammatory Pseudotumor of the Peripheral Nerve

Localized or diffuse swelling of a peripheral nerve may also be due to a number of inflammatory or infectious diseases. Inflammatory pseudotumor, also described as nodular lymphoid hyperplasia, plasma cell granuloma, and fibrous xanthoma, are all reactive and non-neoplastic processes that can mimic a peripheral nerve mass lesion. Inflammatory pseudotumors are characterized by chronic infiltration of inflammatory cells, primarily the T-cell population, as well as extensive fibrosis and collagen...

Antibody Mediated Immunotherapy

Antibody-mediated immunotherapy is the use of monoclonal antibodies targeted against specific TAAs to mediate tumor cell destruction. Binding of antibody to a TAA can lead to cell death through the traditional pathways of phagocytosis or complement-mediated lysis or by serving as the delivery system for a tumoricidal compound conjugated to the antibody. A variety of tumoricidal compounds such as ricin and diphtheria toxin, radionuclides, and chemotherapeutic agents have been attempted. To date...

Chemotherapy Principles

Chemotherapy can be defined as administering pharmacologic agents that either destroy tumor cells directly or modify their biology such that tumor growth is impaired. Chemotherapy plays an important role in treating tumors affecting the nervous system. This role is expanding, and many options are now available to treat a wide variety of tumors. For example, whereas chemotherapy has been widely accepted in the management of highly malignant tumors such as glioblastoma multiforme, its use is now...

Malignant Peripheral Nerve Sheath Tumors

MPNSTs include all malignancies arising from the connective tissues of the peripheral nerves. Attempts to subdivide these tumors based on cell of origin have not yielded consistent results. They are extremely rare in the general population, with an incidence of 0.001 2 however, they are a major cause of mortality in individuals with NF1. These patients exhibit a lifetime risk of 8 to 13 .3 In patients with NF1, MPNSTs are thought to arise from preexisting plexiform neurofibromas and...

Intra Arterial Chemotherapy

Intra-arterial chemotherapy is the process by which chemother-apeutic agents are delivered directly into the arterial tumor supply (usually in gliomas) via a microcatheter. Most gliomas are treated by a combination of surgery, radiation therapy, and standard intravenous chemotherapy. However, selective intra-arterial chemotherapy was rendered possible by introduction of the microcatheter and has been undertaken in limited fashion in an effort to expose the tumor to the largest concentration of...

Clinical Presentations Of Tuberous Sclerosis Complex

Vogt's classic triad of seizures, mental retardation, and adenoma sebaceum is present in less than 50 of all cases of TSC. Moreover, a wide variety of clinical manifestations and their variable expressions make the diagnosis more difficult. So in 1992 the National Tuberous Sclerosis Association formulated the diagnostic criteria for TSC.46 For a definitive diagnosis the presence of one primary criterion, two secondary criteria, or one secondary and three tertiary criteria are required (Table...

A

See Anaplastic astrocytoma AA spine, 523 AAV, 94 Abnormal karyotype, 364 ABR testing, 55-56 ABTA, 138 Accelerated fractionation, 72 Accommodative paralysis, 58 Acetaminophen, 103, 143 Acetylcholine (ACH), 103 ACNS 0121, 657 ACNS 0126, 608 ACNS 0231, 609 Acoustic neuroma, 321-329 anesthesia, 324-325 clinical presentation, 322 complications, 326-327 defined, 322 diagnosis, 322-323 functional results rehabilitation, 327-328 outcome quality of life, 328-329 postsurgical treatment, 325-326...

Myxopapillary Ependymoma

Myxopapillary ependymomas occur in the cauda equina and directly involve the filum terminale. Extension may occur into the conus medullaris, along the nerve roots, or through the sub-arachnoid space. The most common initial symptom in children with myxopapillary ependymoma is an exacerbation of low-back pain, but other symptoms include lower extremity weakness, numbness or pain, difficulty with ambulation, and bowel or bladder incontinence.34 Symptoms of 13 months, and up to 18 months, have...

Dermoid And Epidermoid

Dermoids and epidermoids (also referred to as dermoid and epi-dermoid cysts) make up a small subsection of pediatric spinal cord tumors. Constantini and Epstein report an incidence of a little more than 6 in an analysis of their own series and the current literature.7 DeSousa et al found that 8 of 81 (9.8 ) children with spinal cord tumors had either a dermoid or epider-moid.9 Predictably, as age demographics increase, the relative incidence of these two lesions decreases as the number of other...

Multifocal Langerhans Cell Histiocytosis

When making the diagnosis of multifocal LCH, it is important to fully assess the extent of body involvement. The prognosis is thought to be substantially worse in the presence of soft-tissue involvement (e.g., lymph nodes, liver, spleen, skin) than if only multifocal bone lesions are present.12 A typical screening for extent of systemic involvement would include a detailed history and physical examination, complete blood count, coagulation and liver profiles, nuclear scintigraphy, and skeletal...

Lipomas of the Filum Terminale without Spinal Dysraphism

Lipomas of the filum terminale occur at the caudal tip of the spinal canal. These malformations are more common than lipomyelomeningoceles and greater than 90 are recognizable on CT or MRI.23 The finding of a thickened filum terminale and a caudally descended conus medullaris are sufficient for the Patients with lipomas of the filum terminale may have similar symptoms to patients with lipomyelomeningocele and other tethered cord syndromes, including motor weakness, sensory disturbances, bladder...

Histology Light Microscopy

Ependymal cells are ciliated columnar epithelium of neuroec-todermal origin that line the cerebral ventricles and the central canal of the spinal cord. These cells are embryologically related to astrocytes and oligodendroglia but at maturation demonstrate an epithelial appearance. The World Health Organization (WHO) divides ependymomas into ependymomas (WHO grade II), anaplastic ependy-momas (WHO grade III), subependymomas (WHO grade I), and myxopapillary ependymomas (WHO grade I). There are...

Neurofibroma And Schwannoma

Intradural extramedullary neurofibromas and schwannomas are considered together here differences are emphasized when appropriate. Although sporadically occurring spinal schwannomas exist, most of these lesions in children are associated with the autosomal dominant hereditary disorder neurofibromato-sis.19,21 The reader is referred to Chapter 108, Neurocutaneous Syndromes, for further details on the diagnostic criteria and associated central nervous system lesions found in neurofibromatosis type...

Surgical Resection Of Tumor And Other Types Of Mass Lesions

In general, the main steps in resecting peripheral nerve tumors consist of (1) exposing the nerve proximal and distal to the tumor by external neurolysis (2) directly exposing the tumor capsule, usually after incising several encircling connective tissue layers (3) electrically mapping the tumor capsule to localize the distribution of functioning nerve fibers (4) longitudinally (i.e., in the direction of the course of adjacent nerve fibers) cutting the tumor capsule where there are no...

Preoperative Tests

Nerve conduction and electromyography evaluations are not routinely undertaken in the management of peripheral nerve Figure 74-2 Coronal T2- (A) and T1- (B) weighted gadolinium-enhanced magnetic resonance images of a lesion in the right buttock growing rapidly, producing severe neuropathic pain and progressive sciatic neuropathy, in a woman with neurofibromatosis 1. The tumor, which shows regions of signal inhomogeneity and enhancement, is located deep to the gluteal muscle mass adjacent to or...

Therapeutic Options

Therapeutic considerations for patients with intracranial lipomas must be made within a risk-benefit analysis. Although there are limited data on the treatment of intracranial lipomas, surgical resection versus observation alone has been the standard of care. There are no reports of attempts to treat intra-cranial lipomas with radiation or chemotherapy. Because intracranial lipomas are benign entities whose contribution to the patient's underlying symptomatology is often unclear, the...

Amyloidoma of the Peripheral Nerve

Amyloidoma or amyloid tumor consists of a localized deposit of amyloid material, which is composed of extracellular fibril-lar proteins with a b-pleated sheet secondary structure. Amy-loidomas are rare and usually located in the lung and upper respiratory tract. Occasionally, these tumors have been detected in the PNS, most commonly in the trigeminal or gasserian ganglion or one of its three branches.96,129 The etiology and patho-genesis of amyloidomas in peripheral nerves are unknown. Some...

Vascular Tumors or Hemangioma

Hemangiomas of peripheral nerves are rare tumors arising from vascular endothelial cells. Histologically, hemangiomas show erythrocytes populated within thin-wall blood vessels within or surrounding the peripheral nerve.18,75,94 These tumors occur mostly in younger patients. Clinical features include tenderness, pain, and the presence of a mass along with sensory or motor neurologic deficits. The surgical treatment of these tumors is similar to that of lipomas. Local recurrence sometimes...

Angiolipoma

The first spinal angiolipoma was described by Berenbruch in 1890.1,13 Andaluz et al have recently reviewed the subject of angiolipomas within the CNS.1 They reported 94 documented cases 86 within the spinal canal (predominantly extradural) and 8 found intracranially.1 Table 37-3 outlines the 8 reported intracranial angiolipomas. The majority of cases of spinal angiolipomas are found in the posterior epidural compartment. This is in contrast to the infiltrating angiolipomas that occur more...

Arkansas Cancer Research Center Neuro Oncology Program Strategy

What follows is a description of our current treatment philosophy and priority ranking in the Neuro-Oncology Program at the Arkansas Cancer Research Center. A breakdown of our case distribution for each treatment modality over the past 3.5 years according to tumor number and the tumor location relative to eloquent brain is given in Figure 55-6. All patients with breast cancer and solid brain tumor metastases receive WBRT at our institution. A single brain metastasis as the only evidence of...

Immunohistochemical Features

Cytokeratins and vimentin are expressed by virtually all CPPs and most CPCs. GFAP can be found focally in approximately 25 to 55 of CPPs and 20 of CPCs.31 Most of the GFAP-positive cells are simultaneously positive for cytokeratin.33 S-100 protein is present in almost all cases of CPP and less commonly in CPCs. The staining for S-100 is often stronger and more diffuse compared with GFAP staining.33 Synapto-physin has been used as a marker for choroid plexus epithelium, but staining of tumors...

Lipomas of the Spinal Cord Associated with Spinal Dysraphism

The term lipomyelomeningocele refers to a congenital lesion of the meninges or spinal cord plus meninges and the presence of benign fat, both of which exist with a dysraphic spinal column. Depending on the classification system used, these lesions may be divided into as many as five subtypes as they appear on neuroimaging.2 In general, a lipomyelomeningocele is recognized on MRI as a caudally descended conus medullaris invested in fat. Arai et al, using conventional myelogram and MRI, define...

Carcinomatous Meningitis

Breast cancer is one of the most common cancer types leading to leptomeningeal involvement, along with melanoma, lymphoma, and lung cancer. The frequency of leptomeningeal disease in clinical series of breast cancer patients is 2 to 5 , with similar rates of 3 to 6 seen at autopsy. Lobular carcinoma has a predilection to spread to the subarachnoid space, as compared with ductal disease (16 versus 0.3 in one study).35 Patients with solid tumor metastatic brain disease can develop carcinomatous...

Epidermoid and Dermoid

Epidermoid and dermoid cysts are the most common tumors, accounting for up to 60 of the calvarial masses in the pediatric population.52,72 The lesions result when cutaneous ecto-dermal rests are included in the developing cranium. They grow within the diploe and expand and erode the outer and inner tables of the skull. Both epidermoid and dermoid cysts are lined by stratified squamous epithelium and contain keratin from desquamation. Because a dermoid also has skin appendages, its cyst may...

Schwannomas of the Jugular Foramen Nerves

The jugular foramen forms an acute-angle triangle in the skull base. An anterior fibrous or bony band separates the jugular foramen into an anteromedial pars nervosa, which contains the glossopharyngeal nerve, and a posterolateral pars venosa, which contains the vagus and accessory nerves, as well as the jugular bulb. Schwannomas of these nerves are very rare. They usually affect the glossopharyngeal nerve. The nerve of origin in the jugular foramen cannot always be identified (Figure 44-3)....

Chemotherapy

The usefulness of chemotherapy has been established in the pediatric medulloblastoma population. However, for adults with medulloblastoma, the role of chemotherapy is not yet established. The most commonly used regimen is the Packer regimen, which consists of weekly vincristine during CSI and eight cycles of CCNU, cisplatin, and vincristine (CCV) after CSI for children with medulloblastoma. This has become the standard against which all other chemotherapy regimens are measured in the United...

Intradural Intramedullary and Intradural Extramedullary Lipomas Unassociated with Spinal Dysraphism

Spinal cord intradural, intramedullary lipomas are rare entities, usually found along the dorsal aspect of the thoracic spine and in the midline.12,23 In one early report, they were found in decreasing frequency over the cervical-thoracic spine and over the cervical spine. In addition, there are eight reports of intramedullary spinal cord lipomas affecting nearly all levels of the spinal cord, known as holocord lipomas.12 Patients with spinal cord lipomas most often have slowly progressive...

Clinical Presentation

As a first step, it is crucial to obtain a thorough history (including a family history) with a focal and systemic physical examination, including an inquiry and search for the clinical findings associated with NF, because half of the cases are associated with NF1.52 Approximately a third of neurogenic sarcomas arise de novo, whereas the reminder represent a sarcomatous degeneration of a preexisting plexiform neurofibroma in an NF1 or non-NF1 patient.52 Only rarely has neurogenic sarcoma been...

Intracranial Lipoma Embryologic Origin

Theories regarding the pathogenesis of intracranial lipomas have evolved since the first report of a chiasmatic lipoma by Meckel in 1818.23 A contemporary consensus holds that intracranial lipomas result from the abnormal persistence and maldifferentiation of the meninx primitiva during the development of the subarachnoid space.20 This theory, first proposed by Verga in 1929 and well summarized and further supported by Truwit and Barkovich in 1990,20 accounts for several of the distinctive...