References

Albright AL Intraoperative spinal cord monitoring for intramedullary surgery an essential adjunct Pediatr Neurosurg 29 112, 1998. 2. Auberge C, Ponsot G, Lemerle J, et al Intramedullary tumors in children. Apropos of 30 cases . Arch Fr Pediatr 36 1024-1039, 1979. 3. Aysun S, Cinbis M, Ozcan OE Intramedullary astrocytoma presenting as spinal muscular atrophy. J Child Neurol 8 354-356, 1993. 4. Azzarelli B, Luerssen TG, Wolfe TM Intramedullary secretory gangliocytoma. Acta Neuropathol 82 402-407,...

P

P-glycoprotein, 76 P-glycoprotein antagonists, 137 P53, 96, 111, 564 P53 gene mutations, 95 PA. See Pilocytic astrocytoma. Packer CCV regimen, 263, 264 Paclitaxel, 77 Palladium-103, 70 Palliative radiation therapy, 407 Pallister-Hall syndrome, 652 Pamidronate, 408, 523 Panhypopituitarism, 51-52 Papillary craniopharyngiomas, 713 Papillary edema, 324 Papillary ependymomas, 192 Papillary glioneuronal tumor, 224 Papillary meningiomas, 37, 337 Papilledema, 58, 60 Paraganglioma, 82, 84, 366-370, 546...

Neurofibroma Pathology

The WHO classification system divides neurofibromas into two types a general type, also referred to as solitary, circumscribed, or globular, and a plexiform type (Figure 73-4). The gross appearance of both types of neurofibromas can be similar to schwannomas. In the skin and subcutaneous fat, solitary neu- rofibromas are well circumscribed but may not be encapsulated, as well as being firm with pale gray and translucent cut sur-faces.11,68,93 Neurofibromas developing in deeper nerves are more...

Local Or Intratumoral Chemotherapy

The preceding discussion has focused on systemic chemotherapy delivery. However, local delivery may be an attractive alternative for patients with CNS tumors.12 These tumors are locally recurrent and rarely spread outside the CNS. Local high-dose chemotherapy without systemic administration may be efficacious but avoid systemic toxicities. Furthermore, local delivery may circumvent the BBB. The simplest means to deliver chemotherapeutic agents locally to brain tumors has been direct injection,...

Outcome and Quality of Life

Acoustic neuroma can be cured by complete tumor resection via the suboccipital approach, and the patients will be able to return to their previous quality of life in most cases. Facial nerve function is regarded as the most important outcome criterion by all patients. Some patients who show good recovery will nonetheless suffer from side effects such as synkinesia and aberrant innervation of tear secretion. Synk-inesia can be treated successfully with specialized physiotherapy or with muscular...

Posterior Fossa Meningiomas

Tentorial Meningioma

Posterior fossa meningiomas account for less than 10 of total intracranial meningiomas. Half of these are in the cerebello-pontine angle 40 occur at the tentorium or cerebellar convexity. Approximately 9 are clival and 6 occur at the foramen magnum. Petroclival meningiomas arising medial to the trigeminal nerve have a higher rate of surgical morbidity (Figure 45-11).12 Meningiomas of the petrous pyramid can cause hearing loss, facial pain, and facial weakness. Headaches and ataxia are common...

Diagnosis Clinical Presentation

The clinical characteristics of a glomus jugulare tumor depend on its locally invasive behavior, its anatomic extension, the size of the tumor, and whether it secretes neuropeptide hormones. Most commonly, patients have hearing loss and pulsatile tinnitus or dizziness. Hearing loss is usually unilateral and results from invasion of the middle ear. It can be conductive if the ear canal is obstructed or sensorineural if the cochlea or labyrinth is invaded, and is often accompanied by dizziness....

Charcot MarieTooth Disorder

Charcot-Marie-Tooth (CMT) disorders, also known as hereditary motor and sensory neuropathy or peroneal muscular atrophy, represent an inherited group of neuropathies, some of whose genetic mutations have been characterized. These disorders are associated with diffusely enlarged peripheral nerves, most commonly involving the peroneal nerve. Prevalence of CMT disorder is 1 person per 2500 in the population, or approximately 125,000 patients in the United States. Patients with CMT usually have a...

Rehabilitation Outcome

Outcome depends on size, location, and pathologic type of the tumor. Because most of the lesions treated surgically are either of low-grade malignancy or are benign, the recurrence rates are low (26 ). Surgical mortality and morbidity are also low in the hands of an appropriate team. Because long-term survival is common, the choice of surgical approach and the avoidance of new deficits is very important. This end is attained by having a clear plan of treatment before the initiation of any...

Vinca Alkaloids and Epipodophyllotoxins

Vincristine and vinblastine are vinca alkaloids that act on tubulin to inhibit microtubule assembly. They result in S-phase-specific mitotic arrest. Resistance is mediated by the p170 membrane glycoprotein. These agents are water soluble, must be given intravenously, and have poor BBB penetration.8 Toxicities are primarily neurologic, including peripheral neuropathy and (as a result) constipation. Vinblastine is seldom administered to patients with nervous system tumors. Vin-cristine is...

Phase I Cancer Trials

Phase I studies have the primary objective of establishing a phase II dose.15 For cytotoxic agents, the phase II dose is assumed to be the maximally tolerated dose (MTD) based on dose-limiting toxicities (DLTs). For some phase I cancer studies, the selection of patients may be important. Although patients with refractory disease are often included, the important factor is normal organ function. In some instances, patients who have previously been heavily pretreated with other agents may be...

Phase Ii Cancer Trials

Once a therapeutic dose has been determined, the next step in evaluation of a treatment is to determine if there is evidence of efficacy. Although response to therapy can be described in a phase I study, this is not the primary objective. Also, phase I trials tend to be performed in patients with refractory cancers of various types, so the determination of benefit among a specific tumor type is limited by small numbers and multiple dose levels. In phase II studies, a specified tumor type is...

Brain Metastasis From Nonsmall Cell Lung Cancer

Mamelak, Fredric Grannis, Robert Morgan, Jr., and Richard D. Pezner Brain metastases from tumors arising outside the central nervous system are exceptionally common. There are an estimated 75,000 to 100,000 cases of brain metastasis each year in the United States. Approximately 30 to 60 of all brain metastases originate in a primary lung cancer. In light of the marked frequency with which this illness is encountered in general oncology practice, a thorough understanding of the...

Nonsmall Cell Lung Cancer Staging

Metastatic spread of NSCLC is very common, with bone, adrenal, and brain being the most common sites.14 Brain metastases occur in approximately 33 of patients. Solitary metastases are noted in approximately 30 of cases. NSCLC stages are typically designated I to IV, including subsets IIA, IIB, IIIA, and IIIB. Staging is determined using standard tumor, node, and metastasis (T, N, M) criteria. The extent of staging tests performed depends on a number of factors, including the presenting...

Locally Advanced and Inflammatory Breast Cancer

Current data suggest that patients with advanced stage III disease who are at least T3 (> 5 cm in maximal diameter), T4 (extension to chest wall or skin), or N2 (fixed or matted axillary lymphadenopathy) are best treated with preoperative chemotherapy or hormonal therapy, followed by surgery and then local-regional fractionated radiation therapy. Limited data support the addition of adjuvant chemotherapy or hormonal therapy postoperatively and after radiation therapy. Excellent local control...

Relationship With Specific Systemic Cancers

In patients with an unknown primary site and brain metastases, the lung represents the most common primary tumor site discovered antemortem, accounting for between 45 and 83 of identifiable primary sites (see Table 60-2). The lung also appears to be the most common primary site regardless of whether it becomes known early (within 2 months) or late (after 2 months) after the diagnosis of brain metastases.33,46 In contrast, when the primary site is discovered only after autopsy, there does not...

Management Of Cervicomedullary Tumors

In the past, intrinsic brainstem tumors as a group were treated with irradiation and adjunctive chemotherapy with relatively little success.5 With MRl allowing better differentiation among the different groups of brainstem tumors, the cervicomedullary tumor group has been found to significantly benefit from surgery.5-7,23,25 Although adults with cervicomedullary tumors have been treated successfully with observation or radiation,14 surgery is recommended in children.7,17 If the patient has...

Hypercortisolemia Corticotroph Adenomas and Cushings Disease

The first step in diagnosing a patient with hypercortisolemia (Cushing's syndrome) is to find the etiology. In the subset of causes of Cushing's syndrome in the pediatric population, 85 of cases resulted from Cushing's disease.8 Patients diagnosed with Cushing's disease, thus having ACTH-releasing corti-cotroph adenomas, exhibit cushingoid features. Most often, these features in children are short stature, rapid weight gain, menstrual irregularities, skin striae, and mental status changes.9 To...

Classification And Clinical Presentation

Many classification schemes have been used to stratify pituitary tumors. These have included clinical, pathologic, and imaging-based classifications, many of which are still in use informally. In an attempt to standardize reporting, the World Health Organization approved a five-tier classification based on (1) clinical presentation and secretory activity, (2) size and invasiveness (i.e., microadenoma versus macroadenoma), (3) histologic features, (4) immunohistochemical profile, and (5)...

Complications of Radiation Therapy

Radiation therapy results in endocrine dysfunction and visual defects similar to those observed following surgery, but the severity of these complications, particularly with respect to diabetes insipidus, appears to be reduced. In a retrospective study of 72 patients at UCSF from 1972 to 1999 treated for initial disease, 32 had visual deficits after subtotal resection followed by irradiation, although 81 of these had visual deficits before treatment, and 72 retained the same functional status....

Malignant Peripheral Nerve Tumors

Mubarak Al-Gahtany, Rajiv Midha, Abhijit Guha, and W. Bradley Jacobs I Peripheral nerve tumors are rare lesions that can arise anywhere on the body and as a result have a wide differential diagnosis. They commonly occur as a nonspecific mass lesion that is diagnosed as a peripheral nerve tumor at surgery. Although these tumors may initially be seen by a wide variety of surgeons, early recognition of the nature of the lesion and appropriate surgical treatment by a surgeon with expertise in...

Peripheral Nerve Tumors In Children

Bradley Jacobs, Rajiv Midha, and Mubarak Al-Gahtany Peripheral nerve tumors (PNTs) are not common lesions. Although it is widely recognized that these lesions do occur in the pediatric population, PNTs occur with less frequency there than in the adult population. The differential diagnosis of a suspected PNT in a child follows the same general framework as for adult patients (Table 107-1), but there are several key differences between PNTs in the pediatric population and their adult...

Malignant Peripheral Nerve Sheath Tumors

MPNSTs are nonrhabdomyosarcomatous soft-tissue tumors that arise from the peripheral nerve sheath. Cases in children and adolescents are not common and account for only 13 of all MPNSTs8 and 12 of all peripheral neurogenic tumors of childhood.6 The association of MPNST with NF1 is well established, with MPNSTs occurring in the setting of NF1 in 50 of cases.25 Pediatric MPNST case series reveal that 29 to 66 of pediatric MPNSTs arise in the setting of NF1,5,8,18 comparable to the overall...

Whole Brain Radiation Therapy

The use of fractionated WBRT improves expected patient median survival from 1 to 2 months to 4 to 6 months.80,95 130 Further, it reduces the likelihood of dying directly as a result of CNS disease from 95 to 100 to approximately 50 .95130 Although this is a statistically significant improvement in both overall survival and quality of life achieved (through a reduction in CNS-related death), the magnitude of this effect is modest at best. Once it develops, the brain tumor remains the rate...

Radiologic Characteristics

What Does Brain Mri With Contrast Show

The radiologic characteristics of primary thalamic tumors, although suggestive of certain histologic subtypes, should never replace actual tissue sampling. In addition to being indicative of certain tumor histologies, the radiologic features become very important from the standpoint of dictating therapeutic options. These features are based principally on growth characteristics, because they relate to tumor infiltration and potential for surgical extirpation. Computed tomography (CT), although...

Pharmacologic Interventions

Pharmacologic interventions may be extremely helpful in improving a patient's function and general well-being. Clinicians often use psychostimulants to treat the declines in cognitive and emotional functioning that are so ubiquitous among brain tumor patients. Psychostimulants are effective, in part, because they counter the effects that tumor and treatment produce on the monoamine pathways of the frontal-brainstem reticular system. Methylphenidate, dextroamphetamine, and pemoline have each...

Systemic Chemotherapy General Pharmacologic Principles

For chemotherapy to be effective, it must be delivered to sensitive tumor cells in adequate concentrations. For nervous system tumors, as with all neoplasms, choosing appropriate chemotherapy administration routes and schedules is important in achieving adequate concentrations. In addition, CNS tumors have unique features, such as the blood-brain and blood-tumor barriers, that affect chemotherapeutic options. Systemic chemotherapy may be given orally or intravenously. Oral bioavailability is...

Chemotherapy General Considerations

AAs represent approximately 25 of malignant gliomas. Because they are less common, they typically compose a small part of the study group in clinical trials for malignant gliomas. Misclassification has been problematic as well. In a large phase III trial of PCV chemotherapy with or without BUdR as a radiation sensitizer, 78 of 268 patients enrolled were ineligible primarily on the basis of central pathology review findings, the most common scenario being that tumors classified as anaplastic at...

Chemotherapy for Brain Metastases

Systemic cytotoxic chemotherapy has not been extensively evaluated in the treatment of patients with brain metastasis. A study published in 1986 showed significant response rates in a fairly heterogeneous group of patients with brain metastases due to breast cancer.107 Approximately 63 of these patients had received prior adjuvant chemotherapy, primarily CMF (cyclophosphamide, methotrexate, and 5-Fu) or cyclophos-phamide and doxorubicin. Response rates, including partial and complete responses,...

Adjuvant Radiation Therapy And Chemotherapy

High-grade gliomas in children have conventionally been treated with maximal resection followed by radiation therapy to the tumor bed and a margin of surrounding brain, with a dosage of 5000 to 6000 cGy in 180 to 200 cGy day fractions. Cooperative group studies have demonstrated that the administration of chemotherapy in addition to irradiation improves the chances for long-term survival, although to date the optimal treatment regimen remains uncertain. In the CCG-943 study, children who were...

Chemotherapy

Management options for adults with low-grade astrocytomas (LGAs) include observation, surgery, radiation, or chemotherapy, but there is no well-established standard approach. Patients who have undergone a complete radiographic resection of their tumor are often followed with surveillance MRI scans and treated at progression. In the past, patients who have undergone subtotal resections have been treated with radiation therapy. However, there is now a trend to avoid up-front radiation based on an...

Clinical Trials And Treatment Response

Chemotherapeutic agents generally undergo extensive preclin-ical and clinical studies before becoming widely available. The transition from experimental agent to standard treatment is based on progression through a series of clinical trials. Phase I clinical trials are generally conducted with few patients and are designed to determine toxicity and maximum tolerated dose. Once an appropriate starting dose has been determined, phase II trials are conducted to measure treatment response in a...

Multimodality Treatment Intent And Timing

Patients with nervous system tumors usually receive chemotherapy as part of a multimodality treatment approach also involving surgery and radiation therapy. Chemotherapeutic agents may be given alone or in combination. Combining agents with different mechanisms of action and different toxicities is attractive and has been effective in many tumor types. Unfortunately, few chemotherapy combinations have proved more effective than single agents for nervous system tumors. Chemotherapy can be...

Symptom Clusters That Affect Quality Of Functional Outcomes

Patients with brain tumors experience a number of adverse symptoms that negatively affect their ability to function in usual work, leisure, and social roles. These include cognitive impairment, neurologic signs and symptoms, fatigue, mood disturbance, sleep disturbance, and sexual dysfunction. These symptoms occur in a majority of patients on active therapy, and occasionally are the first symptoms that herald the initial diagnosis. In addition, these symptoms often persist after treatment is...

Treatment Specific Factors

Radiation therapy to the brain is widely known to cause injury to white matter and cognitive impairments related to frontal-subcortical dysfunction. It also causes significant fatigue in fact, cancer patients receiving radiation therapy to non-CNS sites (e.g., prostate or breast cancer) also experience fatigue during treatment that is centrally mediated. Induction of inflammatory cytokines, disruption of the hypothalamic-pituitary-adrenal axis, and alterations of neurotransmitters have been...

Histopathologic Diagnosis Classification and Grading

The generic reference to astrocytomas is that of the diffuse infiltrating, predominantly supratentorial tumor derived from fib-rillary astrocytes. In their normal state, astrocytes are readily found in the neuropil, with many stellate processes, often reaching out to extend foot-processes that rest on nearby blood vessels. The neoplastic transformation of these cells is responsible for the widest range of clinical presentations, an array of histologic features and wide-ranging prognostic...

UpFront Therapy for Anaplastic Astrocytoma

The Northern California Oncology Group (NCOG) stratified a chemotherapy trial based on tumor type. The investigators first compared single-agent BCNU with pCv in patients with malignant glioma. PCV was found to be superior to BCNU but only in the patients with AA.76 This difference was observed in a retrospective analysis of the data. The number of patients with AA was relatively small 37 in the BCNU arm and 36 in the PCV arm. This advantage was not observed for patients with GBM. Retrospective...

Desmoid Tumors

Desmoid tumors, also known as aggressive or deep-seated fibromatosis, are benign fibrous tumors of mesenchymal origin usually located in muscle, particularly in the abdominal wall. When located at extra-abdominal sites such as in the neck, shoulder, or limbs, they often compress, envelop, or infiltrate peripheral nerves such as the brachial plexus, peroneal nerve, radial nerve, and sciatic nerve. Desmoid tumors are considered benign, because they do not metastasize to other parts of the body....

Vestibular Schwannomas

The terminology for acoustic neuroma tumors has undergone several changes along with discoveries through pathology, specifically electron microscopy. The term neuroma refers to hyperplastic proliferation of nerve fibers and nerve sheaths induced by trauma and therefore is not adequate for these neoplasms. The term neurilemoma has been left, because the neurilemma is the Schwann cell plasma membrane, its lamina, and connective tissue ground substance. Acoustic neuromas are correctly termed...

Management

MPNSTs are rare tumors that remain incurable, mainly because of their high metastatic potential, and are managed by several surgical disciplines, including plastic, orthopedic, and neuro-surgery, depending on local interest and expertise. The rarity of MPNSTs usually renders them to be managed according to clinical protocols used for the much more common soft-tissue sarcomas (STSs), of which MPNSTs constitute between 3 and 10 .11,17,26,42 Whether these protocols are the most appropriate for...

Neuromuscular Hamartoma

Neuromuscular hamartomas, also known as benign triton tumors, are characterized by skeletal muscle fibers mixed within bundles of disorganized nerve fascicles, containing myelinated and nonmyelinated nerve fibers. They are very rare peripheral nerve tumors, with only 20 cases reported in the literature. The exact histogenesis of these neoplastic lesions remains unclear, but they may be due to the concurrent proliferation of neuroectodermal-derived or limb-associated meso-dermal tissue in a...

Clinical Presentation and Evaluation

Perineuriomas, also called storiform perineurial fibromas, are rare benign peripheral nerve tumors that occur mostly in adolescents and young adults. The chance of developing these tumors is slightly higher in females. Commonly they are a mononeuropathy, arise from soft tissues deep in the extremities, and are not associated with NF1.3,1718 Extraneural or soft-tissue perineuriomas, the more common form of perineuriomas, are benign tumors that are usually not associated with peripheral nerves...

Outcomes

The histologic subtype may be the most significant predictor of prognosis after treatment in cervicomedullary tumors.10 Bricola reports in his series of children and adults with cervi-comedullary tumors that 6 of 12 patients (one ependymoma, five benign astrocytomas) made excellent neurologic recoveries, although the remainder (four malignant gliomas, one angioreticuloma, and one lipoma) showed no appreciable improvement.2 In Epstein's series,7 there were 24 patients with cervicomedullary...

Other Peripheral Nerve Tumors Not Included In The Who Classification System

We have described benign peripheral nerve tumors arising primarily from Schwann cells, in the case of schwannomas and neurofibromas, and perineural cells in the case of perineuri-omas. Other tumors arising from cells normally found in peripheral nerves that will be discussed include mucosal neuroma, neurothekeoma, granule cell tumor, lipoma, vascular tumors, and neuromuscular hamartoma. Peripheral nerve tumors can also originate from cells not normally found in nerves by local invasion, as in...

Clinical Presentation

More than 30 of patients with intracranial lipomas are asymptomatic.23 The vast majority of the remaining patients have signs and symptoms not attributable to the lipoma but secondary to the associated developmental anomalies. Approximately 30 of patients with supratentorial lipomas have a seizure disorder. The seizure disorder is reportedly often severe, is focal or generalized, and has an average onset age of 15 years. Recurrent headaches are a presenting symptom in 25 percent, and . . . 10...

Gross and Microscopic Appearance

Intracranial lipomas appear as dark-yellow lesions on gross inspection. The meninx primitiva contains within it primitive perivascular reticuloendothelium,6 which may become specialized in the storage of fat, and this may account for the histology of intracranial lipomas. Early precapillary parenchyma may also differentiate into fat cells. Microscopic examination reveals that these lesions are composed of mature adipose cells with peripheral, sometimes indented, small bland nuclei. A...

Miscellaneous Intramedullary Pathology

Metastases account for approximately 2 of intramedullary tumors. This low prevalence is likely due to the small size of the spinal cord and its poor vascular accessibility to hematoge-nous tumor emboli.12 Other non-neoplastic entities can occur as intramedullary spinal lesions.33,38 Vascular malformations, particularly cavernous angiomas, may occur in the spinal cord. Inclusion tumors and cysts are rarely intramedullary. Lipomas are the most common dysembryogenic lesion and account for...

Lipoma

Lipomas, also known as lipofibromatous hamartomas or neural fibrolipomas, are benign tumors arising from the proliferation of adipose or fibrous tissues. Grossly, they are well circumscribed and encapsulated and have a fusiform and yellowish appearance in the subcutaneous layer.92 Histologically, lipomas can be intimately associated with functioning nerve fibers from which they cannot be easily separated. The median nerve is most commonly affected. Larger lipomas can envelop, infiltrate, or...

Prophylactic Cranial Irradiation

The high frequency of brain metastases in patients with SCLC has led to multiple evaluations of PCI. Randomized trials in the 1970s and 1980s evaluating the role of PCI for SCLC have consistently shown decreases in the rate of brain metastases but no clear improvement in survival. In a large, randomized trial of PCI (294 patients), Arriagada3 showed a 45 incidence of brain metastases over 2 years in SCLC patients treated with 2400 cGy in eight fractions, versus a 67 recurrence rate in the...

Fallopian Tube Cancer

Primary tumors of the fallopian tube are extremely rare entities, accounting for less than 1 of gynecologic malignancies.37 These tumors appear to behave in a manner similar to ovarian cancer and are classified and treated in much the same way. Some fallopian tube neoplasms may be prone to early blood-borne micrometastasis, resulting in poor long-term disease-free survival and contributing to the formation of brain metasta-sis.37,47 The few cases of such brain metastases reported in the...

Spontaneous Cerebellar Astrocytoma

In children, astrocytomas constitute approximately 35 to 47 of all primary brain tumors.18,44 They can arise within the optic pathway (5 ), hypothalamus (10 ), cerebellum (15 to 25 ), cerebral hemisphere (12 ), spine (10 to 12 ), or brainstem (12 ).36 Cerebellar astrocytomas represent 12 to 17 of the central nervous system (CNS) tumors in children15 and 25 to 35 of pediatric tumors of the posterior fossa.13 The incidence of cerebellar astrocytomas is highest between ages 4 and 10 years, with a...

Immature Teratoma

In this tumor, one finds mature teratoma along with primitive, malignant elements. Even after total gross-total resection, most cases show recurrence, and adjuvant therapy is essential to prolong survival. If serum HCG or AFP levels are elevated, prognosis is generally less favorable. However, Yoshida et al have described immature teratomas in which HCG and AFP tumor makers are negative, and the tumors are resistant to PE combination chemotherapy.25 Matsutani et al analyzed the long-term...

Asymptomatic Colloid Cysts

Colloid cysts are being discovered incidentally with increasing frequency. Often CT or MRI is performed because of chronic headache in the absence of other symptoms or signs, and in some cases the headaches are not attributed to the cyst or the patient does not have headache. Whether the cyst should be treated is based on various factors, including the size of the cyst, age of the patient, and whether there is associated hydro-cephalus on the CT or MRI scan. In most cases, consultation with a...

Parasagittal and Falcine Meningiomas

Commonly, patients with parasagittal and falcine meningiomas in the anterior third of the falx experience headaches. Gradual progressive mental-status changes, seizures, or increased intra-cranial pressure can also occur. Focal seizures are often seen in patients with meningiomas of the middle third of the falx. They are initially evident in the contralateral foot and leg. Progressive mental decline, visual deterioration secondary to increased intracranial pressure, or generalized seizures can...

Tumor Histology

The histopathologic characteristics of intramedullary spinal cord tumors are not so distinct from those of their intracranial counterparts that description of the microscopic appearances of each type needs repetition in this chapter. The reader may look into the chapters on each corresponding intracranial tumor type elsewhere in this text. Table 106-1 is a list of the various histologies that have been described for intramedullary tumors in childhood with selected citations. A recent trend in...

Choroid Plexus Papillomas

A choroid plexus papilloma is an unusual tumor that arises from choroid plexus cells within the cerebral ventricles. The normal function of the choroid plexus is to produce cerebrospinal fluid (CSF). These highly vascular tumors are usually identified in infants and children, are most often found within the lateral ventricles, and can produce hydrocephalus or ventriculomegaly from ventricular obstruction or overproduction of CSF.22 Imaging studies show a contrast-enhanced mass with irregular...

Normal Anatomy

Neurofibroma Cross Section

The pathology of peripheral nerve tumors can best be understood by studying the normal anatomy and cellular elements that make up a peripheral nerve (Figure 73-2), the cells from which the different types of intrinsic peripheral nerve tumors arise. In contrast, extrinsic tumors arise either from adjacent tissues with local spread or, in the case of metastatic tumors, from cells at a distance. Peripheral nerves are part of the PNS. These axonal pathways connect the brain and spinal cord with...

Germinoma with Syncytiotrophoblastic Giant Cells

Approximately 13 of germinomas contain syncytiotrophoblastic giant cells (STGC) positive for HCG-b.21,22 This type of tumor has been shown to have a different response to chemoradiation therapy. Although the response rate is high, the FIGURE 96-1 A, a 14-year-old boy complained of visual disturbance. Magnetic resonance imaging (MRI) with gadolinium enhancement revealed an enhanced solid mass in the suprasellar (neurohypophysis) region. Histologic examination revealed an immature teratoma....

Tuberous Sclerosis Complex

Tuberous sclerosis complex (TSC), or Bourneville's disease, is an inherited disorder resulting in hamartomatous changes in multiple organ systems, including the brain, kidneys, heart, retina, and skin. The name refers to tubers (areas of swelling or protuberances) and areas of sclerosis, or hardening, in the cortical gyri. In addition to the hamartomatous growths, true neoplasms also occur in TSC, in particular within the brain and kidneys. This disorder was first described by von...

Parasellar or Sphenocavernous Syndrome

Parasellar or sphenocavernous syndrome usually results from metastases to the bone of either the petrous apex or the sella Manifestations of Syndromes Involved in Skull Base Metastases Posterior Skull Base Jugular foramen Extraocular muscles CNs III, IV, Vi, and VI sensory and motor) Possible CNs III, IV, VI, Supraorbital and orbital pain Blurred vision Proptosis No diplopia External ophthalmoplegia Enophthalmos Facial pain numbness along CN V,, 2 Numbness, paresthesias, and pain along CN V2, 3...

Ganglion Cyst

Ganglion cysts are cystic structures filled with viscous fluid and lined by fibrous hypocellular tissue. They are often a palpable mass that may produce symptoms and functional deficits in the involved peripheral nerve. They usually arise from joints or tendon sheaths and may directly or indirectly involve peripheral nerves and thereby cause neurologic symptoms and deficits. These cysts are most commonly located extraneurally, but examples of intraneural ganglion cysts developing within a...

Pineal Parenchymal Tumors

Pineoblastomas commonly affect infants and young children. They tend to recur and disseminate. Pineocytomas, however, are considered benign, and surgical excision can lead to cure. Histologically, pineoblastomas may have multiple cellular differentiations. Among these, neuronal differentiation in pineoblastomas is considered an indicator of benign progno-sis,35 although others did not observe a correlation between the prognosis and the degree of differentiation.32 Nevertheless, the prognosis of...

Optic Pathway Gliomas

Optic Nerve Pathway Gliomas Image

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...

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

Schwanoma Dumbell

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...

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

Tuberous Sclerosis

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

Treating Lipomas Naturally

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

Metastatic Brain Tumours

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)....

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...

Mecanism Of Centripetal Lipid Accumulation Supraclavicular Fat And Face

The pituitary gland is often considered the master gland, regulating most of the body's hormonal balance. The gland itself is regulated by the hypothalamus through stimulatory and inhibitory hormones that travel through the infundibulum and pituitary stalk. The adult pituitary gland measures 12 x 6 x 9 mm and weighs 0.6 g. It enlarges during pregnancy, when it may weigh 1 g or more. The gland is situated near the sella turcica (Turkish saddle) formed by the sphenoid bone and is completely...

Intracranial Lipoma Embryologic Origin

Intracranial Lipoma Sites

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...