Rare indolent tumors comprised of neoplastic glial and neuronal cells which occur primarily in children and young adults. Benign lesions tend to be associated with long survival unless the tumor degenerates into a histologically malignant form. They tend to occur in the optic nerve and white matter of the brain and spinal cord.
Loss of structural differentiation and useful function of neoplastic cells.
A disorder characterized by recurrent partial seizures marked by impairment of cognition. During the seizure the individual may experience a wide variety of psychic phenomenon including formed hallucinations, illusions, deja vu, intense emotional feelings, confusion, and spatial disorientation. Focal motor activity, sensory alterations and AUTOMATISM may also occur. Complex partial seizures often originate from foci in one or both temporal lobes. The etiology may be idiopathic (cryptogenic partial complex epilepsy) or occur as a secondary manifestation of a focal cortical lesion (symptomatic partial complex epilepsy). (From Adams et al., Principles of Neurology, 6th ed, pp317-8)
Neoplasms of the intracranial components of the central nervous system, including the cerebral hemispheres, basal ganglia, hypothalamus, thalamus, brain stem, and cerebellum. Brain neoplasms are subdivided into primary (originating from brain tissue) and secondary (i.e., metastatic) forms. Primary neoplasms are subdivided into benign and malignant forms. In general, brain tumors may also be classified by age of onset, histologic type, or presenting location in the brain.
Primary and metastatic (secondary) tumors of the brain located above the tentorium cerebelli, a fold of dura mater separating the CEREBELLUM and BRAIN STEM from the cerebral hemispheres and DIENCEPHALON (i.e., THALAMUS and HYPOTHALAMUS and related structures). In adults, primary neoplasms tend to arise in the supratentorial compartment, whereas in children they occur more frequently in the infratentorial space. Clinical manifestations vary with the location of the lesion, but SEIZURES; APHASIA; HEMIANOPSIA; hemiparesis; and sensory deficits are relatively common features. Metastatic supratentorial neoplasms are frequently multiple at the time of presentation.
Primary or metastatic neoplasms of the CEREBELLUM. Tumors in this location frequently present with ATAXIA or signs of INTRACRANIAL HYPERTENSION due to obstruction of the fourth ventricle. Common primary cerebellar tumors include fibrillary ASTROCYTOMA and cerebellar HEMANGIOBLASTOMA. The cerebellum is a relatively common site for tumor metastases from the lung, breast, and other distant organs. (From Okazaki & Scheithauer, Atlas of Neuropathology, 1988, p86 and p141)

Ictus expectoratus: a sign of complex partial seizures usually of non-dominant temporal lobe origin. (1/68)

Spitting (or expectoration) is rarely seen with seizures. In Western society, spitting is a striking behavioral aberration. A 13-year-old child had intermittent agitated behavior, episodes of rage, spitting and confusion lasting up to 2 minutes. He stood up in church and told the preacher to 'shut up and sit down'. Epilepsy monitoring revealed spitting with polysharp and spike seizures resolved over the right temporal lobe. Magnetic resonance imaging revealed a right temporal lobe ganglioglioma. Spitting seizures resolved after resection. Ictal expectoration is rare. It may occur with epigastric aura, nausea, chewing, swallowing and fumbling. Literature review disclosed 17 cases, 12 of which arose from the non-dominant hemisphere. Most regressed with surgery and anticonvulsants.  (+info)

Ganglioglioma of the optic chiasm: case report and review of the literature. (2/68)

We report a case of a hypothalamic chiasmatic ganglioglioma in a 21-year-old woman who presented with hyperprolactinemia and developed visual field defects. This circumscribed cystic lesion with an enhancing mural nodule was radiologically indistinguishable from a pilocytic astrocytoma. Although rare, gangliogliomas should be included in the differential diagnosis of lesions occurring in this area of the brain.  (+info)

Mixed conventional and desmoplastic infantile ganglioglioma: an autopsied case with 6-year follow-up. (3/68)

We describe a case of desmoplastic infantile ganglioglioma (DIG) arising in the ventral diencephalon of a 3-1/2-month-old boy. On biopsy, the tumor featured a desmoplastic, S-100 protein and GFAP immunoreactive stromal element, as well as a variable spectrum of ganglion cells. Electron microscopy demonstrated astrocytes, and morphologically fibroblasts, as well as neurons containing 120-nm dense core granules. In addition, tubular structures composed of tightly apposed cells with features of astrocytes and of Schwann-like cells were also noted. Devoid of fibroblasts, the tubular structures were surrounded by a single basal lamina. At autopsy 6 years later, the multinodular, cystic mass had replaced the diencephalon, extended into both temporal lobes as well as the optic nerves, and showed marked leptomeningeal involvement. Microscopically, superficial portions of the tumor consisted of typical DIG, whereas deep, nondesmoplastic portions exhibited pattern variation ranging from pilocytic astrocytoma to ganglioglioma and gangliocytoma. There was also a minor element of small, 'primitive-appearing' neuroepithelial cells. Dysplastic ganglion cells variously reactive for neurofilament protein and synaptophysin were present throughout the tumor. Our study not only confirms DIG as a variant of ganglioglioma, one capable of slow growth, infiltration, and fatal progression but suggests that its differentiating potential includes elements of both the central and peripheral nervous systems. If so, their derivation may be from multipotential cells of the neural plate.  (+info)

Cerebellopontine angle ganglioglioma: MR findings. (4/68)

We present a case of cerebellopontine (CP) angle ganglioglioma in a young child with developmental delay and no trigeminal nerve symptoms. MR imaging demonstrated a mass of homogeneous low signal intensity in the left CP angle on T1-weighted images with no enhancement with gadolinium, and of relatively homogeneous high signal intensity on T2-weighted images.  (+info)

Ex vivo pediatric brain tumors express Fas (CD95) and FasL (CD95L) and are resistant to apoptosis induction. (5/68)

Fas (APO-1/CD95/TNFRSF6) is a member of the tumor necrosis/nerve growth factor receptor family that signals apoptotic cell death in sensitive cells. Expression of Fas and its agonistic ligand (FasL/TNFSF6) was investigated in ex vivo pediatric brain tumor specimens of various histologic types. Fas expression was identified in all of the 18 tumors analyzed by flow cytometry and immunohistochemistry. FasL expression was identified in most of the 13 tumors analyzed by both Western analysis and immunohistochemistry. Nine of these tumor specimens were treated with either the agonistic anti-Fas antibody (APO-1) in combination with protein A or FasL in short-term cytotoxicity assays. Sensitivity to apoptosis induced by the topoisomerase II inhibitor, etoposide, was also assessed. Despite the presence of Fas, all the specimens analyzed demonstrated a high degree of resistance to Fas-mediated apoptosis. These 9 specimens also showed a high degree of resistance to etoposide. Only 2 of the 9 specimens were susceptible to etoposide-induced cell death, whereas only 3 were sensitive to Fas-mediated apoptosis. One brain tumor was sensitive to both Fas ligation and etoposide treatment. This contrasted with the high degree of susceptibility to both etoposide- and Fas-induced apoptosis observed in the reference Jurkat cell line. The results suggest that Fas expression may be a general feature of tumors of the CNS and that a significant degree of resistance to Fas-mediated apoptosis may exist in ex vivo pediatric brain tumor specimens.  (+info)

Language processing during natural sleep in a 6-year-old boy, as assessed with functional MR imaging. (6/68)

Functional MR imaging for language lateralization was performed in a 6-year-old child before neurosurgical intervention. A passive story-listening task was used; this revealed a bilateral language network. The task was repeated during the same session when the child had fallen asleep and surprisingly yielded strong activation in similar language areas. Our findings suggest that language processing does occur during natural sleep, even in young children. This potentially allows for an assessment of language functions, even in sleeping children.  (+info)

Gangliogliomas: A report of five cases. (7/68)

Gangliogliomas are rare tumors of the Central Nervous System. Five gangliogliomas were diagnosed out of 1560 brain tumours surgically resected out in a period of 5 years accounting for 0. 32%. We have tried to discuss in detail the pathological features of these tumours and have mentioned the clinical and radiological features associated with them. All the slides, tissue blocks and pathology reports of the surgical specimens of gangliglioma were reviewed and the clinical and radiological data reviewed. The ages of the patients ranged from 7-65 years with 4 males and 1 female. The tumors were located in the lateral ventricle (a rare site), temporal, parietal and the frontal lobes with duration of seizures varying from 1-9 years. The tumors were diagnosed by the presence of a dual population of neoplastic ganglionic and glial components. The glial components consisted of pilocytic astrocytes (l case), fibrillary astrocytes (2 cases), oligodendrocytes (1 case) and anaplastic astrocytes and oligodendrocytes (1 case). There was one-grade I GG, three-Grade II GGs and one-grade III GG. Astrocytes were the commonest glial component of GGs, either pilocytic or fibrillary. Oligodendrocytes as the glial component of GGs was seen in 2 cases one of which was anaplastic and this is a rare finding.  (+info)

MR imaging in the diagnosis of desmoplastic infantile tumor: retrospective study of six cases. (8/68)

BACKGROUND AND PURPOSE: Desmoplastic infantile tumors (DITs) are rare supratentorial tumors of infancy with a favorable prognosis. Radiologic and histologic features of DIT are misleading, and DIT may be misinterpreted as a malignant lesion. We have studied the usefulness of MR imaging in the diagnosis of these tumors. METHODS: Between 1995 and 2002, six DITs were diagnosed in young children at our institution. Neuroimaging, age at diagnosis, sex, clinical presentation, symptoms duration, follow-up, and development were studied retrospectively. Contrast-enhanced CT and MR images were available. MR study included T1-, T2-, and postgadolinium T1-weighted sequences in the axial, sagittal, and coronal planes. RESULTS: These tumors were massive and predominantly cystic, with preferential frontal and parietal involvement. Typically, a DIT appears as a hypointense cystic mass with an isointense peripheral solid component on T1-weighted MR images. The peripheral solid component enhances after gadolinium administration. On T2-weighted MR images, the cystic component is hyperintense and the solid portion isointense or heterogeneous. The cystic portion is usually located deep inside the lesion, whereas its solid portion is peripheral. Meningeal enhancement and thickening adjacent to the solid portion of the tumor, calcifications, bone abnormalities adjacent to the tumor consisting of thinning and deformation were noted in 50% of our cases. Edema was usually absent or moderate. Median follow-up was 32 months, and no recurrence was noted except for one atypical case with incomplete excision, which led to the patient's death. CONCLUSION: Despite their malignant appearance, MR imaging features of DIT may help in the diagnosis and obviate unnecessary chemotherapy or radiation therapy.  (+info)

Ganglioglioma is a rare, typically slow-growing tumor that occurs in the brain or spinal cord. It is composed of both neuronal (ganglion cell) and glial elements. These tumors most commonly occur in the temporal lobe of the brain and are usually found in children and young adults.

Gangliogliomas can be benign or malignant, with the majority being low-grade (benign). Symptoms vary depending on the location of the tumor but may include seizures, headaches, changes in behavior or cognition, and motor weakness or paralysis. Treatment typically involves surgical removal of the tumor, and in some cases, radiation therapy or chemotherapy may be recommended.

It's important to note that while I strive to provide accurate information, my responses should not be used as a substitute for professional medical advice, diagnosis, or treatment. Always consult with a qualified healthcare provider for any medical concerns.

Anaplasia is a term used in pathology to describe the loss of differentiation and cellular organization in malignant tumors. It is characterized by the presence of large, pleomorphic cells with high mitotic activity, absence of mature tissue architecture, and lack of functional specialization. Anaplastic tumors are often aggressive and have a poor prognosis due to their rapid growth and tendency to metastasize. The term "anaplasia" is derived from the Greek words "ana," meaning "back" or "against," and "plasis," meaning "formation" or "molding."

Complex partial epilepsy, also known as temporal lobe epilepsy or focal impaired awareness epilepsy, is a type of epilepsy characterized by recurrent, unprovoked seizures that originate in the temporal lobe or other localized areas of the brain. These seizures typically involve alterations in consciousness or awareness, and may include automatisms (involuntary, repetitive movements), such as lip smacking, fidgeting, or picking at clothes. Complex partial seizures can last from a few seconds to several minutes and may be followed by a post-ictal period of confusion or fatigue.

Complex partial epilepsy is often associated with structural abnormalities in the brain, such as hippocampal sclerosis, tumors, or malformations. It can also be caused by infectious or inflammatory processes, vascular disorders, or genetic factors. The diagnosis of complex partial epilepsy typically involves a thorough neurological evaluation, including a detailed history of seizure symptoms, neuroimaging studies (such as MRI or CT scans), and electroencephalography (EEG) to record brain activity during and between seizures.

Treatment for complex partial epilepsy usually involves medication therapy with antiepileptic drugs (AEDs). In some cases, surgery may be recommended if medications are not effective in controlling seizures or if there is a structural lesion that can be safely removed. Other treatment options may include dietary modifications, such as the ketogenic diet, or vagus nerve stimulation.

Brain neoplasms, also known as brain tumors, are abnormal growths of cells within the brain. These growths can be benign (non-cancerous) or malignant (cancerous). Benign brain tumors typically grow slowly and do not spread to other parts of the body. However, they can still cause serious problems if they press on sensitive areas of the brain. Malignant brain tumors, on the other hand, are cancerous and can grow quickly, invading surrounding brain tissue and spreading to other parts of the brain or spinal cord.

Brain neoplasms can arise from various types of cells within the brain, including glial cells (which provide support and insulation for nerve cells), neurons (nerve cells that transmit signals in the brain), and meninges (the membranes that cover the brain and spinal cord). They can also result from the spread of cancer cells from other parts of the body, known as metastatic brain tumors.

Symptoms of brain neoplasms may vary depending on their size, location, and growth rate. Common symptoms include headaches, seizures, weakness or paralysis in the limbs, difficulty with balance and coordination, changes in speech or vision, confusion, memory loss, and changes in behavior or personality.

Treatment for brain neoplasms depends on several factors, including the type, size, location, and grade of the tumor, as well as the patient's age and overall health. Treatment options may include surgery, radiation therapy, chemotherapy, targeted therapy, or a combination of these approaches. Regular follow-up care is essential to monitor for recurrence and manage any long-term effects of treatment.

Supratentorial neoplasms refer to tumors that originate in the region of the brain located above the tentorium cerebelli, which is a dual layer of dura mater (the protective outer covering of the brain) that separates the cerebrum from the cerebellum. This area includes the cerebral hemispheres, basal ganglia, thalamus, hypothalamus, and pineal gland. Supratentorial neoplasms can be benign or malignant and may arise from various cell types such as neurons, glial cells, meninges, or blood vessels. They can cause a variety of neurological symptoms depending on their size, location, and rate of growth.

Cerebellar neoplasms refer to abnormal growths or tumors that develop in the cerebellum, which is the part of the brain responsible for coordinating muscle movements and maintaining balance. These tumors can be benign (non-cancerous) or malignant (cancerous), and they can arise from various types of cells within the cerebellum.

The most common type of cerebellar neoplasm is a medulloblastoma, which arises from primitive nerve cells in the cerebellum. Other types of cerebellar neoplasms include astrocytomas, ependymomas, and brain stem gliomas. Symptoms of cerebellar neoplasms may include headaches, vomiting, unsteady gait, coordination problems, and visual disturbances. Treatment options depend on the type, size, and location of the tumor, as well as the patient's overall health and age. Treatment may involve surgery, radiation therapy, chemotherapy, or a combination of these approaches.

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