scholarly journals Subependymal giant cell astrocytoma with high choline/creatine ratio on proton MR spectroscopy

2006 ◽  
Vol 64 (3b) ◽  
pp. 877-880 ◽  
Author(s):  
Arnolfo de Carvalho Neto ◽  
Emerson L. Gasparetto ◽  
Isac Bruck
Keyword(s):  
Tuberous Sclerosis ◽  
Giant Cell ◽  
Mr Spectroscopy ◽  
Subependymal Giant Cell Astrocytoma ◽  
Foramen Of Monro ◽  
Proton Mr Spectroscopy ◽  
History Of ◽  
The Right ◽  
Subependymal Nodules ◽  
Giant Cell Astrocytoma

OBJECTIVE: To report a case of subependymal giant cell astrocytoma (SEGA) in a patient with tuberous sclerosis, emphasizing the proton MR spectroscopy (MRS) findings. CASE: A three year-old boy with tuberous sclerosis presented with a 3-month history of mild headache. The physical examination showed discrete mental retardation and multiple hypomelanic macules in the legs and back. MRI showed many cortical tubers and subependymal nodules. At the left foramen of Monro, there was a 15 mm nodule, with heterogeneous signal on T1 and T2-weighted images, and strong enhancement after contrast administration. MRS study (multi-voxel PRESS, TE=144ms) showed the following ratios: a) left foramen of Monro nodule N-acetylaspartate (NAA)/creatine (Cr)=0.93 and Choline (Cho)/Cr=1.6 and b) at the right Monro foramen NAA/Cr=1.56 and Cho/Cr=1.29. CONCLUSION: The MRS performed at the SEGA may show high Cho/Cr and low NAA/Cr ratios, similar to the other brain neoplasms. As a consequence, MRS may be a valuable tool for the early detection of neoplastic transformation of subependymal nodules near the foramina of Monro in patients with tuberous sclerosis.

scholarly journals Subependymal giant cell astrocytoma in the absence of tuberous sclerosis complex: case report

2015 ◽  
Vol 16 (2) ◽  
pp. 134-137 ◽  
Author(s):  
Thomas L. Beaumont ◽  
Jakub Godzik ◽  
Sonika Dahiya ◽  
Matthew D. Smyth
Keyword(s):  
Tuberous Sclerosis ◽  
Giant Cell ◽  
Clinical Features ◽  
Tuberous Sclerosis Complex ◽  
Left Ventricular ◽  
Complex Case ◽  
Subependymal Giant Cell Astrocytoma ◽  
Clinical Approach ◽  
Foramen Of Monro ◽  
Giant Cell Astrocytoma

The authors report the case of a 14-year-old male with a subependymal giant cell astrocytoma (SEGA) that occurred in the absence of tuberous sclerosis complex (TSC). The patient presented with progressive headache and the sudden onset of nausea and vomiting. Neuroimaging revealed an enhancing left ventricular mass located in the region of the foramen of Monro with significant mass effect and midline shift. The lesion had radiographic characteristics of SEGA; however, the diagnosis remained unclear given the absence of clinical features of TSC. The patient underwent gross-total resection of the tumor with resolution of his symptoms. Although tumor histology was consistent with SEGA, genetic analysis of both germline and tumor DNA revealed no TSC1/2 mutations. Similarly, a comprehensive clinical evaluation failed to reveal any clinical features characteristic of TSC. Few cases of SEGA without clinical or genetic evidence of TSC have been reported. The histogenesis, genetics, and clinical approach to this rare lesion are briefly reviewed.

scholarly journals A Subependymal Giant Cell Astrocytoma in a Cat

2000 ◽  
Vol 37 (3) ◽  
pp. 275-278 ◽  
Author(s):  
S. Duniho ◽  
F. Y. Schulman ◽  
A. Morrison ◽  
H. Mena ◽  
A. Koestner
Keyword(s):  
Giant Cell ◽  
Neuron Specific Enolase ◽  
Subependymal Giant Cell Astrocytoma ◽  
Neurofilament Protein ◽  
Neoplastic Cells ◽  
S 100 ◽  
History Of ◽  
The Right ◽  
Pleomorphic Cells ◽  
Giant Cell Astrocytoma

A 6-year-old spayed female Domestic Shorthair cat presented with a 1 to 2-month history of blindness and altered behavior. At necropsy, a 1-cm-diameter, firm white mass was found arising from the subependymal region of the right lateral ventricular wall that protruded into and partially filled the lumen. Histologically, there was a well-demarcated, expansile paraventricular neoplasm composed of moderately pleomorphic cells within a richly fibrillar matrix arranged in interlacing streams and perivascular pseudorosette-like patterns. Neoplastic cells varied in morphology from small spindloid cells to larger polygonal cells with eccentric vesicular nuclei to neuronlike cells with vesicular nuclei and prominent nucleoli. The mitotic index was low. Immunohistochemically, neoplastic cells were positive for S-100 protein, glial fibrillary acidic protein, and neuron-specific enolase and negative for neurofilament protein. Ultrastructurally, the cells contained few to abundant bundles of intermediate filaments with variable numbers of mitochondria, endoplasmic reticulum, and ribosomes. These features are characteristic of subependymal giant cell astrocytoma (SEGA) in humans. To our knowledge, this is the first reported case of SEGA in domestic animals.

scholarly journals PC3 - 157 Subependymal Giant Cell Astrocytoma (SEGA) in Adults without Tuberous Sclerosis

2016 ◽  
Vol 43 (S4) ◽  
pp. S22-S22
Author(s):  
D.R. MacDonald ◽  
M.J. MacDonald ◽  
J.F. Megyesi ◽  
R.R. Hammond
Keyword(s):  
Family History ◽  
Tuberous Sclerosis ◽  
Giant Cell ◽  
Lateral Ventricle ◽  
Subependymal Giant Cell Astrocytoma ◽  
Cutaneous Manifestations ◽  
Caudate Head ◽  
Frontal Horn ◽  
History Of ◽  
Giant Cell Astrocytoma

Subependymal giant cell astrocytoma (SEGA) is typically seen in children with tuberous sclerosis (TS), who present with headaches and seizures, and characteristic clinical and cutaneous manifestations of TS. Surgical resection, CSF diversion (for hydrocephalus), radiotherapy, and chemotherapy with a mammalian target of rapamycin (mTOR) inhibitor are treatment options. SEGA can occur in adults without TS, raising diagnostic and therapeutic challenges. A 53 year old man presented with headaches and diplopia. An exophytic, enhancing mass in the left lateral ventricle was resected, confirming SEGA. There was no recurrence on MRI 13 months later. He previously had an “astrocytoma” involving the left frontal horn resected at age 19. Pathology review was SEGA. He had no clinical or cutaneous findings of TS, and no family history of TS. An identical twin was well. A 66 year old man presented with “weakness”, due to diuretic-induced hypokalemia. CT showed a hypodense mass from the right caudate head deforming the frontal horn. MRI showed an exophytic, enhancing mass from the caudate head into the frontal horn. A stereotactic biopsy confirmed SEGA. He had no clinical or cutaneous findings of TS, and negative family history. His mother had a meningioma resected at age 66. Observation is planned. These 2 adult patients had SEGA without clear clinical findings or family history of TS. SEGA should be in the differential diagnosis of tumors involving the lateral ventricle.

scholarly journals Subependymal Giant Cell Astrocytoma without Clinically or Genetically Identifiable Tuberous Sclerosis Complex: A Case Report

2020 ◽  
pp. 1-4
Author(s):  
Christopher J. Farrell ◽  
Karim Hafazalla ◽  
Donald Y. Ye ◽  
Timothy Chao ◽  
Mark Curtis ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Giant Cell ◽  
Clinical Features ◽  
Tuberous Sclerosis Complex ◽  
The Body ◽  
World Health ◽  
Subependymal Giant Cell Astrocytoma ◽  
Foramen Of Monro ◽  
Clinical Criteria ◽  
Giant Cell Astrocytoma

Background: Subependymal giant cell astrocytoma (SEGA) is a World Health Organization grade I tumor most commonly located in the lateral ventricles near the foramen of Monro. They are primarily associated with tuberous sclerosis complex (TSC), an autosomal dominant inherited condition that leads to a variety of tumors throughout the body. TSC must be diagnosed via either genetic criteria or clinical criteria. Cases of SEGA in patients without both genetically or clinically identifiable TSC are scarce, with only two instances identified of those genetically screened in the literature. We describe what we believe is only the third such case, a 22-year-old patient with no other clinical or genetic indicators for tuberous sclerosis. Case Description: The patient is a 22-year-old Caucasian female who experienced progressive symptoms of headaches, tinnitus and visual obscuration. A magnetic resonance imaging study of the brain revealed a homogeneously enhancing mass in the right lateral ventricle with obstruction of the foramen of Monro. The patient underwent a right frontal craniotomy for transcortical-transventricular resection of the mass. Histologic examination of the tumor revealed a diagnosis of SEGA. Germline genetic tests for both TSC1 and TSC2 were negative, and no additional clinical features were present. The patient was thus not felt to meet the criteria for a diagnosis of TSC based on the absence of clinical features, family history, and negative genetic testing. Conclusion: Although a rare phenomenon, SEGA should remain on the differential diagnosis for patients with newly found periventricular tumors and absence of TSC clinical features.

Recurrent subependymal giant-cell astrocytoma in the absence of tuberous sclerosis

1979 ◽  
Vol 50 (1) ◽  
pp. 106-109 ◽  
Author(s):  
G. Michael Halmagyi ◽  
Leon P. Bignold ◽  
John L. Allsop
Keyword(s):  
Tuberous Sclerosis ◽  
Giant Cell ◽  
Parietal Lobe ◽  
Mural Nodule ◽  
Subependymal Giant Cell Astrocytoma ◽  
Content Type ◽  
Fine Print ◽  
Giant Cell Astrocytoma

✓ A case is described of a subependymal giant-cell astrocytoma that occurred as a mural nodule within a cyst in the parietal lobe. The tumor recurred twice over a period of 47 years despite two extensive surgical resections. Neither the patient nor any of his children suffered tuberous sclerosis, a disease with which this type of astrocytoma is associated.

scholarly journals Clinical Characteristics of Subependymal Giant Cell Astrocytoma in Tuberous Sclerosis Complex

2019 ◽  
Vol 10 ◽  
Author(s):  
Anna C. Jansen ◽  
Elena Belousova ◽  
Mirjana P. Benedik ◽  
Tom Carter ◽  
Vincent Cottin ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Giant Cell ◽  
Tuberous Sclerosis Complex ◽  
Clinical Characteristics ◽  
Subependymal Giant Cell Astrocytoma ◽  
Giant Cell Astrocytoma
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