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LETTER TO EDITOR
Year : 2011  |  Volume : 59  |  Issue : 6  |  Page : 933-935

Hemorrhagic subependymal giant cell astrocytoma in a patient with tuberous sclerosis: Case report and review of the literature


1 Department of Neurosurgery, LSU Health Sciences Center, Shreveport, LA, USA
2 Department of Pathology, LSU Health Sciences Center, Shreveport, LA, USA

Date of Submission13-Sep-2011
Date of Decision07-Oct-2011
Date of Acceptance26-Oct-2011
Date of Web Publication2-Jan-2012

Correspondence Address:
Bharat Guthikonda
Department of Neurosurgery, LSU Health Sciences Center, Shreveport, LA
USA
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DOI: 10.4103/0028-3886.91394

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How to cite this article:
Wadhwa R, Khan IS, Thomas JO, Nanda A, Guthikonda B. Hemorrhagic subependymal giant cell astrocytoma in a patient with tuberous sclerosis: Case report and review of the literature. Neurol India 2011;59:933-5

How to cite this URL:
Wadhwa R, Khan IS, Thomas JO, Nanda A, Guthikonda B. Hemorrhagic subependymal giant cell astrocytoma in a patient with tuberous sclerosis: Case report and review of the literature. Neurol India [serial online] 2011 [cited 2014 Dec 18];59:933-5. Available from: http://www.neurologyindia.com/text.asp?2011/59/6/933/91394


Sir,

Subependymal giant cell astrocytoma (SEGA) commonly occurs in patients with tuberous sclerosis (TS), and only rarely do these tumors hemorrhage. We report one such case.

A 50-year-old African-American female, diagnosed case of TS at birth, presented with a 5-day history of progressive headache, nausea and vomiting. The patient had a history of seizure disorder and known periventricular calcified tubers, which were being followed with serial magnetic resonance imaging (MRI) scans since 2003 [Figure 1]a. She also had a larger contrast-enhancing lesion in the left frontal horn, extending to the left foramen of Monroe. On the day of presentation, cranial computed tomography showed enlargement of the left frontal mass with significant intratumoral hemorrhage [Figure 1]b. MRI showed a hemorrhagic lesion [Figure 1]c. Patient was taken up for a left frontal transcortical intraventicular resection of the hemorrhagic mass. Neuronavigation was used, and the cortical entry point was that which is used for a left frontal ventriculostomy. The tumor was encountered and was noted to have significant intratumoral hemorrhage along with an adjacent hemorrhagic clot [Figure 1]d. The dissection was carried down into the frontal horn of the lateral ventricle, where we were able to remove the tumor in its entirety. Ventricular hemostasis was achieved, and the septum pellucidum was identified and fenestrated to allow biventricular spinal fluid communication. Post-operatively, the patient did well and was discharged after a short hospital stay, and has required no further intervention. Histopathological examination revealed findings consistent with hemorrhagic SEGA [Figure 2].
Figure 1a: Axial T1 with contrast magnetic resonance imaging with contrast showing left frontal horn lesion from 2003
Figure 1b: Computed tomography head showing left frontal horn mass with hemorrhage at the time of admission
Figure 1c: Axial T2 gradient-recalled echo (GRE) magnetic resonance imaging showing hemorrhage within tumor
Figure 1d: Intraoperative photo showing intratumoral hemorrhage


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Figure 2: Microscopic findings: (a) Cellular tumor composed of large polygonal, round and spindly cells with abundant eosinophilic cytoplasm, eccentric nuclei with prominent nucleoli, some resembling large neuronal cells or gemistocytes. (b) The tumor shows prominent vascularity with marked perivascular hyalinization and pseudo-rosette pattern with condensation of cell processes around fribrovascular stroma. (c-g) There is associated vascular microthrombi with old and recent hemorrhage, aggregates of foamy histiocytes, infarctive necrosis and nodular sclerotic areas

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TS is an autosomal-dominant phakomatoses (neuro-cutaneous disorders). [1] Subependymal nodules may occasionally give rise to SEGAs. Histologically, these tumors can have necrosis and abundant mitotic figures. In spite of these aggressive pathological features, however, most are not biologically aggressive. [1] Because of this tumor's propensity to grow near the foramen of Monroe and disturb the natural circulation of the cerebrospinal fluid, a majority of symptomatic patients have hydrocephalus. In one series of 15 patients, 12 had hydrocephalus at presentation, [2] and in another series of 11 patients, hydrocephalus was present in seven patients. [3] The altered anatomy of the subependymal region is another reason for the increased intracranial pressure (ICP) in patients with SEGA. [2],[3] The mechanism of intratumoral hemorrhage remains unclear. In malignant pathologies, rapid rate of growth with distortion of the vasculature and endothelial proliferation leading to obliteration of the vessel lumen seem to be plausible causes of bleeding. Another reason, and a more likely one in a benign tumor such as SEGA, is increased venous pressure (secondary to an increased ICP) leading to necrosis and hemorrhage. [4] Prominent vascular proliferation with ectasia is also a feature in some of these tumors, as was with our patient. This may also put them at a high risk of spontaneous hemorrhage. Large SEGAs also distort the foramen of Monroe and pose a challenge for intraoperative hemostasis. [5] Kumar and Singh reported difficulty in controlling intraoperative hemorrhage in one very vascular SEGA. [6] Mortality due to massive hemorrhage post-operatively has been reported as well. [7] In conclusion, it is important to realize that these tumors can rarely hemorrhage, and may subsequently lead to increased mass effect and/or obstructive hydrocephalus, potentially requiring urgent treatment.

 
  References Top

1.Rengachary S. Principles of neurosurgery. 2 nd ed. Philadelphia: Elsevier Mosby; 2005.  Back to cited text no. 1
    
2.Cuccia V, Zuccaro G, Sosa F, Monges J, Lubienieky F, Taratuto AL. Subependymal giant cell astrocytoma in children with tuberous sclerosis. Childs Nerv Syst 2003;19:232-43.  Back to cited text no. 2
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3.Goh S, Butler W, Thiele EA. Subependymal giant cell tumors in tuberous sclerosis complex. Neurology 2004;63:1457-61.  Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.Waga S, Yamamoto Y, Kojima T, Sakakura M. Massive hemorrhage in tumor of tuberous sclerosis. Surg Neurol 1977;8:99-101.  Back to cited text no. 4
[PUBMED]    
5.Berhouma M. Management of subependymal giant cell tumors in tuberous sclerosis complex: The neurosurgeon's perspective. World J Pediatr 2010;6:103-10.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Kumar R, Singh V. Subependymal giant cell astrocytoma: A report of five cases. Neurosurg Rev 2004;27:274-80.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  
7.Shepherd CW, Scheithauer BW, Gomez MR, Altermatt HJ, Katzmann JA. Subependymal giant cell astrocytoma: A clinical, pathological, and flow cytometric study. Neurosurgery 1991;28:864-8.  Back to cited text no. 7
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