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LETTER TO EDITOR
Year : 2012  |  Volume : 60  |  Issue : 6  |  Page : 674--676

Pediatric gliosarcoma of thalamus

R Neelima1, Mathew Abraham2, TR Kapilamoorthy3, Divyata Rajendra Hingwala3, VV Radhakrishnan1,  
1 Department of Pathology, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Trivandrum, Kerala, India
2 Department of Neurosurgery, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Trivandrum, Kerala, India
3 Department of Imaging sciences and Interventional Radiology, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Trivandrum, Kerala, India

Correspondence Address:
V V Radhakrishnan
Department of Pathology, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Trivandrum, Kerala
India




How to cite this article:
Neelima R, Abraham M, Kapilamoorthy T R, Hingwala DR, Radhakrishnan V V. Pediatric gliosarcoma of thalamus .Neurol India 2012;60:674-676


How to cite this URL:
Neelima R, Abraham M, Kapilamoorthy T R, Hingwala DR, Radhakrishnan V V. Pediatric gliosarcoma of thalamus . Neurol India [serial online] 2012 [cited 2020 Apr 9 ];60:674-676
Available from: http://www.neurologyindia.com/text.asp?2012/60/6/674/105222


Full Text

Sir,

Occurrence of pediatric gliosarcoma (GS) in the thalamic region has not been documented, hence this report.

A male child aged 11 years presented with intermittent headache and vomiting of one-month duration. Neurologic examination revealed bilateral early papilledema. Magnetic resonance imaging (MRI) brain showed a large round well-defined mass lesion in the left thalamic region extending into the occipital horn of the lateral ventricle, involving the left temporal lobe, left internal capsule, pineal region, splenium of corpus callosum and left cerebral peduncle. The tumor was heterogeneously intense on T2-weighted and FLAIR images and was seen extending into the occipital horn of the left lateral ventricle [Figure 1]a and b. Evidence of periventricular cerebrospinal fluid (CSF) seepage, midline shift and mass effect over third ventricle were seen [Figure 1]b. Focal areas of diffusion restriction, perifocal edema and contrast ring enhancement were seen [Figure 1]c and d. The preoperative diagnosis was high-grade glioma of thalamus. Intraoperatively, a yellowish necrotic suckable tumor was seen arising from the posterolateral surface of the thalamus extending anteriorly to the temporal lobe, lateral ventricle and posteriorly till the atrium and posterior part of the quadrigeminal cistern with peduncular involvement. Near-total decompression of the tumor was done. Histopathology showed features of an oligoastrocytoma with marked nuclear atypia, mitotic figures, pseudopalisading necrosis and vascular endothelial proliferation [Figure 2]a-c. Interspersed with this glial component were pleomorphic spindle cells seen in fascicles or arranged around blood vessels indicating sarcomatous transformation [Figure 2]d. Gordon and Sweet reticulin stain done [Figure 3]a showed increased reticulin fibers in the sarcomatous component whereas the glial component was devoid of reticulin fibers. Both histological components expressed high MIB index upto 10-15% [Figure 3]b. The neoplastic glial cells were intensely positive for GFAP (glial fibrillary acidic protein) (monoclonal, 1:100) [Figure 3]c and the sarcomatous component negative for GFAP. Vimentin (monoclonal, 1:100) done showed strong and diffuse positivity in both glial and sarcomatous elements indicating cross-reactivity [Figure 3]d. These histomorphological features along with immunohistochemistry and reticulin stain findings suggested a diagnosis of gliosarcoma.{Figure 1}{Figure 2}{Figure 3}

Only 23 cases of pediatric GS have been documented in the literature till date with none in the thalamus. [1] GS are commonly seen in the frontal lobe followed by temporal and parietal lobes. [2] GS occurring in the thalamic region have rarely been described in adult patients. [3] The neuroimaging features may show a heterogeneously enhancing lesion with a central area of necrosis, suggesting a glioblastoma or may show hyperdense lesions with well-defined margins and contrast enhancement, mimicking a meningioma. [4] Histomorphologic al features, supported by immunohistochemical staining for GFAP and Vimentin as well as Gordon and Sweet's reticulin stain will help in arriving at the final diagnosis. Reticulin stain clearly demarcates the sarcomatous and glial components. Hence it is highly useful for the diagnosis of gliosarcoma in those laboratories where there are constraints of laboratory resources and technical expertise to use immunohistochemical techniques.

Karremann et al., had reported a bipolar age distribution for GS, with one peak in the first year of age (congenital GS) and a second broader peak in late childhood and adolescence [1] The authors have reported four cases of GS in young infants (1-4 months) who had a long recurrence-free period following surgery. The second group comprised six patients who developed GS following radiotherapy for other neoplasms such as low-grade glioma, giant cell glioblastoma, medulloblastoma, acute lymphoblastic leukemia and angioma. The third group consisted of 13 patients in late childhood and adolescent age groups with features of GS de novo. Both the second and third categories of patients had a poor prognosis, quite similar to that seen in adult Glioblastoma or GS. [1] Kramm et al., had pointed that thalamic high-grade gliomas in the pediatric age group represent clinically and prognostically a different subgroup compared to pediatric high-grade gliomas arising from supratentorial and pontine regions. [5] GS is one of the rare primary intracranial neoplasms with a well-known tendency to metastasize to extracranial sites such as lungs, liver. [2],[6] All these variables may point towards different genetic mechanisms that can play a distinct role in the pathogenesis and biological behavior of GS. [1],[5] Hence prospective molecular studies are needed which will enhance our concepts and promote in developing targeted therapy for pediatric GS.

 Acknowledgment



We express our sincere gratitude to the Director of our institute for permitting to publish this article. We also express our sincere thanks to Mrs Sushamakumari for her expert technical help in the laboratory.

References

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