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LETTER TO EDITOR
Year : 2011  |  Volume : 48  |  Issue : 1  |  Page : 129-131
 

Gliosarcoma with rhabdomyomatous differentiation: A case report


Department of Pathology, Sir Ganga Ram Hospital, New Delhi - 110 060, India

Date of Web Publication10-Feb-2011

Correspondence Address:
S Sethi
Department of Pathology, Sir Ganga Ram Hospital, New Delhi - 110 060
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-509X.76643

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How to cite this article:
Sethi S, Siraj F, Roy S. Gliosarcoma with rhabdomyomatous differentiation: A case report. Indian J Cancer 2011;48:129-31

How to cite this URL:
Sethi S, Siraj F, Roy S. Gliosarcoma with rhabdomyomatous differentiation: A case report. Indian J Cancer [serial online] 2011 [cited 2019 Dec 11];48:129-31. Available from: http://www.indianjcancer.com/text.asp?2011/48/1/129/76643


Sir,

Gliosarcomas are uncommon malignant brain tumors consisting of an admixture of neoplastic glial and sarcomatous elements. [1] They account for up to 8% of all glioblastoma multiforme. [1] These tumors are common in elderly individuals [1] In most cases, the sarcomatous areas of the tumors have the appearance of fibrosarcoma and only rarely other types of differentiation, such as, malignant fibrous histiocytoma, rhabdomyosarcoma, leiomyosarcoma, chondrosarcoma, and osteochondrosarcoma have been reported.[1] We recently found a case of gliosarcoma with rhabdomyoblastic differentiation in a young individual, occurring in cerebellum and because such an occurrence is rare we consider it worthwhile to report this case.

A 25-year-old man presented with a two-month history of increasingly severe headaches associated with vomiting and gradual loss of vision. A Computed Tomography scan (CT Scan) and Magnetic Resonance Imaging (MRI) revealed a large left cerebellar tumor [Figure 1]. On craniotomy the tumor was diffusely infiltrating the left cerebellar hemisphere.
Figure 1: MRI showing a large left cerebellar lesion

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Brain tumor biopsy was received in multiple fragments, together measuring 2 × 1.5 × 0.2 cm.

H and E-stained sections showed a highly cellular tumor with a biphasic pattern. There was an intimate admixture of malignant glial and sarcomatous components [Figure 2]. The glial component had predominant gemistocytes, which showed marked pleomorphism. These cells stained positively for glial fibrillary acidic protein (GFAP), shown in [Figure 3]a. These areas intermingled with the spindle cells, showing a strong positive reaction to reticulin [Figure 3]b. Also seen within these sarcomatous areas were large cells with abundant dense eosinophilic cytoplasm, with a strong positive reaction to desmin [Figure 4] and [Figure 5], but negative staining for GFAP [Figure 5]. Tumor cells showed strong positivity, with Epidermal Growth Factor Receptor (EGFR) and p53 [Figure 6], further confirming the diagnosis of gliosarcoma. No microvascular proliferation was seen.
Figure 2: Gliomyosarcoma showing an admixture of small round malignant glial cells with sarcomatous areas (˜ 100, H and E stain)

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Figure 3a: Glial fibrillary acidic protein (GFAP) positivity is less in the same region, which shows high positivity for reticulin (× 40, Immunohistochemical stain : GFAP)
b: Gliomyosarcoma showing reticulin positivity in the sarcomatous areas (×40, Reticulin Stain)


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Figure 4: Tumor showing rhabdomyosarcomatous differentiation in the sarcomatous areas, with large cells positive for Desmin (× 400, Immunohistochemical stain : Desmin)

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Figure 5: Large cells stain negative with GFAP and positive with Desmin protein in the same area (Immunohistochemical stain for Desmin is positive in large cells seen on the left side and the same cells on the right side are stained negative with GFAP; × 400)

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Figure 6: Tumor cells show a strong cytoplasmic positivity with the epidermal growth factor receptor and strong nuclear positivity with p53 favoring gliosarcoma (Right : EGFR ; Left : p53)

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On the basis of these morphological and immunohistochemical findings, a diagnosis of gliosarcoma showing rhabdomyoblastic differentiation (Gliomyosarcoma) was made.

The term gliosarcoma was first used by Stroebe in 1895, to describe the sarcomatous change occurring in the glioblastoma. [2] In 1969, Goldman described a case of gliosarcoma with light microscopic evidence of rhabdomyoblastic differentiation and coined the term gliomyosarcoma. [3] Later Shuangshoti and Netsky, and Barnard and Stapleton reported similar cases and described the electron microscopy and immunohistochemical properties of this tumor. [4]

The occurrence of rhabdomyoblastic differentiation in various CNS tumors is well-recognized, although rare, as in medullomyoblastoma, malignant triton tumor, and malignant ectomesanchymoma. [2] Primary cerebral rhabdomyosarcoma within the cranium and spinal canal has also been described. [2]

Sarcomatous change in the gliosarcoma is proposed to occur from the neoplastic proliferation of the vascular mesenchyme. [4] However, recent studies have suggested that both the glial and sarcomatous components arise from glial cells. Progressive loss of GFAP in some parts of the glioma results in acquisition of the sarcomatous phenotype.

Postoperatively, the patient made a good recovery with a resolution of his symptoms. He was treated further with radiotherapy.

 
 » References Top

1.Sarkar C, Sharma MC, Sudha K, Gaikwad S, Varma A. A clinico-pathological study of 29 cases of gliosarcoma with special reference to two unique variants. Indian J Med Res 1997;106:229-35.  Back to cited text no. 1
    
2.Stapleton SR, Harkness W, Wilkin PR, Uttley D. Gliomyosarcoma: An Immunohistochemical analysis. J Neurol Neurosurg Psychiatry 1992;55:728-30.  Back to cited text no. 2
    
3.Goldman RL. Gliomyosarcoma of the cerebrum. Am J Clin Path 1969;52:741-4.  Back to cited text no. 3
[PUBMED]    
4.Barnard RO, Bradford R, Scott T, Thomas DG. Gliomyosarcoma. Report of a case of rhabdomyosarcoma arising in a malignant glioma. Acta Neuropathol (Berl.) 1986;69:23-7.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

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