Indian Journal of Cancer Medknow Publications on behalf of Indian Cancer Society ISSN: 0019-509X EISSN: 1998-4774 Vol. 48, Num. 1, 2011, pp. 129-131

Indian Journal of Cancer, Vol. 48, No. 1, January-March, 2011, pp. 129-131

Letter to Editor

Gliosarcoma with rhabdomyomatous differentiation: A case report

S Sethi, F Siraj, S Roy

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

Correspondence Address:
S Sethi
Department of Pathology, Sir Ganga Ram Hospital, New Delhi - 110 060
India
somika@gmail.com

Code Number: cn11031

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.

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.

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

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