|Year : 2015 | Volume
| Issue : 4 | Page : 917-919
Gliosarcoma with leiomyomatous differentiation: A case report with an emphasis on histogenesis
Saumya Shukla1, Namrata Punit Awasthi1, Pradyumn Singh1, Nuzhat Husain1, Bal Krishna Ojha2
1 Department of Pathology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
2 Department of Neurosurgery, King George's Medical University, Lucknow, Uttar Pradesh, India
|Date of Web Publication||15-Feb-2016|
Department of Pathology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow - 226 010, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Gliosarcoma is an uncommon high-grade tumor which constitutes about 2% of all glioblastomas. These tumors have the histological hallmark of a biphasic pattern of high-grade glial and sarcomatous components. The histogenesis of these tumors is controversial. We report a case of primary gliosarcoma in an adult male with leiomyomatous differentiation and discuss the histogenesis as it appears in our case. Primary gliosarcomas of the brain are clinically challenging with a poor clinical outcome.
Keywords: Gliosarcoma, leiomyomatous differentiation, histogenesis
|How to cite this article:|
Shukla S, Awasthi NP, Singh P, Husain N, Ojha BK. Gliosarcoma with leiomyomatous differentiation: A case report with an emphasis on histogenesis. J Can Res Ther 2015;11:917-9
|How to cite this URL:|
Shukla S, Awasthi NP, Singh P, Husain N, Ojha BK. Gliosarcoma with leiomyomatous differentiation: A case report with an emphasis on histogenesis. J Can Res Ther [serial online] 2015 [cited 2019 Sep 21];11:917-9. Available from: http://www.cancerjournal.net/text.asp?2015/11/4/917/158026
| > Introduction|| |
Gliosarcoma is an uncommon high-grade tumor which constitutes about 2% of all glioblastomas. It is considered as Grade IV tumor (World Health Organization, 2007). , These tumors have the histological hallmark of a biphasic pattern of high-grade glial and sarcomatous components. Gliosarcoma commonly affects adults in fifth to sixth decades of life and has a male predominance.  The glial component is usually astrocytic in nature showing the morphological features of glioblastoma. 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. , The histogenesis of the mesenchymal component has been a matter of debate. We report a case of primary gliosarcoma in an adult male with leiomyomatous differentiation and discuss the histogenesis as it appears in our case.
| > Case report|| |
A 47-year-old male presented with a history of headache, loss of consciousness, nausea, and vomiting. The magnetic resonance imaging revealed a large space-occupying lesion in the right frontal region with mass effect [Figure 1]. Craniotomy revealed a large tumor infiltrating diffusely into the cerebral parenchyma. A biopsy was taken, and the histopathological examination revealed a cellular tumor with a characteristic biphasic pattern. The tumor was composed of an admixture of glial and sarcomatous elements. The neoplastic astrocytic component was composed of cells with moderate amount of cytoplasm and pleomorphic vesicular nuclei. The sarcomatous element was composed of spindle-shaped cells with a moderate amount of eosinophilic cytoplasm and pleomorphic hyperchromatic nuclei. Mitosis, microvascular proliferation, and necrosis were also identified [Figure 2]. On immunostaining, glial fibrillary acidic protein (GFAP) was expressed in the malignant glial tissue and sarcomatous component was positive for vimentin and smooth muscle actin (SMA) while S-100 and desmin were negative [Figure 3]. A diagnosis of gliosarcoma with leiomyomatous differentiation was rendered based on the histomorphology and immunohistochemical findings. The patient was referred for radiotherapy, but the patient expired within a week of diagnosis due to massive intracerebral hemorrhage.
|Figure 1: Magnetic resonance imaging: A large space-occupying lesion in the right frontal region with mass effect|
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|Figure 2: (a) A cellular tumor composed of an admixture of glial and sarcomatous elements with foci of necrosis (arrow) (H and E, ×50), (b) the sarcomatous component is composed of moderately pleomorphic spindle-shaped cells (H and E, ×100) (c) the spindle cells with moderate amount of eosinophilic cytoplasm and pleomorphic hyperchromatic nuclei (H and E, ×200)|
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|Figure 3: (a) Glial fibrillary acidic protein: Positive in the malignant glial component (diaminobenzidine tetrachloride [DAB], ×50), (b) vimentin: Positive in the sarcomatous component (DAB, ×50), (c) smooth muscle actin (SMA): Diffuse positive in the sarcomatous component (DAB, ×100), (d) SMA: The sarcomatous element is in continuity with the blood vessel wall within the tumor cells (arrow) (DAB, ×200)|
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| > Discussion|| |
The occurrence of gliosarcoma was first documented in 1895 by Ravisankar et al. However, it was accepted as a distinct entity when Feigin and Gross reported the occurrence of sarcoma in glioblastoma. ,, Gliosarcoma is usually located in the cerebral hemispheres involving the temporal, frontal, parietal, and occipital lobes in decreasing order of frequency. The presenting signs and symptoms include a rapidly expanding intracranial tumor leading to aphasia, headache, hemiparesis, seizures, and cognitive decline depending on the location. ,,,
The glial component is usually astrocytic in nature showing the morphological features of glioblastoma, and the sarcomatous component is commonly a fibrosarcoma exhibiting morphological features of malignancy. However rarely mesenchymal components such as smooth muscle, skeletal muscle, bone, and cartilage constituting the sarcomatous component have also been reported. ,, Haddad et al. and Jones et al. have also reported rare cases of gliosarcomas with smooth muscle differentiation which has also been documented in our case.
The origin of the mesenchymal component has been controversial. Feigin et al. first suggested that the sarcomatous component develops from the neoplastic transformation of the hyperplastic vessels which are commonly found in glioblastoma. The sarcomatous component generally originates from the endothelial, pericytic or the undifferentiated mesenchymal cells. This histogenesis has been widely accepted as the sarcomatous elements show expression of endothelial markers such as factor VIII-related antigen and Ulex europaeus agglutinin. , In our case, it was noted that the SMA positive sarcomatous element was in continuity with the blood vessel wall within the tumor cells. Hence, it implies that this theory of histogenesis appears to be true in our case.
However, some studies have also suggested that the sarcomatous component is due to the loss of GFAP from some parts of the glioma along with the acquisition of the sarcomatous phenotype. These studies indicate that both the components of a gliosarcoma originate from the glial cells. As a result of this phenotypic change, the sarcomatous element acquires antigenic characteristics of other cells with a loss of GFAP expression. Thus, this theory indicates that both components in gliosarcoma are monoclonal in origin. However, the histogenesis of gliosarcoma is debatable and alternate pathways cannot be ruled out. ,,,,
Primary gliosarcoma is a clinically challenging and a very rare tumor with a poor prognosis in untreated cases. The prognosis is sometimes more wane as gliosarcomas can metastasize through the hematogenous route to extracranial sites like the lung and liver. The sarcomatous component has a high propensity to metastasize. ,, The treatment of gliosarcomas is almost similar to glioblastomas consisting of surgical resection and depending on clinical status, radiotherapy and/or chemotherapy. However, a multidisciplinary approach (surgery, radiotherapy, and chemotherapy) seems to be associated with slightly prolonged survival times. ,
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[Figure 1], [Figure 2], [Figure 3]