Gliosarcoma in patients under 20 years of age. A clinicopathologic study of 11 cases and detailed review of the literature

Abstract

Background Gliosarcoma is a rare variant of IDH- wild type glioblastoma with both glial and mesenchymal differentiation. It accounts for approximately 2% of glioblastomas and has a poor prognosis similar to that of classic glioblastoma. It is seen mostly between 40 and 60 years of age with a mean age over 50 years. Pediatric gliosarcoma is even rarer than gliosarcoma in adults. We describe the clinicopathological features of gliosarcoma in patients under 20 years of age and determine whether there are significant differences from gliosarcoma in adults. We also present detailed review of published literature on pediatric gliosarcoma. Methods Slides of gliosarcomas in patients under 20 years of age were reviewed. Clinicopathological features were noted in detail and follow up was obtained. Results Eleven cases of gliosarcoma were reported in patients under 20 years of age. Ages ranged from three to 19 years (mean age 13 years). Frontal, parietal and temporal lobes were the commonest locations. Mean and median tumor size was six and five cm respectively. All 11 cases demonstrated the classic biphasic pattern. In 10 cases, glial component was astrocytic and was highlighted on GFAP. Sarcomatous component in most cases resembled fibrosarcoma and was high grade in 72.7%. Glial areas were reticulin poor while sarcomatous areas were reticulin rich. In over 45% cases, bizarre tumor giant cells were seen in the sarcomatous areas. In 1 case, sarcomatous areas showed extensive bone and cartilage formation. Other histologic features included hyalinized blood vessels, hemorrhage, infarction, gemistocytic cells, rhabdoid cells etc. Follow up was available in nine patients, five received chemoradiation post resection while three received radiotherapy only. Prognosis was dismal and eight patients died within one to 14 months following resection. Conclusions Gliosarcomas in patients under 20 comprised 13% of all gliosarcomas reported during the study period. Frequency and mean age were higher compared to other published reports. Pathological features were similar to those described in literature. Clinicopathological features and prognosis of pediatric gliosarcomas were similar to adult gliosarcomas.

A Unique Case Report of Infant-Type Hemispheric Glioma (Gliosarcoma Subtype) with <b><i>TPR</i></b>-<b><i>NTRK1</i></b> Fusion Treated with Larotrectinib

“…To the best of our knowledge, only 13 cases have been reported in the literature [10,11]. Although very few cases exhibited longer outcomes, infantile gliosarcomas share a gloomy prognosis with adult gliosarcomas [10,11]. Different from adults and children, infant-type gliomas do not show clear association of histologic tumor grade and clinical outcome.…”

“…Infantile (<3 years old) gliosarcomas are extremely rare. To the best of our knowledge, only 13 cases have been reported in the literature [10, 11]. Although very few cases exhibited longer outcomes, infantile gliosarcomas share a gloomy prognosis with adult gliosarcomas [10, 11].…”

“…Few reports of infantile gliosarcoma have been reported, and due to its rarity, there are no available data regarding its etiology and genetics. Compared to adults, the prognosis of infantile gliosarcoma is dismal [10, 11], and the few cases with better outcomes may suffer severe neurological and neurocognitive deficits as a consequence of surgery and chemotherapy [12]. Therefore, more effective treatment strategies for infantile gliosarcoma are urgently needed.…”

Incidência de gliomas do sistema nervoso central e sua correlação com a ocupação profissional na região do Planalto Norte Catarinense

“…Até o momento, mais de 63% dos casos analisados são Gliomas, afetando mais, frequentemente, o hemisfério direito, no lobo frontal. Na literatura não há nenhum artigo que traga a frequência quanto ao hemisfério e lobo como a aqui apresentada, porém, há evidências quanto a estruturas afetadas e, comumente, associadas a casos de câncer infantil 11,12 .…”

Optic Pathway–Hypothalamic Glioma Apoplexy: A Report of Two Cases and Systematic Review of the Literature

“…Ten deaths have been reported in the included literature, most of which were related to intraventricular hemorrhage or hemorrhage into the hypothalamus. Out of these 10 deaths, three cases were due to high-grade gliomas (glioblastoma and gliosarcoma) ( 11 , 24 , 35 ). On the other hand, van Baarsen and colleagues suggested that hemorrhage into glioma of the optic nerves carries a more benign course than hypothalamic and chiasmal ones since this type of hemorrhage is confined to the orbit and does not cause intracerebral damage ( 6 ).…”