Primary Intracranial pPNET/Ewing Sarcoma: Diagnosis, Management, and Prognostic Factors Dilemma–A Systematic Review of the Literature

Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor Mimicking Meningioma: A Case Report and Literature Review

“…Our study showed that clinical symptoms include intracranial pressure, increasing presenting headache, and vomiting (5 cases, [50.0%]), hemiplegia and muscle strength decrease (3 cases, [30.0%]), facial palsy (3 cases, [30.0%]), deafness and hearing disturbance (2 cases, [20.0%]), drowsiness and fatigue (2 cases, [20.0%]), epilepsy (1 case, [10.0%]), memory decline (1 case, [10.0%]), and ataxia (1 case, [10.0%]). The mean duration of clinical symptoms was 5.9 months (range: 1–18 months), and the duration in our results is longer than what has been described in the literature (with a median of 9 weeks) ( 14 ). This might associate with the compensation of unclosed or not firmly integrated cranial sutures in children.…”

“…Intracranial ES/pPNETs are very rare malignant tumors, accounting for 0.03% of the total number of intracranial tumors ( 3 , 4 ), with only 57 cases reported until now. ES/pPNETs mostly occur in children and adolescents with a median age at first onset of disease being 15 years of age, and the peak of disease is prominent in the second decade, ranging from 5 months to 67 years of age, with a slight male predisposition ( 14 , 16 – 18 ). The case presented here is an ES/pPNET in the left frontal lobe of a 55-year-old female patient.…”

“…In our study, the mean patient age was 50.7 years (range: 34–67 years), and there were seven females and four males ( Table 1 ). Moreover, supratentorial cerebral hemispheres are predilection sites for ES/pPNET ( 14 ). In our study, the presenting sites include the hemispheric surface (6 cases), cerebellopontine angle (2 cases), cavernous sinus (1 case), and posterior fossa (1 case).…”

“…Our case was consistent with these findings. As for post-contrast MRI, Cherif et al reviewed all reported ES/pPNETs in 2018, and presented a heterogeneous enhancement of about 40.0%, intense enhancement of about 52.5%, and moderate enhancement of 7.5% of these lesions ( 14 ). In our study, we showed heterogeneous enhancement of about 60.0% (6 cases), and intense enhancement of 40.0% (4 cases) of these lesions.…”

“…They are mainly composed of small, round or oval, undifferentiated cells, with hyperchromatic nuclei, increased mitotic activity, and a slightly basophilic cytoplasm ( 1 , 3 , 4 ). Moreover, the tumor cells are markedly fibrotic, highly mitotic, and separated by groups of cells containing collagen bands ( 1 , 3 , 4 , 14 , 18 ). Membranous expression of CD99 is a highly reliable and sensitive diagnostic biomarker for primary intracranial ES/pPNETs ( 3 , 4 , 13 – 15 , 18 ), and was detected in nearly all patients.…”