Primary intracranial peripheral primitive neuroectodermal tumor in an adult patient with aphasia: A rare case report

Primary intracranial peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare malignancies that commonly affect children and adolescents. Only 10 cases over the age of 33 have been reported. pPNETs have an aggressive behavior and a high tendency for local recurrence and distant metast...

Full description

Saved in:
Bibliographic Details
Published inClinical case reports Vol. 10; no. 3; pp. e05508 - n/a
Main Authors Ghaemi, Kazem, Rajabi‐Moghaddam, Mahdieh, Abbaszadeh, Hamid
Format Journal Article
LanguageEnglish
Published England John Wiley & Sons, Inc 01.03.2022
John Wiley and Sons Inc
Wiley
Subjects
Age
Aphasia
Biopsy
Brain cancer
brain neoplasms
Case Report
Case Reports
Chemotherapy
Cytogenetics
Lymphoma
Medical imaging
Medical prognosis
Metastasis
Neuroblastoma
primitive neuroectodermal tumors
Radiation therapy
skull
Surgery
Tumors
brain neoplasms
primitive neuroectodermal tumors
skull
Online AccessGet full text

Cover

More Information
Summary:Primary intracranial peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare malignancies that commonly affect children and adolescents. Only 10 cases over the age of 33 have been reported. pPNETs have an aggressive behavior and a high tendency for local recurrence and distant metastasis. Here, we present a case of supratentorial pPNET that affected the left frontoparietal lobe of a 36‐year‐old female patient. The patient complained of aphasia during the last 2 months. Aphasia is reported for the first time as a result of a pPNET. In T1‐weighted MRI, a large mass with mixed isointense to hypointense signals was observed. The tumor was completely removed. Histopathologic examination was indicative of a small round cell tumor. Immunohistochemical analysis showed positivity for CD99. Presence of EWSR1 gene rearrangement confirmed the diagnosis. The patient's aphasia was gradually resolved post‐surgery. Six months follow‐up showed no evidence of local recurrence or metastasis. Primary intracranial primitive neuroectodermal tumors (PNETs) are extremely rare malignancies that, in particular, we should consider it in patients over 33 years of age. According to diverse clinical characteristics, we must keep in mind that these tumors can also manifest as aphasia, which was first mentioned in this case report.
Bibliography:Funding information
No funding.
ObjectType-Case Study-2
SourceType-Scholarly Journals-1
content type line 23
ObjectType-Report-1
ISSN:2050-0904
2050-0904
DOI:10.1002/ccr3.5508