Clinical Features and Long-Term Outcome of Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumors: 14 Cases From a Single Institution

Abstract Background Calvarial Ewing tumor is a relatively rare differential among bony neoplasms. We present our experience of managing primary calvarial Ewing sarcoma (EWS), highlighting their clinical and radiological findings. Method In a retrospective analysis, we evaluated our 12-year database for pathologically proven EWS. A literature search was conducted for the comparative presentation and update on the management and outcome. Result From January 2008 to December 2020, we managed eight patients (male:female = 5:3; age range 6 months to 19 years, mean 11.5 years) harboring primary calvarial EWS. All cases underwent wide local excision; two patients required intradural tumor resection, while one required rotation flap for scalp reconstruction. Mean hospital stay was 8 days. All patients received adjuvant chemo- and radiotherapy. Three patients remained asymptomatic at 5 years of follow-up, while two patients died. Conclusion Primary calvarial EWS is a rare entity. It usually affects patients in the first two decades of life. These tumors can be purely intracranial, causing raised intracranial pressure symptoms, which may exhibit rapidly enlarging subgaleal tumors with only cosmetic deformities or symptoms of both. Radical excision followed by adjuvant therapy may offer a favorable long-term outcome.

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Intraventricular infusion test accuracy in predicting short- and long-term outcome of iNPH patients: a 10-year update of a three-decade experience at a single institution

Neurosurgical Review ◽

10.1007/s10143-021-01495-4 ◽

2021 ◽

Author(s):

Gianluca Trevisi ◽

Francesco Signorelli ◽

Chiara de Waure ◽

Vito Stifano ◽

Cosimo Sturdà ◽

...

Keyword(s):

Test Accuracy ◽

Infusion Test ◽

Single Institution ◽

Term Outcome ◽

Long Term Outcome ◽

Short And Long Term ◽

Intraventricular Infusion

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Intracranial Peripheral Primitive Neuroectodermal Tumors of the Cavernous Sinus: A Diagnostic Peculiarity

Archives of Pathology & Laboratory Medicine ◽

10.5858/2005-129-e11-ippnto ◽

2005 ◽

Vol 129 (1) ◽

pp. e11-e15 ◽

Cited By ~ 1

Author(s):

Muhammad Idrees ◽

Chirag Gandhi ◽

Simone Betchen ◽

James Strauchen ◽

Wesley King ◽

...

Keyword(s):

Cavernous Sinus ◽

Ewing Sarcoma ◽

Moderate Amount ◽

Primitive Neuroectodermal Tumors ◽

Neuroectodermal Tumors ◽

Male Preponderance ◽

Poorly Differentiated ◽

Immunohistochemical Techniques ◽

The Right ◽

Peripheral Primitive Neuroectodermal Tumors

Abstract Peripheral primitive neuroectodermal tumors (pPNETs) are aggressive, poorly differentiated neoplasms that occur in children and young adults. These tumors are associated with a peak incidence in the second decade and a slight male preponderance. Recently, Ewing sarcoma and pPNET tumors have been proven to carry identical translocations, the most common being t(11;22)(q24;q12). Intracranial Ewing sarcoma/pPNETs have rarely been described in the literature. We studied a case of intracranial pPNET arising in the right cavernous sinus of a 46-year-old man. On imaging, the tumor had both sellar and suprasellar components and was centered within the right parasellar region. Histologically, the tumor was composed of intermediate to large cells with round to oval hyperchromatic nuclei with distinct nucleoli. The cells contained a moderate amount of slightly basophilic cytoplasm. The tumor was markedly fibrotic and had collagen bands surrounding both individual and groups of cells. A large immunohistochemical panel was positive only for CD99 and vimentin. Fluorescence in situ hybridization did not show translocations associated with Ewing sarcoma/pPNET. However, a small percentage of these tumors can be negative for this translocation. In these cases, histology and immunohistochemical techniques in the absence of an alternative diagnosis are the only tools available to establish the diagnosis.

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