Peripheral Primitive Neuroectodermal Tumors (pPNET)

2011 ◽  
pp. 1911-1911
Keyword(s):  
Primitive Neuroectodermal Tumors ◽  
Neuroectodermal Tumors ◽  
Peripheral Primitive Neuroectodermal Tumors

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

2005 ◽  
Vol 129 (1) ◽  
pp. e11-e15 ◽  
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.

Peripheral primitive neuroectodermal tumors in adults: documentation by molecular analysis.

1998 ◽  
Vol 16 (3) ◽  
pp. 1150-1157 ◽  
Author(s):  
E R Lawlor ◽  
J A Mathers ◽  
T Bainbridge ◽  
D E Horsman ◽  
A Kawai ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Molecular Analysis ◽  
Fusion Gene ◽  
Molecular Diagnostic ◽  
Gene Fusions ◽  
Primitive Neuroectodermal Tumors ◽  
Pathologic Features ◽  
Neuroectodermal Tumors ◽  
Objective Evidence ◽  
Peripheral Primitive Neuroectodermal Tumors

PURPOSE The Ewing tumor (ET) family of peripheral primitive neuroectodermal tumors (pPNETs) are primitive small round-cell tumors (SRCTs) of the bone and soft tissue that occur predominantly in children and adolescents. However, pPNETs only rarely enter the differential diagnosis of bone and soft tissue SRCTs in adults. Recently, gene fusions between the EWS gene and different members of the ETS transcription factor family have been shown to occur in virtually all pPNETs and thus constitute a pathognomonic marker for this tumor subclass. The aim of the present study was to document EWS/ETS fusion gene expression in suspected pPNETs of adults as objective evidence for the existence of this tumor family in older patients. PATIENTS AND METHODS The three contributing molecular diagnostic laboratories retrospectively compiled a cohort of all SRCT cases in which EWS/ETS gene fusions had been shown by molecular analysis. This cohort was surveyed for cases that occurred in patients aged 40 years or older, which were then analyzed for their clinical and pathologic features. RESULTS Nine patients between 40 and 65 years of age were found to have tumors positive for EWS/ETS gene fusions. Standard histopathologic and clinical features of these cases, other than age, were similar to those of childhood pPNETs. Patients were initiated on appropriate therapy after molecular analysis confirmed the diagnosis of pPNET. CONCLUSION Identification of an EWS/ETS gene fusion is useful in providing objective evidence of the diagnosis of pPNET in patients over the age of 40 years. This diagnosis should be considered in adults who present with bone and soft tissue SRCTs and appropriate biopsy specimens should be collected for molecular analysis at the time of diagnosis.

Clinical presentation and long-term outcome of primary spinal peripheral primitive neuroectodermal tumors

2015 ◽  
Vol 124 (3) ◽  
pp. 455-463 ◽  
Author(s):  
Xianzeng Tong ◽  
Xiaofeng Deng ◽  
Tao Yang ◽  
Chenlong Yang ◽  
Liang Wu ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Primitive Neuroectodermal Tumors ◽  
Neuroectodermal Tumors ◽  
Peripheral Primitive Neuroectodermal Tumors
Sign in / Sign up

Export Citation Format

Share Document