scholarly journals Treatment Outcomes and Prognostic Factors of Patients With Primary Spinal Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumors

2019 ◽  
Vol 9 ◽  
Author(s):  
Jun Chen ◽  
Mengxue Li ◽  
Yifeng Zheng ◽  
Lei Zheng ◽  
Fanfan Fan ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Treatment Outcomes ◽  
Ewing Sarcoma ◽  
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.

scholarly journals Primary Ewing sarcoma/peripheral primitive neuroectodermal tumors in the cranial bone and mobile spine: what is the difference?

BMC Surgery ◽  
2022 ◽  
Vol 22 (1) ◽  
Author(s):  
Jun Chen ◽  
Shi-Zhou Wu ◽  
Jie Tan ◽  
Qing-Yi Zhang ◽  
Bo-Quan Qin ◽  
...  
Keyword(s):  
Ewing Sarcoma ◽  
Survival Rates ◽  
Patient Characteristics ◽  
Cranial Bone ◽  
Primitive Neuroectodermal Tumors ◽  
Kaplan Meier ◽  
Neuroectodermal Tumors ◽  
Significant Difference ◽  
Mobile Spine ◽  
Peripheral Primitive Neuroectodermal Tumors

Abstract Background Primary Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are aggressive bone tumors that rarely occur in the axial skeleton, including the cranial bone and mobile spine. The purpose of this study was to investigate whether there were any differences in patient characteristics, treatment strategies, and outcomes between patients with ES/pPNETs of the cranial bone and those with ES/pPNETs of the mobile spine. Methods A retrospective study was performed on 33 patients with ES/pPNETs who had been surgically treated and pathologically confirmed at our institution between 2010 and 2020. Patient characteristics were compared using Fisher exact tests or independent t tests. Survival rates were estimated via Kaplan–Meier survival analysis and compared using log-rank tests. Results Thirteen patients had ES/pPNETs of the cranial bone (39.4%), while 20 patients had ES/pPNETs of the mobile spine (60.6%). Patients with ES/pPNETs of the cranial bone had a younger mean age (14.8 vs 22.6 years; p = 0.047) and longer mean disease duration (2.5 vs 1.9 months; p = 0.008) compared with those of patients with ES/pPNETs of the mobile spine. Kaplan–Meier analysis showed that gross total resection (GTR) and radiotherapy resulted in a longer median survival time. The overall survival rates and progression-free survival rates of patients with ES/pPNETs of the cranial bone versus those of the mobile spine were not significantly different (p = 0.386 and p = 0.368, respectively). Conclusions Patients with ES/pPNETs of the cranial bone were younger compared to patients with ES/pPNETs of the mobile spine. There was no significant difference in the prognosis of patients with ES/pPNETs of the cranial bone versus those of the mobile spine. Taken together, our findings suggest that GTR and radiotherapy offer the best prognosis for improved long-term survival.

scholarly journals The Ews/Fli-1 Fusion Gene Switches the Differentiation Program of Neuroblastomas to Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumors

2004 ◽  
Vol 64 (4) ◽  
pp. 1266-1277 ◽  
Author(s):  
Checo J. Rorie ◽  
Venetia D. Thomas ◽  
Pengchin Chen ◽  
Heather Hanson Pierce ◽  
John P. O’Bryan ◽  
...  
Keyword(s):  
Ewing Sarcoma ◽  
Fusion Gene ◽  
Primitive Neuroectodermal Tumors ◽  
Neuroectodermal Tumors ◽  
Gene Switches ◽  
Peripheral Primitive Neuroectodermal Tumors

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.

scholarly journals Massive Embryonal Rhabdomyosarcoma of the Hand in an Infant With Metastasis at Birth: Management Dilemma

Hand ◽  
2017 ◽  
Vol 12 (5) ◽  
pp. NP109-NP112
Author(s):  
Jayakrishnan K. Narayana Kurup ◽  
Vinay C. Kamble ◽  
Ashwath M. Acharya ◽  
Anil K. Bhat
Keyword(s):  
Ewing Sarcoma ◽  
Lymphatic Metastasis ◽  
Striated Muscle ◽  
Malignant Tumors ◽  
Embryonal Rhabdomyosarcoma ◽  
Unusual Feature ◽  
Primitive Neuroectodermal Tumors ◽  
Histological Features ◽  
The Poor ◽  
Neuroectodermal Tumors

Background: Rhabdomyosarcomas are malignant tumors arising from striated muscle but can be often confused with primitive neuroectodermal tumors and Ewing sarcoma. They are often classified based on age of presentation and histological features. Three major types of rhabdomyosarcomas are embryonal, alveolar, and pleomorphic with characteristic presentations. Method: Here, we present a case of embryonal rhabdomyosarcoma in the hand of a 5-month-old child with lymphatic metastasis, the age, site, and metastasis being unusual feature for this type of rhabdomyosarcoma. Result: The child succumbed to the complications soon after initiation of chemotherapy. Conclusion: Age, site, metastasis at birth and treatment related complications contribute to the poor outcome.

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