scholarly journals Vaginal Ewing Sarcoma: An Uncommon Clinical Entity in Pediatric Patients

2017 ◽  
Vol 7 ◽  
pp. 17 ◽  
Author(s):  
Nathan M Cross ◽  
A Luana Stanescu ◽  
Erin R Rudzinski ◽  
Douglas S Hawkins ◽  
Marguerite T Parisi
Keyword(s):  
Surgical Approach ◽  
Ewing Sarcoma ◽  
Pediatric Patients ◽  
Multimodality Imaging ◽  
Primary Site ◽  
Primitive Neuroectodermal Tumors ◽  
Radiographic Features ◽  
Ewing Tumors ◽  
Neuroectodermal Tumors ◽  
Prompt Diagnosis

Ewing sarcoma, including classical Ewing sarcoma of the bone and primitive neuroectodermal tumors arising in bone or extraosseous primary sites, is a highly aggressive childhood neoplasm. We present two cases of Ewing sarcoma arising from the vagina in young girls. Previously reported cases in literature focused on their pathologic rather than radiographic features. We describe the spectrum of multimodality imaging appearances of Ewing sarcoma at this unusual primary site. Awareness of vaginal Ewing tumors may facilitate prompt diagnosis and lead to a different surgical approach than the more commonly encountered vaginal rhabdomyosarcoma.

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 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.

scholarly journals Multiple Ewing Sarcoma/Primitive Neuroectodermal Tumors in the Mediastinum

Medicine ◽  
2016 ◽  
Vol 95 (7) ◽  
pp. e2725 ◽  
Author(s):  
Sung Hwan Bae ◽  
Jung Hwa Hwang ◽  
Bo Da Nam ◽  
Hyun Jo Kim ◽  
Ki-Up Kim ◽  
...  
Keyword(s):  
Ewing Sarcoma ◽  
Primitive Neuroectodermal Tumors ◽  
Neuroectodermal Tumors

scholarly journals Ewing Sarcoma/Primitive Neuroectodermal Tumors In The Mediastinum: A Case Report And Review Of Literature

2020 ◽  
Vol 6 (2) ◽  
pp. 1-4
Author(s):  
Qiang Du ◽  
Keyword(s):  
Ewing Sarcoma ◽  
Soft Tissues ◽  
Survival Rates ◽  
Surgical Excision ◽  
Malignant Neoplasms ◽  
Embryonic Cells ◽  
Free Survival ◽  
Primitive Neuroectodermal Tumors ◽  
Neuroectodermal Tumors ◽  
Ewing Family Of Tumors

Ewing Sarcoma/Primitive Neuroectodermal Tumors (ES/PNETs) are highly malignant neoplasms originating from primitive neural tube embryonic cells, and grouped in the Ewing family of tumors. These malignancies are relatively uncommon, most frequently arising from bones or soft tissues and rarely reported in the mediastinum. The present study describes a case of a 22-year-old woman with unresectable ES/PENT in the middle mediastinum. The patient died the next day after bronchoscopy. A review of the literature showed reports of 9 patients with mediastinal PENTs in the past 20 years. The therapy of choice for ES/PENT in mediastinum consists of combinations of surgical excision with neoadjuvant or adjuvant chemotherapy and radiation to improve event-free survival rates.

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.

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