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Extraosseous Primary Intracranial Ewing Sarcoma/peripheral Primitive Neuroectodermal Tumor: Series of Seven Cases and Review of Literature
| Content Provider | Semantic Scholar |
|---|---|
| Author | Singh, Amit Kumar Srivastava, Avdhesh Kumar Pal, Lily Sardhara, Jayesh Chunnilal Yadav, Rajan Singh, Shalini Bhaisora, Kamlesh Singh Das, Kuntal Kanti Mehrotra, Anant Sahu, Rabi Narayan Jaiswal, Awadhesh Kumar Behari, Sanjay |
| Copyright Year | 2018 |
| Abstract | Background The Ewing sarcoma peripheral PNET (ES-pPNET) is very rare small round cell tumour that involves the CNS as either a primary dural neoplasm or by direct extension from contiguous bone or soft tissue. Materials and Methods Biopsy proven cases of intracranial ES/pPNET with orbital involvement operated during Jan 2010-Jan 2014 were retrospectively included and their clinical data, operative and histological findings were reviewed from institutional oncology register. Results seven patients (4 males; 3 female) were studied with mean age at presentation of 13 years. Six patients had orbital involvement in one or other form. Surgical excision was gross total in five, near total in one, and subtotal in one patient. All patients received adjuvant therapy, only chemotherapy in 2, only Radiotherapy in four, both in one. MRI characteristics were studied in six patients. Four patients died with average survival of 33.2 months and three patients are having Progression free survival of average 23.3 months. Conclusions The EWS-pPNET is very rare tumour and very poorly described in literature. These tumours are showing special predilection for the frontotemporal dura and erode through the flat bone of cranium like orbital roof and lateral wall of the orbit. These tumours are aggressive, multi compartmental, vascular and very rapidly growing, so missing or overlooking the primary symptoms of dural stretching/bony involvement leads to delay in management and poor outcome. |
| Starting Page | 288 |
| Ending Page | 296 |
| Page Count | 9 |
| File Format | PDF HTM / HTML |
| PubMed reference number | 29682023v1 |
| Alternate Webpage(s) | https://doi.org/10.4103/1793-5482.228570 |
| DOI | 10.4103/1793-5482.228570 |
| Journal | Asian journal of neurosurgery |
| Volume Number | 13 |
| Language | English |
| Access Restriction | Open |
| Subject Keyword | Bifunctional Expression Vector Plasmid DNA-bi-shRNA EWS/FLI1 Type 1 Lipoplex Bone Tissue Bone structure of cranium Cessation of life Conflict (Psychology) Direct Extension Dura Mater Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor Ewings sarcoma Ewings sarcoma-primitive neuroectodermal tumor (PNET) Intracranial Hemorrhages Numerous Patients Peripheral Nervous System Neoplasms Pharmaceutical Adjuvants Skeletal bone Stimulation (motivation) Structure of flat bone Subtotal Therapeutic radiology procedure Uterine Corpus Sarcoma adjuvant therapy soft tissue |
| Content Type | Text |
| Resource Type | Article |
