9260/3 Ewing tumor


Related terms

Ewing sarcoma

Definitions

Nasal cavity and Sinuses
ICD-O-3 topography code: C30-C31
ICD10: C30-C31
           

Ewing sarcoma is a small round cell tumour that lacks morphologic, immunohistochemical, and electron microscopic evidence of neuroectodermal differentiation. It affects mostly males under age 20, and it can occur in soft tissue or bone.



Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

Ewing sarcoma is a small round cell tumour that lacks morphologic, immunohistochemical, and electron microscopic evidence of neuroectodermal differentiation. It affects mostly males under age 20, and it can occur in soft tissue or bone.



Bone
ICD-O-3 topography code: C40-C41
ICD10: C40-C41
           

Ewing sarcoma (EWS) and primitive neuroectodermal tumours (PNET) represent a group of small round cell neoplasms with variable degrees of neuroectodermal differentiationis. EWS typically shows little or no neurodectodermal features.The Ewing family of tumours (EFT) is characterized by a recurrent (t11;22)(q24;q12) chromosomal translocation, detectable in approximately 85% of cases that results in an in-frame fusion between the EWS and FLI1 genes.

Ewing sarcoma of bone most frequently arises in the diaphysis or metaphyseal-diaphyseal portion of long bones. The tumours are ill-defined osteolytic lesions and often necrotic and haemorrhagic. Permeative or motheaten bone destruction, frequently associated with "onion-skin"-like multilayered periosteal reaction, is characteristic. The cortex overlying the tumour is irregularly thinned or thickened. .
Ewing sarcoma is the second most common sarcoma of bone and soft tissues in children. It shows a peak incidence in the second decade of life; patients older than 30 years are very rare. The current 5-years survival rate is estimated to be 41% 1
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Pancreas
ICD-O-3 topography code: C25
ICD10: C25
           

A small round cell tumor that lacks morphologic, immunohistochemical, and electron microscopic evidence of neuroectodermal differentiation. It represents one of the two ends of the spectrum called Ewing's sarcoma/peripheral neuroectodermal tumor. It affects mostly males under age 20, and it can occur in soft tissue or bone.

Tumours of this family (peripheral primitive neuroectodermal tumours [PNETs]) have been reported in the pancreas
2
Click to access Pubmed
Movahedi-Lankarani S, Hruban RH, Westra WH, Klimstra DS (2002)
Primitive neuroectodermal tumors of the pancreas: a report of seven cases of a rare neoplasm.
Am J Surg Pathol 26: 1040-7



. These small round blue cell tumours have rearrangement of the EWSR1 gene, usually a EWSR1-FLI1 fusion with t(11;22) translocation, and are strongly immunoreactive for CD99. They often express synaptophysin, and occasionally keratins. Before making a diagnosis of Ewing sarcoma, other small round blue cell tumours and pancreatic neuroendocrine neoplasms must be excluded 3
 
Hruban RH, Pitman MB, and Klimstra DS
Tumors of the Pancreas. Armed Forces Institute of Pathology
Washington, DC 2007



4
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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Vagina
ICD-O-3 topography code: C52
ICD10: C52
           

Ewing sarcoma is a small round cell tumour that lacks morphologic, immunohistochemical, and electron microscopic evidence of neuroectodermal differentiation. It affects mostly males under age 20, and it can occur in soft tissue or bone.



Vulva
ICD-O-3 topography code: C51
ICD10: C51
           

Ewing sarcoma is a small round cell tumour that lacks morphologic, immunohistochemical, and electron microscopic evidence of neuroectodermal differentiation. It affects mostly males under age 20, and it can occur in soft tissue or bone.



Skin
ICD-O-3 topography code: C44
ICD10: C44
           

Ewing sarcoma is a small round cell tumour that lacks morphologic, immunohistochemical, and electron microscopic evidence of neuroectodermal differentiation. It affects mostly males under age 20, and it can occur in soft tissue or bone.