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Askin Tumour: A rare childhood thoraco -pulmonary malignancy of Ewing’s family of tumours

Year 2012, Volume: 34 Issue: 3, 352 - 355, 26.02.2012

Abstract

 

Abstact

Primitive neuro-ectodermal tumour of chest wall or the Askin tumour is a rare, malignant small cell neuroepithelioma that arises from the chest wall or lung. It is seen predominantly in children and adolescents. Although its appearance by light microscopy is similar to Ewing’s sarcoma, rhabdomyosarcoma, neuroblastoma and lymphoma, which account for bulk of chest wall neoplasms in this age group, yet the Askin tumour is a distinct entity. We present a case of Askin tumour in a child, a rare malignant tumour in the thoraco-pulmonary area with highly aggressive clinical course.

Keywords: Askin Tumour, primitive neuro-ectodermal tumour, malignant small cell neuroepithelioma

 

Özet

Göğüs duvarının primitif nöro-ektodermal tümörü veya diğer bir deyişle Askin tümörü nadir görülen bir küçük hücreli nöroepitelyoma olup göğüs duvarı veya akciğer kökenlidir. Ağırlıklı olarak çocuk ve adolesanlarda saptanır. Her ne kadar ışık mikroskopisindeki görünümü Ewing sarkomu, rabdomyosarkom, nöroblastom ve lenfoma gibi bu yaş grubunda çoğunlukla görülen göğüs duvarı neoplasmlarına benzese de Askin tümörü farklı bir antitedir. Bu makalede yüksek derecede agresif klinik seyirli, torakopulmoner alan yerleşimli nadir bir malignite olan Askin tümörlü bir çocuk hasta sunulmuştur.

Anahtar sözcükler: Askin tümörü, primitif nöroektodermal tümör, malign küçük hücreli nöroepitelyoma

References

  • Harimaya K, Oda Y, Matsuda S, Tanaka K, Chuman H, Iwamoto Y. Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: report of four cases and a review of the literature. Spine 2003; 28: E408-12.
  • Montgomery E. Soft tissue tumours. In: Silverberg SG, Delellis RF, Frable WJ, Livdsi VA, Wick MR, Editors. Silverberg’s Principles and Practice of Surgical Pathology and cytopathology. 4th ed. Churchill Livingstone: Elsevier; 2006; pp: 385-6. 3. Ultrasound in Askin Tumour. 2009 Jun 03. Available from: URL:http://www.mypacs.net/cases/ULTRASOUND-IN-ASKIN-TUMOUR- 24469293.html (Accessed on August 09, 2012)
  • Askin FB, Rosai J, Sibley RK, Dehner LP, McAlister WH. Malignant small cell tumor of the thoracopulmonary region in childhood: a distinctive clinicopathologic entity of uncertain histogenesis. Cancer 1979; 43: 2438-51.
  • Sallustio G, Pirronti T, Lasorella A, Natale L, Bray A, Marano P. Diagnostic imaging of primitive neuroectodermal tumour of the chest wall (Askin tumour). Pediatric Radiology 1998; 28: 697-702.
  • Fink IJ, Kurtz DW, Cazenave L, Lieber MR, Miser JS, Chandra R, Triche TJ. Malignant thoracopulmonary small-cell ("Askin") tumor. Am J Roentgenol 1985; 145: 517-20.
  • Cabezalí R, Lozano R, Bustamante E, Castiella T, Güemes A, Ramírez J, Moncada E, Sousa R, Gil I. Askin's tumor of the chest wall: A case report in an adult. J Thorac Cardiovasc Surg 1994; 107: 960-2.

Askin Tümörü: Ewing tümör ailesinin nadir görülen çocukluk çağı torakopulmoner malignitesi

Year 2012, Volume: 34 Issue: 3, 352 - 355, 26.02.2012

Abstract

Göğüs duvarının primitif nöro-ektodermal tümörü veya diğer bir deyişle Askin tümörü nadir görülen bir küçük hücreli nöroepitelyoma olup göğüs duvarı veya akciğer kökenlidir. Ağırlıklı olarak çocuk ve adolesanlarda saptanır. Her ne kadar ışık mikroskopisindeki görünümü Ewing sarkomu, rabdomyosarkom, nöroblastom ve lenfoma gibi bu yaş grubunda çoğunlukla görülen göğüs duvarı neoplasmlarına benzese de Askin tümörü farklı bir antitedir. Bu makalede yüksek derecede agresif klinik seyirli, torakopulmoner alan yerleşimli nadir bir malignite olan Askin tümörlü bir çocuk hasta sunulmuştur

References

  • Harimaya K, Oda Y, Matsuda S, Tanaka K, Chuman H, Iwamoto Y. Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: report of four cases and a review of the literature. Spine 2003; 28: E408-12.
  • Montgomery E. Soft tissue tumours. In: Silverberg SG, Delellis RF, Frable WJ, Livdsi VA, Wick MR, Editors. Silverberg’s Principles and Practice of Surgical Pathology and cytopathology. 4th ed. Churchill Livingstone: Elsevier; 2006; pp: 385-6. 3. Ultrasound in Askin Tumour. 2009 Jun 03. Available from: URL:http://www.mypacs.net/cases/ULTRASOUND-IN-ASKIN-TUMOUR- 24469293.html (Accessed on August 09, 2012)
  • Askin FB, Rosai J, Sibley RK, Dehner LP, McAlister WH. Malignant small cell tumor of the thoracopulmonary region in childhood: a distinctive clinicopathologic entity of uncertain histogenesis. Cancer 1979; 43: 2438-51.
  • Sallustio G, Pirronti T, Lasorella A, Natale L, Bray A, Marano P. Diagnostic imaging of primitive neuroectodermal tumour of the chest wall (Askin tumour). Pediatric Radiology 1998; 28: 697-702.
  • Fink IJ, Kurtz DW, Cazenave L, Lieber MR, Miser JS, Chandra R, Triche TJ. Malignant thoracopulmonary small-cell ("Askin") tumor. Am J Roentgenol 1985; 145: 517-20.
  • Cabezalí R, Lozano R, Bustamante E, Castiella T, Güemes A, Ramírez J, Moncada E, Sousa R, Gil I. Askin's tumor of the chest wall: A case report in an adult. J Thorac Cardiovasc Surg 1994; 107: 960-2.
There are 6 citations in total.

Details

Primary Language English
Journal Section Case Reports
Authors

S. Mathur

Promil Jain

Sunita Singh

K. Rattan

Narinder Dahiya

Sneh Singh

Publication Date February 26, 2012
Published in Issue Year 2012Volume: 34 Issue: 3

Cite

AMA Mathur S, Jain P, Singh S, Rattan K, Dahiya N, Singh S. Askin Tumour: A rare childhood thoraco -pulmonary malignancy of Ewing’s family of tumours. CMJ. December 2012;34(3):352-355.