Abstract
Primitive neuroectodermal tumours (PNETs) are a group of malignant soft tissue tumours of neuroepithelial origin that arise from primitive neural crest cells. Most of the PNETs occur in the central nervous system (CNS). If the origin is outside the CNS it is called peripheral primitive neuroectodermal tumour (pPNET). Histopathologically, PNETs consist of small round cells with a hyperchromatic nuclei, high nuclear-cytoplasmic ratio and varying degrees of neural differentiation detectable by immunohistochemical or ultrastructural techniques. pPNETs occur predominantly in children and young adults and show no gender difference. Occurrences of pPNETs in the orbit are infrequent and to the best of our knowledge only eighteen cases have been reported in the literature up to now. In this study, we present clinic, radiologic and histopathologic features of an orbital mass in an 8-year-old boy, which was diagnosed as a primary orbital pPNET confirmed by immunohistochemistry.
References
- 1. Garrity JA, Henderson JW, Cameron JD. Henderson’s orbital tumors. Primitive Neuroectodermal Tumors. 4th edition. Philadelphia: Lippincott Williams & Wilkins, 2007:179–81.
- 2. Jürgens H, Bier V, Harms D, et al. Malignant peripheral neuroectodermal tumors. A retrospective analysis of 42 patients. Cancer 1988;61:349-57.
- 3. Santra G, Sinha PK, De D, Phaujdar S. Peripheral type of primitive neuroectodermal tumors arising from the left orbital flor. Singapore Med J 2011;52:138-40.
- 4. Dipankar D, Ganesh CK, Panna D, Akshay CD. Primary primitive neuroectodermal tumor of the orbit. Indian J Ophthalmol 2009;57: 391-3.
- 5. Hemalatha AL, Asha U. Primary extraosseous Ewing sarcoma/ PNET at an extraordinary site - the orbit. NJBMS 2012;1:83-4.
- 6. Kim UR, Arora V, Devanand J, Khazei HM. Multimodality treatment approach in management of primary peripheral primitive neuroectodermal tumor of the orbit. Indian J Ophthalmol 2009;57:395– 8. doi: 10.4103/0301-4738.55067. 2009.
- 7. Tamer C, Oksuz H, Hakverdi S, Karazincir S, Balci A, Yaldiz M. Primary peripheral primitive neuroectodermal tumor of the orbit. Can J Ophthalmol 2007;42:138–40.
- 8. Khoury JD. Ewing sarcoma family of tumors. Adv Anat Pathol 2005; 12:212-20.
- 9. Folpe AL, Goldblum JR, Rubin BP, et al. Morphologic and immunophenotypic diversity in Ewing family tumors: a study of 66 genetically confirmed cases. Am J Surg Pathol 2005;29: 1025-33.
- 10. Ishii N, Hiraga H, Sawamura Y, Shinohe Y, Nagashima K. Alternative EWS-FLI1 fusion gene and MIC2 expression in peripheral and central primitive neuroectodermal tumors. Neuropathology 2001;21:40-4.
- 11. Romero R, Castano A, Abelairas J, et al. Peripheral primitive neuroectodermal tumor of the orbit. BJO 2011;95: 915-20. doi:10.1136/bjo.2010.186833.
- 12. Miser JS, Krailo MD, Tarbell NJ, et al. Treatment of metastatic Ewing’s sarcoma or primitive neuroectodermal tumor of bone: evaluation of combination ifosfamide and etoposide-a Children’s Cancer Group and Pediatric Oncology Group study. J Clin Oncol 2004;22:2873-6.
- 13. Romero R, Abelairas J, Sanz J, Sendagorta E. Recurrence of peripheral primitive neuroectodermal tumor of the orbit with systemic metastases. Arch Soc Esp Oftalmol 2006;81:599-602.
Çok nadir bir pediatrik periferik primitif nöroektodermal tümör vakası: Orbital primitif nöroektodermal tümör
Abstract
Primitif nöroektodermal tümörler (PNET) primitif nöral krest
hücrelerinden köken alan bir grup malign yumuşak doku
tümörleridir. Tümör, santral sinir sistemi ya da beyin dışındaki
yumuşak dokulardan köken alırsa, periferik primitif nöroektodermal
tümör (pPNET) adını almaktadır. Histopatolojik olarak PNET
hiperkromatik nükleuslu, yüksek nükleus/stoplazma oranına
sahip, immünohistokimyasal ve/veya ultrastrüktürel teknikler
ile saptanabilen değişik derecelerde nöral diferansiasyona sahip
küçük yuvarlak hücrelerden oluşmaktadır. pPNET genellikle
genç erişkin ve çocuklarda ortaya çıkmakta olup cinsiyet ayrımı
göstermez. Orbital yerleşim nadir izlenmektedir ve şimdiye kadar,
literatürde yalnızca on sekiz vaka bildirimine ulaşılmaktadır. Biz,
bu çalışmamızda immunohistokimyasal olarak pPNET tanısı
onaylanmış 8 yaşında erkek çocuğun gözünde saptanan kitlenin
klinik, radyolojik ve histopatolojik özelliklerini sunmayı amaçladık
References
- 1. Garrity JA, Henderson JW, Cameron JD. Henderson’s orbital tumors. Primitive Neuroectodermal Tumors. 4th edition. Philadelphia: Lippincott Williams & Wilkins, 2007:179–81.
- 2. Jürgens H, Bier V, Harms D, et al. Malignant peripheral neuroectodermal tumors. A retrospective analysis of 42 patients. Cancer 1988;61:349-57.
- 3. Santra G, Sinha PK, De D, Phaujdar S. Peripheral type of primitive neuroectodermal tumors arising from the left orbital flor. Singapore Med J 2011;52:138-40.
- 4. Dipankar D, Ganesh CK, Panna D, Akshay CD. Primary primitive neuroectodermal tumor of the orbit. Indian J Ophthalmol 2009;57: 391-3.
- 5. Hemalatha AL, Asha U. Primary extraosseous Ewing sarcoma/ PNET at an extraordinary site - the orbit. NJBMS 2012;1:83-4.
- 6. Kim UR, Arora V, Devanand J, Khazei HM. Multimodality treatment approach in management of primary peripheral primitive neuroectodermal tumor of the orbit. Indian J Ophthalmol 2009;57:395– 8. doi: 10.4103/0301-4738.55067. 2009.
- 7. Tamer C, Oksuz H, Hakverdi S, Karazincir S, Balci A, Yaldiz M. Primary peripheral primitive neuroectodermal tumor of the orbit. Can J Ophthalmol 2007;42:138–40.
- 8. Khoury JD. Ewing sarcoma family of tumors. Adv Anat Pathol 2005; 12:212-20.
- 9. Folpe AL, Goldblum JR, Rubin BP, et al. Morphologic and immunophenotypic diversity in Ewing family tumors: a study of 66 genetically confirmed cases. Am J Surg Pathol 2005;29: 1025-33.
- 10. Ishii N, Hiraga H, Sawamura Y, Shinohe Y, Nagashima K. Alternative EWS-FLI1 fusion gene and MIC2 expression in peripheral and central primitive neuroectodermal tumors. Neuropathology 2001;21:40-4.
- 11. Romero R, Castano A, Abelairas J, et al. Peripheral primitive neuroectodermal tumor of the orbit. BJO 2011;95: 915-20. doi:10.1136/bjo.2010.186833.
- 12. Miser JS, Krailo MD, Tarbell NJ, et al. Treatment of metastatic Ewing’s sarcoma or primitive neuroectodermal tumor of bone: evaluation of combination ifosfamide and etoposide-a Children’s Cancer Group and Pediatric Oncology Group study. J Clin Oncol 2004;22:2873-6.
- 13. Romero R, Abelairas J, Sanz J, Sendagorta E. Recurrence of peripheral primitive neuroectodermal tumor of the orbit with systemic metastases. Arch Soc Esp Oftalmol 2006;81:599-602.
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | Case Reports |
| Authors | |
| Publication Date | September 30, 2015 |
| Published in Issue | Year 2013 Volume: 26 Issue: 3 |
