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Malignant Pediatric Tumors

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Orbital Tumors

Abstract

The most common pediatric orbital malignancy in the developed countries is rhabdomyosarcoma. In the developing world, however, the most common orbital malignancies in the pediatric group are not primary malignant tumors, but rather secondary and metastatic lesions, such as retinoblastoma, Burkitt’s lymphoma, leukemia, and neuroblastoma. The clinical, histopathological, radiologic, and therapeutic aspects of these tumors are detailed in this chapter.

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References

  1. Katowitz JA. Pediatric oculoplastic surgery. New York: Springer; 2002. p. 491–2.

    Book  Google Scholar 

  2. Garrity JA, Henderson JW, Cameron JD. Orbital tumors. Philadelphia: Lippincott Williams & Wilkins; 2007.

    Google Scholar 

  3. Rootman J. Diseases of the orbit. Philadelphia: Lippincott Williams & Wilkins; 2003.

    Google Scholar 

  4. Kodsi SR, Shetlar DJ, Campbell RJ, et al. A review of 340 orbital tumors in children during a 60-year period. Am J Ophthalmol. 1994;117:177–82.

    Article  PubMed  CAS  Google Scholar 

  5. Chuka-Okosa CM, Uche NJ, Kizor-Akaraiwe NN. Orbito-ocular neoplasms in Enugu, South-Eastern, Nigeria. West Afr J Med. 2008;27(3):144–7.

    PubMed  CAS  Google Scholar 

  6. Shinder R, Al-Zubidi N, Esmaeli B. Survey of orbital tumors at a comprehensive cancer center in the United States. Head Neck. 2011;33(5):610–4.

    Article  PubMed  Google Scholar 

  7. Frezzotti R, Hadjistilianou T, Tosi P. Rhabdomyosarcoma of the orbit: clinical and histopathological analysis of a personal series of 19 cases. Orbit. 1990;9:307–12.

    Article  Google Scholar 

  8. Karcioglu ZA, Hadjistilianou D, Rozans M, et al. Diagnosis and management of orbital rhabdomyosarcoma. Cancer Control. 2004;11:328–33.

    PubMed  Google Scholar 

  9. Shields JA, Shields CL. Rhabdomyosarcoma: review for the ophthalmologist. Surv Ophthalmol. 2003;48:39–57.

    Article  PubMed  Google Scholar 

  10. Finger P, Harbour JW, Karcioglu ZA. Risk factors for metastasis in retinoblastoma [major review]. Surv Ophthalmol. 2002;47:1–16.

    Article  PubMed  Google Scholar 

  11. Günalp I, Gündüz K. Pediatric orbital tumors in Turkey. Ophthal Plast Reconstr Surg. 1995;11(3):193–9.

    Article  PubMed  Google Scholar 

  12. Adeyemi BF, Kolude BM, Ogun GO, Akang EE. Paediatric head and neck malignancies in Ibadan, Nigeria. Afr J Med Med Sci. 2009;38(1):55–62.

    PubMed  CAS  Google Scholar 

  13. Gupta R, Yadav JS, Yadav S, Wadood A. Orbital involvement in nonendemic Burkitts lymphoma. Orbit. 2012;31(6):441–5.

    Article  PubMed  Google Scholar 

  14. Omoregie FO, Saheeb BD, Onasanya PO. Periradicular Burkitt’s lymphoma: a report of 2 cases from Nigeria and review of the literature. Afr J Med Sci. 2008;37(3):279–83.

    CAS  Google Scholar 

  15. Newton WA, Gehan EA, Webber BL, et al. Classification of rhabdomyosarcomas and related sarcomas. Cancer. 1995;76:1073–85.

    Article  PubMed  Google Scholar 

  16. Warner MA, Weber AL, Jakobiec FA. Benign and malignant tumors of the orbital cavity including the lacrimal gland. In: Imaging of the globe, orbit and visual pathway. Philadelphia: WB Saunders; 1996. p. 123–42.

    Google Scholar 

  17. Shaib SA, Moseley I, Wright JE. Orbital rhabdomyosarcoma—the radiological characteristics. Clin Radiol. 1998;53:357–62.

    Article  Google Scholar 

  18. Cota N, Chandna A, Abernethy LJ. Orbital abscess masquerading as a rhabdomyosarcoma. J AAPOS. 2000;4:318–20.

    Article  PubMed  CAS  Google Scholar 

  19. Mafee MF, Pai E, Binu P. Rhabdomyosarcoma of the orbit. Evaluation with MR imaging and CT. Radiol Clin North Am. 1998;36:1215–27.

    Article  PubMed  CAS  Google Scholar 

  20. Roshdy N, Shahin M, Kishk H, et al. MRI in diagnosis of orbital masses. Curr Eye Res. 2010;35(11):986–91.

    Article  PubMed  Google Scholar 

  21. Crist W, Gehan EA, Ragad AH, et al. The third intergroup Rhabdomyosarcoma study. J Clin Oncol. 1995;13:610–30.

    PubMed  CAS  Google Scholar 

  22. Shields JA, Shields CL, Honavar SG, Demirci H. Clinical spectrum of primary ophthalmic rhabdomyosarcoma. Ophthalmology. 2001;108:2284–92.

    Article  PubMed  CAS  Google Scholar 

  23. Shields CL, Shields JA, Honavar SG, et al. Primary ophthalmic rhabdomyosarcoma in 33 patients. Trans Am Ophthalmol Soc. 2001;99:133–42.

    PubMed  CAS  PubMed Central  Google Scholar 

  24. Buwalda J, Schouwenburg PF, Blank LE, et al. A novel local treatment strategy for advanced stage head and neck rhabdomyosarcomas in children: results of the AMORE protocol. Eur J Cancer. 2003;39:1594–602.

    Article  PubMed  CAS  Google Scholar 

  25. Raney RB, Anderson JR, Kollath J, et al. Late effects of therapy in 94 patients with localized rhabdomyosarcoma of the orbit: report from the Intergroup Rhabdomyosarcoma Study (IRS)-III, 1984–1991. Med Pediatr Oncol. 2000;34:413–20.

    Article  PubMed  CAS  Google Scholar 

  26. Breneman JC, Wiener ES. Issues in the local control of rhabdomyosarcoma. Med Pediatr Oncol. 2000;35:104–9.

    Article  PubMed  CAS  Google Scholar 

  27. Oberlin O, Rey A, Anderson J, et al. Treatment of orbital rhabdomyosarcoma: survival and late effects of treatment. Results of an international workshop. J Clin Oncol. 2001;19:197–204.

    PubMed  CAS  Google Scholar 

  28. Fiorillo A, Migliorati R, Vassallo P, et al. Radiation late effects in children treated for orbital rhabdomyosarcoma. Radiother Oncol. 1999;53:143–8.

    Article  PubMed  CAS  Google Scholar 

  29. Michalski JM, Sur RK, Harms WB, et al. Three-dimensional conformal radiation therapy in pediatric parameningeal rhabdomyosarcomas. Int J Radiat Oncol Biol Phys. 1995;33:985–91.

    Article  PubMed  CAS  Google Scholar 

  30. Janss AJ, Grundy R, Caan A, et al. Optic pathway and hypothalamic/chiasmatic gliomas in children younger than age 5 years with 6 years follow-up. Cancer. 1995;75:1051–9.

    Article  PubMed  CAS  Google Scholar 

  31. von Graefe A. Geschwulste des Schenerven. Arch Ophthalmol. 1864;10:193.

    Google Scholar 

  32. Dutton JJ. Gliomas of the anterior visual pathway. Surv Ophthalmol. 1994;38:427–52.

    Article  PubMed  CAS  Google Scholar 

  33. Karcioglu ZA, Kirzhner M. Clinicopathologic correlates in orbital disease. In: Tasman W, Jaeger EA, editors. Duane’s Foundations of Clinical Ophthalmology, vol. 4. Philadelphia: Lippincott; 2012. p. 28–30.

    Google Scholar 

  34. Chaundry IA, Morales J, Shamsi FA, et al. Orbitofacial neurofibromatosis: clinical characteristics and treatment outcome. Eye (Lond). 2011;26:593–2.

    Google Scholar 

  35. Erb HM, Uzcategui N, See RF, Burnstine MA. Orbitotemporal neurofibromatosis: classification and treatment. Orbit. 2007;26:223–38.

    Article  PubMed  Google Scholar 

  36. Dutton J. Optic nerve glioma. In: Dutton JJ, editor. Diagnostic atlas of orbital diseases. Philadelphia: WB Saunders; 2000. p. 120–1.

    Google Scholar 

  37. Listernick R, Charrow J, Greenwald MJ, et al. Optical pathway tumors in children: the effect of neurofibromatosis type 1 on clinical manifestation and natural history. J Pediatr. 1995;127:718–22.

    Article  PubMed  CAS  Google Scholar 

  38. Kuenzle CH, Weissert M, Roulet Bode H, et al. Follow-up of optic pathway gliomas in children with neurofibromatosis type 1. Neuropediatrics. 1994;25:295–300.

    Article  PubMed  CAS  Google Scholar 

  39. Lee AG, Dutton J. A practice pathway for the management of gliomas of the anterior visual pathway: an update and an evidence-based approach. Neuro-ophthalmology. 1999;22:139–55.

    Article  Google Scholar 

  40. Balcer L, Liu G, Heller G, et al. Visual loss in children with neurofibromatosis type 1 and optic pathway gliomas: relation to tumor location by magnetic resonance imaging. Am J Ophthalmol. 2001;131:442–5.

    Article  PubMed  CAS  Google Scholar 

  41. Liu GT. Optic gliomas of the anterior visual pathway. Curr Opin Ophthalmol. 2006;17(5):427–31.

    Article  PubMed  CAS  Google Scholar 

  42. Gaye G, Scott IU, Feuer W, et al. Long-term visual outcome in patients with anterior visual pathway gliomas. J Neuroophthalmol. 2001;21:1–7.

    Article  Google Scholar 

  43. Parsa CF, Hoyt CS, Lesser RL, et al. Spontaneous regression of optic gliomas. Arch Ophthalmol. 2001;119:516–29.

    Article  PubMed  CAS  Google Scholar 

  44. Sartor K. MR imaging of the skull and brain. A correlative text-atlas. Berlin: Springer; 1992.

    Book  Google Scholar 

  45. Chateil JF, Sousotte C, Pedespan JM, et al. MRI and clinical differences between optic pathway tumours in children with and without neurofibromatosis. Br J Radiol. 2001;74:24–31.

    Article  PubMed  CAS  Google Scholar 

  46. De Potter P, Shields CL, Shields JA. Optic nerve and meningeal tumors. In: De Potter P, Shields CL, Shields JA, editors. MRI of the eye and orbit. Philadelphia: JB Lippincott; 1995. p. 193–201.

    Google Scholar 

  47. Hollander MD, FitzPatrick M, O’Connor SG, et al. Optic gliomas. Radiol Clin North Am. 1999;37:59–71.

    Article  PubMed  CAS  Google Scholar 

  48. Karcioglu ZA, Yulug A, Haik BG. Tumors of the optic nerve. In: Margo CE, Hamed LM, Mames RN, editors. Diagnostic problems in clinical pathology. Philadelphia: WB Saunders; 1994. p. 105–14.

    Google Scholar 

  49. Grabenbauer G, Schuchardt U, Buchfelder M, et al. Radiation therapy of opticohypothalamic gliomas (OHG): radiographic response, vision and late toxicity. Radiother Oncol. 2000;54:239–45.

    Article  PubMed  CAS  Google Scholar 

  50. Khafaga Y, Hassounah M, Kandil A, et al. Optic gliomas: a retrospective analysis of 50 cases. Int J Radiat Oncol Biol Phys. 2003;56:807–12.

    Article  PubMed  Google Scholar 

  51. Shapey J, Danesh-Meyer HV, Kaye AH. Diagnosis and management of optic nerve glioma. J Clin Neurosci. 2011;18(12):1585–91.

    Article  PubMed  CAS  Google Scholar 

  52. Fuss M, Hug EB, Schaefer RA, et al. Proton radiation therapy (PRT) for pediatric optic pathway gliomas: comparison with 3D planned conventional photons and standard photon technique. Int J Radiat Oncol Biol Phys. 1999;45:1117–26.

    Article  PubMed  CAS  Google Scholar 

  53. Han SR, Kim KN, Yee GT, et al. Fractionated stereotactic radiotherapy using Novalis for confined intra-orbital optic nerve glioma in pediatric patient. Childs Nerv Syst. 2013;29(5):867–71.

    Article  PubMed  Google Scholar 

  54. Oystreck DT, Morales J, Chaudhry I. Visual loss in orbitofacial neurofibromatosis type 1. Ophthalmology. 2012;119(10):2168–73.

    Article  PubMed  Google Scholar 

  55. Shofty B, Ben-Sira L, Freedman S, et al. Visual outcome following chemotherapy for progressive optic pathway gliomas. Pediatr Blood Cancer. 2011;57(3):481–5.

    Article  PubMed  Google Scholar 

  56. Musarella MA, Chan HS, DeBoer G, Gallie BL. Ocular involvement in neuroblastoma: prognostic implications. Ophthalmology. 1984;91(8):936–40.

    Article  PubMed  CAS  Google Scholar 

  57. Amemiya T, Hayashida H, Dake Y. Metastatic orbital tumors in Japan: a review of the literature. Ophthalmic Epidemiol. 2002;9:35–47.

    Article  PubMed  Google Scholar 

  58. Metha V, Balachandran C, Lonikar V. Blueberry muffin baby: a pictorial differential diagnosis. Dermatol Online J. 2008;14(2):8.

    Google Scholar 

  59. Belgaumi AF, Kauffman WM, Cordoba J, et al. Blindness in children with neuroblastoma. Cancer. 1997;80:1997–2004.

    Article  PubMed  CAS  Google Scholar 

  60. Ahmed S, Goel S, Khandwala M, et al. Neuroblastoma with orbital metastasis: ophthalmic presentation and role of ophthalmologists. Eye (Lond). 2006;20:466–70.

    Article  CAS  Google Scholar 

  61. Bullock J, Goldberg SH, Rakes SM, et al. Primary orbital neuroblastoma. Arch Ophthalmol. 1989;107:1031–3.

    Article  PubMed  CAS  Google Scholar 

  62. Dutton JJ. Neuroblastoma. In: Dutton JJ, editor. Diagnostic atlas of orbital diseases. Philadelphia: WB Saunders; 2000. p. 118–9.

    Google Scholar 

  63. Parsons K, Bernhardt B, Strickland B. Targeted immunotherapy for high-risk neuroblastoma–the role of monoclonal antibodies. Ann Pharmacother. 2013;47(2):210–8.

    Article  PubMed  Google Scholar 

  64. Russo V, Scott IU, Querques G, et al. Orbital and ocular manifestations of acute childhood leukemia: clinical and statistical analysis of 180 patients. Eur J Ophthalmol. 2008;18(4):619–23.

    PubMed  CAS  Google Scholar 

  65. Stein Wexter R, Wooton-Gorges SL, West DC. Orbital granulocytic sarcoma: an unusual presentation of acute myelocytic leukaemia. Pediatr Radiol. 2003;33:136–9.

    Article  Google Scholar 

  66. Shields JA, Stopyra GA, Marr PB, et al. Bilateral orbital myeloid sarcoma as initial sign of acute myeloid leukaemia: case report and review of the literature. Arch Ophthalmol. 2003;121:138–42.

    Article  PubMed  Google Scholar 

  67. Noh BW, Park SW, Chun JE, et al. Granulocytic sarcoma in the head and neck: CT and MR imaging findings. Clin Exp Otorhinolaryngol. 2009;2(2):66–71.

    Article  PubMed  PubMed Central  Google Scholar 

  68. Zhou J, Bell D, Medeiros LJ. Myeloid sarcoma of the head and neck region. Arch Pathol Lab Med. 2013;137(11):1560–8.

    Article  PubMed  Google Scholar 

  69. Klco JM, Welch JS, Nguyen TT, et al. State of the art in myeloid sarcoma. Int J Lab Hematol. 2011;33(6):555–65.

    Article  PubMed  CAS  Google Scholar 

  70. Maka E, Lukáts O, Tóth J, Fekete S. Orbital tumour as initial manifestation of acute myeloid leukemia: granulocytic sarcoma: case report. Pathol Oncol Res. 2008;14(2):209–11.

    Article  PubMed  Google Scholar 

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Correspondence to Zeynel A. Karcioglu M.D. .

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Karcioglu, Z.A., Hadjistilianou, D. (2015). Malignant Pediatric Tumors. In: Karcioglu, Z. (eds) Orbital Tumors. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1510-1_26

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  • DOI: https://doi.org/10.1007/978-1-4939-1510-1_26

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