Skip to main content
SpringerLink
Log in

Primary Orbital Malignancies

  • Chapter
  • First Online:
Emergencies of the Orbit and Adnexa

  • 837 Accesses

Abstract

Malignancies of the orbit can be classified as primary, secondary, or metastatic. Primary orbital malignancies arise within the orbit. Secondary orbital malignancies are extensions of locally invasive tumors from adjacent structures, whereas metastatic tumors travel from distant sites. Evaluation of all potential orbital malignancies requires a detailed systematic workup including clinical history, examination, ancillary testing, and imaging. This information can help to narrow the differential diagnosis and direct diagnostic procedures and treatment. Specialized radiologists and ophthalmic pathologists can be invaluable in determining the correct diagnosis. Additional specialists may be needed to direct medical therapies or for collaborative surgical management.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 149.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 199.00
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 199.00
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

References

  1. Rootman J. Diseases of the orbit: a multidisciplinary approach. Philadelphia: Lippincott; 1988. p. xxiv. 628 p.

    Google Scholar 

  2. Demirci H, Shields CL, Shields JA, Honavar SG, Mercado GJ, Tovilla JC. Orbital tumors in the older adult population. Ophthalmology. 2002;109(2):243–8.

    Article  PubMed  Google Scholar 

  3. Margo CE, Mulla ZD. Malignant tumors of the orbit. Analysis of the Florida Cancer Registry. Ophthalmology. 1998;105(1):185–90.

    Article  CAS  PubMed  Google Scholar 

  4. Shields JA, Shields CL. Atlas of eyelid and conjunctival tumors. Philadelphia: Lippincott Williams & Wilkins; 1999. p. xvi. 350 p.

    Google Scholar 

  5. Hoyt WF, Meshel LG, Lessell S, Schatz NJ, Suckling RD. Malignant optic glioma of adulthood. Brain. 1973;96(1):121–32.

    Article  CAS  PubMed  Google Scholar 

  6. Shields JA, Shields CL. Rhabdomyosarcoma: review for the ophthalmologist. Surv Ophthalmol. 2003;48(1):39–57.

    Article  PubMed  Google Scholar 

  7. Chess J, Ni C, Yin RQ, Wang WJ, Cassady JR, Weichselbaum RR, et al. Rhabdomyosarcoma. Int Ophthalmol Clin. 1982;22(1):163–82.

    Article  CAS  PubMed  Google Scholar 

  8. Shields CL, Shields JA, Honavar SG, Demirci H. Clinical spectrum of primary ophthalmic rhabdomyosarcoma. Ophthalmology. 2001;108(12):2284–92.

    Article  CAS  PubMed  Google Scholar 

  9. Abramson DH, Ellsworth RM, Tretter P, Wolff JA, Kitchin FD. The treatment of orbital rhabdomyosarcoma with irradiation and chemotherapy. Ophthalmology. 1979;86(7):1330–5.

    Article  CAS  PubMed  Google Scholar 

  10. Jakobiec FA, Howard GM, Rosen M, Wolff M. Leiomyoma and leiomyosarcoma of the orbit. Am J Ophthalmol. 1975;80(6):1028–42.

    Article  CAS  PubMed  Google Scholar 

  11. Meekins BB, Dutton JJ, Proia AD. Primary orbital leiomyosarcoma. A case report and review of the literature. Arch Ophthalmol. 1988;106(1):82–6.

    Article  CAS  PubMed  Google Scholar 

  12. Yeniad B, Tuncer S, Peksayar G, Mete O, Minareci O. Primary orbital leiomyosarcoma. Ophthal Plast Reconstr Surg. 2009;25(2):154–5.

    Article  PubMed  Google Scholar 

  13. Lin IC, Wu CT, Liao SL, Lin LL. Primary orbital leiomyosarcoma. Ophthal Plast Reconstr Surg. 2005;21(6):451–3.

    Article  PubMed  Google Scholar 

  14. Jakobiec FA, Rini F, Char D, Orcutt J, Rootman J, Baylis H, et al. Primary liposarcoma of the orbit. Problems in the diagnosis and management of five cases. Ophthalmology. 1989;96(2):180–91.

    Article  CAS  PubMed  Google Scholar 

  15. Lane CM, Wright JE, Garner A. Primary myxoid liposarcoma of the orbit. Br J Ophthalmol. 1988;72(12):912–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. McNab AA, Moseley I. Primary orbital liposarcoma: clinical and computed tomographic features. Br J Ophthalmol. 1990;74(7):437–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Naeser P, Mostrom U. Liposarcoma of the orbit: a clinicopathological case report. Br J Ophthalmol. 1982;66(3):190–3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Khurana S, Gupta AK, Sen S, Kashyap S. Primary liposarcoma of the orbit. Indian J Pathol Microbiol. 2014;57(4):617–9.

    Article  PubMed  Google Scholar 

  19. Eifrig DE, Foos RY. Fibrosarcoma of the orbit. Am J Ophthalmol. 1969;67(2):244–8.

    Article  CAS  PubMed  Google Scholar 

  20. Yanoff M, Scheie HG. Fibrosarcoma of orbit. Report of two patients. Cancer. 1966;19(11):1711–6.

    Article  CAS  PubMed  Google Scholar 

  21. Jakobiec FA, Tannenbaum M. The ultrastructure of orbital fibrosarcoma. Am J Ophthalmol. 1974;77(6):899–917.

    Article  CAS  PubMed  Google Scholar 

  22. Bahrami A, Folpe AL. Adult-type fibrosarcoma: a reevaluation of 163 putative cases diagnosed at a single institution over a 48-year period. Am J Surg Pathol. 2010;34(10):1504–13.

    Article  PubMed  Google Scholar 

  23. Dhir SP, Munjal VP, Jain IS, Banerjee AK, Sharma SK. Osteosarcoma of the orbit. J Pediatr Ophthalmol Strabismus. 1980;17(5):312–4.

    CAS  PubMed  Google Scholar 

  24. Alio 2nd JL, Sales-Sanz M, Vaz MA, Barrancos C, Reguero ME, Diamantopoulus J, et al. Primary extraosseous Ewing sarcoma of the orbit. Ophthal Plast Reconstr Surg. 2013;29(3):e91–3.

    Article  PubMed  Google Scholar 

  25. Lam DS, Li CK, Cheng LL, Teo JG, Chik KW, Kwan WH, et al. Primary orbital Ewing’s sarcoma: report of a case and review of the literature. Eye (Lond). 1999;13(Pt 1):38–42.

    Article  Google Scholar 

  26. Vlasak R, Sim FH. Ewing’s sarcoma. Orthop Clin N Am. 1996;27(3):591–603.

    CAS  Google Scholar 

  27. Demirci H, Shields CL, Karatza EC, Shields JA. Orbital lymphoproliferative tumors: analysis of clinical features and systemic involvement in 160 cases. Ophthalmology. 2008;115(9):1626–31. 31 e1-3.

    Article  PubMed  Google Scholar 

  28. Jenkins C, Rose GE, Bunce C, Cree I, Norton A, Plowman PN, et al. Clinical features associated with survival of patients with lymphoma of the ocular adnexa. Eye (Lond). 2003;17(7):809–20.

    Article  CAS  Google Scholar 

  29. Piro LD, White CA, Grillo-Lopez AJ, Janakiraman N, Saven A, Beck TM, et al. Extended Rituximab (anti-CD20 monoclonal antibody) therapy for relapsed or refractory low-grade or follicular non-Hodgkin’s lymphoma. Ann Oncol Off J Eur Soc Med Oncol/ESMO. 1999;10(6):655–61.

    Article  CAS  Google Scholar 

  30. Woolf DK, Kuhan H, Shoffren O, Akinnawo EM, Sivagurunathan B, Boyce H, et al. Outcomes of primary lymphoma of the ocular adnexa (orbital lymphoma) treated with radiotherapy. Clin Oncol. 2014.

    Google Scholar 

  31. Williams MD, Al-Zubidi N, Debnam JM, Shinder R, DeMonte F, Esmaeli B. Bone invasion by adenoid cystic carcinoma of the lacrimal gland: preoperative imaging assessment and surgical considerations. Ophthal Plast Reconstr Surg. 2010;26(6):403–8.

    Article  PubMed  Google Scholar 

  32. Esmaeli B, Golio D, Kies M, DeMonte F. Surgical management of locally advanced adenoid cystic carcinoma of the lacrimal gland. Ophthal Plast Reconstr Surg. 2006;22(5):366–70.

    Article  PubMed  Google Scholar 

  33. Ahmad SM, Esmaeli B, Williams M, Nguyen J, Fay A, Woog J, et al. American Joint Committee on Cancer classification predicts outcome of patients with lacrimal gland adenoid cystic carcinoma. Ophthalmology. 2009;116(6):1210–5.

    Article  PubMed  Google Scholar 

  34. Tellado MV, McLean IW, Specht CS, Varga J. Adenoid cystic carcinomas of the lacrimal gland in childhood and adolescence. Ophthalmology. 1997;104(10):1622–5.

    Article  CAS  PubMed  Google Scholar 

  35. Tse DT, Benedetto P, Dubovy S, Schiffman JC, Feuer WJ. Clinical analysis of the effect of intraarterial cytoreductive chemotherapy in the treatment of lacrimal gland adenoid cystic carcinoma. Am J Ophthalmol. 2006;141(1):44–53.

    Article  CAS  PubMed  Google Scholar 

  36. Tse DT, Kossler AL, Feuer WJ, Benedetto PW. Long-term outcomes of neoadjuvant intra-arterial cytoreductive chemotherapy for lacrimal gland adenoid cystic carcinoma. Ophthalmology. 2013;120(7):1313–23.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Meldrum ML, Tse DT, Benedetto P. Neoadjuvant intracarotid chemotherapy for treatment of advanced adenocystic carcinoma of the lacrimal gland. Arch Ophthalmol. 1998;116(3):315–21.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, CA, USA

    Seanna R. Grob MD, MAS

  2. Ophthalmic Plastic Surgery Service, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA, USA

    Lora Rabin Dagi Glass MD

  3. Ophthalmic Plastic Surgery Service, Department of Oculoplastic and Reconstructive Surgery, Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles Street, 10th Floor Oculoplastics Suite, Boston, MA, 02114, USA

    Suzanne K. Freitag MD

Authors
  1. Seanna R. Grob MD, MAS
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Lora Rabin Dagi Glass MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Suzanne K. Freitag MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Suzanne K. Freitag MD .

Editor information

Editors and Affiliations

  1. Medical Research Foundation, Sankara Nethralaya, Chennai, India

    Bipasha Mukherjee

  2. Hong Kong Eye Hospital, Hong Kong, China

    Hunter Yuen

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer India

About this chapter

Cite this chapter

Grob, S.R., Glass, L.R.D., Freitag, S.K. (2017). Primary Orbital Malignancies. In: Mukherjee, B., Yuen, H. (eds) Emergencies of the Orbit and Adnexa. Springer, New Delhi. https://doi.org/10.1007/978-81-322-1807-4_42

Download citation

  • DOI: https://doi.org/10.1007/978-81-322-1807-4_42

  • Published:

  • Publisher Name: Springer, New Delhi

  • Print ISBN: 978-81-322-1806-7

  • Online ISBN: 978-81-322-1807-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation