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J Neurol Surg B Skull Base 2021; 82(01): 081-090
DOI: 10.1055/s-0040-1722635
Typical Orbital Pathologies
Review Article

Primary Malignant Orbital Tumors

Jacquelyn Laplant
1   Department of Ophthalmology, Tulane University, New Orleans, Louisiana, United States
,
Kimberly Cockerham
2   Stanford Department of Ophthalmology, Byers Eye Institute, Palo Alto, California, United States
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Abstract

Objective Primary orbital malignancy is rare. Awareness of the characteristic clinical and imaging features is imperative for timely identification and management. Surgery remains an important diagnostic and treatment modality for primary orbital malignancy, but determining the optimal surgical approach can be challenging. The purpose of this article is to explore recent advances in the diagnosis, management, and surgical approaches for primary orbital malignancies.

Design In this review, the clinical presentation, imaging features, and medical and surgical management of primary orbital malignancies with representative cases will be discussed.

Setting Outpatient and inpatient hospital settings.

Participants Patients with diagnosed primary orbital malignancies.

Main Outcome Measures Descriptive outcomes.

Results Advancements in orbital imaging, microsurgical techniques, and multimodal therapy have improved the diagnosis and management of primary orbital malignancies. Special considerations for biopsy or resection are made based on the tumor's location, characteristics, nearby orbital structures, and goals of surgery. Minimally invasive techniques are supplanting traditional approaches to orbital surgery with less morbidity.

Conclusions Advances in imaging technologies and surgical techniques have facilitated the diagnosis and management of primary orbital malignancies. Evolution toward less invasive orbital surgery with focus on preservation and restoration of function is underway.

Keywords

primary orbital tumors - malignant orbital tumors - lymphoma - rhabdomyosarcoma - adenoid cystic carcinoma


Publikationsverlauf

Artikel online veröffentlicht:
18. Februar 2021

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • References

  • 1 Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1. Ophthalmology 2004; 111 (05) 997-1008
    CrossrefPubMedGoogle Scholar
  • 2 Margo CE, Mulla ZD. Malignant tumors of the orbit. Analysis of the Florida Cancer Registry. Ophthalmology 1998; 105 (01) 185-190
    CrossrefPubMedGoogle Scholar
  • 3 Bonavolontà G, Strianese D, Grassi P. et al. An analysis of 2,480 space-occupying lesions of the orbit from 1976 to 2011. Ophthal Plast Reconstr Surg 2013; 29 (02) 79-86
    CrossrefPubMedGoogle Scholar
  • 4 Shields JA, Bakewell B, Augsburger JJ, Flanagan JC. Classification and incidence of space-occupying lesions of the orbit. A survey of 645 biopsies. Arch Ophthalmol 1984; 102 (11) 1606-1611
    CrossrefPubMedGoogle Scholar
  • 5 Rootman J, White V, Connors J, Gascoyne R. Lymphoproliferative, leukemic, and histiocytic lesions of the orbit. In: Rootman J. ed. Diseases of the Orbit: A Multidisciplinary Approach. 2nd ed.. Philadelphia, PA: Lippincott Williams & Wilkins; 2003: 385-416
    Google Scholar
  • 6 Pastore G, Peris-Bonet R, Carli M, Martínez-García C, Sánchez de Toledo J, Steliarova-Foucher E. Childhood soft tissue sarcomas incidence and survival in European children (1978-1997): report from the Automated Childhood Cancer Information System project. Eur J Cancer 2006; 42 (13) 2136-2149
    CrossrefPubMedGoogle Scholar
  • 7 Karcioglu ZA, Hadjistilianou D. Malignant pediatric tumors. In: Karcioglu ZA. ed. Orbital Tumors: Diagnosis and Treatment. New York, NY: Springer; 2015: 349-359
    Google Scholar
  • 8 Mirzai H, Baser EF, Tansug N, Nese N, Isisag A. Primary orbital neuroblastoma in a neonate. Indian J Ophthalmol 2006; 54 (03) 206-208
    CrossrefPubMedGoogle Scholar
  • 9 Guzowski M, Tumuluri K, Walker DM, Maloof A. Primary orbital Ewing sarcoma in a middle-aged man. Ophthal Plast Reconstr Surg 2005; 21 (06) 449-451
    CrossrefPubMedGoogle Scholar
  • 10 Patel BC. Malignant orbital tumors. In: Hwang CJ, Patel BC, Singh AD. eds. Clinical Ophthalmic Oncology: Orbital Tumors. Berlin: Springer; 2019: 201-215
    CrossrefGoogle Scholar
  • 11 Al-Qudah M. Image-guided sinus surgery in sinonasal pathologies with skull base/orbital erosion. J Craniofac Surg 2015; 26 (05) 1606-1608
    CrossrefPubMedGoogle Scholar
  • 12 Felippu A, Mora R, Guastini L, Peretti G. Transnasal approach to the orbital apex and cavernous sinus. Ann Otol Rhinol Laryngol 2013; 122 (04) 254-262
    CrossrefPubMedGoogle Scholar
  • 13 Shields CL, Shields JA, Honavar SG, Demirci H. Clinical spectrum of primary ophthalmic rhabdomyosarcoma. Ophthalmology 2001; 108 (12) 2284-2292
    CrossrefPubMedGoogle Scholar
  • 14 Esmaeli B, Yin VT, Hanna EY. et al. Eye-sparing multidisciplinary approach for the management of lacrimal gland carcinoma. Head Neck 2016; 38 (08) 1258-1262
    CrossrefPubMedGoogle Scholar
  • 15 Bejjani GK, Cockerham K. Diagnosis and management of orbital tumors. Contemp Neurosurg 2003; 25 (02) 1-8
    CrossrefPubMedGoogle Scholar
  • 16 Kennerdell JS, Cockerham K. Orbital diagnosis. In: Nesi FA. ed. Smith's Textbook of Ophthalmic Plastic and Reconstructive Surgery. 2nd ed.. St Louis, MO: Mosby; 1997: 705-712
    Google Scholar
  • 17 Karcioglu ZA. Orbital Tumors: Diagnosis and Treatment. New York, NY: Springer; 2014
    Google Scholar
  • 18 Tailor TD, Gupta D, Dalley RW, Keene CD, Anzai Y. Orbital neoplasms in adults: clinical, radiologic, and pathologic review. Radiographics 2013; 33 (06) 1739-1758
    CrossrefPubMedGoogle Scholar
  • 19 Karcioglu ZA. Surgical techniques for orbital tumors. In: Karcioglu ZA. ed. Surgical Ophthalmic Oncology. Cham, Switzerland: Springer; 2019: 117-127
    CrossrefGoogle Scholar
  • 20 Cockerham K. Meningiomas and sarcomas. In: Miller NR. ed. Walsh and Hoyt's Clinical Neuro-Ophthalmology. Philadelphia, PA: Lippincott Williams & Wilkins; 1997: 2017-2082
    Google Scholar
  • 21 Shinder R, Esmaeli B. Primary orbital cancers in adults. In: Esmaeli B. ed. Ophthalmic Oncology. Berlin: Springer; 2010: 3-12
    CrossrefGoogle Scholar
  • 22 Cockerham KP, Hong SH, Browne EE. Orbital inflammation. Curr Neurol Neurosci Rep 2003; 3 (05) 401-409
    CrossrefPubMedGoogle Scholar
  • 23 Priel A, Oh S-R, Kikkawa DO, Korn BS. Surgical approaches to the orbit and optic nerve. In: Smith and Nesi's Ophthalmic Plastic and Reconstructive Surgery. New York, NY: Springer; 2012: 793-805
    Google Scholar
  • 24 Kennerdell JS, Cockerham K. Computed tomography of the orbit. In: Nesi FA. ed. Smith's Textbook of Ophthalmic Plastic and Reconstructive Surgery. 2nd ed.. St. Louis, MO: Mosby; 1997: 778-806
    Google Scholar
  • 25 Cockerham K, Kennerdell JS. Magnetic resonance imaging of the orbit. In: Nesi FA. ed. Smith's Ophthalmic Plastic and Reconstructive Surgery. 2nd ed.. St. Louis, MO: Mosby; 1997: 807-827
    Google Scholar
  • 26 Nasr AM, Chacra GIA. Ultrasonography in orbital differential diagnosis. In: Karcioglu Z. ed. Orbital Tumors. New York, NY: Springer; 2015: 69-81
    Google Scholar
  • 27 Ben Simon GJ, Annunziata CC, Fink J, Villablanca P, McCann JD, Goldberg RA. Rethinking orbital imaging establishing guidelines for interpreting orbital imaging studies and evaluating their predictive value in patients with orbital tumors. Ophthalmology 2005; 112 (12) 2196-2207
    CrossrefPubMedGoogle Scholar
  • 28 Campbell AA, Grob SR, Yoon MK. Novel surgical approaches to the orbit. Middle East Afr J Ophthalmol 2015; 22 (04) 435-441
    CrossrefPubMedGoogle Scholar
  • 29 Ali MJ, Naik MN, Kaliki S, Dave TV. Interactive navigation-guided ophthalmic plastic surgery: the usefulness of computed tomography angiographic image guidance. Ophthal Plast Reconstr Surg 2016; 32 (05) 393-398
    CrossrefPubMedGoogle Scholar
  • 30 Shields JA, Shields CL. Eyelid, Conjunctival, and Orbital Tumors: An Atlas and Textbook. Philadelphia, PA: Lippincott Williams & Wilkins; 2008
    Google Scholar
  • 31 Verity DH, Rose GE. Principles of orbital surgery. In: Hwang CJ, Patel BC, Singh AD. eds. Clinical Ophthalmic Oncology: Orbital Tumors. Berlin: Springer; 2019: 261-274
    CrossrefGoogle Scholar
  • 32 Bejjani GK, Cockerham K, Maroon JC, Kennerdell JS. Orbital tumors. In: Youmans JR. ed. Youmans Neurologic Surgery. 5th ed.. Philadelphia, PA: Saunders; 2004: 1371-1381
    Google Scholar
  • 33 Paluzzi A, Gardner PA, Fernandez-Miranda JC. et al. “Round-the-clock” surgical access to the orbit. J Neurol Surg B Skull Base 2015; 76 (01) 12-24
    PubMedGoogle Scholar
  • 34 Murchison AP, Rosen MR, Evans JJ, Bilyk JR. Endoscopic approach to the orbital apex and periorbital skull base. Laryngoscope 2011; 121 (03) 463-467
    CrossrefPubMedGoogle Scholar
  • 35 Tsirbas A, Kazim M, Close L. Endoscopic approach to orbital apex lesions. Ophthal Plast Reconstr Surg 2005; 21 (04) 271-275
    CrossrefPubMedGoogle Scholar
  • 36 Matsuda M, Akutsu H, Tanaka S, Matsumura A. Combined simultaneous transcranial and endoscopic endonasal resection of sphenoorbital meningioma extending into the sphenoid sinus, pterygopalatine fossa, and infratemporal fossa. Surg Neurol Int 2017; 8: 185
    CrossrefPubMedGoogle Scholar
  • 37 Bejjani GK, Cockerham KP, Kennerdel JS, Maroon JC. A reappraisal of surgery for orbital tumors. Part I: extraorbital approaches. Neurosurg Focus 2001; 10 (05) E2
    CrossrefPubMedGoogle Scholar
  • 38 Demirci H, Shields CL, Shields JA, Honavar SG, Mercado GJ, Tovilla JC. Orbital tumors in the older adult population. Ophthalmology 2002; 109 (02) 243-248
    CrossrefPubMedGoogle Scholar
  • 39 Demirci H, Shields CL, Karatza EC, Shields JA. Orbital lymphoproliferative tumors: analysis of clinical features and systemic involvement in 160 cases. Ophthalmology 2008; 115 (09) 1626-1631 1631.e1–1631.e3
    CrossrefPubMedGoogle Scholar
  • 40 Aronow ME, Hill BT, Singh AD. Orbital and adnexal lymphoma. In: Perry JD, Singh AD. eds. Clinical Ophthalmic Oncology: Orbital Tumors. Berlin: Springer; 2014: 123-139
    Google Scholar
  • 41 Hatef E, Roberts D, McLaughlin P, Pro B, Esmaeli B. Prevalence and nature of systemic involvement and stage at initial examination in patients with orbital and ocular adnexal lymphoma. Arch Ophthalmol 2007; 125 (12) 1663-1667
    CrossrefPubMedGoogle Scholar
  • 42 Mulay K, Narula R, Honavar SG. Primary vitreoretinal lymphoma. Indian J Ophthalmol 2015; 63 (03) 180-186
    CrossrefPubMedGoogle Scholar
  • 43 Mochizuki M, Singh AD. Epidemiology and clinical features of intraocular lymphoma. Ocul Immunol Inflamm 2009; 17 (02) 69-72
    CrossrefPubMedGoogle Scholar
  • 44 White WL, Ferry JA, Harris NL, Grove Jr AS. Ocular adnexal lymphoma. A clinicopathologic study with identification of lymphomas of mucosa-associated lymphoid tissue type. Ophthalmology 1995; 102 (12) 1994-2006
    CrossrefPubMedGoogle Scholar
  • 45 Cockerham KP, Bejjani GK, Kennerdell JS, Maroon JC. Surgery for orbital tumors. Part II: transorbital approaches. Neurosurg Focus 2001; 10 (05) E3
    CrossrefPubMedGoogle Scholar
  • 46 Shields JA, Shields CL, Epstein JA, Scartozzi R, Eagle Jr RC. Review: primary epithelial malignancies of the lacrimal gland: the 2003 Ramon L. Font lecture. Ophthal Plast Reconstr Surg 2004; 20 (01) 10-21
    CrossrefPubMedGoogle Scholar
  • 47 Milman T, Shields JA, Husson M, Marr BP, Shields CL, Eagle Jr RC. Primary ductal adenocarcinoma of the lacrimal gland. Ophthalmology 2005; 112 (11) 2048-2051
    CrossrefPubMedGoogle Scholar
  • 48 Verity DH, Rose GE. Lacrimal gland tumors. In: Hwang CJ, Patel BC, Singh AD. eds. Clinical Ophthalmic Oncology: Orbital Tumors. Berlin: Springer; 2019: 165-175
    CrossrefGoogle Scholar
  • 49 Lorenzano D, Rose GE. The “wedge sign”: an imaging sign for aggressive lacrimal gland disease. Ophthalmology 2017; 124 (07) 1081-1083
    CrossrefPubMedGoogle Scholar
  • 50 Ramakrishna R, Raza SM, Kupferman M, Hanna E, DeMonte F. Adenoid cystic carcinoma of the skull base: results with an aggressive multidisciplinary approach. J Neurosurg 2016; 124 (01) 115-121
    CrossrefPubMedGoogle Scholar
  • 51 Zoumalan CI, Zoumalan RA, Cockerham KP. Surgical management of lacrimal gland tumors. Oper Tech Otolaryngol Head Neck Surg 2008; 19 (04) 252-257
    CrossrefPubMedGoogle Scholar
  • 52 Abugo UE, Cockerham K. Exenteration. In: Levine MR. ed. Manual of Oculoplastic Surgery. Berlin: Springer; 2018
    CrossrefGoogle Scholar
  • 53 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 (05) 366-370
    CrossrefPubMedGoogle Scholar
  • 54 Ahmad SM, Esmaeli B, Williams M. et al. American Joint Committee on Cancer classification predicts outcome of patients with lacrimal gland adenoid cystic carcinoma. Ophthalmology 2009; 116 (06) 1210-1215
    CrossrefPubMedGoogle Scholar
  • 55 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 (07) 1313-1323
    CrossrefPubMedGoogle Scholar
  • 56 Font RL, Gamel JW, Jacobiec FA. Epithelial tumors of the lacrimal gland: an analysis of 265 cases. In: Jakobiec FA. ed. Ocular and Adnexal Tumors. Birmingham, AL: Aesculapius Publishing; 1978: 787-805
    Google Scholar
  • 57 Lai T, Prabhakaran VC, Malhotra R, Selva D. Pleomorphic adenoma of the lacrimal gland: is there a role for biopsy?. Eye (Lond) 2009; 23 (01) 2-6
    CrossrefPubMedGoogle Scholar
  • 58 Currie ZI, Rose GE. Long-term risk of recurrence after intact excision of pleomorphic adenomas of the lacrimal gland. Arch Ophthalmol 2007; 125 (12) 1643-1646
    CrossrefPubMedGoogle Scholar
  • 59 Vagefi MR, Hong JE, Zwick OM, Bedrossian Jr EH, Seiff SR, Cockerham KP. Atypical presentations of pleomorphic adenoma of the lacrimal gland. Ophthal Plast Reconstr Surg 2007; 23 (04) 272-274
    CrossrefPubMedGoogle Scholar
  • 60 Ammirati M, Zarzour H. Overview of skull base tumors. In: Winn HR. ed. Youmans and Winn Neurological Surgery. Philadelphia, PA: Saunders Elsevier; 2017: 1222-1242
    Google Scholar
  • 61 Perry JD, Patel BC. Orbital rhabdomyosarcoma. In: Hwang CJ, Patel BC, Singh AD. eds. Clinical Ophthalmic Oncology: Orbital Tumors. Berlin: Springer; 2019: 217-229
    CrossrefGoogle Scholar
  • 62 Chung EM, Smirniotopoulos JG, Specht CS, Schroeder JW, Cube R. From the archives of the AFIP: pediatric orbit tumors and tumorlike lesions: nonosseous lesions of the extraocular orbit. Radiographics 2007; 27 (06) 1777-1799
    CrossrefPubMedGoogle Scholar
  • 63 Katowitz WR, Fries PD. Malignant pediatric orbital tumors. In: Katowitz JA, Katowitz WR. eds. Pediatric Oculoplastic Surgery. Berlin: Springer; 2018: 743-770
    CrossrefGoogle Scholar
  • 64 Crist W, Gehan EA, Ragab AH. et al. The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol 1995; 13 (03) 610-630
    CrossrefPubMedGoogle Scholar
  • 65 Raney RB, Maurer HM, Anderson JR. et al. The Intergroup rhabdomyosarcoma study group (IRSG): major lessons from the IRS-I through IRS-IV studies as background for the current IRS-V treatment protocols. Sarcoma 2001; 5 (01) 9-15
    CrossrefPubMedGoogle Scholar
  • 66 Malempati S, Hawkins DS. Rhabdomyosarcoma: review of the Children's Oncology Group (COG) Soft-Tissue Sarcoma Committee experience and rationale for current COG studies. Pediatr Blood Cancer 2012; 59 (01) 5-10
    CrossrefPubMedGoogle Scholar
  • 67 Hawkins DS, Spunt SL, Skapek SX. COG Soft Tissue Sarcoma Committee. Children's Oncology Group's 2013 blueprint for research: Soft tissue sarcomas. Pediatr Blood Cancer 2013; 60 (06) 1001-1008
    CrossrefPubMedGoogle Scholar
  • 68 Reilly BK, Kim A, Peña MT. et al. Rhabdomyosarcoma of the head and neck in children: review and update. Int J Pediatr Otorhinolaryngol 2015; 79 (09) 1477-1483
    CrossrefPubMedGoogle Scholar
  • 69 Turner JH, Richmon JD. Head and neck rhabdomyosarcoma: a critical analysis of population-based incidence and survival data. Otolaryngol Head Neck Surg 2011; 145 (06) 967-973
    CrossrefPubMedGoogle Scholar
  • 70 Donaldson SS, Anderson JR. Rhabdomyosarcoma: many similarities, a few philosophical differences. J Clin Oncol 2005; 23 (12) 2586-2587
    CrossrefPubMedGoogle Scholar
  • 71 Bompas E, Campion L, Italiano A. et al. Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study. Cancer Med 2018; 7 (08) 4023-4035
    CrossrefPubMedGoogle Scholar
  • 72 Bernardini FP, de Conciliis C, Schneider S, Kersten RC, Kulwin DR. Solitary fibrous tumor of the orbit: is it rare? Report of a case series and review of the literature. Ophthalmology 2003; 110 (07) 1442-1448
    CrossrefPubMedGoogle Scholar
  • 73 Wilson WB, Feinsod M, Hoyt WF, Nielsen SL. Malignant evolution of childhood chiasmal pilocytic astrocytoma. Neurology 1976; 26 (04) 322-325
    CrossrefPubMedGoogle Scholar
  • 74 de Keizer RJW, de Wolff-Rouendaal D, Bots GTA, Thomeer RT, Brouwer OF, Vielvoye GJ. Optic glioma with intraocular tumor and seeding in a child with neurofibromatosis. Am J Ophthalmol 1989; 108 (06) 717-725
    CrossrefPubMedGoogle Scholar
  • 75 Katowitz WR, Fries PD, Kazim M. Benign pediatric orbital tumors. In: Katowitz JA, Katowitz WR. eds. Pediatric Oculoplastic Surgery. Berlin: Springer; 2018: 667-741
    CrossrefGoogle Scholar
  • 76 Dutton JJ. Tumors of the optic nerve. In: Hwang CJ, Patel BC, Singh AD. eds. Clinical Ophthalmic Oncology: Orbital Tumors. Berlin: Springer; 2019: 137-148
    CrossrefGoogle Scholar
  • 77 Dutton JJ. Gliomas of the anterior visual pathway. Surv Ophthalmol 1994; 38 (05) 427-452
    CrossrefPubMedGoogle Scholar
  • 78 Alireza M, Amelot A, Chauvet D, Terrier LM, Lot G, Bekaert O. Poor prognosis and challenging treatment of optic nerve malignant gliomas: literature review and case report series. World Neurosurg 2017; 97: 751 e751-751 e756
    CrossrefPubMedGoogle Scholar
  • 79 Minovi A, Basten O, Hunter B, Draf W, Bockmühl U. Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review. Head Neck 2007; 29 (05) 439-445
    CrossrefPubMedGoogle Scholar
  • 80 Lyons CJ, McNab AA, Garner A, Wright JE. Orbital malignant peripheral nerve sheath tumours. Br J Ophthalmol 1989; 73 (09) 731-738
    CrossrefPubMedGoogle Scholar
  • 81 Erzurum SA, Melen O, Lissner G. et al. Orbital malignant peripheral nerve sheath tumors. Treatment with surgical resection and radiation therapy. J Clin Neuroophthalmol 1993; 13 (01) 1-7
    PubMedGoogle Scholar