Skip to main content
SpringerLink
Log in

Retinoblastoma associated with orbital pseudocellulitis and high-risk retinoblastoma: a study of 32 eyes

  • Original Paper
  • Published:
International Ophthalmology Aims and scope Submit manuscript

Abstract

Purpose

To study the correlation between retinoblastoma (RB) associated with orbital pseudocellulitis and high-risk histopathology features.

Methods

Retrospective study of 32 patients who underwent primary enucleation for RB presenting with orbital pseudocellulitis.

Results

All RB patients presented with orbital pseudocellulitis. The mean age at presentation of RB was 30 months (median, 24 months; range, 3–70 months). There were 14 (44%) males and 18 (56%) females. All patients were referred with a diagnosis of RB with orbital pseudocellulitis. Tumor was bilateral in 12 (38%) patients but orbital pseudocellulitis was unilateral in all cases. The pseudocellulitis features included proptosis (n = 9; 28%), eyelid edema (n = 22; 69%), conjunctival congestion (n = 23; 72%), and conjunctival chemosis (n = 15; 47%). Based on clinical features and orbital imaging, all patients were diagnosed to have group E intraocular RB. All patients received intravenous steroids prior to enucleation. On histopathology, tumor necrosis was present in all cases with a mean % necrosis of 60% (median, 60%; range, 10% to 90%). Most tumors (72%) were poorly differentiated. High-risk histopathology features were noted in 23 (72%) cases and adjuvant chemotherapy was advised for all these patients. The most common high-risk histopathology features included post-laminar optic nerve infiltration (34%) and scleral infiltration (22%). Over a mean follow-up period of 34 months (median, 9 months; range, < 1–188 months), there was no event of metastasis or death in any patient.

Conclusion

RB presenting with orbital pseudocellulitis is associated with high incidence of high-risk histopathology features.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

Data availability and material

Data are available with the authors.

References

  1. Abramson DH, Frank CM, Susman M, Whalen MP, Dunkel IJ, Boyd NW 3rd (1998) Presenting signs of retinoblastoma. J Pediatr 132(3 Pt 1):505–508

    Article  CAS  Google Scholar 

  2. Agarwal M, Biswas J, Shanmugam MP (2004) Retinoblastoma presenting as orbital cellulitis: report of four cases with a review of the literature. Orbit 23(2):93–98

    Article  Google Scholar 

  3. Al Rashaed S, Elkhamary SM, Al Sheikh O, Al Mesfer S, Al-Dhibi H, Al MA (2020) Adult presentation of retinoblastoma in Saudi patient. Saudi J Ophthalmol 34(2):136–138

    Article  Google Scholar 

  4. Balasubramanya R, Pushker N, Bajaj MS, Ghose S, Kashyap S, Rani A (2004) Atypical presentations of retinoblastoma. J Pediatr Ophthalmol Strabismus 41(1):18–24

    Article  Google Scholar 

  5. Ben Meir A, Bardenstein D, Peiffer R (1995) Retinoblastoma presenting with orbital cellulitis: a mechanical hypothesis. Invest Ophthalmol Vis Sci 36:492

    Google Scholar 

  6. Chong EM, Coffee RE, Chintagumpala M, Hurwitz RL, Hurwitz MY, Chévez-Barrios P (2006) Extensively necrotic retinoblastoma is associated with high-risk prognostic factors. Arch Pathol Lab Med 130(11):1669–1672

    Article  Google Scholar 

  7. Foster BS, Mukai S (1996) Intraocular retinoblastoma presenting as ocular and orbital inflammation. Int Ophthalmol Clin 36(1):153–160

    Article  CAS  Google Scholar 

  8. Kaliki S (2018) How to do an enucleation for retinoblastoma. Community Eye Health 31(101):20–22

    PubMed  PubMed Central  Google Scholar 

  9. Kaliki S, Gupta S, Ramappa G, Mohamed A, Mishra DK (2020) High-risk retinoblastoma based on age at primary enucleation: a study of 616 eyes. Eye (Lond) 34(8):1441–1448

    Article  Google Scholar 

  10. Kaliki S, Patel A, Iram S, Palkonda VAR (2017) Clinical presentation and outcomes of stage III or stage IV retinoblastoma in 80 Asian Indian patients. J Pediatr Ophthalmol Strabismus 54(3):177–184

    Article  Google Scholar 

  11. Kaliki S, Patel A, Iram S, Ramappa G, Mohamed A, Palkonda VAR (2019) Retinoblastoma in India: clinical presentation and outcome in 1,457 patients (2,074 Eyes). Retina 39(2):379–391

    Article  Google Scholar 

  12. Kaliki S, Shields CL, Eagle RC Jr, Iram S, Shields JA (2018) High-risk intraocular retinoblastoma: comparison between Asian Indians and Americans from two major referral centers. Retina 38(10):2023–2029

    Article  Google Scholar 

  13. Martino F, Gelmi MC, Galluzzi P, De Francesco S, Miracco C, Hadjistilianou D (2020) Orbital pseudocellulitis: a retinoblastoma-associated masquerade syndrome. Ocul Oncol Pathol 6(6):430–437

    Article  Google Scholar 

  14. Mullaney PB, Karcioglu ZA, Huaman AM, Al-Mesfer S (1998) Retinoblastoma associated orbital cellulitis. Br J Ophthalmol 82(5):517–521

    Article  CAS  Google Scholar 

  15. Nadol JB Jr (1977) Pseudocellulitis of the orbit. Ann Otol Rhinol Laryngol 86(1 Pt 1):86–88

    Article  Google Scholar 

  16. Nalcı H, Gündüz K, Erden E (2018) Necrotic intraocular retinoblastoma associated with orbital cellulitis. Surv Ophthalmol 63(1):114–118

    Article  Google Scholar 

  17. Read RW, Zamir E, Rao NA (2002) Neoplastic masquerade syndromes. Surv Ophthalmol 47(2):81–124

    Article  Google Scholar 

  18. Sachdeva R, Schoenfield L, Marcotty A, Singh AD (2011) Retinoblastoma with autoinfarction presenting as orbital cellulitis. J AAPOS 15(3):302–304

    Article  Google Scholar 

  19. Shields JA, Shields CL, Suvarnamani C, Schroeder RP, DePotter P (1991) Retinoblastoma manifesting as orbital cellulitis. Am J Ophthalmol 112(4):442–449

    Article  CAS  Google Scholar 

  20. Walinjkar J, Krishnakumar S, Gopal L, Ramesh A, Khetan V (2013) Retinoblastoma presenting with orbital cellulitis. J AAPOS 17(3):282–286

    Article  Google Scholar 

Download references

Funding

This work is supported by Hyderabad Eye Research Foundation, Hyderabad, India and The Operation Eyesight Universal Institute for Eye Cancer, Hyderabad, India.

Author information

Authors and Affiliations

  1. The Operation Eyesight Universal Institute for Eye Cancer (SK, VSV, AM), L V Prasad Eye Institute, Hyderabad, 500034, India

    Swathi Kaliki, Vijitha S. Vempuluru & Akhila Mallu

  2. Ophthalmic Pathology Laboratory (SJ, DKM), L V Prasad Eye Institute, Hyderabad, India

    Saumya Jakati & Dilip K. Mishra

Authors
  1. Swathi Kaliki
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Saumya Jakati
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Vijitha S. Vempuluru
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Akhila Mallu
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Dilip K. Mishra
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

SK was responsible for the concept and formulation of the work, data analysis, and manuscript preparation. VSV and AM were responsible for data collection and reviewing the manuscript. SJ and DKM were responsible for histopathological correlation and reviewing the manuscript.

Corresponding author

Correspondence to Swathi Kaliki.

Ethics declarations

Conflict of interest

No conflicts of interest exist for any author.

Consent for publication

Consent for publication was obtained from the patients.

Consent to participate

Consent to participate was obtained from the patients.

Ethical approval

This study has been approved by the Institute Ethics Committee.

Human and animal rights

This work involved demographic and clinical information of human participants. Research involved demographic and clinical information of human participants. All procedures performed were in accordance with the ethical standards of the institutional ethical standards of the institutional ethics committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent has been obtained from all study participants.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Précis Of the 32 patients who underwent primary enucleation for retinoblastoma presenting as orbital pseudocellulitis, tumor necrosis was noted in all cases (median, 70%; range, 10–90%) and high risk histopathology features in 23 (72%) cases.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Kaliki, S., Jakati, S., Vempuluru, V.S. et al. Retinoblastoma associated with orbital pseudocellulitis and high-risk retinoblastoma: a study of 32 eyes. Int Ophthalmol 42, 19–26 (2022). https://doi.org/10.1007/s10792-021-01993-w

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10792-021-01993-w

Keywords

Search

Navigation