Neurological

Cross-practice variability for definitions of risk retinoblastoma

A small but wide-ranging survey of clinicians treating retinoblastoma found that there is little consistency in defining high-risk cases of the disease. Without a recognized definition and clearly defined characteristics, consistent treatment of retinoblastoma remains difficult, according to a report published in JAMA Ophthalmology.

The high-risk histopathological features of retinoblastoma are used to determine the risk of systemic metastasis. However, among 27 oncologists and pathologists from 16 countries on 6 continents who were surveyed electronically, postlaminar optic nerve infiltration, involvement of optic nerve transection, and extrascleral tumor extension were the only features that were consistently described as high-risk signals for metastases.

The researchers submitted a 10-question survey to 27 clinicians from 24 retinoblastoma practices in 16 countries on 6 continents. The survey asked objective yes-or-no questions, each with a free text field for additional comments. Questions centered on what histopathological factors clinicians looked for in determining whether a patient with retinoblastoma is at high risk.

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Of those surveyed, 18 (67%) were ophthalmologists or researchers practicing ocular oncology, 2 (7%) were ophthalmic pathologists, 3 (11%) were trained in both ocular oncology and pathology, and 4 (15%) were medical oncologists who treat retinoblastoma; 5 respondents (19%) were from North America.

According to the study, high-risk characteristics of retinoblastoma include:

  • Postlaminar optic nerve infiltration (27 [100%])
  • Involvement of the transection of the optic nerve (27 [100%])
  • Extra-scleral tissue infiltration (27 [100%])
  • Massive (≥3 mm) choroidal invasion (25 [93%])
  • Microscopic scleral infiltration (23 [85%])
  • Ciliary body infiltration (20 [74%])
  • Trabecular Meshwork Invasion (18th Century) [67%])
  • Iris infiltration (17 [63%])
  • Anterior chamber seeds (14 [52%])
  • Infiltration of the laminar optic nerve (13th century) [48%])
  • Combination of prelaminar and laminar optic nerve infiltration and small choroidal invasion (11 [41%])
  • Minor (<3 mm) choroidal invasion (5th [19%])
  • Prelaminar optic nerve infiltration (2 [7%])

The data showed that the other histopathological features that were rated as high risk by the respondents involved invasion of Schlemm’s canal (4th [15%]) and severe anaplasia (1 [4%]). In addition, 4 respondents (15%) said that the presence of more than one high risk feature, particularly massive peripapillary choroidal invasion paired with postlaminar optic nerve infiltration, should be viewed as a “very high risk” of metastasis.

“Since the decision to offer adjuvant chemotherapy and radiation therapy depends on the definition of high-risk RBC in each individual treatment center, this inequality leads to a wide range of practical variations in the treatment of high-risk RBC with very different results.” Study says.

The researchers note some limitations to their work, including the fact that the survey was not validated and respondents had different backgrounds and ways to treat, track, and examine their patients.

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Kaliki S, Shields CL, Cassoux N, et al. Definition of high-risk retinoblastoma: a multicenter global survey. JAMA Ophthalmol. Published online 11 November 2021. doi: 10.1001 / jamaophthalmol.2021.4732

This article originally appeared on Ophthalmology Advisor

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