Retinoblastoma
Retinoblastoma
Epidemiology:
Retinoblastoma is one of the most common pediatric malignancies, with an estimated incidence of approximately 1 in 15,000 live births. The global prevalence and advancements in early detection and treatment have contributed to an overall survival rate exceeding 90% for patients with localized disease. Spontaneous remission (SR) in retinoblastoma is a rare phenomenon, documented in approximately 1% of cases 1,2. Documenting these cases can be challenging, as many may go unreported, especially when cases involve less severe manifestations or incompletely regressed tumors 3.
Clinical Characteristics:
To date, thirteen cases of SR of retinoblastoma have been documented in the medical literature. These reports collectively underscore the extraordinary rarity of SR in this pediatric ocular malignancy and its occurrence across a broad age range, from infancy to adulthood. Reported cases involved both male and female patients, with no apparent gender predilection observed. See Table 1 below for further information.
Histological Characteristics:
Of the cases analyzed, the tumors were exclusively reported as originating from the eye, with remission occurring within the same ocular site in nearly all instances. No cases involving extraocular or metastatic disease were included, as only those demonstrating definitive intraocular spontaneous remission were considered. This selection ensured diagnostic accuracy and maintained the clinical specificity of spontaneous remission in retinoblastoma.
Proposed Contributing Mechanisms:
Among the reported cases, proposed mechanisms included immune response, ischemic necrosis, tumor maturation, febrile illness, and calcium inhibition, though several reports did not specify a cause. Overall, immune-mediated processes, ischemic injury, and tumor differentiation appear to contribute to the spontaneous remission of retinoblastoma.
Site and Extent of Remission:
The majority of documented cases exhibited complete remission localized to the primary ocular site, with no evidence of extraocular involvement or metastatic spread. In a few reports, remission was noted bilaterally, reflecting simultaneous regression in both eyes. The longest reported remission persisted for 44 years, indicating durable disease control in the absence of therapeutic intervention. Although follow-up durations varied among reports, these findings suggest that spontaneous remission in retinoblastoma can occasionally result in long-term and clinically significant tumor resolution.
Table 1: Retinoblastoma SR Cases and Clinical Characteristics
Age/sex | Primary site | Remission site | Proposed mechanisms | Follow-up | |
|---|---|---|---|---|---|
Not reported | Eye | Eye | Immune response; Febrile illness | Not reported | |
Not reported | Eye | Eye | Immune response; Febrile illness | Not reported | |
62/M | Eye | Eye | Not reported | Not reported | |
Not reported | Eye | Eye | Immune response; Systemic factors | Not reported | |
5/F | Right eye | Right eye | Ischemic necrosis; Immune response | Not reported | |
9/F | Eye | Eye | Not reported | Not reported | |
18/M | Eye | Eye | Tumor maturation | 6.5 years | |
24/M | Eyes | Eyes | Not reported | Not reported | |
Not reported | Eye | Eye | Tumor arrest | Not reported | |
52/M | Left eye | Left eye | Ischemia; Immune response; Febrile illness; Calcium inhibition | Not reported | |
Not reported | Eyes | Eyes | Not reported | Not reported | |
3/F | Eye | Eye | Not reported | 44 years | |
24/M | Fundus | Fundus | Not reported | Not reported | |
Retinoblastoma | |||||
46/F | Grape diet | ||||
4 months/Not reported | Orbit |
References:
- Kao L. , Meng Y. Spontaneous regression of retinoblastoma in a taiwan series. J Pediatr Ophthalmol Strabismus. 2005;42(4):228-232. doi:10.3928/01913913-20050701-05
- Sanborn G., Augsburger J., & Shields J. Spontaneous regression of bilateral retinoblastoma.. British Journal of Ophthalmology. 1982;66(11):685-690. doi:10.1136/bjo.66.11.685
- Gallie B., Ellsworth R., Abramson D., & Phillips R. Retinoma: spontaneous regression of retinoblastoma or benign manifestation of the mutation?. Br J Cancer. 1982;45(4):513-521. doi:10.1038/bjc.1982.87
- Steward JK, Smith JLS, Arnold EL. Spontaneous regression of retinoblastoma. Br J Ophthalmol. 1956;40:449-461.
- Boniuk M, Zimmerman LE. Spontaneous regression of retinoblastoma. Int Ophthalmol Clin. 1962;2(2):525-542.
- Boniuk M, Girard LJ. Spontaneous regression of bilateral retinoblastoma. Trans Am Acad Ophthalmol Otolaryngol. 1969;73(2):194-198.
- Ellsworth RM. The practical management of retinoblastoma. Trans Am Ophthalmol Soc. 1969;67:462-534.
- Rubin ML, Kaufman HE. Spontaneously regressed probable retinoblastoma. Arch Ophthalmol. 1969;81:442-445.
- Mortimer CB. Unusual retinoblastoma. Appl Ther. 1970;12:22.
- Karsgaard AT. Spontaneous regression of retinoblastoma; a report of two cases. Can J Ophthalmol. 1971;6(3):218-222.
- Morris WE, Lapiana FG. Spontaneous regression of bilateral multifocal retinoblastoma with preservation of normal visual acuity. Ann Ophthalmol. 1974;6(11):1192-1194
- Smith JLS. Histology and spontaneous regression of retinoblastoma. Trans Ophthalmol Soc U K. 1974;94:953-967.
- Nehen JH. Spontaneous regression of retinoblastoma. Acta Ophthalmol. 1975;53:647-651.
- Khoudadoust A, Roozitalab HM, Smith RE, Green WR. Spontaneous regression of retinoblastoma. Surv Ophthalmol. 1977;21(6):467-478.
- Brodwall J. Spontaneous regression of a retinoblastoma. Acta Ophthalmol. 1981;59(3):430-434.
- Sanborn GE, Augsburger JJ, Shields JA. Spontaneous regression of bilateral retinoblastoma. Br J Ophthalmol. 1982;66(11):685-690.
- Boniuk & Zimmerman, 1962. Spontaneous Regression of Retinoblastoma. International Ophthalmology Clinics 2(2): June 1962; 525-542
- Niethe, U. 1975. Spontaneous Healing of a Malignoma?. Klinische Monatsblatter für Augenheilkunde und Augenarztliche Fortbildung 166(1): Jan 1975; 137-138
- Yancey, W. A. 1968. Spontaneous Regression of Orbital and Facial Hemangioma. Western Journal of Medicine 108(4): April 1968; 300