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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

Author–year

Age/sex

Primary site

Remission site

Proposed mechanisms

Follow-up

Steward et al., 19564

Not reported

Eye

Eye

Immune response; Febrile illness

Not reported

Steward et al., 19565

Not reported

Eye

Eye

Immune response; Febrile illness

Not reported

Boniuk et al., 19696

62/M

Eye

Eye

Not reported

Not reported

Ellsworth, 19697

Not reported

Eye

Eye

Immune response; Systemic factors

Not reported

Rubin et al., 19698

5/F

Right eye

Right eye

Ischemic necrosis; Immune response

Not reported

Mortimer, 19709

9/F

Eye

Eye

Not reported

Not reported

Karsgaard, 197110

18/M

Eye

Eye

Tumor maturation

6.5 years

Morris et al., 197411

24/M

Eyes

Eyes

Not reported

Not reported

Smith, 197412

Not reported

Eye

Eye

Tumor arrest

Not reported

Nehen, 197513

52/M

Left eye

Left eye

Ischemia; Immune response; Febrile illness; Calcium inhibition

Not reported

Khoudadoust et al., 197714

Not reported

Eyes

Eyes

Not reported

Not reported

Brodwall, 198115

3/F

Eye

Eye

Not reported

44 years

Sanborn et al., 198216

24/M

Fundus

Fundus

Not reported

Not reported

Boniuk & Zimmerman et al., 196217

retrogressed retinoblastoma

retrogressed retinoblastoma

Niethe, 197518

46-year-old female

Symptoms disappeared, pigment release decreased, opaqueness shrank to a small scar, small pink spot remains

Grape diet

Symptoms disappeared, pigment release decreased, opaqueness shrank to a small scar, small pink spot remains

Yancey, 196819

patient, first seen at four months of age

The hemangioma regressed (without any treatment) to such an extent that the globe had receded into the orbit and the lids functioned sufficiently well to protect the cornea. Also, the child appeared to have fusion and good extraocular muscle function in all fields of gaze.

The hemangioma regressed (without any treatment) to such an extent that the globe had receded into the orbit and the lids functioned sufficiently well to protect the cornea. Also, the child appeared to have fusion and good extraocular muscle function in all fields of gaze.

Galimova et al.20

Balmer et al.21

Rzalieva et al.22

Ovestad et al., 201123

Lam et al., 200524

Shirai et al., 201025

Kao26

Gallie et al.27

References:

  1. 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
  2. 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
  3. 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
  4. Steward JK, Smith JLS, Arnold EL. Spontaneous regression of retinoblastoma. Br J Ophthalmol. 1956;40:449-461.
  5. Boniuk M, Zimmerman LE. Spontaneous regression of retinoblastoma. Int Ophthalmol Clin. 1962;2(2):525-542.
  6. Boniuk M, Girard LJ. Spontaneous regression of bilateral retinoblastoma. Trans Am Acad Ophthalmol Otolaryngol. 1969;73(2):194-198.
  7. Ellsworth RM. The practical management of retinoblastoma. Trans Am Ophthalmol Soc. 1969;67:462-534.
  8. Rubin ML, Kaufman HE. Spontaneously regressed probable retinoblastoma. Arch Ophthalmol. 1969;81:442-445.
  9. Mortimer CB. Unusual retinoblastoma. Appl Ther. 1970;12:22.
  10. Karsgaard AT. Spontaneous regression of retinoblastoma; a report of two cases. Can J Ophthalmol. 1971;6(3):218-222.
  11. Morris WE, Lapiana FG. Spontaneous regression of bilateral multifocal retinoblastoma with preservation of normal visual acuity. Ann Ophthalmol. 1974;6(11):1192-1194
  12. Smith JLS. Histology and spontaneous regression of retinoblastoma. Trans Ophthalmol Soc U K. 1974;94:953-967.
  13. Nehen JH. Spontaneous regression of retinoblastoma. Acta Ophthalmol. 1975;53:647-651.
  14. Khoudadoust A, Roozitalab HM, Smith RE, Green WR. Spontaneous regression of retinoblastoma. Surv Ophthalmol. 1977;21(6):467-478.
  15. Brodwall J. Spontaneous regression of a retinoblastoma. Acta Ophthalmol. 1981;59(3):430-434.
  16. Sanborn GE, Augsburger JJ, Shields JA. Spontaneous regression of bilateral retinoblastoma. Br J Ophthalmol. 1982;66(11):685-690.
  17. Boniuk & Zimmerman, 1962. Spontaneous Regression of Retinoblastoma. International Ophthalmology Clinics 2(2): June 1962; 525-542
  18. Niethe, U. 1975. Spontaneous Healing of a Malignoma?. Klinische Monatsblatter für Augenheilkunde und Augenarztliche Fortbildung 166(1): Jan 1975; 137-138
  19. Yancey, W. A. 1968. Spontaneous Regression of Orbital and Facial Hemangioma. Western Journal of Medicine 108(4): April 1968; 300
  20. Galimova RZ; Zuikova TP; Buriakova ZA. Clinico-Morphological Features of Retinoblastoma with Spontaneous Regression (Kliniko-Morfologiches-
  21. Balmer A; Munier F; Gailloud C. Retinoma and Phthisis Bulbi: Benign Expression of Retinoblastoma (Retinomes et Phthisis Bulbi: Retinoblastoma (Retinomes et Phthisis Bulbi: Klinische Monatsblatter für Augenheilkunde und
  22. Rzalieva RG; Gabbasov UA. A Case of an Atypical Course of Retinoblastoma (Sluchai Atipichnogo Techeniia Retinoblastomy) Oftal’Mologicheskii Zhurnal 5: 1990; 314-315
  23. Ovestad, I. T., Gudlaugsson, E., Skaland, I., Malpica, A., Munk, A. C., Janssen, E. A., & Baak, J. P. (2011). The impact of epithelial biomarkers, local immune response and human papillomavirus genotype in the regression of cervical intraepithelial neoplasia grades 2-3. Journal of Clinical Pathology, 64(4), 303-307. doi:10.1136/jcp.2010.083626
  24. Lam, A., Shields, C. L., Manquez, M. E., & Shields, J. A. (2005). Progressive resorption of a presumed spontaneously regressed retinoblastoma over 20 years. Retina (Philadelphia, Pa.), 25(2), 230-231.
  25. Shirai, K., Okada, Y., & Saika, S. (2010). Immunohistochemical observation of anterior subcapsular cataract in eye with spontaneously regressed retinoblastoma. Journal of Cataract and Refractive Surgery, 36(3), 503-507. doi:10.1016/j.jcrs.2009.08.042
  26. Kao L. , Meng Y. Spontaneous regression of retinoblastoma in a taiwan series. J Pediatr Ophthalmol Strabismus. 2005;42(4):228-232
  27. 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
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