Back
Year : 2015 | Volume : 3 | Issue : 3 | Page : 232 - 234  


Case Reports
Spontaneous regression of retinoblastoma – A Case Report

M.Padma1, N.Shivanand2, K.Vishwanath3

 

1Assistant Professor of Ophthalmology, Sarojini Devi Eye Hospital, Osmania Medical College, Hyderabad

2 & 3Former Superintendent, HOD and Professor of Ophthalmology, Sarojini Devi Eye Hospital, Osmania Medical College, Hyderabad

Corresponding Author:

Dr. M. Padma

Email: malladipadma9@gmail.com

Abstract:

Spontaneously regressing retinoblastoma is a retinal lesion consisting of one or moregray, translucent retinal masses, calcified nodules and retinal pigment migration and clumping in adjacent areas. Female child of 7 years presenting with esotropia LE was referred from pediatric Ophthalmology department to Vitreo-Retinal clinic of Sarojini Devi Eye Hospital, Hyderabad for fundus evaluation. Vision in RE is 6/6. LE –Perception of light positive, PR inaccurate in all quadrants. RE anterior segment examination is unremarkable and in LE – relative afferent pupillary defect.  On fundus examination, RE – Normal, LE – Large circumscribed excavated area temporal to optic disc noticed. Spontaneously regressed retinoblastoma carries the same genetic implication as an active retinoblastoma.

Key words:Esotropia, Retinoblastoma, Spontaneous regression.

INTRODUCTION:

 

Complete spontaneous necrosis leading to regression and “Cure” is a well known phenomenon that is said to occur more frequently in retinoblastoma than in any other malignant neoplasm1,2. It is characterized by a severe inflammatory reaction in one eye with retinoblastoma, sometimes followed by development of phthisis bulbi. The fundus picture of spontaneously regressing retinoblastoma has been described characteristic and even pathognomic, having multiple scars of chorieoretinal atrophy with chalky white areas of calcification, surrounded by pigmentary changes. It is not certain whether the tumour regression occurs secondary to vascular ischemia to the tumour or whether more complex immunopathologic mechanisms play a role. Spontaneous regression of Retinoblastoma is rare. 71 cases are reported in literature1.

 

Active retinoblastoma appears as a flat, transparent slightly white lesion in the sensory retina. Larger tumours are less transparent and appear solid white with dilated tortuous retinal arteries and veins that supply and drain the tumour. Seeding of tumour cells into overlying vitreous may be seen (Fig-1).

 

 

 

 

  1. Assistant Professor of Ophthalmology.
  2. Former post graduate
  3. Former Professor & HOD of Ophthalmology.

 

CASE REPORT:

Careful history taken from the parents, suggesting,  No h/o consanguity, No family history of retinoblastoma.3rd child among 3 female children.

Visual acuity in RE is 6/6 and VA in LE- Perception of light present, Projection of rays inaccurate in all quadrants.

Child was examined under slitlamp biomicroscopy Anterior segment examination of RE is unremarkable. LE – relative afferent pupillary defect present.

Fundus examination:  RE normal.

LE – clear media with normal optic disc, macula shows large circumscribed area of 30mm vertically and 25 mm horizontally from the disc, adjacent to temporal aspect of disc reaching up to mid periphery, with elevated, vascular cheesy mass, irregular, dense chalky white calcific deposition (cottage cheese appearance) pigment clumping at the margins observed (vertically 4-5 disc diameters, horizontally 8-10 disc diameters). Choroidal vessels seen in the circumscribed area.(Fig-2-6)

Examination of fundus in parents and siblings normal.

LE enucleation was planned for Histopathological confirmation of diagnosis, but parents refused.

 

INVESTIGATIONS:

 

Hemotological :with in normal limits.

 

B-Scan: LE large highly echogenic area seen temporal to disc.

 

CT Brain and both orbits   

 

Brain                                                       : Normal study

Orbits                                                     : Irregular soft tissue density mass lesion in the left eyeball.

Mass measuring 19.5 X 10.6 mm with specks of calcification.

 

CT impression:     Suggestive of retinoblastoma. (Fig-7)

 

DIFFERENTIAL DIAGNOSIS:

 

Retinocytoma: described by Gallie and associates3.4. Clinical picture similar to retinoblastoma, carries same genetic risk and risk to fellow eye as retinoblastoma.  Mitosis not seen, calcification observed but no necrosis

 

DISCUSSION:

Retinoblastoma is the most common malignant intraocular tumour of childhood. Spontaneous regression was diagnosed on the basis of stable pathognomic fundus appearance, positive family history, retinoblastoma in fellow eye or calcified tumour cells observed histologically in the enucleated fellow eye.

 

The tumours is a homogenous, gray, translucent, elevated vascularized retinal mass with opaque white calcific nodules and underlying or adjacent retinal pigment epithelial migration and proliferation. 

The mechanism of regression of retinoblastoma is not known. Char and associates reported an increasing lymphocytotoxicity against the retinoblastoma cell line. The two hit theory of Knudson and Meadows correlate well with clinical manifestations of retinoblastoma. Retinoblastoma is an age specific   malignancy with a peak incidence in the first few years of life. This is in accord with the hypothesis that the target cells disappear and the malignant cells occur if such a cell receives two hits before it fully differentiates.

 

If the final mutation occurs in immature retinoblast, retinoblastoma results.If the carciogenic event occurs in a partially differentiated cell before final differentiation, a benign hyperplastic nodule or retinoma results. If the final hit occurs in a terminally differentiated cell, it has no effect, for this cell cannot proliferate.

The diagnosis of spontaneous regression of retinoblastoma is not difficult if histopathologic examination of an enucleated specimen done. The fundus picture also is characteristic and shows multiple scars of chorio retinal atrophy with chalky white areas of calcification, surrounded by RPE pigment clumping.

The clinical picture in our case is typical.

 

CLINICAL IMPORTANCE:

 

Spontaneous regressed retinoblastoma is clinically and histologically benign lesion but carries same genetic implication as retinoblastoma and does not imply benign course if retinoblastoma develops in contralateral eye or family member.  Therefore careful observation of these lesions is necessary as of treated retinoblastomas.

 

RECOMMENDATIONS:

  1. Essential to examine all children by indirect ophthalmology to identity spontaneous regression
  2. Careful follow – up of these lesions is necessary as of treated retinoblastomas

 

REFERENCES:

 

  1. Guyer, Yannuzzi, Chang, Shields, Green,: Textbook of Retina-Vitreous-macula volume-2-Retinoblastoma: 1999, chapter 99: pp1139-1143.
  2. Shields JA, Shields CL: Retinoblastoma.: Clinical and pathologic features. In shields JA, Shields CL ( eds) : Intraocular tumours. A text and Atlas, Philadelphia, WB-Saunders co. 1992, pp305-33.
  3. Aazy a. Aaby, Ronald L PriceandZ.Nicolaszakov-spontaneously regressing Retinoblastoma,retinoma AJO 96;315-320,1983
  4. DN Gangwar,, I.S. Jain, Amod Gupta, P.C. Sharma- “ Bilateral spontaneous regression of

retinoblastoma with dominant transmission” Ann of Ophthalmol May 1982 479-480.

  1. William E Benson, J. Douglas Cameron, Francis P. Furiuele et al – Scheie Eye Institute – “ Presumed spontaneously Regressed Retinoblastoma.
  2. Gallie BL, Ellsworth KM, Abramson DH, Philips RA: Retinoma : Spontaneous regression of retinoblastoma or benign manifestation of amutation ? Br J Cancer 45: 513-521, 1982.
  3. Steward, J.K. Smith J. L and Arnold, E. L.: Spontaneous regression on of retinoblastoma Br. J Ophthalmol, 40:449, 1966.
  4. Smith, J L : Histology and spontaneous regression of retinoblastoma Trans. Ophthalmol. Soc. U.K. 94: 953, 1974.
  5. Merin S, Robinson E, and Landau J: Spontaneous healed retinoblastoma as a factor in dominant heredity J. Pediatric Opthalmol 243, 1965.
  6. Nehan J.H. Spontaneous regression of retinoblastoma, ActaOphthalmol 53: 647, 1975.
  7. Khodadoust, AA, Roozitalab, H.M., Smith R E and Green, W R: Spontaneous regression of retinoblastoma. Surv, Ophthalmol 21: 467, 1977.

 

Figure 1

 

Figure 2

 

Figure 3

 

Figure 4

 

 

Figure 5

 

Figure 6

 

 

 Figure 7

 





img

Important links

adv apply rec

Open Access Journal

MRIMS Journal of Health Sciences is an open access journal which means that all content is freely available without charge to the user or his/her institution. Users are allowed to read, download, copy, distribute, print, search, or link to the full texts of the articles in this journal without asking prior permission from the publisher of the author. This is in accordance with the BOAI definition of open access.

Visitor Count


756805
© 2020 Chandramma Education society . All Rights Reserved.