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Atypical retinocytoma with diffuse vitreous seeds: An insight.
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MedLine Citation:
PMID:  21897624     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Retinocytoma is a rare benign form of retinoblastoma. It is usually found on routine examinations and also while screening families of patients with retinoblastoma. Distinctive features are a translucent retinal mass with calcification, retinal pigment epithelial disturbance, chorioretinal atrophy and absence of growth. We report a case with all the above features along with diffuse vitreous seeds and optical coherence tomographic documentation of intralesional cavitary lesions.
Authors:
Parag K Shah; V Narendran; George J Manayath; Somya Chowdhary
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Oman journal of ophthalmology     Volume:  4     ISSN:  0974-7842     ISO Abbreviation:  Oman J Ophthalmol     Publication Date:  2011 May 
Date Detail:
Created Date:  2011-09-07     Completed Date:  2011-11-10     Revised Date:  2013-05-29    
Medline Journal Info:
Nlm Unique ID:  101519430     Medline TA:  Oman J Ophthalmol     Country:  India    
Other Details:
Languages:  eng     Pagination:  81-3     Citation Subset:  -    
Affiliation:
Department of Pediatric Retina and Ocular Oncology, Aravind Eye Hospital, Coimbatore, India.
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Full Text
Journal Information
Journal ID (nlm-ta): Oman J Ophthalmol
Journal ID (publisher-id): OJO
ISSN: 0974-620X
ISSN: 0974-7842
Publisher: Medknow Publications, India
Article Information
© 2011 Shah PK, et al.
open-access:
Print publication date: Season: May-Aug Year: 2011
Volume: 4 Issue: 2
First Page: 81 Last Page: 83
ID: 3160075
PubMed Id: 21897624
Publisher Id: OJO-4-81
DOI: 10.4103/0974-620X.83659

Atypical retinocytoma with diffuse vitreous seeds: An insight
Parag K. Shahaff1
V. Narendranaff1
George J. Manayathaff1
Somya Chowdhary1
Department of Pediatric Retina and Ocular Oncology, Aravind Eye Hospital, Coimbatore, India
1Department of Pediatric Ophthalmology, Shyamlal Charitable Trust Eye Hospital, New Delhi, India
Correspondence: Correspondence: Dr. George J Manayath, Department of Pediatric Retina and Ocular Oncology, Aravind Eye Hospital, Avinashi Road, Coimbatore – 641 014, Tamil Nadu, India. E-mail: gjmanayath@yahoo.com

Introduction

Retinocytoma was originally described by Gallie et al.[1] as a benign form of retinoblastoma which undergoes spontaneous growth arrest. Although intralesional cavitations[2] and localized vitreous seeds have been reported,[3] we report a patient with optical coherence tomographic (OCT) documentation of cavitary lesions and diffuse vitreous seeds, which have not been documented till date to the best of our knowledge.


Case Report

A 33-year-old male patient presented with divergent squint and diminution of vision in the right eye (OD) since early childhood. Best corrected visual acuity was 20/80 OD and 20/20 in left eye (OS). Family history was not significant. Except for exotropia OD, rest of the anterior segment examination was normal in both eyes. Fundus examination OD showed whitish elevated retinal lesion arising from the disc, with surrounding chorioretinal scarring [Figure 1] and diffuse vitreous seeds seen on wide field imaging [Figure 2]. It was 3 mm in height and 5.5 mm as the largest basal diameter. OS was normal. OCT showed two large cavitary lesions within the tumor with two calcified vitreous seeds [Figure 3]. B-scan ultrasound showed a mass lesion with multiple high-intensity spikes within the mass and in the vitreous, indicative of intralesional and vitreous seed calcifications [Figure 4]. No subretinal fluid was seen. Ultrasound did not show evidence of intralesional cavities. A-scan ultrasound showed high internal reflectivity in these regions, confirming the presence of calcifications. Past history revealed that the patient was examined at our satellite hospital 19 years back. Patient records showed unchanged clinical findings. Fundus fluorescein angiography done at that time showed superficial network of fine vessels in early phase with leakage in the mid phases and intense staining of mass in late phase. Computerized tomography scan showed mass lesion in right eye with calcification. A diagnosis of retinocytoma with vitreous seedings was made and the patient was advised four monthly follow-up. Since he had unilateral involvement and negative family history, the 8% risk of retinoblastoma in offspring was explained. Also, the patient was advised importance of regular follow-up for the rare, but possible risk of malignant transformation. However, he was lost to follow-up after 5 years.


Discussion

The incidence of retinocytoma in general population is not known. However, it is between 2% and 10% among the retinoblastoma population.[1, 3] Retinocytoma has benign histopathological features, but can undergo malignant transformation into retinoblastoma in about 4% cases and thus requires periodic follow-up.[4] Majority of these patients are asymptomatic and the diagnosis is made only on routine eye examination or while screening families of patients with retinoblastoma. Distinctive features are translucent retinal mass (88%), calcification (63%), retinal pigment epithelial disturbance (54%), chorioretinal atrophy (54%) and absence of growth.[5] In our patient, apart from these typical findings, intralesional cavitation documented by OCT and diffuse vitreous seeds were noted. After 19 years, the lesion remained stable with no documented growth. Mere presence of vitreous seeds does not signify a malignant transformation, as other criteria have to be taken into account. Even though localized vitreous seeds have previously been described in three patients with retinocytoyma,[6] there have been no reports where seedings were as diffuse as in our patient. The diagnosis of malignant transformation, however, can be based on progressive opacification, and increase in size of the tumor and presence of irregular fine tumor vessels.[6] We have reported a case of retinocytoma with diffuse vitreous seeds and cavitary lesions on OCT; both these findings have not been described in the previous literature. It is important to recognize the atypical features of diffuse vitreous seeding and intralesional cavitary lesions of this benign retinal tumor, which does not require aggressive treatment, but regular periodic follow-up.

Despite the characteristic ophthalmoscopic features of retinocytoma as mentioned above, certain entities can closely resemble retinocytoma, like retinoblastoma and astrocytic hamartoma. Retinoblastoma is diagnosed prior to age 5 years and retinocytoma is usually diagnosed in adults. Although calcification is seen in both tumors, areas of chorioretinal atrophy and associated retinal pigment epithelial changes are uncommon in untreated retinoblastoma. In addition, dilated, tortuous retinal feeder vessels are a feature of retinoblastoma rather than retinocytoma. Characteristically, retinoblastoma will show growth within 4–6 weeks, whereas retinocytoma will appear unchanged. Astrocytic hamartoma is a benign retinal tumor and can also closely resemble retinocytoma because both lesions may be calcified. Calcification, when present, can demonstrate subtle differences, as it tends to be dull and chalky white in retinocytoma, whereas in astrocytoma it is more glistening yellow, resembling fish eggs. Surrounding retinal pigment epithelium alterations, a common finding in retinocytoma, is typically absent in astrocytic hamartomas as they are situated superficially in the retina.


Notes

Source of Support: Nil

Conflict of Interest: None declared.

References
1. Gallie BL,Ellsworth RM,Abramson DH,Philips RA. Retinoma: Spontaneous regression of retinoblastoma or benign manifestation of the mutation?Br J CancerYear: 198245513217073943
2. Morris WE,LaPiana FG. Spontaneous regression of bilateral multifocal retinoblastoma with preservation of normal visual acuityAnn OphthalmolYear: 19746119244429324
3. Singh AD,Santos CM,Shields CL,Shields JA,Eagle RC Jr. Observations on 17 patients with retinocytomaArch OphthalmolYear: 200011819920510676785
4. Gallie BL,Philips RA,Ellsworth RM,Abramson DH. Significance of retinoma and phthisis bulbi for retinoblastomaOphthalmologyYear: 198289139397162783
5. Singh AD,Balmer A,Munier F. Singh AD. Retinocytoma or retinomaClinical ophthalmic oncologyYear: 2007PhiladelphiaSaunders Elsevier48790
6. Lueder GT,Héon E,Gallie BL. Retinoma associated with vitreous seedingAm J OphthalmolYear: 199511952237709984

Figures

[Figure ID: F1]
Figure 1 

Fundus photograph OD showing the translucent mass lesion with surrounding chorioretinal atrophy (black arrow)



[Figure ID: F2]
Figure 2 

Wide field imaging OD showing the mass lesion associated with diffuse vitreous seeds



[Figure ID: F3]
Figure 3 

OCT of the same eye showing a cavitation within the tumor (thick white arrow) with two calcified vitreous seeds on the left side (thin white arrows)



[Figure ID: F4]
Figure 4 

Ultrasound B-scan picture showing retinocytoma tumor with calcifications and vitreous seeds



Article Categories:
  • Case Report

Keywords: Cavitary lesions, diffuse vitreous seeds, optical coherence tomography, retinocytoma.

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