RETINOBLASTOMA IN LATE CHILDHOOD

Badhu B, Sah S P, Kumar S, Sah R P


INTRODUCTION
Retinoblastoma, the commonest intra-ocular tumour in childhood, is diagnosed before 5 years of age in 90% of cases. 1,2It has, however, been reported in adults 3,4 even in a 74-year-old man. 5 Presentation of retinoblastoma with a fungating proptosed mass in late childhood or in adults is unusual. 1 We found a case of retinoblastoma presenting as a proptosed fungating orbital mass in a 10-year-old boy at B.P.Koirala Institute of Health Sciences, Dharan, Nepal.

CASE REPORT
A 10-year-old boy presented to the Department of Ophthalmology, B.P.Koirala Institute of Health Sciences, Dharan, Nepal with the complaint of

Fig. 3, Higher magnification showing rossets and fleurettes (H & E stain, X100).
painful rapidly growing foul smelling mass involving his left eye for three months.It was preceded by the presence of a white pupillary reflex associated with diminution of vision in the affected eye for six months.Family history was unremarkable.Past medical history was not contributory.Examination revealed a fungating proptosed tender mass of firm consistency with irregular surface and necrotic areas involving the entire left eye and orbit (Fig. 1).The right eye was normal in all aspects.The preauricular and submandibular lymph nodes were not palpable.CT scan of the orbit showed a mass involving the entire eyeball with areas of calcification as well as the involvement of optic nerve and periorbital tissue.CT of the head disclosed normal gray and white matter and no signs of intracranial masses.CSF and bone marrow aspirates did not reveal any malignant cells.A clinical diagnosis of retinoblastoma was made.The patient underwent exenteration of the left orbit under general anesthesia.Grossly the exenterated mass with oedematous and erythematous eyelids measured 7x6x3 cm.The tumor was gray white in color, occupied the entire globe and extended beyond the sclera.Focal areas of necrosis and haemorrhages were also identified.Sections examined showed small round cells with hyperchromatic nuclei and scanty cytoplasm.At places, the cells were arranged in rossets and fleurettes (Fig. 2, Fig. 3).Resected end of the optic nerve showed tumour infiltration.The diagnosis of well differentiated retinoblastoma with involvement of the optic nerve section was offered.In the postoperative period, the orbit was allowed to granulate.The patient was referred for combined chemotherapy and radiotherapy.

DISCUSSION
The history of painless leukocoria initially for 6 months in the case presented here was significant to suspect retinoblastoma by health workers.Medical consultation, however, at that stage was not, sought for which shows poor awareness about the disease.However, what is highlighted in this Badhu, Sah, Kumar, Dulal, Sah : Retinoblastoma in late childhood paper is the delayed presentation of retinoblastoma with fungating mass in late childhood.
Reported modes of presentation of retinoblastoma are leukocoria, squint, hypopyon, hyphema, proptosis, secondary glaucoma, and orbital cellulitis. 6Because of its rarity in late childhood, the fungating proptosed mass or leukocoria due to retinoblastoma may be misdiagnosed.Primary health care professionals 7 as well as the parents require education about the importance of ocular symptoms in pediatric patients.
Management of retinoblastoma should be guided by the objectives to save life, to retain anatomical integrity of the eye, to preserve vision, and to obtain good cosmetic results. 7If retinoblastoma could be diagnosed in its early stage, less aggressive treatment modalities like photocoagulation, cryosurgery, cobalt plaques, external beam radiation, and diathermy would be extremely useful. 8ucleation and exenteration are more invasive modalities, which are applied if the disease is too far advanced to salvage useful vision in the affected eye, or when other treatments have failed. 9nucleation has to be carried out with special care not to spread tumor into the orbit during surgery and to obtain a long section of optic nerve by applying traction suture through the medial and lateral rectus insertions 10 or a hemostat to the medial rectus stump. 11Lateral canthotomy has been advised in a tight orbit. 11No clamps or snares on the optic nerve stump should be used as they can leave crush artifact. 11e removal of all the orbital structures including lids, eyes, extraocular muscles, nerves and orbital fat is termed as exenteration.It is reserved for treatment of primary orbital mass or orbital recurrence of retinoblastoma. 10Orbital retinoblastoma can be treated with exenteration, local irradiation and systemic chemotherapy in combination.Studies have shown that among the patients with orbital retinoblastoma, 5% survive when treated with exenteration alone, while 38% survive with adjuvant chemotherapy. 12The "cure" rate of retinoblastoma with exenteration and local irradiation is 30.9%vs 9% with exenteration alone. 13A long-term survivor with orbital recurrence of retinoblastoma treated with radiation and systemic chemotherapy has been reported. 14xenteration alone may not be effective in removing the entire recurrent tumor, but in combination with radiation may affect a more complete local cure. 15The role of chemotherapy is to treat any microscopic systemic metastasis. 15

CONCLUSION
1. Health education on the modes of presentation of retinoblastoma particularly leukocoria should be promoted among the population for the early detection and management of retinoblastoma.2. Retinoblastoma is uncommon in late childhood, but its diagnosis should be considered in cases of fungating proptosed orbital mass preceded by leukocoria.

Fig. 1 ,
Fig. 1, The clinical picture of fungating proptosed mass in the left orbit.