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Retinoblastoma
is the most common intraocular malignancy in children. It has been reported
through studies and researches that survival rate of extra ocular
retinoblastoma ranges between 50% and 70% which is still low. Therefore better
research should be carried out so that we can improve the survival rate and
visual outcome of the patient. The survival of retinoblastoma patients still
remains low, primarily due to a delayed presentation, resulting in larger
proportions of extra ocular disease compared with the developed world, where
majority of the disease is intraocular. This is a case of 6 year boy presenting
with pain, swelling along with inflammation of the right orbit for 15 days
which was enucleated six month ago. Recommendations are made on the role of
chemotherapy and radiotherapy to reduce the morbidity of patient. After the
case presentation, a review of therapeutic modalities for treatment of
retinoblastoma will be discussed, with particular focus on enucleation and the
management of recurrent retinoblastoma.
Keywords: Retinoblastoma,
Recurrence, Treatment
INTRODUCTION
Retinoblastoma is an
intraocular tumor of neuroectodermal origin. It most commonly occurs unilaterally, more
than 70% of the cases. It is a life threatening and sight threatening disease.
Enucleation is the main modality of treatment. Among all the enucleated eyes
4.2% cases shows recurrence [1]. Most important risk factor for
recurrence is compromised surgical margin and extra scleral invasion [2,3].
Retro laminar Optic nerve invasion and choroid invasion is the most important
risk factor for recurrence. Adjuvant therapy required in those situations [4].
CASE REPORT
A boy of 6 years age coming to the outpatient
department of Ophthalmology, Agartala Govt. Medical college, Agartala, with the
complain of pain and swelling of the right orbit for 15 days. It was associated
with itching and discharge from the right eye. It was associated with fever
also. The patient had undergone enucleation of the right eye 6 month back
elsewhere.
The parents of the baby noticed a white
pupillary reflex six month ago after which they consulted with an
ophthalmologist. After all the necessary investigations, he was diagnosed as a
case of intraocular retinoblastoma. He was advised enucleation and adjuvant
chemotherapy. The baby underwent enucleation of the right eye. Specimens were
sent to the pathology department and it was diagnosed as a case of
retinoblastoma. Prothesis was implanted during the time of enucleation surgery.
Parents were advised for follow up and chemotherapy after 1 month. But the parents were disinterested in any of the follow up procedures
and so unfortunately the patient did not receive any chemotherapy after
enucleation. After three months the patient developed severe pain and swelling
of the enucleated eye, associated with watering and discharge. On examination,
a large proptotic mass was noted which was spreading the eyelids apart. It was
Purple red in color, irregular surface with ulcero proliferative nature, hard
in consistency, fixed to underlying structures, movements restricted. Lids were
swollen and vessels over the skin were engorged. On
contrast enhanced CT scan on the orbit shows soft tissue mass lesion
approximately 5.6 cm × 4.7 cm × 4 cm seen completely filling the intraconal and
extraconal compartments of the left orbit. CT scan of the brain showed no signs
of intracranial extension. Lumber puncture, body scan and bone marrow biopsy
were performed to find out any metastasis. But there were no signs of
metastasis at that moment. Initially the patient was managed conservatively.
After the diagnosis was confirmed we planned for combined radiotherapy and
chemotherapy. But the patient only received 10 Gy radiotherapy, after that the
parents refused
DISCUSSION
Retinoblastoma
is the most common intraocular malignant neoplasm of childhood affecting
1:17,000 to 1:20,000 children [5]. Most cases are unilateral; though up to 25%
cases can be bilateral. It presents with white pupillary reflex, strabismus,
nystagmus, proptosis, palpable orbital mass and lid swelling with ecchymosis.
Sometimes extrusion of prosthesis after enucleation can represent recurrence.
Goal
of treatment is to maintain life, to retain the eye, to preserve vision and to
facilitate good cosmetic result. Management is based on the size and
involvement of the tumor. Small sized tumors can be managed by trans pupillary
thermotherapy, laser photocoagulation and cryotherapy for tumors anterior to
ora serrate [6].
Medium
sized tumors can be managed by plaque brachytherapy and EBRT along with
chemotherapy. Invasive treatment modalities like enucleation and exenteration
are reserved for large tumors with or without optic nerve involvement. It
should be followed by chemotherapy along with radiotherapy. Chemotherapy is of
paramount importance in its management [7].
After
enucleation, orbital bed and optic nerve examination is important. Inadequate
amount of resection of optic nerve and/or spillage of tumor cells during
surgery can cause recurrence. Goble et al. demonstrated the effectiveness of
combination therapy enucleation, radiation and systemic chemotherapy in large
tumors [8]. They conclude that aggressive therapy for orbital retinoblastoma
should include local surgical biopsy, radical orbital radiotherapy and systemic
chemotherapy.
If
after careful enucleation, the surgical margin of the optic nerve is positive
or if an orbital recurrence is present, removal of any tumor mass surgically
with local irradiation and systemic chemotherapy is the management of choice.
In review of our case presentation, it is hard to comment on the enucleation
that took place. It is not known whether the optic nerve was extracted with as
much length as possible.
Nevertheless,
the patient certainly should have completed his course of initial radiation.
When the patient presented with a large proptotic mass, he should have
undergone the combination therapy of exenteration, radiation and chemotherapy.
Unfortunately, the patient succumbed to a disease which still has a high
mortality rate even with aggressive treatment.
CONCLUSION
If
a child presents with ocular retinoblastoma, the utmost step is to decide
whether the affected eye can be saved with the non-invasive procedures which
are listed above. If however, the eye cannot be saved, then careful enucleation
with extraction of a long optic nerve sample is advisable.
1.
Kim
JW, Kathpalia V, Dunkel IJ, Wong RK, Riedel E, et al. (2009) Orbital recurrence
of retinoblastoma following enucleation. Br J Ophthalmol 93: 463-467.
2.
Khelfaoui
F, Validire P, Auperin A, Quintana E, Michon J, et al. (1996) Histopathologic
risk factors in retinoblastoma: A retrospective study of 172 patients treated
in a single institution. Cancer 77: 1206-1213.
3.
Messmer
EP, Heinrich T, Höpping W, Sutter E, Havers W, et al. (1991) Risk factors for
metastases in patients with retinoblastoma. Ophthalmology 98: 136-141.
4.
Chantada
GL, Gonzalez A, Fandino A, de Davila MT, Demirdjian G, et al. (2009) Some
clinical findings at presentation can predict high-risk pathology features in
unilateral retinoblastoma. J Pediatr Hematol Oncol 31: 325-329.
5.
Klintworth
GK (1994) The eye. In: Rubin, E, Farber, JL, ed. Pathology. Philadelphia: J.B.
Lippincott, Chapter 29: 1480-1482.
6.
Abramson
DH (1985) Treatment of retinoblastoma. In: Blodi, FC, ed. Contemporary Issues
in Ophthalmology, Vol. 2, retinoblastoma. New York: Churchill Livingstone;
Chapter 4: 63-93.
7.
Shields
JA, Shields, CL, De Potter P (1992) Enucleation technique for children with
retinoblastoma. J Pediatr Ophthalmol Strabismus 29: 213-215.
8.
Finger
PT, Czechonska G, Demirci H, Rausen A (1999) Chemotherapy for retinoblastoma: A
current topic. Drugs 58: 983-996.
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