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The main orbital benign and malignant tumours include orbital cyst, capillary hemangioma, neurofibroma, optic nerve gliomas, rhabdomyosarcomas, bone tumours and metastatic tumours.

Most pediatric orbital tumours are benign. Developmental cysts comprise half of orbital cases, with capillary hemangioma being the second most common orbital tumour.

The most common orbital malignancy is rhabdomyosarcoma.

The most common intraocular malignant lesion is retinoblastoma. Choroidal melanoma, which is common in adults, is extremely rare in children.

The orbit is the most common location for metastases in children, whereas the choroid is the predominant site in adults.

Pediatricians play a vital role in diagnosis of pediatric ocular tumours. They are the first to recognize ocular problems that may not be apparent to parents.

Certain malignant neoplasms can threaten the child's life as well as the child's sight.

Hence, prompt recognition of the signs and symptoms of ocular tumours of childhood and referral to an ocular oncologist are crucial for proper clinical management.

Retinoblastoma

Rhabdomyosarcoma

Cellular Hemangioma of Infancy

Neurofibroma

                 

Pediatric tumors of the eye and orbit. Pediatr Clin North Am. 2003;50 (1):149-72.

Most ocular and orbital tumors of childhood are distinct from tumors that occur in adults. Many are congenital with early presentations. Most pediatric orbital tumors are benign; developmental cysts comprise half of orbital cases, with capillary hemangioma being the second most common orbital tumor. The most common orbital malignancy is rhabdomyosarcoma. The most common intraocular malignant lesion is retinoblastoma. Choroidal melanoma, which is common in adults, is extremely rare in children. The orbit is the most common location for metastases in children, whereas the choroid is the predominant site in adults. Pediatricians play a vital role in diagnosis of pediatric ocular tumors. They are the first to recognize ocular problems that may not be apparent to parents. It is therefore important to recognize the signs and symptoms of ocular tumors of childhood so that prompt ophthalmologic evaluation and treatment may be undertaken. Whereas the malignant tumors may be life-threatening, both malignant and benign tumors may be vision-threatening.

Ophthalmological tumors in children: diagnosis and therapeutic strategy. J Fr Ophtalmol. 2000;23(9):926-39.

Clinical aspects and diagnosis features of retinoblastoma are reported. The most important differential diagnosis are discussed. The diseases that can cause difficulties in diagnosis are uveitis and Coats disease. The principles of the treatment of retinoblastoma are exposed especially the side effects of radiotherapy that lead to a limitation of the use of external beam if possible. Treatments methods include external beam radiotherapy, chemotherapy, thermochemotherapy cryotherapy, curietherapy and photocoagulation. Results and indications of these treatments are given. We describe the diagnosis approach, for orbital tumors in children. The main orbital benign and malignant tumors and their treatment are listed: orbital cyst, neurofibroma; optic nerve gliomas; rhabdomyosarcomas, bone tumors and metastatic tumors.

Ocular tumors of childhood. Pediatr Clin North Am.1993;40(4):805-26.

It is important for the pediatrician to be aware of the benign and malignant ophthalmic tumors that can occur in children. This article covers some general concepts related to ocular tumors in children and briefly describes the clinical features and management of some of the more important tumors of the eyelids, conjunctiva, intraocular structures, and orbit. Although most ocular tumors in children are benign, certain malignant neoplasms can threaten the child's life as well as the child's sight. Hence, prompt recognition and patient referral to an ocular oncologist are crucial for proper clinical management.

Eye tumors in children. Histopathological aspects of differential diagnosis.Klin Oczna. 1991;93(2-3):59-62.

Ocular tumors in children, due to their biological characteristics, possess a special position in ophthalmology. Most of these tumors are today curable with early diagnosis and correct treatment. Therefore their differential diagnosis should be familiar to all pediatricians and ophthalmologists. Tumors were divided into three groups, concerning their localization (lid and orbit, epibulbar, intraocular). In each group histopathologic and clinical pictures of most important benign (hamartomas, choristomas and pseudotumors) and malignant (primary and metastatic) tumors were separately described and correlated, with special attention paid to differential diagnosis.

Considerations of malignant ocular tumours in children (author's transl).Arch Ophtalmol (Paris).1977;37(10):629-40.

The authors undertake a clinico-anatomical study of 27 malignant tumours of the orbit and eye in children seen at the Ophthalmological Clinic in Jassy over the last 12 years. The majority (18 cases) were intra-ocular tumours which were clinically and anatomically retinoblastomas. After presenting the clinical symptoms, drawing attention to the signs of the disease presenting to paediatricians, the treatment is described. This was surgical in all cases followed by radiotherapy according to the case. Analysis of fatal cases and those which survived demonstrate certain degrees of seriousness in clinical, histopathological and therapeutic aspects. It can be appreciated that the long-term prognosis of these cases depends on the early diagnosis and the institution of the correct treatment as well as the anatomopathological form.

                   

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