Most common childhood malignant orbital tumor
Presents with rapid onset proptosis, globe displacement, blepharoptosis, conjunctival and eyelid swelling, palpable mass, and pain
MRI demonstrates circumscribed mass of uniform density
Biopsy required for confirmation, FNAB should not be performed initially
Embryonal: most common
Alveolar: worst prognosis
Pleomorphic: best prognosis
Botryoid: well differentiated, grapelike tumors that arise from conj
Rx with excision, chemo, radiation, or surgery. Exteneration rarely required.
Abnormal proliferation of histiocytes (APCs of the dendritic type) in bone
Eosinophilic granuloma: Most localized and benign form, produces bony lesions that involve the orbit, skull, ribs, and long bones. Can produce proptosis, ptosis, and periorbital swelling. Rx with excision, observation, steroids, radiation.
Hand-Shuller-Christian Disease: disseminated and aggressive form. Diabetes insipitus common. Presents btw ages 2-5. Rx with chemo, prognosis good.
Letterer-Siwe disease: most severe and malignant variety. Presents younger than 2 years, rare involves eye.
Most common malignant ocular childhood tumor.
Due to mutation in RB1 gene on chromosome 13 which prevents cell proliferation from G1 to S phase.
Typically diagnosed during 1st year of life in familial bilateral cases and age 1-3 in sporadic unilateral cases.
Presents with leukocoria (most common), strabismus, vit heme, hyphema, ocular or periocular inflammation, glaucoma, proptosis, and pseudohypopyon.
Diagnosis usually clinical. Histo shows Flexner-Wintersteiner rosettes, fleurettes, and Homer-Write rosettes
Spontaneous regression possible.
Rx with systemic chemo + local radiation (most common), external beam radiation (less common due to risk of cancer), and primary enucleation.