FAMILIAL RETINOBLASTOMA
\famˈɪlɪəl ɹˌɛtɪnˌə͡ʊblastˈə͡ʊmə], \famˈɪlɪəl ɹˌɛtɪnˌəʊblastˈəʊmə], \f_a_m_ˈɪ_l_ɪ__ə_l ɹ_ˌɛ_t_ɪ_n_ˌəʊ_b_l_a_s_t_ˈəʊ_m_ə]\
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A malignant tumor arising from the nuclear layer of the retina that is the most common primary tumor of the eye in children. The tumor tends to occur in early childhood or infancy and may be present at birth. The majority are sporadic, but the condition may be transmitted as an autosomal dominant trait. Histologic features include dense cellularity, small round polygonal cells, and areas of calcification and necrosis. An abnormal pupil reflex (leukokoria); NYSTAGMUS; STRABISMUS; and visual loss represent common clinical characteristics of this condition. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, p2104)
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