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Introduction: Primitive neuroectodermal tumors arise from the progenitor cells of the neural crest, in the central nervous system or other peripheral locations., Case Presentation: We report a rare case of a congenital malignant tumor, diagnosed as a primary orbital primitive neuroectodermal tumor on histopathological examination., Conclusion: Multidisciplinary management with adjuvant chemotherapy needed for the management of these cases.

Phacomatosis pigmentovascularis (PPV) is a rare congenital disease characterized by the co-existence of cutaneous vascular malformation and pigmentary nevi with or without extracutaneous systemic involvement. Here, we present a 2-month old child diagnosed with phacomatosis cesioflammea type of PPV with Sturge-Weber syndrome and secondary congenital glaucoma of the left eye. She underwent combined trabeculotomy and trabeculectomy in the left eye for glaucoma and was started on anti-epileptics for seizure control following pediatric evaluation. Early screening and treatment initiation can prevent blindness and other systemic complications associated with PPV.

A 50-year-old female patient presented with protrusion of the left eye for 1 month. Examination showed abaxial proptosis, restriction of extraocular movements, and elevated intraocular pressure. Computed tomography of the orbits showed soft tissue enhancing lesion in the superolateral aspect of the left orbit with lytic lesions in calvarium. Fine needle aspiration cytology of the lesion revealed a diagnosis of plasmacytoma with positive CD138 and CD38 immunohistochemical stains. Erythrocyte sedimentation rate, C-reactive protein and serum lactate dehydrogenase were elevated. Serum protein electrophoresis revealed hypergammaglobulinemia, and bone marrow biopsy revealed 6% plasma cells. The patient was started on chemotherapy with bortezomib, dexamethasone and lenalidomide by the medical oncologist. Significant improvement in proptosis and extraocular movements noted on follow-up. Orbital myeloma may be the first manifestation of systemic disease.