Orbital embryonal rhabdomyosarcoma in association with neurofibromatosis type 1.
A 20-month-old child with systemic features of neurofibromatosis type 1 presented with sudden-onset proptosis. Biopsy and histopathology confirmed the diagnosis of an embryonal rhabdomyosarcoma. The tumor regressed completely with chemotherapy and external beam radiotherapy. This case highlights the association of rhabdomyosarcoma with neurofibromatosis type 1.
Shome
Debraj
D
Department of Ophthalmic Plastic Surgery, Orbit and Ocular Oncology, L.V. Prasad Eye Institute, Hyderabad, India.
Honavar
Santosh G
SG
Reddy
Vijay Anand P
VA
Vemuganti
Geeta K
GK
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003131:Combined Modality Therapy; D019583:Dose Fractionation, Radiation; D005094:Exophthalmos / Q000175:diagnosis; D005260:Female; D006801:Humans; D007223:Infant; D016609:Neoplasms, Second Primary / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D009456:Neurofibromatosis 1 / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D009918:Orbital Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D018233:Rhabdomyosarcoma, Embryonal / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D014057:Tomography, X-Ray Computed