Intraocular rhabdomyosarcoma in the sibling of a patient with a cerebellar medulloepithelioma.
A 12-year-old girl with a three-year history of right eye disease was referred to our service after enucleation of her right eye. Histology showed a pleomorphic high-grade rhabdomyosarcoma. As there was extrascleral extension, we elected to treat her with local radiotherapy and adjuvant vincristine, dactinomycin, cyclophosphamide, and adriamycin. She remains disease free 35 months from diagnosis. Notably, a younger sibling died in early childhood as a result of a cerebellar medulloepithelioma. Although there were no teratoid elements in the rhabdomyosarcoma, our patient's unusual disease course would be consistent with rhabdomyosarcomatous differentiation in a teratoid medulloepithelioma.
Davidson
Alan
A
Haematology-Oncology Service, Red Cross Children's Hospital, School of Child and Adolescent Health, Cape Town, South Africa. davidson@ich.uct.ac.za
Desai
Farieda
F
Stannard
Clare
C
Ivey
Andrew
A
Solomon
Rustum
R
Sinclair-Smith
Colin
C
D002528:Cerebellar Neoplasms / Q000235:genetics*; D002648:Child; D003131:Combined Modality Therapy; D005134:Eye Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D018242:Neuroectodermal Tumors, Primitive / Q000235:genetics*; D012208:Rhabdomyosarcoma / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D035781:Siblings; D016896:Treatment Outcome