Orbital Leiomyosarcoma After Bilateral Retinoblastoma Treated With Chemotherapy And Radiotherapy



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Submitted 2020-06-04
Published 2020-06-23
10.24018/ejmed.2020.2.3.321

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Article Main Content

A 23-year old man presented with a swelling medially in his left orbit. He had had bilateral retinoblastoma as an infant and was treated with bilateral enucleation, chemotherapy and radiotherapy. Histological examination confirmed the lesion to be leiomyosarcoma. A genetic defect in the RB1 tumour suppressor gene underlies the development of hereditary retinoblastoma and renders patients at substantially increased risk of developing subsequent non-ocular malignancies including soft tissue sarcomas. This risk is enhanced by radiotherapy particularly if administered before the age of one year. Awareness, by both patients and healthcare professionals, of this risk of secondary malignancy, is extremely important. Identification and aggressive investigation of new symptoms and signs may allow for the earlier detection of secondary malignancy which may, in turn, improve outcomes.

Keywords: Leiomyosarcoma; Radiotherapy; Retinoblastoma

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