Chiasmatic infiltration secondary to late malignant transformation of retinoma

Asimina Mataftsi, Leonidas Zografos, Aubin Balmer, Sylvie Uffer, Roger Stupp, Robert C. Janzer, Alessia Pica, Daniel F. Schorderet, Francis L. Munier*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

2 Scopus citations


Purpose: To report the lethal course of malignant transformation of retinoma in an adult. Methods: Case report. A 40-year-old patient presented with retinoma in his right eye and retinoblastoma in his left eye. Enucleation was recommended but refused and the patient received whole eye radiotherapy elsewhere. Five years later he presented again, with temporal hemianopsia of the left eye secondary to chiasmatic invasion. Results: Diagnosis of retinoblastoma infiltration was confirmed by stereotactic biopsy of the chiasmatic lesion. Treatment with intravenous and intrathecal chemotherapy led to partial remission, and was followed by stereotactic irradiation of the chiasmatic mass and right optic nerve. The left eye was enucleated. Death occurred one year later due to cerebrospinal fluid metastases. Conclusion: Extraocular extension of retinoblastoma diagnosed in adulthood has never, to our knowledge, been reported. This case stresses the importance of lifelong retinoma monitoring and the necessity for radical treatment in the event of malignant transformation.

Original languageEnglish (US)
Pages (from-to)155-158
Number of pages4
JournalOphthalmic Genetics
Issue number3
StatePublished - Sep 2012


  • Adult
  • Malignant transformation
  • Retinoblastoma
  • Retinocytoma
  • Retinoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Ophthalmology
  • Genetics(clinical)


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