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Multiple malignancies in a patient with bilateral retinoblastoma

Published online by Cambridge University Press:  29 June 2007

H. M. Eha
Affiliation:
Department of Radiotherapy, The Netherlands Cancer Institute (Antoni van Leeuwenhoek Hospital), Amsterdam, The Netherlands.
A. J. M. Balm*
Affiliation:
Departments of Otolaryngology/Head and Neck SurgeryThe Netherlands Cancer Institute (Antoni van Leeuwenhoek Hospital), Amsterdam, The Netherlands.
D. de Jong
Affiliation:
Department of Pathology, The Netherlands Cancer Institute (Antoni van Leeuwenhoek Hospital), Amsterdam, The Netherlands.
L. J. Van't Veer
Affiliation:
Department of Pathology, The Netherlands Cancer Institute (Antoni van Leeuwenhoek Hospital), Amsterdam, The Netherlands.
*
Address for correspondence: A. J. M. Balm, M.D., Department of Otolaryngology/Head and Neck Surgery, The Netherlands Cancer Institute, Plesmanlaan 121, 1066 CX Amsterdam, The Netherlands. Fax: +31-20-5122554 e-mail: [email protected]

Abstract

A case is presented of a patient with bilateral retinoblastoma, treated at infancy with surgery, chemotherapy and radiotherapy, who subsequently developed at least four additional histologically distinct malignancies: a Ewing sarcoma of the left fibula, two extraskeletal osteosarcomas of the left lower extremity, a mucoepidermoid carcinoma of the right parotid gland and a squamous cell carcinoma of the left paranasal cavity.

In addition to retinoblastoma, patients with a germline RB-1 mutation are at high risk of second primary malignancies. An additive carcinogenic effect of cytotoxic therapy in these patients has been assumed. Patients with hereditary retinoblastoma should be under life-long follow-up programmes including a regular head and neck examination for detection of new primaries, especially in the radiation field of the presenting retinoblastoma.

Type
Clinical Records
Copyright
Copyright © JLO (1984) Limited 1998

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