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Multiple malignancies in a patient with bilateral retinoblastoma

Published online by Cambridge University Press:  29 June 2007

H. M. Eha ,
A. J. M. Balm ,
D. de Jong  and
L. J. Van't Veer
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: FBalm@NK.NL
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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.

Keywords

RetinoblastomaNeoplasms, multiple primary
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 112 , Issue 2 , February 1998 , pp. 189 - 192
Copyright
Copyright © JLO (1984) Limited 1998

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