Multicentric osteosarcoma presenting as retrobulbar mass: A diagnostic enigma

Osteosarcoma is the most common primary malignant bone tumor of children and adolescents. It often presents as a solitary lesion; multicentric osteosarcoma with synchronous lesions occurring at multiple skeletal sites is very rare. We report a 9‐year‐old boy with multicentric osteosarcoma who presen...

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Bibliographic Details
Published in:Pediatric Blood & Cancer 2006-06, Vol.46 (7), p.815-819
Main Authors: Wong, K.F., Shu, S.J., Luk, I.S.C., Yuen, H.L., Leung, J.N.S., Chan, J.K.C.
Format: Article
Language:English
Subjects:
Biological and medical sciences
bone marrow
Bone Marrow - pathology
Bone Neoplasms - genetics
Bone Neoplasms - pathology
Child
Cytogenetic Analysis
cytogenetics
cytology
Diagnosis, Differential
Eye Neoplasms - genetics
Eye Neoplasms - pathology
Eye Neoplasms - secondary
General aspects
Humans
Male
Medical sciences
multicentric osteosarcoma
Neoplasms, Multiple Primary - genetics
Neoplasms, Multiple Primary - pathology
Osteosarcoma - genetics
Osteosarcoma - pathology
Osteosarcoma - secondary
radiology
Tomography, X-Ray Computed
Tumors
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Summary:Osteosarcoma is the most common primary malignant bone tumor of children and adolescents. It often presents as a solitary lesion; multicentric osteosarcoma with synchronous lesions occurring at multiple skeletal sites is very rare. We report a 9‐year‐old boy with multicentric osteosarcoma who presented with a left retrobulbar non‐sclerotic mass. The multiple lesions in bone were mostly non‐sclerotic on radiological examination except for a single lesion in the left tibia. Biopsy of the retrobulbar mass showed an unclassifiable poorly differentiated malignant tumor. Marrow aspiration smears showed many large, often segregated, round cells that expressed NB84a. However, trephine biopsy showed the formation of tumoral osteoid by the malignant cells, finally permitting the definitive diagnosis of osteosarcoma to be made. A hypertetraploid clone with complex structural abnormalities was demonstrated by cytogenetic study. © 2005 Wiley‐Liss, Inc.
ISSN:1545-5009
1545-5017
1096-911X
DOI:10.1002/pbc.20431