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Precursor B-cell lymphoblastic lymphoma presenting as an orbital mass
Mark A. Alford1, Jeffrey A. Nerad, Ronan M. Conlan
1Department of Ophthalmology & Visual Sciences, University of Iowa Hospitals & Clinics, Iowa City, Iowa, U.S.A.
Orbit (Amsterdam, Netherlands)
|June 6, 2002
Summary
A rare childhood cancer, precursor B-cell lymphoblastic lymphoma, presented as eye proptosis in a 12-year-old boy. This case highlights orbital involvement and emphasizes prompt diagnosis and chemotherapy for this potentially curable condition.
Area of Science:
- Pediatric Oncology
- Ophthalmology
- Hematology
Background:
- Precursor B-cell lymphoblastic lymphoma is a rare non-Hodgkin's lymphoma primarily affecting children and young adults.
- Orbital involvement by this lymphoma is exceptionally uncommon, posing diagnostic challenges.
Purpose of the Study:
- To report the first case of precursor B-cell lymphoblastic lymphoma presenting with orbital mass and proptosis in a pediatric patient.
- To review the clinical features, diagnosis, and multidisciplinary management of childhood non-Hodgkin's lymphomas with orbital involvement.
Main Methods:
- Case presentation of a 12-year-old boy with acute proptosis.
- Diagnostic imaging including CT scan to delineate the orbital and sinonasal mass.
- Incisional biopsy for histopathological confirmation of precursor B-cell lymphoblastic lymphoma.
Main Results:
- CT revealed a mass in the left orbit, maxillary, and ethmoid sinuses, extending into the anterior cranial fossa.
- Incisional biopsy confirmed precursor B-cell lymphoblastic lymphoma.
- The patient's condition required systemic chemotherapy, which is potentially curative.
Conclusions:
- Precursor B-cell lymphoblastic lymphoma can present primarily in the orbit, necessitating ophthalmologist involvement for early diagnosis.
- Multidisciplinary management involving chemotherapy is crucial for potentially curative outcomes in pediatric orbital lymphomas.
- This case underscores the importance of considering rare hematologic malignancies in the differential diagnosis of pediatric orbital masses.