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Orbital precursor B-lymphoblastic lymphoma involving the extraocular muscles in a 56-year-old male and a review of the literature

The aim of the present study was to describe a rare case of orbital precursor B-lymphoblastic lymphoma (B-LBL) in an adult. A 56-year-old male in complete remission of a gastric precursor B-LBL was referred to our orbital clinic due to rapid development of left-sided painless periorbital swelling, d...

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Published in:Oncology letters 2019-02, Vol.17 (2), p.1477-1482
Main Authors: Ejstrup, Rasmus, Mikkelsen, Lauge Hjorth, Andersen, Mette Klarskov, Clasen-Linde, Erik, Gjerdrum, Lise Mette Rahbek, Safavi, Setareh, Heegaard, Steffen
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container_title Oncology letters
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creator Ejstrup, Rasmus
Mikkelsen, Lauge Hjorth
Andersen, Mette Klarskov
Clasen-Linde, Erik
Gjerdrum, Lise Mette Rahbek
Safavi, Setareh
Heegaard, Steffen
description The aim of the present study was to describe a rare case of orbital precursor B-lymphoblastic lymphoma (B-LBL) in an adult. A 56-year-old male in complete remission of a gastric precursor B-LBL was referred to our orbital clinic due to rapid development of left-sided painless periorbital swelling, diplopia, and proptosis. Complete ophthalmoplegia was observed. Notably, magnetic resonance imaging showed swelling of the medial and inferior rectus muscles in the left orbit and biopsies were performed. Following histological diagnosis of precursor B-LBL, the patient was treated with radiotherapy (2Gy × 20) and chemotherapy according to the NOPHO ALL 2008 protocol. The disease progressed and the patient succumbed after 5 months. Histomorphologically, a lymphoblastic infiltrate was observed within the skeletal muscle tissue. The tumor cells were small and immature, and stained strongly for cluster of differentiating (CD)10, CD79a, paired box 5 and B cell lymphoma-2. The Ki-67 proliferative index was 90%. Multiplex ligation-dependent probe amplification and array comparative genomic hybridization detected whole chromosomal gain of X and 12, and both hemizygous and homozygous deletion on 9p comprising cyclin dependent kinase inhibitor 2A/B. Furthermore, array comparative genomic hybridization detected copy number imbalances consisting of focal or smaller deletions on chromosomes 1, 9, 10, 11 and 20. The final diagnosis was precursor B-LBL relapse in the extraocular muscles. Orbital precursor B-LBL is extremely rare in adults, and the diagnosis may be challenging to make. It is recommended to obtain material for cytogenetic and molecular analyses.
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subjects Age
Bone marrow
Care and treatment
Case studies
Chemotherapy
Chromosomes
Cloning
Deoxyribonucleic acid
Diagnosis
DNA
Genomes
Hybridization
Lymphoma
Lymphomas
NMR
Nuclear magnetic resonance
Oncology
Pain
Tumors
Young adults
title Orbital precursor B-lymphoblastic lymphoma involving the extraocular muscles in a 56-year-old male and a review of the literature
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