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

Ejstrup,Rasmus; Mikkelsen,Lauge Hjorth; Andersen,Mette Klarskov; Clasen‑Linde,Erik; Gjerdrum,Lise Mette Rahbek; Safavi,Setareh; Heegaard,Steffen

doi:10.3892/ol.2018.9725

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

Authors:
- Rasmus Ejstrup
- Lauge Hjorth Mikkelsen
- Mette Klarskov Andersen
- Erik Clasen‑Linde
- Lise Mette Rahbek Gjerdrum
- Setareh Safavi
- Steffen Heegaard
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Affiliations: Department of Ophthalmology, Rigshospitalet, Copenhagen University Hospital, 2100 Copenhagen, Denmark, Department of Clinical Genetics, Rigshospitalet, Copenhagen University Hospital, 2100 Copenhagen, Denmark, Department of Pathology, Rigshospitalet, Copenhagen University Hospital, 2100 Copenhagen, Denmark, Department of Pathology, Zealand University Hospital, 4000 Roskilde, Denmark
Published online on: November 19, 2018 https://doi.org/10.3892/ol.2018.9725
Pages: 1477-1482
Copyright: © Ejstrup et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

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 x 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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