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Management of a Complex Orbital Post-transplantation Lymphoproliferative Disorder: A Case Report and Literature Review

TESSHO MARUYAMA, YOHEI HOKAMA, KAZUTAKA KISHABA, TOMOMI KUNINAKA, SHIGETAKA KOBAYASHI, HIDEKI NAGAMINE, HIROFUMI MIYAHIRA, KEI TANAKA, KAZUHO MORICHIKA, FUMI KAWAKAMI, MARIKO TOMITA, NAOKI WADA, AKIRA YOGI, HIROAKI MASUZAKI and TADASHI HAMASAKI
In Vivo May 2026, 40 (3) 1824-1831; DOI: https://doi.org/10.21873/invivo.14336

Article Figures & Data

Figures

  • Figure 1.
    Figure 1.

    Overview of diagnosis, treatment, and outcome in a patient with orbital post-transplant lymphoproliferative disorder.

  • Figure 2.
    Figure 2.

    Transcranial biopsy guided by preoperative image simulation. (A, B) Image simulation showing the planned approach and the intraorbital mass. (C) Intraoperative view after creation of a burr hole. (D) Biopsy of the intraorbital mass.

  • Figure 3.
    Figure 3.

    Pathological findings. (A) Hematoxylin and eosin staining reveals diffuse infiltration of lymphocytes without evident plasma cell differentiation (objective magnification, ×20). (B) Immunohistochemical staining for cluster of differentiation 20 demonstrates scattered medium- to large-sized B cells (objective magnification, ×20). (C) Epstein–Barr virus (EBV)-encoded small RNA in situ hybridization shows scattered EBV-positive cells (objective magnification, ×20). Scale bar: 50 μm. (D-F) Immunoglobulin heavy-chain gene rearrangements were analyzed with GeneScan software and BIOMED-2 multiplex polymerase chain reaction (PCR). Fluorescently labeled PCR products targeting variable region–joining region recombination in framework regions FR1 (D), FR2 (E), and FR3 (F) were separated by capillary electrophoresis. The multiple clonal peaks (arrows) in the electropherograms indicate clonal immunoglobulin heavy-chain gene rearrangements, confirming oligoclonal B-cell proliferation. The double-headed arrow indicates the evaluation range. BIOMED-2: Biomedical and Health Research Programme - 2 (The BIOMED-2 network later evolved into the EuroClonality consortium).

  • Figure 4.
    Figure 4.

    Serial magnetic resonance imaging (MRI) findings before and after reduction in immunosuppressant treatment (RIT). (A) MRI obtained 20 days before biopsy and initiation of RIT. (B) MRI obtained 65 days after biopsy, showing marked lesion regression. Day 0 indicates biopsy and initiation of RIT. (C) Serial computed tomography images showing changes in left orbital proptosis. From left to right, images were captured on days 1, 44, 57, and 279. Day 0 corresponds to the day biopsy was performed and RIT was initiated. Follow-up imaging showing gradual resolution of proptosis.

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Management of a Complex Orbital Post-transplantation Lymphoproliferative Disorder: A Case Report and Literature Review
TESSHO MARUYAMA, YOHEI HOKAMA, KAZUTAKA KISHABA, TOMOMI KUNINAKA, SHIGETAKA KOBAYASHI, HIDEKI NAGAMINE, HIROFUMI MIYAHIRA, KEI TANAKA, KAZUHO MORICHIKA, FUMI KAWAKAMI, MARIKO TOMITA, NAOKI WADA, AKIRA YOGI, HIROAKI MASUZAKI, TADASHI HAMASAKI
In Vivo May 2026, 40 (3) 1824-1831; DOI: 10.21873/invivo.14336
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