PMC9877327_01
Preoperative condition of the left eye. (A) A funduscopic examination of the left eye revealed a hazy fundus. (B) Automated perimetry showed total visual field defect in the left eye. (C) F-VEPs of the left eye showed a reduced amplitude.
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The funduscopic examination, perimetry, and F-VEPs showed damage to his left optic nerve ( Figure 1 ).
Enhanced nuclear MRI images of the patient during different periods. (A) Pre-op sagittal MRI images showed that the mass invaded the nasal cavity, ethmoid sinus, and sphenoid sinus, and compressed the optic nerve; pre-op axial MRI image revealed that the mass invaded the middle and posterior cranial fossa. (B) Post-op MRI images showed that intraoperative decompression of the optic nerve was performed. (C) Post-op MRI images showed the condition of the optic nerve and intracranial tumor after 1 month of HSCT.
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On imaging, the diagnosis was highly likely lymphoma ( Figure 2A ). The optic nerve canal was further completely exposed, and the tumor around the optic canal was removed to decompress the optic nerve ( Figure 2B ). Tocilizumab and dexamethasone were given, and ulinastatin was added to improve capillary permeability, and norepinephrine was used to increase blood pressure. There was no further deterioration of vision in both eyes after the surgery ( Figure 2C ) and he remained disease free for 1 year after comprehensive treatment.
Representative images of translocation statuses by FISH analysis for three genes.(A) Tumor nuclei were negative for translocation of BCL-2. (B) Tumor nuclei were positive for translocation of BCL-6. Multiple tumor nuclei were seen with distinct red and green signals (highlighted by the red arrows). (C) Tumor nuclei were negative for translocation of C-MYC.
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Genetic testing of the malignant cells was positive for BCL-6-related gene translocation but negative for BCL-2- and C-MYC-related genes, and the gene translocations were detected by fluorescence in situ hybridization (FISH) ( Figure 3 ).
Timeline scheme of the major clinical events of the patient.WAS, Wiskott-Aldrich syndrome; ONSF, optic nerve sheath fenestration; COP, cyclophosphamide, vincristine, and prednisolone; VP, vinorelbine and prednisone; TBI, total body irradiation; FC, fluorouracil and carboplatin; CAR-T therapy, chimeric antigen receptor T-cell immunotherapy; CCNU, lomustine; FA, fludarabine; Dex, dexamethasone; Ara-C, cytosine arabinoside; G-CSF, granulocyte colony-stimulating factor; BU, busulfan; CTX, cyclophosphamide; RTX, rituximab; GVHD, graft-versus-host disease; MMF, mycophenolate mofetil; MTX, methotrexate; HSCT, hematopoietic stem-cell transplantation.
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The condition regimens patient used for CAR-T therapy and allogeneic HSCT are shown in  Figure 4 . On the basis of radiographic evidence of tumor shrinkage and the absence of distant metastasis, we considered this patient to be in "partial remission." The main treatment steps for the patient are shown in  Figure 4 .