To the Editor: Classical Hodgkin lymphoma (cHL) represents approximately 10% of all lymphomas, with nodular sclerosis (NSHL) being the most common subtype 1-3. While NSHL typically presents with localized lymphadenopathy and systemic B-symptoms, extranodal involvement can occur via direct extension or hematogenous spread 2, 4, 5. Common extranodal sites include the lungs, liver, and spleen; however, ocular involvement is exceptionally rare, with an incidence of less than 0.5% 6. We present a unique case of NSHL involving a discrete subfoveal choroidal infiltrate. A 19-year-old male with a past medical history significant for gastroesophageal reflux disease initially presented to the oncology clinic for worsening chronic cough for the past year and a weight loss exceeding 60 pounds, along with decreased appetite, dysphagia, and mild dysphonia. He denied fatigue, fevers, chills, and night sweats in the previous year. Physical examination revealed no marked cervical or supraclavicular lymphadenopathy. Further work-up and imaging revealed a new large right anterosuperior mediastinal mass of 9.4 × 5.9 × 7.2 cm. This mass was seen to extend to the level of the clavicle into the thoracic inlet. Significant mass effect upon the right lung apex was observed. An interventional radiology-guided right paratracheal mass biopsy revealed scattered large atypical cells in the background of eosinophilia with overall architecture effaced by nodular sclerosis. Subsequent immunohistochemical staining was performed, which demonstrated neoplastic cells positive for CD30 and MUM-1, consistent with the diagnosis of NSHL. Comprehensive infectious work-up was unremarkable. Approximately 1 month later, the patient presented to the emergency department with fever (Tmax 103°F), worsening cough, and new-onset blurry vision in the right eye. Subsequent chest imaging revealed a persistent right upper mediastinal mass from previous studies and contiguous extension to the right suprahilar region, with positron emission tomography scan results further corroborating these results. Neuroophthalmology consultation showed a discrete solitary subfoveal choroidal infiltrate in the right macula (Figure 1A). Optical coherence tomography (OCT) revealed a hyperreflective choroidal mass with overlying retinal elevation of the right eye, significant perifoveal edema, and associated subretinal hemorrhage (Figure 1B). The lesion exhibited homogenous reflectivity, suggestive of neoplastic infiltration rather than inflammatory pathology. Fundoscopic examination demonstrated vitreous haze with scattered inflammatory cells, central scotoma, and loss of color vision (Figure 1C). Given clinical signs concerning worsening cHL, chemotherapy consisting of brentuximab, prednisone, etoposide, and doxorubicin was initiated. After two cycles of the chemotherapy regimen, the patient reported decreased blurriness and improved vision in his right eye. Repeat OCT demonstrated resolution of the choroidal lesion, with persistent foveal atrophy, retinal thinning, and disruption of the ellipsoid zone, indicative of prior neoplastic infiltration. The reduction in choroidal thickening and disappearance of subretinal fluid further supported a successful therapeutic response (Figure 2A, B). The absence of new lesions on follow-up imaging and stabilization of visual acuity reinforces post-treatment regression, aligning with research documenting similar outcomes in choroidal lymphoma regression post-chemotherapy 7, 8. The patient is in remission as of his 33-month off-therapy visit and has a lingering central scotoma in his right eye. This case presents a rare choroidal metastasis in cHL, underscoring atypical metastatic patterns in HL 3, 5. Unlike common extranodal sites, choroidal metastasis, as seen in this patient, is less frequent than orbital or adnexal involvement, which may present with proptosis or conjunctival masses 8, 9. Hematogenous dissemination, likely responsible for this case's metastasis, involves vascular invasion by Reed–Sternberg cells 4. The mechanisms of such spread remain poorly understood, making this presentation particularly notable for its rarity and clinical implications 4, 7, 10. The patient's OCT findings, including a hyperreflective choroidal mass, retinal elevation, and subretinal fluid, align with reported choroidal lymphoma characteristics 7, 8. Mashayekhi et al. 8 described similar imaging features, emphasizing OCT's diagnostic utility. The lesion's homogenous reflectivity distinguished it from inflammatory conditions, such as Vogt–Koyanagi–Harada disease, highlighting the need for precise diagnostic tools 11. Post-chemotherapy, the choroidal lesion resolved, leaving residual foveal atrophy and retinal thinning, consistent with prior studies 7, 8, 10. Stabilized visual acuity and no new lesions confirm therapeutic success, aligning with reported choroidal lymphoma regression 9, 10. This case presents a highly unusual presentation of cHL metastasizing to the retina, reinforcing the need for vigilance in identifying unexpected sites of disease involvement. The patient's initial mediastinal involvement, subsequent subfoveal choroidal metastasis, and positive therapeutic response align with literature findings and lymphoma pathogenesis 12. Given the rarity of hematogenous spread to the eye, early identification and prompt intervention with systemic chemotherapy are crucial to preserving visual function and optimizing patient outcomes 7. The authors declare no conflicts of interest. Data sharing not applicable to this article as no data sets were generated or analyzed during the current study.
Ambuj et al. (Thu,) studied this question.