안와에 발생한 원발성 지방층염양 말초 T세포 림프종 1예
A Case of Primary Orbital Peripheral T-cell Lymphoma with Panniculitis-like Features
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목적: 안와에 원발성으로 발생한 지방층염양 말초 T세포 림프종 진단 및 화학항암요법 후 완전관해에 도달한 증례 1예를 경험하였기에 이를 보고하고자 한다. 증례요약: 57세 여자가 복시증상을 동반한 우측 안와주위의 부종을 주소로 내원하였다. 안와 가성종양 의심하에 전신 고용량 스테로이드요법을 시행하였으나 호전이 없어 눈확절개술을 통해 절개생검을 시행하였다. 절개된 종괴는 조직학적 검사상 국소적으로 치밀한 림프구의 침윤 및 세포학적 비정형을 보였고, 면역조직화학검사상 CD3, CD8 양성이었으며, CD4, CD20, CD56 음성소견을 보여 지방층염양 말초 T세포 림프종으로 확진되었다. 또한 분자병리학적 검사상 Epstein-Barr 바이러스 양성이었다. 이후 화학항암치료를 시행하였으며 완전관해에 도달하였다. 결론: 말초 T세포 림프종에서는 림프절종대, B 증상과 같은 전신증상이 흔하게 나타나며, 안와에 원발성으로 발생하는 경우는 매우 드물고 병의 진행이 빠르며 치사율이 높아 예후가 매우 불량하다. 저자들은 특이 과거력 없이 건강한 사람에게 전신증상 없이 발생한 안와 원발성 말초 T세포 림프종 진단 및 치료 후 완전관해에 도달한 드문 경우를 경험하였기에 보고하고자 한다.
Abstract
Purpose: To report a case of complete remission of primary orbital peripheral T-cell lymphoma with panniculitis-like features after chemotherapy. Case summary: A 57-year-old healthy female presented with periorbital swelling and symptoms of diplopia. The patient was first treated with high-dose systemic corticosteroids, however, symptoms persisted. Therefore, anterior orbitotomy with excisional biopsy was performed for diagnostic purposes. On microscopic examination, the excised mass showed localized dense lymphocyte infiltrates, and cytologic atypia was observed under a high-power field. On immunehistochemical examination, tumor cells were positive for CD3 and CD8 but negative for CD4, CD20 and CD56. Based on histopathological results, primary orbital peripheral T-cell lymphoma with panniculitis-like features was diagnosed. Additionally, molecular pathological testing was positive for Epstein-Barr virus. Subsequently, the patients underwent chemotherapy and complete remission was obtained. Conclusions: Peripheral T-cell lymphoma often manifests as systemic symptoms, including lymph node enlargement and B symptom. The primary form of the disease in an orbit is very rare, and has a poor prognosis with a high mortality rate because the disease quickly progresses. Herein, the authors report a rare case of a healthy patient without any past medical history who achieved complete remission of a fast-growing primary orbital T-cell lymphoma with no preceding systemic symptoms.
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