眼眶软组织复合性淋巴瘤表现为伴有套细胞和滤泡细胞成分的淋巴结淋巴瘤复发：病例报告、文献复习及患者治疗指南。
Orbital soft tissue composite lymphoma presenting as recurrence of a nodal lymphoma with mantle and follicular cell components: A case report, literature review and guideline for the treatment of patients.
机构信息
Department of Pathology, University General Hospital, Valencia, Spain.
Department of Pathology, University General Hospital, Valencia, Spain.
出版信息
Rev Esp Patol. 2020 Jan-Mar;53(1):48-54. doi: 10.1016/j.patol.2019.07.002. Epub 2019 Aug 22.
Composite lymphoma with mantle and follicular cell components is a challenging diagnosis. Flow cytometry, immunohistochemistry and molecular genetics are required to distinguish the two components, as often the more aggressive one is predominant and masks the other. A 58-year-old man with history of nodal composite lymphoma presented with right exophthalmos and diplopia. A head CT scan showed an orbital tumor. A biopsy of the tumor revealed a mantle cell lymphoma predominating over a follicular lymphoma. Immunoglobulin heavy chain and light chain rearrangements analysis by PCR proved that both components of the orbital tumor were recurrences of the same nodal composite lymphoma diagnosed two years earlier. The nodal lymphoma was composed of a follicular lymphoma and an in situ mantle cell neoplasia. Consensus view is that dominant lymphoma should be treated when needed but taking into account if the mantle cell lymphoma is an in situ neoplasia and if it expresses CD5 and SOX11.
复合性淋巴瘤伴套细胞和滤泡细胞成分是一种具有挑战性的诊断。为了区分这两种成分，需要进行流式细胞术、免疫组织化学和分子遗传学检查，因为通常侵袭性更强的成分占主导地位，掩盖了另一种成分。一位 58 岁的男性，有淋巴结复合性淋巴瘤病史，表现为右眼突出和复视。头部 CT 扫描显示眼眶肿瘤。肿瘤活检显示套细胞淋巴瘤为主，滤泡性淋巴瘤次之。通过 PCR 进行免疫球蛋白重链和轻链重排分析证明，眼眶肿瘤的两个成分均为两年前诊断的同一淋巴结复合性淋巴瘤的复发。淋巴结淋巴瘤由滤泡性淋巴瘤和原位套细胞肿瘤组成。共识观点认为，当需要时应治疗主导性淋巴瘤，但要考虑套细胞淋巴瘤是否为原位肿瘤，以及是否表达 CD5 和 SOX11。
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