Zheng Xiao-Dan, Zhang Yan-Lin, Xie Jian-Lan, Zhou Xiao-Ge
Department of Pathology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.
World J Clin Cases. 2020 Apr 26;8(8):1507-1514. doi: 10.12998/wjcc.v8.i8.1507.
We describe the case of a 74-year-old man diagnosed with primary cutaneous mantle cell lymphoma (MCL), an extremely rare and controversial condition that is not included in the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas.
The patient presented diffuse cutaneous erythematous plaques and nodules throughout the body. Skin lesions were biopsied and histopathological examination showed diffuse monomorphic lymphocyte infiltration in the dermal and subcutaneous layers, sparing the epidermis. Immunohistochemical staining revealed CD20, cyclin-D1, CD5, and SOX-11 expression. Fluorescence hybridization showed / gene rearrangement. Correct diagnosis of primary cutaneous MCL requires ensuring that no other parts are involved; these cases require close follow-up to monitor their possible progression to systemic disease and for treating relapsed cutaneous disease. In this case, positron emission tomography scanning and clinical staging revealed no systemic involvement, and follow-up examination at 20 mo after diagnosis showed no evidence of systemic disease. The prognosis of primary cutaneous MCL is relatively good. Our patient received six cycles of chemotherapy, and the cutaneous manifestations presented almost complete remission.
Primary cutaneous MCL is rare, and its prognosis is relatively favorable. However, correct diagnosis is a prerequisite for proper treatment.
我们描述了一例74岁男性原发性皮肤套细胞淋巴瘤（MCL）的病例，这是一种极为罕见且存在争议的疾病，未被世界卫生组织 - 欧洲癌症研究与治疗组织的皮肤淋巴瘤分类所涵盖。
患者全身出现弥漫性皮肤红斑斑块和结节。对皮肤病变进行活检，组织病理学检查显示真皮和皮下层有弥漫性单形性淋巴细胞浸润，表皮未受累。免疫组织化学染色显示CD20、细胞周期蛋白D1、CD5和SOX - 11表达。荧光杂交显示 / 基因重排。原发性皮肤MCL的正确诊断需要确保无其他部位受累；这些病例需要密切随访，以监测其可能进展为系统性疾病以及治疗复发性皮肤疾病。在本病例中，正电子发射断层扫描和临床分期显示无系统性受累，诊断后20个月的随访检查未发现系统性疾病的证据。原发性皮肤MCL的预后相对较好。我们的患者接受了六个周期的化疗，皮肤表现几乎完全缓解。
原发性皮肤MCL罕见，其预后相对良好。然而，正确诊断是恰当治疗的前提。
