Wantavornprasert Kittipong, Asawapanumas Thiti, Asawanonda Pravit
Division of Dermatology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
Division of Hematology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
Case Rep Dermatol. 2022 Jul 5;14(2):188-193. doi: 10.1159/000525348. eCollection 2022 May-Aug.
Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) is a rare condition which has a very aggressive clinical outcome. The most common and typical presentation is the destructive tumor involving the nasal cavity or nasopharynx, referred to as lethal midline granuloma, while cutaneous involvement is found to be the second most involved site. In this report, we describe a case of an otherwise healthy 40-year-old female solely presenting with ulcerative plaques and subcutaneous nodules on her lower extremities. Although the rimming of adipocytes by atypical lymphocytes, which resembles subcutaneous panniculitis-like T-cell lymphoma, was found on histopathology, immunohistochemistry revealed the diagnosis of cutaneous ENKTCL-NT which portends a much worse prognosis. A positron emission tomography scan also detected a hypermetabolic mass on her nasal cavity despite negative findings on history and initial examination. After thorough investigation, her final diagnosis was ENKTCL-NT with extranasal involvement (cutaneous) stage IV.
结外NK/T细胞淋巴瘤,鼻型(ENKTCL-NT)是一种罕见疾病,临床预后极具侵袭性。最常见且典型的表现是累及鼻腔或鼻咽部的破坏性肿瘤,称为致死性中线肉芽肿,而皮肤受累是第二常见的部位。在本报告中,我们描述了一例40岁健康女性,仅表现为下肢溃疡性斑块和皮下结节。尽管组织病理学检查发现非典型淋巴细胞围绕脂肪细胞,类似于皮下脂膜炎样T细胞淋巴瘤,但免疫组化显示诊断为皮肤ENKTCL-NT,其预后要差得多。正电子发射断层扫描在其鼻腔也检测到一个高代谢肿物,尽管病史和初步检查结果为阴性。经过全面检查,她的最终诊断为ENKTCL-NT伴鼻外受累(皮肤)IV期。