Althoff Ashley, Bibliowicz Michael
Medical Student, LECOM Bradenton.
Otolaryngology, Florida Hospital-Orlando.
Cureus. 2017 May 19;9(5):e1260. doi: 10.7759/cureus.1260.
Extranodal natural killer/T-cell lymphoma (ENKTCL) is a rare form of non-Hodgkin lymphoma. This neoplasm is more prevalent in regions of Asia and Latin America and most commonly involves the sinonasal tract, presenting with signs of nasal obstruction, epistaxis, or sinus infection. It is a locally destructive and angioinvasive neoplasm. The treatment of ENKTCL is dependent on the extent of the tumor. For localized disease, the treatment is chemoradiation. For disseminated disease, treatment is mainly chemotherapy-based. This report describes a case of a 41-year-old Hispanic woman who initially presented with signs of nasal congestion for four weeks and was subsequently diagnosed and treated for chronic sinusitis. The patient underwent endoscopic surgery for persistent chronic sinusitis, with a presumptive diagnosis of allergic fungal rhinosinusitis based on clinical and radiographic presentation. The pathologic exam revealed a diagnosis of ENKTCL. The patient underwent three cycles of chemotherapy comprised of steroid (hydrocortisone), methotrexate, ifosfamide, pre-asparaginase, and etoposide (SMILE) followed by radiation, resulting in clinical and radiographic remission. On review of the literature, ENKTCL is very rare in the United States and diagnosis is commonly delayed due to non-specific signs. We report this case to increase awareness of this disease entity and remind clinicians to include this in the differential diagnosis of nasal obstruction.
结外自然杀伤细胞/T细胞淋巴瘤(ENKTCL)是一种罕见的非霍奇金淋巴瘤。这种肿瘤在亚洲和拉丁美洲地区更为常见,最常累及鼻窦,表现为鼻塞、鼻出血或鼻窦感染症状。它是一种具有局部破坏性和血管侵袭性的肿瘤。ENKTCL的治疗取决于肿瘤的范围。对于局限性疾病,治疗方法是放化疗。对于播散性疾病,治疗主要以化疗为主。本报告描述了一例41岁的西班牙裔女性病例,该患者最初出现鼻塞症状四周,随后被诊断并接受慢性鼻窦炎治疗。患者因持续性慢性鼻窦炎接受了内镜手术,根据临床和影像学表现初步诊断为变应性真菌性鼻-鼻窦炎。病理检查显示诊断为ENKTCL。患者接受了三个周期的化疗,化疗药物包括类固醇(氢化可的松)、甲氨蝶呤、异环磷酰胺、门冬酰胺酶前体和依托泊苷(SMILE方案),随后进行放疗,实现了临床和影像学缓解。经查阅文献,ENKTCL在美国非常罕见,由于症状不具特异性,诊断通常会延迟。我们报告此病例以提高对这种疾病实体的认识,并提醒临床医生在鼻塞的鉴别诊断中考虑到这种疾病。