累及胰腺并以急性胰腺炎为表现的结外鼻型NK/T细胞淋巴瘤一例报道及文献复习

Extranodal NK/T-cell lymphoma,nasal type with pancreas involvement presenting as acute pancreatitis: report of a case and literature review

目的侵及胰腺的NK/T细胞淋巴瘤极为罕见,临床表现无特异性,容易被临床医生误诊或漏诊。该文拟提高对以急性胰腺炎为表现的结外鼻型NK/T细胞淋巴瘤的临床表现及诊治要点的认识。方法报道以急性胰腺炎为表现的结外鼻型NK/T细胞淋巴瘤1例,并以"NK/T Cell"及"Pancreas"、或"NK/T细胞"及"胰腺"等为检索词在Pubmed数据库、中国期刊全文数据库(CNKI)、万方数据知识服务平台检索相关文献,收集资料完整的侵及胰腺的NK/T细胞淋巴瘤的病例,分析其临床特点、治疗方案及预后情况等。结果该例为38岁男性患者,因上腹痛及血清淀粉酶、脂肪酶升高收入我科。腹部MRI提示胰体部囊实性病变,行超声引导下胰腺穿刺提示恶性肿瘤细胞,倾向于NK/T淋巴瘤细胞。通过鼻咽部肿物的穿刺活检,最终确诊为结外鼻型NK/T细胞淋巴瘤。患者先后进行8个疗程化学治疗及2次放射治疗,胰腺病灶在6个疗程化学治疗后完全消失,鼻咽部病灶在6个疗程化学治疗后明显缩小,8个疗程化学治疗后几乎消失。检索文献后共收集国内外8例侵及胰腺的NK/T细胞淋巴瘤患者的资料,其中5例诊断为结外鼻型NK/T细胞淋巴瘤,3例原发于胰腺。共有7例有明确的随访资料,仅1例获得完全缓解,其余在诊断后8个月内死亡。结论NK/T细胞淋巴瘤尽管罕见,临床医生应意识到其可作为急性胰腺炎的发病原因之一,影像学检查可发现病灶,确诊需依靠病理和免疫组织化学检测。治疗以化学治疗为主,辅以放射治疗。

Objective Natural killer T （NK/T） -cell lymphoma with pancreas involvement is ex-tremely rare with non-specific clinical manifestations, which is likely to be misdiagnosed or missed by clini-cians. This study aimed to deepen the understanding of clinical manifestations, diagnosis and treatment of ex-tranodal NK/T-cell lymphoma with pancreas involvement presenting as acute pancreatitis. Methods We re-ported one case of extranodal NK/T-cell lymphoma with pancreas involvement presenting as acute pancreatitis. Literature review was performed using the researching keywords of “ NK/T Cell” and “ Pancreas” in both Eng-lish and Chinese from PubMed, China national knowledge infrastructure （ CNKI） and Wanfang databases. Clinical presentations, therapeutic regime and clinical prognosis of patients with NK/T-cell lymphoma with pan-creas involvement were retrospectively analyzed. Results A 38- year-old male was hospitalized due to epigas-tric pain coupled with elevated serum amylase and lipase levels. Abdominal magnetic resonance imaging （MRI） demonstrated a cystic and solid lesion in the pancreas. Ultrasound-guided percutaneous fine needle as-piration （FNA） from the pancreas revealed malignant ce l ls, which were highly suggestive of lymphoma cells. The diagnosis of NK/T-cell lymphoma was finally confirmed by the biopsy of nasal cavity. He received 8 cycles of chemotherapy combined with 2 sessions of radiotherapy. The pancreatic lesions disappeared after 6 cycles of chemotherapy. The mass in the nasopharynx was shrank and almost disappeared after the 8 cycles of chemother-apy. Clinical data of 8 patients diagnosed with NK/T-cell lymphoma with pancreas involvement were retrieved.Five cases were diagnosed with extranodal NK/T-cell lymphoma with nasal type and the other 3 patients had primary involvement of the pancreas. Seven patients had explicit follow-up data. Only one case received com-plete remission and the remaining patients died within 8 months after diagnosis. Conclusions NK/T-cell lym-phoma with pancreas involvement is extremely rare. Clinicians should emphasize the possibility of NK/T-cell lymphoma as one of the causes of acute pancreatitis. Lesions can be identified by imaging examination. The di-agnosis depends on pathological examination and immunohistochemistry. Chemotherapy is the main treatment assisted by radiotherapy.