PDF(Pubmed)
Abstract：
BACKGROUND: Extranodal natural killer/T-cell lymphoma, nasal type (ENKL) of the small intestine, is a disease with extremely poor prognosis. We describe treatment in a case which is novel in that it demonstrated long-term survival.
METHODS: A 68-year-old man was admitted to the emergency department of our hospital with the complaint of severe umbilical pain with tenderness and muscular defense. An abdominal computed tomography scan revealed a thick-wall mass on the small intestine and intra-abdominal free air. He was suspected of perforation of a small intestinal tumor and underwent emergency surgery. The surgery revealed a perforated tumor ulcer, and ENKL was diagnosed from the postoperative pathological findings. The patient\'s postoperative course was uneventful. He was further treated with adjuvant chemotherapy by hematologist comprising six courses of dexamethasone, etoposide, ifosfamide, and carboplatin. The patient demonstrated long-term survival and was in remission at the time of writing, four years and five months after surgery.
CONCLUSIONS: We report a rare case of long-term survival of perforated ENKL of the small intestine achieved by surgery and adjuvant chemotherapy with dexamethasone, etoposide, ifosfamide, and carboplatin. It is essential to consult with a hematologist to determine the most appropriate chemotherapy such as DeVIC if one encounters rare postoperative pathological findings of ENKL. To elucidate the pathophysiology of this disease and to prolong survival of affected patients, accumulation of cases of long-term survival and examination of associated characteristics is necessary.
METHODS: A 68-year-old man was admitted to the emergency department of our hospital with the complaint of severe umbilical pain with tenderness and muscular defense. An abdominal computed tomography scan revealed a thick-wall mass on the small intestine and intra-abdominal free air. He was suspected of perforation of a small intestinal tumor and underwent emergency surgery. The surgery revealed a perforated tumor ulcer, and ENKL was diagnosed from the postoperative pathological findings. The patient\'s postoperative course was uneventful. He was further treated with adjuvant chemotherapy by hematologist comprising six courses of dexamethasone, etoposide, ifosfamide, and carboplatin. The patient demonstrated long-term survival and was in remission at the time of writing, four years and five months after surgery.
CONCLUSIONS: We report a rare case of long-term survival of perforated ENKL of the small intestine achieved by surgery and adjuvant chemotherapy with dexamethasone, etoposide, ifosfamide, and carboplatin. It is essential to consult with a hematologist to determine the most appropriate chemotherapy such as DeVIC if one encounters rare postoperative pathological findings of ENKL. To elucidate the pathophysiology of this disease and to prolong survival of affected patients, accumulation of cases of long-term survival and examination of associated characteristics is necessary.
摘要：
背景：结外自然杀伤/T细胞淋巴瘤，小肠的鼻型（ENKL），是一种预后极差的疾病。我们描述了一种新颖的病例的治疗方法，因为它证明了长期生存。
方法：一名68岁的男子因患有压痛和肌肉防御的严重脐痛而入院。腹部计算机断层扫描扫描显示小肠上有厚壁肿块和腹内自由空气。他被怀疑患有小肠肿瘤穿孔，并接受了紧急手术。手术显示肿瘤溃疡穿孔,根据术后病理结果诊断为ENKL。患者术后病程顺利。他接受了血液学家的辅助化疗，包括六个疗程的地塞米松，依托泊苷,异环磷酰胺,和卡铂。患者表现出长期生存，在撰写本文时处于缓解状态，手术后4年5个月.
结论：我们报告了一例罕见的小肠穿孔ENKL长期存活的病例，通过手术和地塞米松辅助化疗，依托泊苷,异环磷酰胺,和卡铂。如果遇到罕见的ENKL术后病理发现，必须咨询血液学家以确定最合适的化学疗法，例如DeVIC。为了阐明这种疾病的病理生理学并延长患病患者的生存期，长期生存病例的积累和相关特征的检查是必要的。
方法：一名68岁的男子因患有压痛和肌肉防御的严重脐痛而入院。腹部计算机断层扫描扫描显示小肠上有厚壁肿块和腹内自由空气。他被怀疑患有小肠肿瘤穿孔，并接受了紧急手术。手术显示肿瘤溃疡穿孔,根据术后病理结果诊断为ENKL。患者术后病程顺利。他接受了血液学家的辅助化疗，包括六个疗程的地塞米松，依托泊苷,异环磷酰胺,和卡铂。患者表现出长期生存，在撰写本文时处于缓解状态，手术后4年5个月.
结论：我们报告了一例罕见的小肠穿孔ENKL长期存活的病例，通过手术和地塞米松辅助化疗，依托泊苷,异环磷酰胺,和卡铂。如果遇到罕见的ENKL术后病理发现，必须咨询血液学家以确定最合适的化学疗法，例如DeVIC。为了阐明这种疾病的病理生理学并延长患病患者的生存期，长期生存病例的积累和相关特征的检查是必要的。
