Sanders B M, West K W, Gingalewski C, Engum S, Davis M, Grosfeld J L
Section of Pediatric Surgery and Pediatric Pathology, Indiana University School of Medicine and JW Riley Hospital for Children, Indianapolis, IN, USA.
J Pediatr Surg. 2001 Jan;36(1):169-73. doi: 10.1053/jpsu.2001.20045.
BACKGROUND/PURPOSE: Initially described in 1937, inflammatory pseudotumor (IPT) inflammatory myofibroblastic tumor (IMT) or plasma cell granulomas are synonymous for an inflammatory solid tumor that contains spindle cells, myofibroblasts, plasma cells, and histocytes. Common sites of presentation include lung, mesentary, liver, and spleen; intestinal presentations are rare, and the etiology remains obscure. This report details the clinical and surgical experiences in 4 children with alimentary tract IPT at a single institution.
A retrospective chart review was conducted of pediatric patients with the pathologic diagnosis of IPT.
Between 1990 and 1999, 4 patients (4 girls, ages 5 to 15 years) were identified with gastrointestinal tract origins of IPT. Symptoms at presentation included anemia (n = 4), intermittent abdominal pain (n = 3), fever (n = 3), weight loss (n = 2), diarrhea (n = 2), dysphagia (n = 1). Two patients had comorbid conditions of juvenile rheumatoid arthritis and mature B cell lymphoma. Three of 4 patients had elevated sedimentation rates. The sites of origin were the gastroesophageal junction, the colon, the rectum, and the appendix, with the referral diagnosis achalasia, perforated appendix, inflammatory bowel disease, and recurrent lymphoma, respectively. All were treated with aggressive surgical resection, and 3 girls have had no recurrences since the initial surgery. One patient had 3 recurrences within 8 months of presentation; she remains disease free 8 years later.
IPT, although rare in the gastrointestinal tract, mimics more common problems. Successful surgical management is possible even in cases of multiple recurrences.
背景/目的：炎性假瘤（IPT）、炎性肌成纤维细胞瘤（IMT）或浆细胞性肉芽肿最初于1937年被描述，是一种包含梭形细胞、肌成纤维细胞、浆细胞和组织细胞的炎性实体瘤的同义词。常见的发病部位包括肺、肠系膜、肝脏和脾脏；肠道发病较为罕见，病因仍不清楚。本报告详细介绍了一家机构中4例消化道IPT患儿的临床和手术经验。
对病理诊断为IPT的儿科患者进行回顾性病历审查。
1990年至1999年间，确定了4例（4名女孩，年龄5至15岁）IPT起源于胃肠道。就诊时的症状包括贫血（4例）、间歇性腹痛（3例）、发热（3例）、体重减轻（2例）、腹泻（2例）、吞咽困难（1例）。2例患者合并幼年类风湿关节炎和成熟B细胞淋巴瘤。4例患者中有3例血沉升高。起源部位分别为胃食管交界处、结肠、直肠和阑尾，转诊诊断分别为贲门失弛缓症、阑尾穿孔、炎性肠病和复发性淋巴瘤。所有患者均接受了积极的手术切除，3名女孩自初次手术后未复发。1例患者在就诊后8个月内复发3次；8年后她仍无疾病。
IPT虽然在胃肠道中罕见，但会模仿更常见的问题。即使在多次复发的情况下，成功的手术治疗也是可能的。
