小儿胰腺恶性肿瘤11例诊治分析

目的探讨小儿胰腺恶性肿瘤的临床特征和诊治方法。方法选取1992年7月-2004年5月收治的11例小儿胰腺恶性肿瘤。男5例,女6例;年龄3～14岁。7例患儿表现为腹痛,5例表现为腹部包块;其他症状包括黄疸3例,呕吐、厌食和发热各2例。11例患儿中,5例行胰十二指肠切除术,3例行脾及胰体尾切除术,2例行肿瘤局部切除术,1例仅行病理活检术,并对3例患儿分别给予化疗或放疗。结果11例患儿病灶术前至少一项影像学检查定位于胰腺,3例术前B超误诊为后腹膜肿块;术中探查可见5例患儿肿瘤位于胰体部,3位于胰头体部,2例位于胰尾部,1例位于胰头部;瘤体直径4～12cm,平均长径约6.5cm;肿瘤主要侵犯十二指肠和脾脏,均无远处转移。肿瘤类型包括囊实性乳头状肿瘤4例,胰母细胞瘤3例,无功能胰腺内分泌肿瘤2例,腺泡细胞癌及不典型胰腺类癌瘤各1例。术后随访2个月～9a,4例死亡,7例健在,其中1例复发后行胰头部和十二指肠切除处理。结论儿童胰腺恶性肿瘤少见,类型多样,完整切除后能长期生存,预后较成人好,但存在复发的可能,同时化疗和放疗的效果还不确定。     

儿童胰母细胞瘤诊治分析

目的 儿童胰母细胞瘤是一种罕见的小儿恶性肿瘤,本文拟通过分析本院近年来收治的儿童胰母细胞瘤病例,探讨其诊断与治疗要点.方法 回顾性分析本院2002～2006年收治的3例儿童胰母细胞瘤患儿临床资料.结果 3例中,除1例因瘤体巨大出现腹痛、呕吐等非特异性症状,就诊发现包块外,其余两例均因偶然原因体检发现腹部包块.人院体查均可扪及左腹部包块,质硬,不活动.CT检查均提示为左腹膜后巨大实性占位性病变,血清AFP值均显著增高.术中发现,3例患儿肿瘤均位于胰尾部,边界清楚,包膜完整,手术完整切除肿瘤.术后病理检查证实为胰母细胞瘤.本组均手术完整切除肿瘤且未查见远处转移,3例患儿术后均未行化疗.所有患儿均生存,随访期间无复发,复查AFP均无异常升高.结论 儿童胰母细胞瘤多无特异性症状、体征及影像学表现,早期发现和诊断非常困难.血清AFP值可作为瘤标用于诊断与随访.儿童胰母细胞瘤往往呈膨胀性生长,有完整包膜,预后相对较好,应积极治疗.在肿瘤发生远处转移之前,早期诊断并手术切除肿瘤是治愈该病的唯一途径.若能一期彻底切除肿瘤,术后可无需化疗.     

保留十二指肠胰头肿块切除术在儿童胰头肿瘤治疗中的应用

目的 通过对比保留十二指肠的胰头肿块切除术与胰十二指肠切除术治疗儿童胰头肿瘤的术后并发症及治疗效果,探讨保留十二指肠胰头肿块切除术在儿童患者中的应用价值.方法 回顾性地分析了复旦大学附属儿科医院从2007年到2015年,收治并行保留十二指肠的胰头肿块切除术与胰十二指肠切除手术治疗的胰头部肿瘤患儿的临床资料.我们定义保留十二指肠肿块切除术为:①肿块剜除术;②保留十二指肠的胰头切除术(duodenum-preserving pancreatic head resection,DPPHR).胰十二指肠切除术均为保留幽门胰十二指肠切除术(pylorus-preserving pancreaticoduode-nectomy,PPPD).结果 共收集11例患儿临床资料,平均年龄(8.4±3.7)岁.其中7例行保留十二指肠手术,术后病理证实4例为实性假乳头状瘤;3例为胰母细胞瘤;4例行PPPD,3例病理结果为实性-假乳头状瘤;1例为炎性肌纤维母细胞瘤.保留十二指肠组患儿平均肿瘤最长径为:(9.4±4.5)cm,PPPD组最长径为(5.9±2.1)cm,两组肿瘤长径无统计学差异(P=0.17).保留十二指肠组手术平均时间为(6.0±3.5)h,而PPPD组平均手术时间为(7.5±1.3)h,两组手术时间差异无统计学意义(P=0.44).根据国际胰漏小组的定义,4例行保留十二指肠手术的患儿出现A级胰瘘,pp-PD组患儿均无术后胰瘘.根据国际胰腺外科研究小组的定义,11例患儿均未出现临床相关胃排空延迟(B级或C级).除1例炎性肌纤维母细胞瘤失访外,余10例患儿术后经B型超声、CT等影像学检查随访时间为4个月到32个月,均未出现肿瘤复发.临床症状随访时间为6个月到4年,10例患儿均存活,且未诉与疾病相关不适.结论 针对儿童胰头肿瘤,根据术中情况,在肿瘤未侵犯十二指肠且能保证胆道及十二指肠血供的前提下,采取保留十二指肠的胰头肿块切除术,能减少手术创伤,保留胆道及胃肠道的完整性.     

儿童胰腺肿瘤精准手术切除策略探讨(附15例报告)

目的 探讨影响小儿胰腺肿瘤手术切除方式的因素及精准手术切除的可行性.方法 回顾性分析2002年10月至2013年7月收治的儿童胰腺肿瘤15例的临床资料.15例中,女10例,男5例;年龄4个月～14岁,平均8岁4个月.肿瘤位于胰头部9例,胰体尾部6例,伴肝转移1例,合并肝、肠系膜病变1例.结果 本组均接受手术治疗.肿瘤位于胰腺头部9例行保留幽门、胰十二指肠切除术7例,行肿瘤切除术2例,此2例肿瘤均来源于胰头钩突部;肿瘤位于胰腺体尾部6例,均行胰体/尾+脾切除术.病理检查报告:胰腺囊实性假乳头状瘤9例,胰腺母细胞瘤3例,浆液性囊腺瘤1例,胰岛细胞癌1例,促纤维增生性小圆细胞瘤1例.15例患儿术后恢复顺利,随访6个月～12年,促纤维增生性小圆细胞瘤1例患儿死亡,余者均存活.结论 手术切除肿瘤是治疗儿童胰腺肿瘤得重要手段,多数患儿仅通过手术切除肿瘤可长期存活.手术原则是尽可能保持胃肠道连续性,精准手术,尽可能多的保留正常的胰腺组织.影响决定小儿胰腺肿瘤术式的主要因素是肿瘤部位、周围重要组织是否受累肿瘤是否累及胆管、主胰管及脾血管.CT、MRCP属非侵袭性检查方法,可多平面分析,且无需注射造影剂,可对胆管及胰管状况肿瘤进行术前评估,为胰腺肿瘤确定精准手术方案提供依据.     

儿童囊性肾瘤和囊性部分分化型肾母细胞瘤的诊治分析

目的总结儿童囊性肾瘤和囊性部分分化型肾母细胞瘤的临床、病理特点,探讨合理的治疗方法。方法回顾性分析本院收治的7例儿童囊性肾瘤、6例囊性部分分化型肾母细胞瘤患儿的临床资料,包括年龄、临床表现、影像学及病理检查结果、治疗和预后。结果13例患儿中,男8例,女5例,年龄4个月至4岁,平均1岁7个月。左侧6例,右侧5例,双侧2例。腹部包块9例,B超偶然发现4例,术前均行B超和增强CT检查。5例7侧行肿瘤剜除术,8例行瘤肾切除术。术后随访6个月至7年,未见肿瘤复发,保留肾脏的5例中,7侧残肾功能良好。结论囊性。肾瘤和囊性部分分化型肾母细胞瘤患儿术前无法鉴别,手术完整切除是主要的治疗方法,肿瘤位于肾脏一极或双侧者可行保留肾脏的肿瘤剜除术。囊性肾瘤为良性病变,术后无需化疗,囊性部分分化型。肾母细胞瘤为低度恶性或潜在恶性,Ⅰ期者可单纯手术治疗,Ⅱ期以上需行手术＋化疗。     

儿童肾上腺皮质癌5例并文献复习

目的总结儿童肾上腺皮质癌的临床特点、诊治措施及预后,以提高对儿童肾上腺皮质癌的诊治水平。方法回顾性分析上海市儿童医院2010年1月至2018年7月收治的5例儿童肾上腺皮质癌患儿的临床资料并随访。5例均为女童,年龄7个月~4岁。肿瘤左侧3例,右侧2例;肿瘤直径4.5~8.5 cm。结果5例患儿术后病理确诊为肾上腺皮质癌,本组患儿均为有内分泌功能的肿瘤;达到完整肉眼切除瘤体4例,与周围组织、血管黏连重,有肿瘤包膜破溃1例;本组患儿术后均安全渡过围术期,无手术死亡病例。5例患儿均未接受术前化疗;1例术后行米托坦治疗,3例仅观察随访,1例患儿术后失访。结论肾上腺皮质癌是一种罕见的且预后较差的恶性肿瘤。完整的肿瘤切除可提高患者无病生存率。     

原发性卵巢肿瘤125例临床病理分析

目的探讨儿童原发性卵巢肿瘤的临床病理特点及其治疗方法和预后。方法回顾性分析1986年8月～2006年12月收治儿童卵巢肿瘤125例的临床表现、腹腔镜、B超及磁共振检查结果,病理资料,血清肿瘤标志物,临床分期,治疗及预后。结果主要症状为腹部肿块（59.2%）,腹胀（50.4%）,腹痛（41.6%）。125例中良性肿瘤52例占41.6%,恶性61例占48.8%,交界性12例占9.6%。良性肿瘤均手术切除,术后预后好,仅1例复发,恶性肿瘤61例采用手术切除＋术后放化疗,术后复发32例,复发病例治疗成功8例,余死亡。结论女童腹部肿块和腹痛应排除卵巢肿瘤可能;良性肿瘤治疗应切除肿瘤,恶性肿瘤可采取手术切除＋术后放化疗。     

儿童胰腺实性-假乳头状瘤的临床病理分析

目的探讨儿童发生的胰腺实性-假乳头状肿瘤的临床、病理特点及鉴别诊断。方法对我院2005年-2016年确诊的21例临床资料及病理特点进行回顾性分析。结果男5例,女16例,发病年龄平均10.3(5～15.5)岁,临床表现:上腹痛12例(5例伴呕吐,1例伴皮肤巩膜黄染、大便色浅及尿色深;2例因急腹症入院),发现腹部包块9例。影像学(超声=21,CT=20,MRI=9)均提示胰腺囊和(或)实性占位。实验室检查:除3例淀粉酶和白细胞升高外,余未见异常。21例均行胰腺肿瘤切除术(其中3例同时行Whipple手术),1例先行胰腺囊肿内引流术后,再择期行肿瘤切除。肿瘤部位:胰头10例,胰体3例,胰体尾4例,胰尾4例。全部患者经术后病理确诊后,仅密切随访未再行化疗。随访结果:仅1例术后11个月复发,再次手术切除;所有患儿仍在随访中。结论胰腺实性-假乳头状肿瘤属较罕见的低度恶性胰腺外分泌肿瘤。临床表现及影像学检查均无特征性。病理组织学见上皮样细胞形成的实性或假乳头状结构。若瘤体较大,且有明确的包膜、血管及周围神经的浸润,异型性明显及核分裂活性增加,以及肿瘤坏死明显等,则提示恶性的可能。肿瘤的完整切除既是主要的治疗手段,也是预后相关的重要因素。     

保留脾脏的胰体尾部肿瘤切除术治疗小儿胰腺实性假乳头状瘤

目的探讨保留脾脏的胰体尾肿瘤切除术治疗小儿胰腺实性假乳头状瘤的可行性及临床疗效评估。方法回顾性分析2009年1月-2012年12月在天津市儿童医院收治的位于胰体尾部胰腺实性假乳头状瘤患儿4例,均成功行保留脾脏的胰体尾肿瘤切除术。术后均在门诊随访,内容包括腹部B超和CT检查,肿瘤标记物和胰腺外分泌功能,并由专人测量其生长发育情况。结果患儿手术过程顺利,切除肿瘤及部分胰体尾,全部成功保留脾脏,肿瘤直径介于4．0～12．5em,包膜完整,手术过程中无脾脏动、静脉损伤及脾实质撕裂伤,无术中输血。术后3d肠功能恢复后开始进食,恢复过程顺利,住院时间为6～9d。无围手术期出血、胰瘘等并发症发生。病理检查结果均为胰腺实性假乳头状瘤,Ki-67〈5％。所有患儿获得随访,时间为6～48个月,均健康存活,生长发育正常。腹部B超、CT检查未见肿瘤复发,肿瘤标记物及胰腺外分泌功能检查未见异常。结论发生在胰体尾部的胰腺实性假乳头状瘤虽然肿瘤直径较大,但是包膜完整,与胰腺血管关系疏松,易于将脾脏动静脉分开而成功保留脾脏。     

婴儿腹腔实体肿瘤临床病理与预后分析

目的探讨婴儿腹腔实体肿瘤的临床病理与预后的关系。方法回顾上海儿童医学中心1998年4月-2007年2月及上海新华医院2001年1月-2007年2月住院的52例1d～1岁腹部肿瘤患儿临床资料。患儿病史资料通过病史采集方式获得。病理类型通过复查病理切片和病理报告核对。随访采用电话和信访方式及患儿来院复诊记录。随访时间5个月～8a。结果52例中年龄1d～1个月7例,≥1个月～1岁45例。其中畸胎瘤23例(44.23%),神经母细胞瘤9例(17.31%),肾母细胞瘤6例(11.54%),肝母细胞瘤5例(9.62%),肝血管内皮瘤、先天性中胚层肾瘤各3例(5.77%),胰头梭形细胞血管内皮瘤、肝错构瘤、后腹膜小细胞恶性肿瘤各1例(1.92%)。良性肿瘤:恶性肿瘤=1:1,良性肿瘤中男:女=1:1,恶性肿瘤中男:女=2.33:1.0。患儿均手术切除肿瘤,恶性肿瘤辅以化疗。良性肿瘤均无复发;恶性肿瘤中复发或转移2例,其中1例死亡。失访4例。结论婴儿腹腔实体肿瘤以畸胎瘤和神经母细胞瘤居多。良性肿瘤数量与恶性肿瘤相当。良性发病无性别差异,手术切除后预后良好。恶性肿瘤中,男性比例显著高于女性,完整手术切除配合术后化疗,复发少,病死率低。早期诊断和治疗对其预后至关重要。     

小儿胰腺实体囊性乳头状瘤三例报告

目的 提高对小儿胰腺实体囊性乳头状瘤的认识。方法 介绍3例小儿胰腺实体囊性乳头状瘤的诊治经验并复习有关本病的文献报道，加以总结。结果 3例患儿术前经B超及CT检查，术中探查及术后病理检查证实为胰腺实体囊性乳头状瘤。并通过手术治疗，取得了良好的效果。通过复习文献加深了对本病的认识，对提高本病的正确诊断有帮助。结论 小儿胰腺实体囊性乳头状瘤很少见，是低度恶性肿瘤。多见于女性。腹部肿物是首要症状。B超及CT检查可帮助诊断。完全切除肿瘤是本病唯一的治疗选择，可取得满意的效果。     

Choledochal cysts: lessons from a 20 year experience

Cystic dilatation of the biliary tree is a rare congenital anomaly. To determine mode of presentation, diagnostic pitfalls, and long term outcome after surgery, 78 children (57 girls, 21 boys) with choledochal cyst treated between 1974 and 1994 were reviewed. Anatomical types were: Ic (n = 44), If (n = 28), IVa (n = 4), and V (n = 2); a common pancreaticobiliary channel was identified in 76% patients. Age at presentation ranged from 0-16 (median 2.2) years, six patients being diagnosed by prenatal ultrasonography. Of the 72 patients diagnosed postnatally, 50 (69%) presented with jaundice, associated with abdominal pain in 25 or a palpable mass in three, 13 (18%) presented with pain alone, and two (3%) with a palpable mass. The classic triad of jaundice, pain, and a right hypochondrial mass was present in only four (6%). Four children presented acutely after spontaneous perforation of a choledochal cyst, two presented with ascites and one cyst was discovered incidentally. Plasma and/or biliary amylase values were raised in 30 of 31 patients investigated for abdominal pain; seven had evidence of pancreatitis at operation. In 35 of 67 (52%) patients referred without previous surgery, symptoms had been present for more than one month, and in 14 of them for more than one year, before diagnosis. Delayed referral was due to misdiagnosis as hepatitis (n = 12), incomplete investigation of abdominal pain (n = 6), and failure to note the significance of ultrasonographic findings (n = 10). Two patients referred late died from liver failure. Of the 76 patients with type I or IV cysts, 59 underwent radical cyst excision and hepaticojejunostomy as a primary procedure and 10 as a secondary operation after previously unsuccessful surgery. Sixteen patients have been lost to follow up but most of the remainder are well after a mean period of 4.1 (0.1-13) years. Choledochal cysts are often misdiagnosed, but prognosis is excellent if radical excision is performed.     

Practical concerns about the diagnosis of Munchausen syndrome by proxy.

Cystic dilatation of the biliary tree is a rare congenital anomaly. To determine mode of presentation, diagnostic pitfalls, and long term outcome after surgery, 78 children (57 girls, 21 boys) with choledochal cyst treated between 1974 and 1994 were reviewed. Anatomical types were: Ic (n = 44), If (n = 28), IVa (n = 4), and V (n = 2); a common pancreaticobiliary channel was identified in 76% patients. Age at presentation ranged from 0-16 (median 2.2) years, six patients being diagnosed by prenatal ultrasonography. Of the 72 patients diagnosed postnatally, 50 (69%) presented with jaundice, associated with abdominal pain in 25 or a palpable mass in three, 13 (18%) presented with pain alone, and two (3%) with a palpable mass. The classic triad of jaundice, pain, and a right hypochondrial mass was present in only four (6%). Four children presented acutely after spontaneous perforation of a choledochal cyst, two presented with ascites and one cyst was discovered incidentally. Plasma and/or biliary amylase values were raised in 30 of 31 patients investigated for abdominal pain; seven had evidence of pancreatitis at operation. In 35 of 67 (52%) patients referred without previous surgery, symptoms had been present for more than one month, and in 14 of them for more than one year, before diagnosis. Delayed referral was due to misdiagnosis as hepatitis (n = 12), incomplete investigation of abdominal pain (n = 6), and failure to note the significance of ultrasonographic findings (n = 10). Two patients referred late died from liver failure. Of the 76 patients with type I or IV cysts, 59 underwent radical cyst excision and hepaticojejunostomy as a primary procedure and 10 as a secondary operation after previously unsuccessful surgery. Sixteen patients have been lost to follow up but most of the remainder are well after a mean period of 4.1 (0.1-13) years. Choledochal cysts are often misdiagnosed, but prognosis is excellent if radical excision is performed.     

Wilms’ Tumor: A 24-year Retrospective Study from a Single Center

Medical records of 71 children with Wilms’ tumor at Sisli Etfal Education and Research Hospital between 1990 and 2014 were reviewed. Mean age at diagnosis was 3.11 years (2 days–7 years). Male to female ratio was M/F = 6/10. The incidence of associated anomaly was 16.9%. Clinical manifestations included abdominal mass (89%), hematuria (30%), hypertansion (25%), abdominal pain (15%), fever (5%), restlessness (2%), weight loss (2%), varicocele (1%). Ultrasound (USG) was the most often initial study in a child presenting with abdominal mass. Doppler USG was also made to evaluate the inferior vena cava (IVC) for the presence of tumor extension in children with renal mass. The left kidney was affected in 33 patients (46.5%), the right was affected in 31 patients (43.7%). Two patients was extrarenal (2.8%). And 5 patients (7.04%) were bilateral on the presentation. Preoperative chemotheraphy was done in 14 cases. In 63 patients with unilateral Wilm tm, unilateral radical nefrectomy is performed. In one patient with solitary kidney, nephron sparing surgery (NSS) is performed. In 3 patients with bilateral tm NSS is performed and in 2 patients with bilateral Wilms’ tm NSS is performed in one side and nefrectomy on the other side. Out of 71 Wilms tumor (WT) patients, 17 of them has been out of our follow. And 4 of them are died. Ten of them has metastases. Forty children are under follow with no metastases. Patients with WT needs a multimodal, multidisiplinary treatment with the cooperation of pediatric oncologist and pediatric surgeon and needs close follow-up.     

儿童双侧肾母细胞瘤手术治疗的探讨

目的 总结探讨儿童双侧肾母细胞瘤综合治疗的疗效及随访结果。方法 回顾性分析我院白1998年8月至2010年8月手术治疗的双侧肾母细胞瘤患儿临床资料。结果 7例双侧肾母细胞瘤中,男性患儿3例,女性患儿4例,发病年龄最大21个月,最小6个月,平均年龄( 12.71±4.89)个月。临床表现腹部肿块7例(7/7),血尿1例(1/6),贫血2例(2/6),4(4/7)例活检明确病理经术前化疗后手术,3例先手术后化疗,1例化疗2个疗程疾病进展,家属放弃治疗后死亡,5例结束整个疗程随访中,4例带瘤生存,1例随访7个月后残余肿瘤病灶消失,随访时间最长10年,最短10个月,1例术后化疗中,2年存活率85.7％。结论 儿童双侧肾母细胞瘤通过手术、化疗及放疗综合治疗,可以带瘤生存。     

胎儿腹腔囊性占位的生后治疗及转归

目的 分析产前诊断胎儿腹腔囊性占位的病因、随访、预后及转归,指导产前会诊咨询工作.方法 收集2006年4月至2009年4月产前发现胎儿腹腔囊性占位的病例32例,分析病因、就诊年龄、占位大小、手术治疗效果及随访情况,总结预后与转归.结果 32例患儿,男女比例为7:25,女性占绝对多数.新生儿期就诊23例,其余生后3个月内就诊.就诊时囊性占位较产前明显增大者7例(21.9%).病因包括卵巢囊肿、畸胎瘤、肠源性囊肿、肾上腺来源性占位及胆总管囊肿.27例经Ⅰ期手术预后良好.2例经Ⅱ期手术恢复良好.2例未行手术预后良好.1例因恶性肿瘤死亡.结论 胎儿腹腔囊性占位以女性多见,以卵巢囊肿最多见.肠重复畸形、囊性畸胎瘤亦是常见病因.多数患儿因产前发现肿块在新生儿早期就诊.巨大腹腔囊性肿块造成消化道梗阻症状是早期就诊的主要原因.腹腔囊性占位的胎儿多数预后良好,转归满意.

Abstract：

Objective To summarize our experience with prenatal diagnosis of the fetus with cystic abdominal mass, and retrospectively analyze their clinical outcome after birth. Methods From April 2006 to April 2009, 32 fetuses were diagnosed with cystic abdominal mass in prenatal tests.Their clinical data including etiology, age at diagnosis, mass size, surgical treatment and follow-up were retrospectively analyzed. Results The 32 cases included 7 males and 25 females. Twenty three (71.9%) patients visited doctors in the neonatal period. The other patients (71.9%) visited doctors in the first 3 months after birth. The chief compliant was intestinal obstruction caused by abdominal mass. Seven patients' (21.9%) cystic abdominal masses enlarged after birth. The origins of the cystic abdominal masses were ovarian cysts, teratorna, enterogenous cysts, adrenal tumors and common bile duct cysts. Among the 32 patients, 27 patients underwent one stage tumor resection, and 2 were performed 2 stage surgery. Two patients didn't receive any surgery. And 1 patient with malignant tumor died. All patients recovered eventually from surgery. Conclusions Cystic abdominal mass in female fetus is more common than that in male fetus. The most common origin of cystic abdominal mass in female fetus is ovarian cysts. Intestinal duplication and cystic teratoma is also the common origins. Most neonates visit doctors for prenatal diagnosis of cystic abdominal mass. The prognosis of the majority of fetus with abdominal cyst is good.     

小儿先天性中胚叶肾瘤

目的：探讨小儿先天性中胚叶肾瘤（ＣＭＮ）的临床特点、诊断及治疗。方法：近２０年间收治ＣＭＮ７例，男４例，女３例，年龄７个月～１０岁。术前均可扪及一腹部肿块，其中１例伴肉眼血尿，病变位于左侧４例，右侧３例。全部病例均接受了手术治疗，２例证实有肾门旁肿瘤转移，病理检查属平滑肌瘤型５例，细胞型２例，因术前及术中疑诊为Ｗｉｌｍ＇ｓ瘤而接受了不同疗程化疗。结果：除１例２个月龄患儿术后死于窒息外，余６例术后均随访，１例术中肿瘤破裂者术后８个月复发，化疗无效后再次手术已无瘤生存９年，余５例无瘤生存至今，最短５年，最长已１５年。结论：ＣＭＮ是一罕见的肾肿瘤，虽好发于新生儿及婴儿早期，但也可见于年长儿，其临床表现和病理改变虽有诸多特点，但有时仍难与Ｗｉｌｍ＇ｓ瘤区别，手术切除是治疗本病最有效的方法     

Patterns of abdominal relapse and role of sonography in Wilms tumor

This study characterizes the patterns of abdominal recurrence of Wilms tumor and describes the role of sonography in its detection. Twelve patients who had initial tumor recurrence in the abdomen were evaluated. Five patients had recurrence in the kidney; all had nephrogenic rests detected by computed tomography (CT) or magnetic resonance (MR) imaging but not by sonography. The remaining 7 patients had recurrence in the peritoneum (4), the nephrectomy site (2), or the regional lymph nodes (1); tumor spillage had occurred in five of these patients. Four recurrences were detected during therapy, and eight within 3 years after completion of therapy. Seven of the 12 recurrences were first detected by sonography. All 11 sonograms obtained at the time of relapse showed tumor recurrence. Nine patients died a median of 10 months after relapse. The results suggest that regular sonographic surveillance for 3 years after therapy is likely to reveal most abdominal recurrences. Supplementation with CT or MR imaging is indicated for detection of nephrogenic rests.     

PATTERNS OF ABDOMINAL RELAPSE AND ROLE OF SONOGRAPHY IN WILMS TUMOR

This study characterizes the patterns of abdominal recurrence of Wilms tumor and describes the role of sonography in its detection. Twelve patients who had initial tumor recurrence in the abdomen were evaluated. Five patients had recurrence in the kidney; all had nephrogenic rests detected by computed tomography (CT) or magnetic resonance (MR) imaging but not by sonography. The remaining 7 patients had recurrence in the peritoneum (4), the nephrectomy site (2), or the regional lymph nodes (1); tumor spillage had occurred in five of these patients. Four recurrences were detected during therapy, and eight within 3 years after completion of therapy. Seven of the 12 recurrences were first detected by sonography. All 11 sonograms obtained at the time of relapse showed tumor recurrence. Nine patients died a median of 10 months after relapse. The results suggest that regular sonographic surveillance for 3 years after therapy is likely to reveal most abdominal recurrences. Supplementation with CT or MR imaging is indicated for detection of nephrogenic rests.     

Diagnosis of ovarian follicular cysts from birth to puberty: a report of twenty cases

Twenty girls aged 1 day to 17 years have been studied for ovarian follicular cysts. Clinical features leading to the discovery of the follicular cyst were different in prepubertal girls and in girls whose cyst was discovered during puberty. Before seven years of age, four girls presented a precocious pseudopuberty where breast development was in contrast with very low pituitary gonadotropin levels; two girls in this age group were diagnosed after complaining about abdominal pain. In two cases the diagnosis was prenatal during routine ultrasonography of the mother. After ten years of age, abnormal menses (5 cases) or acute abdominal pain (5 cases) were the main clinical features. In only one case the cyst presented as an abdominal mass. Follow-up of the 20 patients showed: spontaneous disappearance of the cyst within 3 to 32 weeks in 9 cases; ovariectomy in 8 cases, due to a torsion of a large cyst (over 55 mm) in 7 children and because of the fear of a possible tumor in one; limited resection of the cyst in 4 cases. By systematic ultrasonography, discovery of an ovarian cyst as defined by a non-echogenic area over 20 mm may occur relatively often in young girls. Spontaneous disappearance is frequent when the cyst is small (under 55 mm). Torsion of large cysts remains the major complication.