儿童原发性胸部巨大肿瘤的外科处理

目的 总结儿童原发性胸部巨大肿瘤的治疗经验,探讨更有效的外科治疗方式.方法 对于占据整个胸腔的胸内肿瘤采取胸部正中切口,处理肺门或分离肿瘤;而其他肺肿瘤及后纵隔肿瘤则采取常规后外侧切口行肿瘤根治术.对于前纵隔肿瘤酌情采取胸部正中切口或前外侧切口,锐性加钝性法分离,直至完整切除肿瘤为止.结果 5例术前诊断为肺肿瘤的患儿,2例行左全肺切除,1例行右全肺切除,1例行右中下肺叶切除,病理学诊断均为胸膜肺母细胞瘤;另1例行胸壁肿块大块切除术,免疫组化检查结果显示为＂左胸壁肋骨Ewing肉瘤＂;1例术前诊断为胸腔内巨大肿块患儿行肿块切除后病理报告为软骨肉瘤;4例前纵隔肿瘤中,2例为梭形细胞瘤,另2例来源于胸腺,分别为胸腺瘤和恶性神经鞘瘤,肿瘤均被完整切除;4例后纵隔肿瘤均作肿块切除,病理检查报告均为神经母细胞瘤.结论 手术指征、手术径路的把握及术者的经验直接决定胸部巨大肿瘤患儿的治疗效果.     

小儿纵隔肿块的特点

１９５９～１９９１年治疗２３６例纵隔肿块中，以神经源性肿瘤占首位，其次为食管重复畸形及畸胎类瘤，前两类多数发生在后纵隔。１岁以下患儿基本上无良性肿瘤。一侧纵隔内巨大肿瘤我们采用暴露较好的双肋间切口，巨大肿瘤开胸后用大针粗线贯穿瘤体，由助手握线上提以减轻心、肺受压。１岁以下神经母细胞瘤手术后预后极佳。     

小儿胰母细胞瘤的诊断和治疗

目的：介绍小儿胰母细胞瘤的诊断治疗经验。临床资料：手术病理诊断８例。年龄１～８岁。男６例，女２例。多以腹块（６／８）、腹痛（４／８）就诊。可误诊为腹膜后神经母细胞瘤、畸胎瘤及黄疸性肝炎。５例ＡＦＰ有轻～中度升高（２０．５６～５９８．８７μｇ／Ｌ），Ｘ线平片及ＣＴ均可见不同程度钙化。肿瘤部位：胰头３例、胰体３例、胰尾２例。局部切除５例、扩大切除（Ｗｈｉｐｐｌｅ术）１例、活检２例。术后分期：Ⅰ、Ⅱ期３例，Ⅲ期２例，Ⅳ期３例。组织学特点：分界清，略分叶，肉眼见有包膜，光镜下与胚胎期８周时的胰胚基相似。治疗结果：本组６例化疗者均生存，最长者已８年。１例Ⅲ期患儿活检后化疗肿瘤消失、ＡＦＰ降至正常，无瘤生存至今已４年。１例Ⅳ期患儿化疗３年至今带瘤生存。化疗药物以长春新碱、环磷酰胺、阿霉素为主，复发者加放疗及顺铂。结论：胰母细胞瘤影像学检查有非特异性钙化。血ＡＦＰ值可作为瘤标用于诊断及随访。肿瘤对化疗敏感，难以一期切除者活检后先化疗，再酌情施行根治性手术。其预后比成人胰母细胞瘤及胰腺癌要好，应积极治疗。     

纵隔肿瘤及囊肿的诊断与治疗

目的通过总结本院收治的20例小儿纵隔肿瘤及囊肿的临床资料,探讨小儿纵隔肿瘤及囊肿的临床诊断及治疗经验。方法回顾性分析2001年1月～2006年12月20例小儿纵隔肿瘤及囊肿的临床资料。结果男12例,女8例;就诊年龄为1个月～9岁。恶性膈瘤5例,占25%,畸胎瘤6例,肠源性囊肿4例,神经源性肿瘤2例,淋巴管瘤3例,支气管囊肿2例,淋巴肉瘤1例,胸腺瘤及增生1例。完整切除肿瘤16例,大部分切除3例,取活检1例。5例恶性肿瘤,4例术后常规化疗,良性肿瘤15例,术后随访10例,生长发育正常,预后良好。结论小儿纵隔肿瘤及囊肿诊断根据临床表现及辅助检查,尽早诊断,一旦确诊应尽早手术治疗,术后合理规范的化疗,可提高患儿的治愈率。     

小儿纵隔交感神经源性肿瘤18例报告

本文报道自1964～1982年收治的18例小儿纵隔交惑神经源性肿瘤(神经母细胞瘤12例,神经节母细胞瘤4例,神经节细胞瘤2例),均位于后纵隔。呼吸道及神经压迫为常见症状,术前出现Horner征预示恶性。手术切除12例,本组神经母细胞瘤五年生存率25%。未行手术、单用化疗及Ⅲ、Ⅳ期病例均死亡。生存者均为2岁以下,Ⅰ、Ⅱ期和肿瘤全部切除的病例。强调早期手术,术后坚持化疗和放疗可提高生存率。神经母细胞瘤因具有自然消退特性,不能轻易放弃治疗。     

神经母细胞瘤的诊断和治疗(综述)

神经母细胞瘤是乳幼儿恶性肿瘤中最常见的一种,其发病率仅次于肾胚瘤,居第2位。泽田1973年报导日本每年登记100例以上,认为近来发病率有增加的倾向,比肾胚瘤更多见。渡边1972年统计,在小儿恶性肿瘤中神经母细胞瘤占10％(94/971),可发生在腹膜后、纵隔、颈部及骨盆等部位,但最常发生在腹膜     

恶性实体瘤110例

目的探讨小儿恶性实体瘤的临床特点、治疗措施和预后。方法收集本院住院治疗的小儿恶性实体瘤110例。病例均经病理证实。男64例,女46例。患儿常规进行B超检查、病理检查、增强CT扫描、螺旋CT、MRI,根据情况进行免疫组织化学检查及全身骨扫描等检查,以指导治疗及预后。结果 110例中肾母细胞瘤26例,恶性淋巴瘤26例(非霍奇金淋巴瘤20例,霍奇金淋巴瘤6例),肝母细胞瘤17例,生殖细胞瘤、横纹肌肉瘤各10例,神经母细胞瘤8例,视网膜母细胞瘤6例,畸胎瘤3例,髓母细胞瘤、骨肉瘤各2例,本组总体治愈率为58.18%。结论采取手术、化疗、放疗等综合治疗小儿恶性实体瘤的生存率有很大提高,但由于小儿恶性肿瘤发病比较隐匿,确诊一般都到晚期,小儿实体肿瘤仍是儿童死亡的主要原因之一。     

小儿腹部常见肿瘤的CT诊断（附16例病例分析）

目的 探讨小儿腹部常见肿瘤的CT表现。方法 回顾性分析16例小儿腹部肿瘤的CT表现。结果 肾母细胞瘤、畸胎瘤、神经母细胞瘤为小儿腹部常见肿瘤，各肿瘤CT表现既有相似之处又有各自特点。结论 CT是诊断小儿腹部肿瘤的重要方法。     

胸腺瘤(文献综述)

胸腺瘤(Thymoma)是位于前纵隔的肿瘤。男女发病率相近。任何年龄均可发病,但21～40岁间发病率较高,小儿时期发病却低。国内资料统计胸腺瘤占纵隔肿瘤的20.7%,小儿仅为2.9%。一、病理胸腺是个双叶器官,主要发源于第三对腮囊的内胚层组织,第四对腮囊也参与其组成。     

肿瘤

932410 3 081例儿童肿瘤活检资料分析/来茂德…刀浙江医学一1993,15(2)一78一80 良性肿瘤1 841例,主要为脉管瘤和畸胎瘤。上皮性良性肿瘤主要为甲状腺腺瘤和钙化上皮瘤。恶性肿瘤1 240例,常见的依次为淋巴网状系统肿瘤、肾母细胞瘤、视网膜母细胞瘤、软组织肉瘤、翠丸生殖细胞肿瘤和神经母细胞瘤。畸胎瘤分布常见的依次为髓尾部、卵巢、后腹膜、皋丸、纵隔等。淋巴网状系统恶性肿瘤年龄分布(例):0一4岁62,5一9岁147,10一14岁81,三组比较XZ=7.444,尸<0.05,提示5一9岁年龄组最多.表6参7(杨威) 932411急性白血病患儿超氧化物歧化酶及脂质过氧…     

Imaging diagnosis of retroperitoneal ganglioneuroma in childhood

Purpose. To demonstrate the typical appearance of retroperitoneal ganglioneuromas on CT and MRI.¶Materials and methods. Retrospective analysis of diagnostic imaging (five CT scans, three MRI scans) in five children aged 3–15 years with the histological diagnosis of ganglioneuroma.¶Results. The scans showed large (maximum 11 cm diameter), round or oval tumours with sharply defined margins. Intraspinal tumour involvement occurred in two cases. Comparing CT with MRI, MRI was more accurate in defining the intraspinal involvement. The ganglioneuromas were hypodense on unenhanced CT and showed moderate enhancement with administration of contrast medium. In three patients, CT demonstrated tumour calcification with a disseminated speckled pattern. On T1-weighted MRI the tumours were homogeneous and hypointense, showing marked enhancement after gadolinium administration. On T2-weighted scans the tumours were hyperintense.¶Conclusion. At the time of diagnosis, retroperitoneal ganglioneuromas are generally large tumours that can be shown well by CT and MRI. The appearance on CT more readily suggests the diagnosis, but MRI is superior for documenting local or intraspinal tumour extension and lacks radiation load.     

A review of open biopsy for mediastinal masses

Objective: To review the recent experience with biopsied mediastinal lesions in children and to assess the impact of recent advances in imaging and surgical techniques on diagnosis. Methodology: The clinical and radiological features of 55 patients who had mediastinal biopsies at The Royal Alexandra Hospital For Children (RAHC) over 15 years were reviewed. Results: Fifty-five patients presented to RAHC between 1978 and 1993 with lesions of the mediastinum requiring biopsy of that site. Thirty-one of the 55 (56%) lesions were malignant. Neurogenic tumours were the most common (40%). In order of frequency the following lesions were found: neuroblastoma (15), teratoma (eight), non- Hodgkin's lymphoma (NHL; eight), enteric cyst/duplication (five), ganglioneuroma (five), bronchogenic cyst (three), ganglioneuroblastoma (two), lymphangioma (two), abscess (two), Hodgkin's lymphoma (HL; two), oesophageal granuloma (one), Langerhan's cell histiocytosis (one), congenital fibromatosis (one). Eighty-two per cent of neurogenic tumours were located in the posterior mediastinum, while 75% of teratomas and 100% lymphoid tumours were located anteriorly. Symptoms were generally unhelpful in establishing a specific diagnosis and in 27% of cases the lesions were discovered incidentally. Physical signs, such as thoracic inlet obstruction and neurological findings, were helpful clinically in localizing lesions within the mediastinum. Chest radiography enabled lesions to be subdivided within the mediastinum. This localization, in combination with the age at presentation, predicted the tissue diagnosis. Computerized tomography (CT) and magnetic resonance imaging (MRI) further defined the lesion and demonstrated involvement of adjacent structures. Histology, however, was essential to distinguish benign from malignant lesions. Conclusions: The clinical presentation of mediastinal masses is often non-specific or incidental. Despite recent advances in imaging technology and biopsy techniques, full histological examination is required to exclude malignancy.     

Paediatric thoracic tumours presenting as empyema

Ultrasonography (US) is considered to be adequate for the preoperative evaluation of childhood empyema. This study was aimed to improve awareness that paediatric intra-thoracic tumours can mimic childhood post-pneumonic empyema and highlights the value of computed tomogram (CT) scan with intravenous (IV) contrast in preoperative evaluation of childhood empyema. The data were analysed on eight children (four boys and four girls) presented at the median age of 6.2 years (1.8–15 years) for the management of empyema and later confirmed to have intra-thoracic tumours. Intra-thoracic tumours in 8 (5.3%) children out of 150 cases of post-pneumonic empyema were managed during the study period. All eight had clinical features, increased white cell count, raised inflammatory markers and biochemical parameters suggestive of childhood empyema. Chest X-ray showed localised opacity in 3/8 while in other five suggested significant pleural collection with mediastinal shift. Additional investigations in referring hospital were suggestive of empyema in four children; US in three, CT scan without IV contrast in one. Referring hospital carried out non-diagnostic thoracocentesis in four children with blood stained pleural tap in two. In four children corroborative evidence suggestive of infection within pleural cavity and acute respiratory distress led to an emergency mini-thoracotomy resulting in significant intra-operative bleeding in two children. Histology on biopsy of the infected material showed primitive neuroectodermal tumour (PNET) in one, pleuropulmonary blastoma in one, metastatic malignant melanoma in one and cytology of pleural fluid diagnosed lymphoma in one. Pre-operative CT scan with IV contrast in four children correctly identified underlying intra-thoracic tumour (two benign teratoma, two PNET). In two cases CT with IV contrast was performed because chest X-ray suggested mediastinal loculated empyema while in other two high clinical index of suspicion prompted preoperative evaluation with CT scan with IV contrast. We advocate caution and increased awareness before considering therapeutic options in childhood empyema and recommend preoperative CT scan with IV contrast in some selected and unusual cases.     

Superior mediastinal teratoma containing well-differentiated bowel

A previously fit and well 14-month-old-girl presented with a 2-month history of worsening cough and wheeze. Chest radiograph revealed a widened mediastinum and thoracic CT showed a large mixed density mass in the superior mediastinum, consistent with a mediastinal teratoma. Her tumor markers were within the normal range. The mass was resected and found to be a mature cystic teratoma. Surprisingly, well-formed bowel-like structures were present, containing all bowel wall layers and having a ganglionated myenteric plexus. The identification of complete sections of bowel in this context is a rare finding and to the best of our knowledge has not been published previously for the mediastinal teratoma. The possibility of secondary neoplasia developing in these areas is a complicating factor.     

婴儿原发性纵隔肿瘤58例临床分析

目的通过分析婴儿原发性纵隔肿瘤的发病特点、临床表现、疗效与并发症,探讨其诊治经验。方法回顾性分析2010年1月至2叭4年12月间本院经手术治疗的58例原发性纵隔肿瘤患儿临床资料,总结其临床表现、原发部位、手术及术后并发症。结果 58例中,男33例,女25例,最小年龄15天,最大年龄12个月(平均4.5个月)。原发肿瘤中,神经源性肿瘤占51.7%,生殖细胞瘤占24.1%,间叶组织肿瘤占20.7%。42例出现临床症状,其中气促26例,占46.5%,发热5例,占8.6%,咳嗽5例,占8.6%,呼吸窘迫3例,占5.2%。良性肿瘤24例(占41.4%),恶性肿瘤34例(占58.6%)。均予手术治疗,其中根治性切除手术53例,大部分切除术5例,仅1例予术后化疗。58例已随诊2个月至5年,无手术相关死亡,无一例复发,术后出现霍纳氏综合征2例,膈神经损伤1例。结论婴儿纵隔肿物的早期诊断较困难,需依赖临床症状和胸部CT检查。手术是治疗婴儿纵隔肿瘤安全有效的治疗方案。     

Mediastinal Germ Cell Tumors in Childhood

Background: Mediastinal germ cell tumors (GCTs) are rare and usually located in anterior mediastinum. We aimed to review clinical and pathological characteristics of these tumors. Procedure: Between 1973 and 2011, 24 children with mediastinal GCTs were diagnosed. Hospital files were reviewed for presenting complaints, clinical, radiological and other laboratory data, surgical practices, treatments, and outcomes. Results: Median age was 4.5 years (0.2–16) (male/female: 10/14). Most common initial complaints were dyspnea, cough, anorexia/fatigue, fever, and chest pain. Primary tumors were located in anterior mediastinum (n = 22), posterior mediastinum (n = 1), and sternum (n = 1). Thirteen of 24 cases had mature teratomas (54.2%); four (16.7%) endodermal sinus tumor (EST); four (16.7%) immature teratomas; and one (4.2%) each of embryonal carcinoma, teratocarcinoma, and malignant teratoma. Mature teratomas underwent only surgical resection and were under follow-up without disease. Four cases with ESTs received chemotherapy and radiotherapy (n = 3), three underwent surgical resections: three died, one was followed for 284 months in remission. All but one immature teratomas were treated with surgery and all were under follow-up without disease. Two patients with embryonal carcinoma and malignant teratoma didn't undergo surgery; both received chemotherapy and radiotherapy but died with disease. The patient with teratocarcinoma was treated with surgery and chemotherapy but died with disease. Conclusions: No adjuvant therapy is needed for mature teratomas. Immature teratomas must be under close follow-up for recurrences. Prognosis for mediastinal malignant GCTs was poor. These cases need intensive chemotherapies and effective local control measures as surgery –/+ radiotherapy to ensure long-term survival.     

Comparing axillary and mediastinal lymphadenopathy on CT in children with suspected pulmonary tuberculosis

Background: Radiographic demonstration of mediastinal lymphadenopathy is important for the diagnosis of pulmonary tuberculosis (PTB). Plain radiographs are unreliable for this and CT, which is relatively more expensive and carries a high radiation burden, remains the gold standard. No studies correlating the presence of axillary with mediastinal lymphadenopathy have been reported. Such a correlation would allow for clinical or ultrasound diagnosis of PTB via the axilla. Objective: To correlate the presence of axillary lymphadenopathy with mediastinal lymphadenopathy in children with suspected PTB. Materials and methods: CT scans were performed and reviewed in 100 children (prospectively recruited) with suspected PTB. The axilla and mediastinum were reviewed separately by covering the non-relevant sections on the CT scans prior to reading. Only nodes greater than 1 cm were regarded as pathological. Results: Mediastinal lymphadenopathy was present in 46% of children; 70% had lymphadenopathy in either axilla. Bilateral axillary lymphadenopathy was identified in 47%. Axillary lymphadenopathy showed a sensitivity of 74% and a specificity of 33% for the presence of mediastinal adenopathy. Bilateral axillary adenopathy had a sensitivity of 50% and a specificity of 56%. Conclusions: Axillary lymphadenopathy has a moderate sensitivity and low specificity for the presence of mediastinal and hilar lymphadenopathy in children with suspected PTB. Further research should be aimed at correlating ultrasound-detected axillary lymphadenopathy with FNA results in children.Presented at 41st Annual Congress of The European Society of Paediatric Radiology, Heidelberg, Germany, June 2004.     

Mediastinal teratoma presenting with hemoptysis and pleuritis misdiagnosed as tuberculosis (empyema)

Background

Mediastinal teratoma is uncommon in children. It can be very difficult to diagnose especially in early stage. Rarely, teratoma may rupture into adjacent structures and lead to lung lesions or pleuritis. The main rarity of our reported cases was the dynamic imaging findings very similar to the developmental process of tuberculosis in patients 1 and 2, the pachypleuritis in patients 2 and 3, the extremely elevated inflammatory markers very similar to empyema in patient 3, and the extremely atypical tumor shape in all patients.

Case presentation

We present three pediatric patients presenting predominantly with recurrent hemoptysis and/or chest pain who were ultimately diagnosed with mediastinal teratoma containing pancreatic tissue. All three patients were initially suspected to have tuberculosis or empyema, and underwent relevant treatment, but without improvement. Patient 1 had left hilar enlargement, and subsequently an enlarging calcified cavity within high-density consolidation was identified. Patient 2 initially presented with right-sided pulmonary consolidation and pleuritis, and subsequently developed right lower lobe calcification, pleural thickening, and irregular soft tissue in the right inferior mediastinum. Patient 3 was initially found to have right lobe consolidation accompanied by a massive right-sided pleural effusion with extremely elevated inflammatory markers in serum and pleural effusion. The effusion later acquired heterogeneous density and appeared to become encapsulated. In patients 2 and 3, pleural biopsy identified fibrous tissue (with and without granuloma). Thoracotomy/thoracoscopy revealed mediastinal teratoma in each case, all of which were completely excised and the patients made uneventful recoveries. Histopathologic analysis revealed mature cystic-solid teratoma containing pancreatic tissue in all patients, and calcification in patients 1 and 2.

Conclusions

Clinicians should be mindful that mediastinal teratoma is a potential cause of hemoptysis, lung lesions and pleuritis. Calcification and pachypleuritis on chest imaging especially in patients without fever should be highly suspected of mediastinal teratoma. Pleural biopsy sometimes fails to assist in making a definitive diagnosis.

     

高效液相色谱法检测尿中儿茶酚胺代谢产物含量在神经母细胞瘤早期诊断中的价值

目的　尿中香草扁桃酸 (VMA)及高香草酸 (HVA)的检测是目前早期诊断神经母细胞瘤 (NB)的重要指标 ,以前的检测方法干扰因素多 ,结果不够准确。该文探讨高效液相色谱法 (HPLC)检测随机尿中VMA、HVA的含量在NB诊断和早期诊断中的价值。方法　用HPLC方法 (流动相 pH4 .3,甲醇含量 2 % ,正辛烷磺酸钠溶液 3.0mmol/L)检测 5 0例正常儿童 (正常对照组 )、2 7例NB患儿 (NB组 )及 1 5例急性淋巴细胞性白血病患儿(ALL组 )随机尿中VMA、HVA浓度。用比色法测定同一样品中肌酐浓度。以VMA、HVA与肌酐的比值表示VMA、HVA含量。结果　NB组患儿随机尿VMA/Cr及HVA/Cr的含量 (5 1 .6 0± 4 .5 3μmol/ μmolCr,5 8.0 0±3.75 μmol/ μmolCr)明显高于正常对照组 (8.4 2± 3.6 1 μmol/ μmolCr,1 0 .1 2± 3.88μmol/ μmolCr)及ALL组患儿(8.78± 3.5 0 μmol/ μmolCr,1 1 .5 0± 2 .6 8μmol/ μmolCr) ,其差异均有显著性 (P <0 .0 5 )。以正常对照组儿童随机尿中VMA/Cr及HVA/Cr均值加 2 .5个标准差为异常判定值 ,分别为 1 7.5、1 9.8μmol/ μmolCr,超过此值为异常 ,则尿VMA/Cr或HVA/Cr异常对NB的诊断效率为 96 .3%。结论　HPLC同时测定随机尿中VMA、HVA含量 ,能为NB的早期诊断提供可靠的实验依据 ,并且可用于NB的诊断     

Clinical significance of catecholamine excretion levels in diagnosis and treatment of neuroblastoma.

Bond, J.V. (1975). Archives of Disease in Childhood, 50, 691. Clinical significance of catecholamine excretion levels in diagnosis and treatment of neuroblastoma. The pattern of urinary excretion of vanillylmandelic acid (VMA) was studied in 50 children with neuroblastoma. There were 10 disease-free survivors, and 4 children who survive for 3 or more years with residual nonmetastatic disease, 36 children died with widespread metastic disease. The urinary VMA level was raised at diagnosis in 8 of the 10 disease-free survivors but rapidly returned to normal after treatment. In 3 out of 4 children with residual tumour, VMA remains persistently raised. In 28 of the 36 children who died VMA was raised at diagnosis and remained so throughout the course of their disease. In the majority of the children with metastatic disease the finding of a raised VMA revealed the nature of the primary tumour. Measurement of urinary VMA is a simple diagnostic test which confirms the presence of neurblastoma and avoids the need for more complex investigations. The prompt return to normal levels in the disease-free survivors confirms the importance of serial VMA estimations in assessing response to treatment and ultimate prognosis. The other good prognostic features in the surviving patients were young age at diagnosis, primary tumour in the thorax, and histologically well-differentiated tumours which had not metastasized.