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。     

术前化疗治疗神经母细胞瘤的疗效随访及其影响因素

目的探索术前化疗治疗神经母细胞瘤患儿的远期疗效及对其生存的影响因素。方法对１３例经术前化疗后手术的病例进行２～６年的疗效随访，分析存活组与死亡组的临床分期、化疗时间、香草杏仁酸（ＶＭＡ）、病理标本增殖细胞核抗原（ＰＣＮＡ）、ＤＮＡ指数的差异。结果２年生存率为６９％（６／１３），５年生存率为２３％（３／１３），６例（４６％）无瘤生存至今。存活组化疗后ＶＭＡ、ＰＣＮＡ、ＤＮＡ指数较死亡组明显下降（Ｐ均＜０．０５）。结论术前化疗可以提高晚期神经母细胞瘤远期疗效和生存率，化疗后肿瘤增殖活性的抑制程度与临床疗效密切相关。     

肾母细胞瘤微卫星DNA变化分析

目的 研究分析１３例肾母细胞瘤（Ｗｉｌｍｓ肿瘤）患儿５个染色体点微卫星标记的变化。方法 ５个微卫星标记分别为：Ｄ７Ｓ１８０８、Ｄ９Ｓ１６１、Ｄ１２Ｓ１０５６、Ｄ１７Ｓ７９１和Ｓ２２Ｓ２７４,应用ＰＣＲ方法扩增Ｗｉｌｍｓ肿瘤组织ＤＮＡ,以该患儿正常组织作对照,扩增产物用含脲素的聚丙烯酰胺凝胶电泳及银染技术进行分析。结果 １３例Ｗｉｌｍｓ肿瘤患儿检测５条染色体５个位点的微卫星多态标记,只１例患儿７号染     

Wilms瘤的免疫治疗:附8例报告

为探讨提高Ｗｉｌｍｓ瘤治愈率的方法，采用自体外周血应用体外ＩＬ－２诱导培养ＬＡＫ细胞，分别通过全身输注或区域动脉灌注的方法配合化疗、放疗及栓塞、手术切除治疗Ⅰ至期；包括ＵＨ型２例在内的Ｗｉｌｍｓ瘤８例，迄今完全存活，其中４例２年均生存。ＬＡＫ细胞具有不同增加治疗药物引起的毒副反应的效果，对全身状况有明显帮助。     

小儿常见恶性实体瘤多药耐药性蛋白的定位检测

对４９例小儿常见恶性实体瘤冰冻切片进行多药耐药蛋白（Ｐ－１７０）的免疫组化定位检测。术前化疗肿瘤多药耐药性蛋白阳性率为肾母细胞瘤４４％（４／９），神经母细胞瘤１００％（７／７），横纹肌肉瘤７５％（３／４），术前未化疗肿瘤多药耐药性蛋白的阳性率为肾母细胞瘤２３％（３／１３），神经母细胞瘤４０％（４／１０），横纹肌肉瘤３３％（２／６）。小儿恶性肿瘤多药耐药性检测对揭示肿瘤耐药、复发的原因，估计化疗效果和判断预后以及指导化学治疗具有一定意义。     

显微外科为主的综合治疗对小儿髓母细胞瘤预后的影响

目的 分析显微手术辅以放疗、化疗对小儿髓线细胞瘤治疗效果及其预后的影响。方法 将髓母细胞瘤患儿分为二组。手术全切除组和手术未全切除组。前才术后只接受全神经轴放射治疗，后者术后辅以放疗和化疗，观察二组治疗效果和预后的差异。结果 手术全切除组术后肿瘤复发率２９．３％，１、２、５年生存率分别为１００％、９４．１％、８２．４％；手术未全切出生率且术后肿瘤复发率８５．７％、１、２、５年的生存率分别为７１．４     

现行影像学检查对双侧同时发生的Wilm瘤诊断的正确性

现行影像学检查对双侧同时发生的Ｗｉｌｍ瘤诊断的正确性在Ｗｉｌｍ瘤（ＷＴ）中，双侧肾脏同时发病（ＢＷＴ约占５％，过去的研究发现在ＮＷＴＳ－３病例中双侧患者仅有２／３病例术前能作出准确的诊断。本文作者对１９８６年８月～１９９３年１月ＮＷＴＳ－４注册的２３...     

介入治疗在小儿腹部恶性肿瘤的应用

目的 对巨大腹部肿瘤采用介入治疗,为肿瘤完整切除创造有利条件。方法 对１２例腹部恶性肿瘤患儿进行介入治疗,其中男７例,女５例。年龄２个月￣７岁。肝母细胞瘤６例,神经母细胞瘤３例,肾母细胞瘤２例肾上腺皮质癌１例。采用Ｓｅｌｄｉｎｇｅｒ’ｓ技术进行动脉插管造影,确定肿瘤主要供血动脉,进行肿瘤供血动脉超选择性插管,局部注入化疗药物,然后用碘油、明胶海绵或白芨微球栓塞肿瘤的供血动脉。结果 １２例中７例手术     

rhG-CSF对小儿化疗所致的白细胞缺乏症的临床观察

本文报告１９９４．２～１９９５．３我们用ｒｈＧ－ＣＳＦ（惠尔血．ＧＲＡＮ）对１９例儿童肿瘤进行了３０例次在化疗后出现粒细胞缺乏症时的临床观察。有自身前后疗程对照１３例（１９例次），前后疗程化疗方案完全相同。每例前１疗程单用化疗为对照疗程，后一疗程在化疗结束后，当ｗｂｃ＜２．０×１０９／Ｌ或ＡＮＣ＜０．５×１０９／Ｌ时使用惠尔血７５ｍｃｇ，每日１次，皮下注射。一般用３～５天，ｗｂｃ及ＡＮＣ即可达到临床要求标准（ｗｂｃ≥４．０×１０９／Ｌ，ＡＮＣ≥１．５×１０９／Ｌ）。试验疗程较对照疗程粒细胞恢复时间明显缩短（ｗｂｃ提前９．３天恢复），ＡＮＣ提前８天恢复。在６例患儿化疗后用惠尔血（１１例次）未设自身对照疗程，临床观察亦获得与上述相似的疗效。本组在３０例次中仅见２例次有发热和骨痛，停药后发热和骨痛消失，未见其它不良反应。观察结果显示，惠尔血对小儿肿瘤化疗所致的粒细胞缺乏症疗效肯定。我们采用在停化疗后ｗｂｃ＜２．０×１０９／Ｌ几或ＡＮＣ＜０．５×１０９／Ｌ时开始使用惠尔血７５ｍｃｇ，１／日，皮下注射。一般３天至５天即可达到预期效果。用药量小，毒副作用少，既能减轻患儿痛苦，又能减轻家庭经济负担．是一种适合国情的用药方     

小儿睾丸卵黄囊瘤治疗体会

探讨临床Ⅰ期尤其是〈１岁小儿睾丸卵黄囊瘤治疗方式的选择。临床资料：Ⅰ期１０例，其中〈１岁７例，Ⅱ例４例，均实行高位精索睾丸切除。９例辅以腹膜后淋巴结清扫，Ⅰ期５例，Ⅱ期４例，仅１例有阳性发现，术后均未行规则的化疗。结果；获访１２例中生存９例，死亡３例，均大于１岁，死因为肺和腹膜后转移。     

Nephron-sparing procedures in 11 patients with Wilms' tumor

PURPOSE: In unilateral Wilms' tumor (WT), tumor nephrectomy is the standard surgical approach, whereas partial nephrectomy (PN) is controversially discussed. The aim of our retrospective study was to show that in selected cases of unilateral WT kidney-sparing operations could be a reasonable alternative to nephrectomy and to discuss the results of patients with bilateral WT treated by tumor enucleation. MATERIALS AND METHODS: From 1981 to 1998, seven patients with unilateral nephroblastoma (four stage I, one stage III and two stage IV) had tumor resection by PN (five right side, two left side), which was planned when the tumor volume was reduced after 4 to 6 weeks of chemotherapy by at least 50%, when the tumor occupied one pole or was easily resectable, when 50% or more of the kidney tissue remained and when paraaortic lymph nodes were free by intraoperative histological examination. In four patients with bilateral WT (stage V) bilateral tumor enucleation was carried out-except in one patient in whom the contralateral kidney had to been removed because of extension of the tumor via the inferior vena cava to the right atrium. All patients ( n = 11) received pre- and postoperative chemotherapy followed by radiotherapy in four patients. RESULTS: All patients with unilateral WT ( n = 7) are still alive and disease free (follow-up time: mean 6.6 years, range: 28 months to 11 years) with normal renal function, although two patients with secondary nephrectomy revealed creatinine clearance levels at the lower range. In six patients primary PN was performed successfully. In a stage III tumor patient (intraperitoneal metastasis, free lymph nodes), secondary nephrectomy was necessary due to renal arterial thrombosis 2 days after PN. In one stage IV tumor patient (lung metastasis, free lymph nodes), the primary resection was not far enough away from the tumor margin so that an additional slice of tissue with then tumor-free margins had to be resected. This patient evolved a local relapse 19 months after PN and had to be nephrectomised thereafter. In the group of bilateral WT patients ( n = 4), one child died 2 months after surgery during chemotherapy because of central venous line sepsis. One patient who additionally suffered from inferior vena cava tumor thrombosis extending to the right atrium making nephrectomy of the right kidney necessary developed chronic renal failure 4.7 years postoperatively. The other two stage V tumor patients have creatinine clearance levels within the normal range. CONCLUSIONS: Kidney-sparing procedures remain the operative approach of choice in patients with bilateral WT, but bear the risk of chronic renal failure when one kidney has to be removed. PN in children with unilateral WT, carried out by an experienced surgeon, is a reasonable alternative to nephrectomy if strict guidelines such as excellent tumor response to preoperative chemotherapy and easy resectability far away from the tumor margins through healthy kidney tissue are followed. Paraaortic lymph nodes must be free of tumor invasion in order to avoid local radiotherapy. PN prevents the patient from having to have dialysis in cases of contralateral nephrectomy resulting from metachronous WT or subsequent renal trauma.     

Pediatric surface osteosarcoma: clinical, pathologic, and radiologic features

BACKGROUND: Osteosarcoma (OS) arising from the surface of bone is far less common than its intramedullary counterpart. Although surface OSs share some radiographic and clinical features, they can be divided into three distinct histologic subtypes. PROCEDURE: We reviewed the clinical, radiographic, and pathologic features of 14 cases of pediatric surface OS treated at St. Jude Children's Research Hospital between 1970 and 2003. RESULTS: Seven patients had parosteal, five had periosteal, and two had high-grade surface OS. The median age at diagnosis was 16.2 years (range, 13.6-18.5 years). Nine patients were male; 11 were Caucasian. None had metastatic disease at diagnosis. Primary tumor sites included distal femur (n = 6), mid to proximal femur (n = 4), and mid to proximal tibia (n = 4). All 14 patients were treated with surgery, and 7 (1 with parosteal, 4 with periosteal, 2 with high-grade tumors) received chemotherapy. One patient experienced pulmonary metastasis of periosteal OS 16 months and 43 months after diagnosis; long-term disease-free survival followed resection of the metastatic tumors. Twelve patients remained alive and disease-free a median of 10 years (range, 1.5-25.4 years) after diagnosis. One patient died of high-grade surface OS 1.8 years after diagnosis, and one patient with periosteal OS died of gastric cancer 18.2 years after diagnosis of OS. CONCLUSIONS: The histologic grade predicts the clinical behavior of pediatric surface OS. Complete resection is the treatment of choice regardless of tumor subtype. Whereas chemotherapy is not indicated for parosteal OS, its role in periosteal OS remains controversial.     

Residual or recurrent cerebellar low-grade glioma in children after tumor resection: is re-treatment needed? A single center experience from 1983 to 2003

PURPOSE: The aim of this study was to report on children with cerebellar low-grade glioma (LGG), who were found to have progressive or nonprogresssive residual tumors or tumor recurrence after tumor resection. PATIENTS AND METHODS: Medical records and magnetic resonance imaging (MRI) studies of children (<16 years) with cerebellar LGG were retrospectively analyzed. RESULTS: Of 289 patients with CNS tumors referred between 1983 and 2003, 28 (9.7%) (15 male, 13 female; median age at diagnosis: 71 months) had cerebellar LGG (pilocytic astrocytoma grade I: n = 21; fibrillary astrocytoma grade II: n = 5; mixed hamartoma/pilocytic astrocytoma: n = 1; radiographic diagnosis: n = 1). Total resection was initially performed in 16 patients (57.1%), near total resection in 4 (14.3%), and partial resection in 6 patients (21.4%). One patient underwent biopsy. At a median follow-up of 112 months, 25 patients (89.3%) were alive, 18 of them being in complete remission. Three patients died, 2 due to symptoms related to brain stem compression/infiltration and 1 patient due to postoperative cerebral edema. Presently 5 patients have nonprogressive residual tumors and 2 patients developed nonprogressive recurrences 10 years and 20 months after initial total resection, respectively. None of them required second surgery and none received additional nonsurgical therapies. Only 1 additional patient had to undergo second surgery due to disease progression. CONCLUSIONS: A 'wait and see' strategy is justified in patients with nonprogressive recurrent or residual cerebellar LGG after primary tumor resection. However, long-term follow-up with repeated MRI is mandatory in these patients to detect disease progression. Second surgery is indicated only in patients with unequivocal disease progression, as documented by MRI.     

Congenital mesoblastic nephroma: a clinicoradiologic study of 17 cases representing the pathologic spectrum of the disease

Congenital mesoblastic nephroma (CMN) is a rare infantile renal tumor with a generally excellent prognosis. We describe 17 tumors that fit into the pathologic spectrum of CMN proposed by Beckwith, which ranges from benign renal tumors, through atypical "gray zone" lesions of more aggressive potential, to "crossover" tumors akin to clear cell sarcoma of kidney. Nine patients with histologically typical CMN were significantly younger and had smaller tumors than did eight patients with atypical CMN. Clinical features did not differ in the two groups of patients. A distinctive "ring sign" on renal sonography was commonly seen in patients with typical intrarenal CMN. All 17 patients were alive with no evidence of disease at a mean follow-up of 10 years. Nephrectomy was adequate therapy for younger infants and for those with typical CMN. Nephrectomy was probably also adequate therapy for infants 3 months of age or younger with atypical CMN, even if the tumor extended to the surgical resection margins and into the perinephric connective tissues. Adjuvant chemotherapy or radiation or both should be reserved for patients older than 3 months who have grossly unresected tumors and for those patients whose tumors have an unequivocally malignant histologic appearance or evidence of aggressive biologic behavior.     

畸胎瘤样肾母细胞瘤治疗体会及文献回顾

目的 探讨儿童畸胎瘤样肾母细胞瘤临床诊断和治疗方法,提高临床对该病的认识.方法 回顾我院2007年至2016年间诊治的4例儿童畸胎瘤样肾母细胞瘤,男2例,女2例,年龄2～40个月,平均年龄21个月;原发灶:右肾3例,左肾1例;根据COG分期为Ⅰ、Ⅱ、Ⅲ、Ⅳ期(肺及骨髓)各1例,分析其临床表现、诊断及治疗方法.结果 4例患儿均行肾脏肿瘤根治性切除术,其中Ⅲ、Ⅳ期患儿术前行化疗再行根治术;3例术后辅以化疗,术后复发1例(化疗不敏感);3例早中期患儿预后可(2例随访时间超9年,1例随访3年),1例Ⅳ患儿再次手术后化疗加放疗中.结论 儿童畸胎瘤样肾母细胞瘤是肾母细胞瘤的罕见病理类型,临床症状通常难与经典肾母细胞瘤鉴别,主要依靠术前影像学及病理诊断,患侧肾脏肿瘤根治性切除术为主要治疗手段,早中期TWT患儿预后良好,至于保肾肿瘤切除术在该类型肾母细胞瘤的应用,暂未见文献报道,本组病例数较少,也未涉及.对于晚期TWT患儿,可能因为其独特的病理特征,其预后可能比经典肾母细胞瘤更差.     

Therapy of advanced ovarian juvenile granulosa cell tumors

BACKGROUND: Gonadal sex cord-stromal tumors are rare tumors that develop from the gonadal non-germ cell component such as granulosa, Sertoli or Leydig cells. Among these, juvenile granulosa cell tumors (JGCT) constitute the largest subgroup of ovarian sex cord-stromal tumors during childhood and adolescence. In local disease (FIGO stage I), the beneficial role of tumor-ovarectomy is well established. In contrast, life expectancy in patients with advanced JGCT (FIGO stage >/= II) is short even after complete tumor resection. The current literature provides only limited and inconclusive data regarding the value of adjuvant chemotherapy in such patients with advanced disease. PATIENTS AND METHODS: Therefore, we analyzed the patients with FIGO stage >/= II JGCT who were prospectively documented as follow-up patients of the German MAKEI trials for non-testicular germ cell tumors and received the recommended cisplatin-based chemotherapy in an adjuvant setting. From 1988 until 2000, 7 patients (age, 4;2 - 18;11 years, median 14;8 years) were registered. Three patients were stage IIc, one stage IIIa, and three stage IIIc. 5 patients underwent laparatomy with adnectomy, which was complete in only two patients. Two patients received laparoscopic tumor resection, which was incomplete in both. All patients received 4 or 6 cycles of adjuvant cisplatin-based three-agent chemotherapy in analogy to the current therapeutic concept applied in malignant germ cell tumors. One patient with a large tumor and multiple peritoneal metastases additionally received 40 Gy abdominal irradiation. RESULTS: All patients achieved complete clinical remission after initial surgery and adjuvant chemotherapy. 4 out of 7 patients are currently remaining in first continuous complete remission after 15 to 111 months follow-up. One patient developed a metachronous tumor of the contralateral ovary after 126 months follow-up and is still alive but currently in therapy of another recurrence. Another patient suffered a tumor recurrence after 12 months but achieved a second complete remission with cisplatin chemotherapy after a follow-up of currently 4 months. One patient achieved complete clinical remission but suffered a diffuse peritoneal tumor recurrence with massive ascites and finally died as a result of tumor progression. In summary, at the time of this report 6 of 7 patients are alive after a median of 47 (15 - 138) months. CONCLUSION: This analysis clearly demonstrates that advanced JGCT can be successfully treated with surgery followed by adjuvant cisplatin-based chemotherapy. Therefore, this study reveals encouraging therapeutic perspectives in these otherwise fatal tumors that merit further investigation in a prospective cooperative trial.     

Childhood meningiomas. Experience in the modern imaging era.

Twenty children with meningiomas (ages 18 months to 17 years) received initial therapy at the Children's Hospital of Philadelphia between January 1975 and June 1991, accounting for 2% of children with primary brain tumors seen during that time interval. All were verified histopathologically, and none had had prior irradiation. Fifteen were male and 5 female. Fifteen tumors were intracranial, all located supratentorially. Two of these also had a component within the optic canal. One tumor was entirely within the orbit. Four meningiomas arose within the spinal canal. Associated conditions were neurofibromatosis (NF) type I (1 patient), NF type II (2 patients), and a facial alveolar rhabdomyosarcoma (1 patient). A gross total resection as documented by postoperative scan and operative note was accomplished in 12 patients. Four of these relapsed, at a mean of 3.5 years from initial surgery. In 4 patients a near-total resection (> 90%) was performed. Of these, 2 progressed at 9 months and 1.5 years. One of these died of complications associated with reoperation. In 4 patients a partial resection (50-90%) was performed. Two of these progressed at 4 months and 1 year, and the other 2 have been followed for less than 2 years. Five patients received radiation therapy (RT). One patient received RT as adjunctive therapy after primary surgery because of papillary histology. The other 4 had RT following reoperation for recurrence at a mean of 1.5 years from diagnosis (range, 7 months to 2 years). These 4 patients remain alive and with stable disease at a mean of 6 years from diagnosis (range 2-8.8 years).(ABSTRACT TRUNCATED AT 250 WORDS)     

Pancreatoblastoma in childhood: clinical course and therapeutic management of seven patients

BACKGROUND: To analyze the clinical course of pancreatoblastoma in children and to propose management and therapy. PROCEDURE: Retrospective review of seven cases of pancreatoblastoma treated in France over a 20-year period and literature review. RESULTS: Median age of patients was 6 years. The tumor was well encapsulated in one patient and had direct extension to adjacent tissues in two others. Three patients had regional lymph node involvement and one had liver metastases. The tumor arose in the head of the pancreas in three children, in the tail and body in two and in the tail and in the body, one each. Elevation of AFP serum levels was noted in four out of 6 cases. Five tumor resections were performed, one initially and 4 after neoadjuvant chemotherapy, and cisplatin plus doxorubicin seemed effective. Two children received post-operative irradiation because of incomplete resection. In all, four children are disease free with a median follow-up of 50 months ( range, 5--120 months) : one had a complete removal of tumor at diagnosis and no further treatment, three had unresectable tumor at diagnosis and received neoadjuvant chemotherapy with cisplatin and doxorubicin. One of them also received post-operative irradiation. CONCLUSIONS: Pancreatoblastoma is a curable tumor. Examination of serum AFP levels may be useful for diagnosis and to follow the course of the disease. Complete resection is the treatment of choice. However, tumor is often unresectable at diagnosis and preoperative chemotherapy is needed to reduce tumor volume. We suggest a regimen that include cisplatin and doxorubicin. In patients with incompletely resected disease, postoperative radiation may be indicated.     

The relevance of surgical therapy for bilateral and/or multiple pulmonary metastases in children.

PURPOSE: Pulmonary surgery is frequently used for the treatment of metastases in children with various malignant diseases. The benefit of an aggressive surgical treatment in children with bilateral and/or multiple pulmonary metastases is still discussed controversially. METHODS: A retrospective analysis of 10 children (7 girls, 3 boys; age range from 2 to 16.5 years) who underwent thoracotomy for bilateral and/or multiple pulmonary metastases was performed. The primary malignancies were osteosarcoma (n = 4), hepatoblastoma (n = 3), malignant peripheral nerve sheath tumor (n = 1), adrenocortical carcinoma (n = 1) and alveolar rhabdomyosarcoma (n = 1). Unilateral but multiple pulmonary metastases were found in 3 children. 7 patients showed bilateral pulmonary metastases. Preoperative induction chemotherapy with tumor regression and a subsequent decrease in the size and number of pulmonary metastases was mandatory for the surgery of metastases. RESULTS: Standardized bilateral thoracotomy was performed in 4 patients in 1 operation (in 1 patient combined with a hemihepatectomy), and in 3 patients, in 2 operations on different days. 5 children underwent re-thoracotomy due to recurrent pulmonary metastases (2 patients: unilateral; 3 patients: bilateral; 1 patient: twice bilateral). All visible and palpable metastases (1 - 25) were excised, either by wedge resection, by segment resection or by lobectomy. Postoperative artificial ventilation was necessary for 0 to 24 hours. Postoperative complications included intrathoracic secondary hemorrhage in 3 cases and pneumonia in 1 patient. 2 patients (20%) died of recurrent metastatic disease (osteosarcoma: 1; adrenocortical carcinoma: 1). During a mean follow-up period of 49 months (14 to 66 months after the last thoracotomy), 8 patients (80%) remained in complete remission without clinically relevant respiratory restrictions. CONCLUSION: Complete surgical resection of pulmonary metastases after response to induction chemotherapy may increase survival in carefully selected children, even in cases with multiple and recurrent metastatic disease. In children, bilateral thoracotomy within a single operation is possible without an increased complication rate.     

Adult type vs. Childhood hepatocellular carcinoma--are they the same or different lesions? Biology,natural history,prognosis, and treatment

BACKGROUND: Differences in the biology, natural history, and treatment results of hepatocellular carcinoma (HCC) in children and adults were sought based on the literature and experience resulting from SIOPEL 1 trial. PROCEDURE: In the SIOPEL1 study, 40 children with HCC were registered from January 1990 to February 1994. Outcome was analyzed in 39. In most cases, disease was advanced at diagnosis: 31% had metastases and 39% had extrahepatic tumor extension and/or vascular invasion. More than 50% of patients had multifocal tumors; 39% of tumors were associated with hepatic cirrhosis. All, but two patients, received preoperative chemotherapy (PLADO--cisplatin and doxorubicin). Outcome, response to treatment, and prognostic factors were analyzed using the SAS statistical package. RESULTS: Overall survival (OS) at 5 years is 28% and EFS is 17% at median follow-up of 75 months (49-90). Partial response to chemotherapy was observed in 18 of 37 cases evaluated (49%). Complete tumor resection was achieved in 14 of the 39 patients (36%). Twenty (51%) never became operable. Multifocality of the tumor, presence of metastases, and PRETEXT grouping adversely influenced OS. A large number of "de novo" HCC cases, fairly high response rate to preoperative chemotherapy (49%) and 54% survival after complete resection constitute a significant difference in comparison with adult HCC series. CONCLUSIONS: Survival for pediatric HCC patients is below 30%. Radical tumor resection remains the only chance for survival. New multi-center prospective studies in children with HCC are required to better results and to allow further study of differences between adult and pediatric HCC should they exist.