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1.
BACKGROUND: The prognosis of craniopharyngioma in children after subtotal surgical removal, followed by irradiation of remaining tumour with 50 Gy, is better than usually reported. In our subjects we found a relapse rate of 5% in the last 20 years. The treatment of recurrences forms a special problem because the possibilities of adjuvant radiotherapy are restricted. We report on a chemotherapeutic treatment after multiple or very rapid recurrences of craniopharyngioma in four children. METHODS: Four children experienced their first tumour recurrence at respectively 3, 8, 50 and 59 months after the initial treatment. New neurosurgical attempts to remove the recurring tumour, and in one patient a second course of radiotherapy, were performed, but there were two or more recurrences in these children, resulting in further restriction of surgical or radiotherapeutical possibilities. Chemotherapy was given, consisting of five intravenous ambulatory courses of Adriamycin (doxorubicin) (33 mg/m2/day, continuously over 3 days) together with oral CCNU (lomustine) (80 mg/m2 at day 1) at 6-weeks intervals. RESULTS: After the chemotherapy there was no further tumour recurrence after 12, 10, 3 and 3 years respectively. In the third patient a cystic relapse occurred after 3 years' remission. In the fourth patient a complete regression was observed of the cystic part of the tumour. The side-effects of the chemotherapy consisted of alopecia and bone marrow depression. No signs of cardiomyopathy have been found. CONCLUSION: Treatment of recurrent craniopharyngioma in children by chemotherapy with anthracyclines and nitrourea-derivates may be effective.  相似文献   

2.
目的:为了提高对儿童颅咽管瘤的诊断率以及减少术后并发症的发生,该研究探讨小儿颅咽管瘤的诊断方法、手术方式及术后并发症的防治。方法:回顾性分析31例小儿颅咽管瘤的临床表现、手术方法和术后并发症治疗的经验。结果:31例患儿主要临床表现为:头痛,视力下降,生长发育迟缓等。CT和MRI可确诊颅咽管瘤。31例颅咽管瘤中,肿瘤全切除 19例(61.3%),次全切除5例(16.1%),大部分切除7例(22.6%)。术后出现尿崩症者19例(61.3%),3例为长期性尿崩。6例(19.4 %)出现下丘脑损伤症状。无死亡病例。随访32.5个月, 5例(16.1%)患儿复发。结论:小儿颅咽管瘤的诊断主要依据临床表现、CT和MRI检查,该病以开颅手术治疗为首选。肿瘤切除的程度应考虑降低肿瘤的复发和减少并发症的发生为原则。[中国当代儿科杂志,2009,11(8):663-665]  相似文献   

3.
The endocrine sequelae of 62 children with craniopharyngioma were studied retrospectively. These patients were followed for a median duration of 3 years (range 1 to 10 years). Eighteen patients had a long-term follow-up for more than 5 years (range 5 to 10 years). Complete surgical resection was achieved in 30 patients and 32 patients had residual tumor. Twenty-five patients had recurrence or progression of the residual tumor and were treated with radiotherapy. Presenting complaints suggestive of endocrinopathy were infrequent. The most common presenting symptoms were headache, nausea and vomiting, followed by growth failure. Pre-operatively, growth hormone deficiency was the most commonly encountered pituitary hormonal deficiency; however postoperatively, most children had diabetes insipidus. Multiple pituitary hormonal deficiencies were more frequently observed in children treated with extensive radical surgery than in those treated with conservative surgery and radiotherapy. The endocrine morbidity associated with craniopharyngioma and its different management modalities remains high; however, it is manageable with appropriate hormonal replacement therapy.  相似文献   

4.
Hydroelectrolytic disorders often complicate surgery of intra/parasellar tumors in children and adolescents. Eighteen patients undergoing microneurosurgical procedures for intra-supra-sellar craniopharyngioma (10 patients), hypothalamic germinomas (3 patients), hypothalamic-chiasmatic astrocytomas (3 patients), pituitary adenomas (2 patients) were studied. The hydroelectrolytic balance was assessed from 8 hours before surgery to 1 week after with a specific protocol in which water metabolism alterations were treated with standard procedure. Diabetes insipidus (DI) was observed in 10/18 patients before surgery and in 15/18 patients after surgery; during surgery it was effectively treated with synthetic desmopressin (DDAVP) and hydroelectrolytic solutions. Hyponatremia, isolated or associated (with diuresis contraction or polyuria), seen during surgery and in the following 24 hours, was treated with variation of the infusion rate. We show that close monitoring and treatment of hydroelectrolytic disorders in patients submitted to neurosurgery for intra/ parasellar tumors may significantly reduce their morbidity and mortality rate.  相似文献   

5.
OBJECTIVES: To evaluate (1) the pattern of post-operative weight gain and (2) the risk factors associated with the development of post-operative weight gain and obesity in children treated for craniopharyngioma. STUDY DESIGN: The records of 43 children who had primary craniopharyngioma resection were reviewed. Neurological, endocrine, anthropometric and radiological risk factors for the development of obesity and for post-operative increase in BMI SDS were analyzed. RESULTS: Twenty-five patients (58%) became obese post-operatively. Obesity was significantly associated with higher BMI SDS at presentation and pre-operative hydrocephalus (p < 0.05). Increased BMI SDS from 0-12 months was significantly associated with post-operative MRI evidenced hypothalamic damage and higher BMI at presentation (p < 0.05). CONCLUSIONS: Children who developed hypothalamic obesity had a significant, rapid BMI increase over the first 6 months, followed by stabilization, with no regression of BMI SDS.  相似文献   

6.
目的 研究小儿隆突性皮肤纤维肉瘤的临床特征与外科治疗方案.方法 以我院2010年1月至2015年12月间治疗的21例隆突性皮肤纤维肉瘤患儿为研究对象,分别调取所选病例的临床资料,并对其进行回顾性分析.本组病例都接受外科手术治疗,若病理切缘呈阳性需要继续补切至切缘呈阴性,分析21例患儿隆突性皮肤纤维肉瘤的临床特征与外科治疗方案.结果 21例患儿均选择手术治疗,其中有12例患儿选择扩大切除术,9例患儿选择扩大切除术联合植皮、转移皮瓣修补术,术后有3例患儿联合放疗治疗.有4例患儿复发,有1例患儿死亡,死亡原因为肿瘤转移.结论 隆突性皮肤纤维肉瘤属于皮肤恶性肿瘤的一种,具有极高的复发率,选择外科治疗方案彻底切除具有重要作用,是临床治疗隆突性皮肤纤维肉瘤的首选方法,同时配合放疗也能提高治疗效果,而肿瘤复发和转移都会影响患儿手术预后效果,外科治疗方案值得推广应用.  相似文献   

7.
Long-term follow-up of children with craniopharyngioma   总被引:2,自引:0,他引:2  
Management of craniopharyngiomas is still controversial. 28 children with this tumor were studied. GH deficiency was present in 22 patients following surgery, 10 of these GH-lacking patients had normal or accelerated growth (usually associated with rapid weight gain) postoperatively. Somatomedin levels were normal in three of six normally growing patients. After craniotomy their basal and TRH-stimulated prolactin levels were in the normal range, but their insulin secretion was markedly increased. Postoperatively there was a significant correlation between peak insulin levels following arginine infusion and growth velocity in all patients.Complete tumor removal could be performed in 28% of our patients. Altogether 36% of all patients had at least one tumor recurrence. Recent literature with the addition of our series showed tumor recurrence in 22% of patients with total tumor excision and in 72% of patients with partial tumor removal. Radiotherapy seems to be capable of destroying craniopharyngioma tissue. The recurrence rate was only 26% in patients with subtotal excision plus radiotherapy. Unless radical tumor removal can be attempted with safety, subtotal tumor removal plus radiotherapy appears to be the treatment of choice for craniopharyngioma.Abbreviations SD Standard Deviation - SDS Standard Deviation Score - SEM Standard Error of the Mean - BA Bone Age - CA chronological Age - RIA Radioimmunoassay - GH Growth Hormone - TRH Thyrotropin Releasing Hormone  相似文献   

8.
Craniopharyngioma is one of the leading causes of hypothalamic-pituitary dysfunction in childhood, caused either by the tumor itself or the consequences of treatment. Tumor management in terms of recurrence rate, quality of life and complications is still controversial. Sixty-six patients with craniopharyngioma at pediatric age were reviewed for symptoms, signs, types of treatment, recurrence rates, complications, and endocrinological outcome. The majority of symptoms was related to the neurological system. Complaints only affecting the endocrinological system were seen in 6% of patients. The most frequent complaints were headache and vomiting (74.2%). The main endocrinological complaints were polyuria and polydipsia (15%), and lassitude (10.6%). Although short stature was a symptom in 9.1% of patients, it was a finding in 39.7% of patients. Plain skull X-rays raised the suspicion of intracranial tumor in more than 90% of children with craniopharyngioma. Recurrence rates were independent of the extent of tumor removal (total or subtotal). The frequency of endocrine dysfunction increased significantly after treatment. The most frequent hypothalamic-pituitary dysfunction was growth hormone deficiency (100%) and gonadotropin deficiency (80%). Hypothyroidism was diagnosed in 74% of patients. The frequency of hypothalamic-pituitary dysfunction was not affected by the extent of tumor removal. Radiotherapy did not increase the frequency of endocrine dysfunctions further. In conclusion, growth follow-up in childhood seems to be an important indicator of craniopharyngioma in early diagnosis. Radiotherapy and extent of tumor removal - either total or subtotal - did not influence endocrine outcome.  相似文献   

9.
Ectopic recurrence of craniopharyngioma 17 years after initial diagnosis is exceedingly rare in pediatric neuro-oncology. Only 23 cases of ectopic recurrence in children with craniopharyngioma are described in the literature with a median time to recurrence of 3 years. We describe a patient diagnosed at 5 years of age, presenting with neck pain and ataxia 17 years after diagnosis. Her original follow-up care was fragmented and included surveillance imaging for 10 years after surgery and endocrine management of panhypopituitarism. Rare, extremely late relapse of this tumor highlights the importance of extended multidisciplinary follow-up care that includes neuro-oncologists in a late-effects/survivorship program.  相似文献   

10.
Salivary gland carcinomas are rare in childhood. We have reviewed the case records of 15 children aged 3–14 years (median 11) identified from children's tumour registries. Primary sites were parotid: 11, submandibular: 3, and base of tongue: 1. The range of histologies was similar to that occurring in adults. Six were treated by complete excision, with one given post-operative radiotherapy (RT). All six remain disease-free at 2 months to 21 years after completion of treatment. Five were treated by partial or sub-total excision. Four were given post-operative RT, of whom 3 are disease-free at 3 years, 6 months - 18 years and 1 lost to follow-up (LTFA). One not given RT developed a local recurrence at 11 months and was given RT and LTFA. Four patients had a biopsy only. Three were treated by RT. One is disease-free at 8 years, one died of metastatic disease at 6 months, and one developed a local recurrence at 11 years and has remained disease-free following salvage surgery. One patient with advanced disease not suitable for RT died 3 months after diagnosis. Complete excision is the treatment of choice. Following sub-total or incomplete excision post-operative RT can prevent recurrence. Careful RT planning is necessary to minimise late effects. © 1993 Wiley-Liss, Inc.  相似文献   

11.
骶尾部畸胎瘤术后复发高危因素分析   总被引:1,自引:0,他引:1  
目的 探讨和分析引起骶尾部畸胎瘤术后复发的高危因素.方法 回顾性分析2003年1月至2012年12月收治的107例骶尾部畸胎瘤患儿的临床资料,使用单因素和多因素危险度Lo-gistic回归法,从Altman分类、手术年龄、肿瘤大小、肿瘤是否完整切除、术中肿瘤是否溃破以及破溃成分、病理类型等方面分析肿瘤复发的高危因素.结果 肿瘤复发共16例,复发时间为6~59个月,平均(16.25±14.01)个月.恶性肿瘤复发均为术后常规随访发现,良性肿瘤仅2例常规随访发现,其余因出现排便困难、骶尾部出现肿块等临床症状就诊.根据Altman分类,Ⅰ~Ⅳ型的复发病例(复发率)分别为8例(15.4%)、2例(10.5%)、1例(10.0%)和5例(31.3%).病理类型中,成熟性畸胎74例中复发9例,其中5例病理性质复发后转变为未成熟或恶性畸胎瘤;未成熟畸胎瘤6例中复发2例;恶性畸胎瘤17例中复发5例.肿瘤复发后的5年生存率(64.8%)与未复发病例(95.0%)相比,差异有统计学意义(P=0.002).单因素及多因素危险度分析提示术中肿瘤实质成分溃破(P=0.028)、肿瘤残留(P=0.000)、未成熟(P=0.029)及恶性畸胎瘤(P=0.026)是骶尾部畸胎瘤复发的危险因素,而Altman分类、手术年龄、肿瘤大小以及囊液破裂并不是肿瘤复发的危险因素.结论 肿瘤复发是影响骶尾部畸胎瘤预后的重要因素.术中避免肿瘤实质成分溃破,尽可能完整切除肿瘤可减少肿瘤复发.重视骶尾部畸胎瘤,特别是成熟性畸胎瘤的术后随访,有利于尽早发现肿瘤复发,提高治愈率.  相似文献   

12.
BACKGROUND: In about one third of patients suffering from a desmoid tumor primary complete resection is not feasible. Furthermore in locally relapsing tumors reoperation alone does not result in cure in many cases. Radiotherapy can be applied in both groups of patients with curative intention. But the indication of radiotherapy is challenging particularly in children and adolescents due to the impending late radiation sequelae such as growth delay, fibrosis and radiation induced secondary malignancy. PATIENTS AND METHOD: The follow up and outcome of five irradiated children/adolescents with desmoid tumors, registered in the German-Cooperative-Soft-Tissue-Sarcoma Study (CWS) was looked at, and the corresponding literature was reviewed. RESULTS: Radiotherapy of gross residual or relapsing tumors resulted in long lasting event free survival in two cases (3/8 years), but in one patient local progression occurred despite irradiation. Postoperative radiotherapy in patients with microscopic residual disease resulted in both, long lasting event free survival (14 years, 1 patient) and in early local relapse (1.5 years, 1 patient). The role of radiotherapy could not be evaluated clearly by the CWS-experience due to the fact that the irradiated patients were treated individually also by chemotherapy and/or tamoxifen. But despite sparse and retrospective data there is evidence in the literature, that radiotherapy is able to control 65-90% of the unresectable desmoid tumors and that the local relapse rate can be reduced by radiotherapy by 10-20% in patients with microscopic residual disease following resection. CONCLUSIONS: Radiotherapy as primary treatment should be given if complete tumor resection is not feasible without mutilation. Radiotherapy can be applied postoperatively if the risk of local relapse seems to be highly life- or function threatening.  相似文献   

13.
OBJECTIVE: To assess the efficacy and toxicity of local radiotherapy in achieving local control in patients with stage 4 or high-risk stage 3 neuroblastoma treated with induction chemotherapy and tandem stem cell transplants. METHODS: Fifty-two children with stage 4 or high-risk stage 3 neuroblastoma were treated on a standardized protocol that included five cycles of induction chemotherapy, surgical resection of the primary tumor when feasible, local radiotherapy, and then consolidation with tandem myeloablative cycles with autologous peripheral blood stem cell rescue. Local radiotherapy (10.5-18 Gy) was administered to patients with gross or microscopic residual disease prior to the myeloablative cycles. Thirty-seven patients received local radiotherapy to the primary tumor or primary tumor bed. Two patients with unknown primaries each received radiotherapy to single, unresectable, bulky metastatic sites. The second of the myeloablative regimens included 12 Gy of total body irradiation. RESULTS: Of the 52 consecutively treated patients analyzed, 44 underwent both transplants, 6 underwent a single transplant, and 2 progressed during induction. Local radiotherapy did not prolong recovery of hematopoiesis following transplants, did not increase peritransplant morbidity, and did not prolong the hospital stay compared with patients who had not received local radiotherapy. Local control was excellent. Of 11 patients with disease recurrence after completion of therapy, 9 failed in bony metastatic sites 3 to 21 months after the completion of therapy, 1 recurred 67 months following therapy in the previously bulky metastatic site that had been irradiated, and 1 had local recurrence concurrent with distant progression 15 months following the second transplant. The three-year event-free survival was 63%, with a median follow-up of 29.5 months. The actuarial probability of local control was 97%. CONCLUSIONS: The use of induction chemotherapy, aggressive multimodality therapy for the primary tumor, followed by tandem myeloablative cycles with stem cell transplant in patients with stage 4 or high risk stage 3 neuroblastoma has resulted in acceptable toxicity, a very low local recurrence risk, and an improvement in survival.  相似文献   

14.
BACKGROUND: The modern era of pediatric craniopharyngioma treatment includes multiple modalities including microsurgical resection, irradiation, brachytherapy or chemotherapy. No clear consensus as to the best therapeutic approach has yet been established. The aim of this study was to describe the techniques and strategies for the treatment of pediatric craniopharyngiomas in light of a literature review with particular attention to the incidence of adverse postoperative effects. METHODS: Twenty-seven pediatric patients (median age 9.0 years) who were surgically treated for craniopharyngiomas were evaluated. We reviewed the recent literature for clinical features of craniopharyngiomas in children, including the present cases. RESULTS: The overall rate of radiographically complete resection of our cases was 92.6%. In the literature we reviewed, the rate of gross total resection was 33-91% (average 57.8%). According to the literature, recurrence rates range from zero to 52.8% (average 16.1%) in the gross total resection group, 51.1% in the subtotal resection without radiation therapy group, and 33.5% in the subtotal resection with radiation therapy group. In six of our most recent cases, for whom we have complete outcome analysis, radiographically complete resection is 100% with only one recurrence so far over 4 years. CONCLUSIONS: For patients in whom early postoperative MRI reveals complete craniopharyngioma removal, a very low rate of recurrence is anticipated. In the authors' experience, radiographically total excision of even large craniopharyngiomas can be safely achieved by one or a combination of several advanced microsurgical techniques, sometimes by a staged strategy. Major morbidities can be avoided, although moderate morbidities occur and require management.  相似文献   

15.
Craniopharyngiomas are benign suprasellar tumors; however, their tendency to recur after resection and the risks associated with aggressive tumor resection pose a surgical dilemma. We reviewed our series of operated craniopharyngiomas and selected pediatric cases operated as first-hand cases and followed in our institution. We studied 37 cases. Resection was total in 65%. No patient died in the operative period; three patients died 6 months to 8 years after surgery of endocrine-related cause or sudden death. Tumor progression occurred in 93% of cases after subtotal resection versus 43% after total resection. Among 20 operations for recurrence, total resection was achieved in 45%. Visual deficits were often stabilized or improved after surgery, but worsened in 30% because of surgical damage or tumor recurrence. Fifty-nine percent of patients follow a normal school curriculum. All patients have some degree of pituitary hormonal replacement and 48% have obesity. Hypothalamic damage was generally associated with intraventricular extension of the tumor recurrence and re-operations, especially through combined approaches. Craniopharyngiomas in children are particularly aggressive tumors. Although the best oncological results are obtained with total resection, in some cases the functional price of surgery may be too high and alternative techniques should be proposed. The life-long management of these patients requires that the best use is made of all potential therapeutic tools.  相似文献   

16.
Pleuropulmonary blastoma (PPB) is a rare and aggressive primary intrathoracic neoplasma of children. The prognosis is extremely poor with frequent metastasis to the brain and bone. We present a 4-year-old girl with a tumor mass in the right hemithorax initially diagnosed as pneumoniae. Tumor resection was performed and the histologic report indicated the diagnosis of PPB. The patient received chemotherapy comprising vincristine, actinomycin D, doxorubicin, cisplatin, and cyclophosphamide. Irradiation was performed with total 45 Gy at the right lower pulmonary lobe. She relapsed 29 months later at the pleura between the right middle and lower pulmonary lobe. Tumor resection and total 45 Gy of irradiation were performed again. High-dose chemotherapy comprising cisplatin, adriamycin, and cyclophosphamide was performed followed by autologous peripheral blood stem cell transplantation (PBSCT). The patient achieved complete hematologic recovery. Thirty-one months after PBSCT, no signs of relapse have been observed. Although it might be that the patient could have been cured with second surgery alone or by the surgery and subsequent chemotherapy, high-dose chemotherapy and PBSCT should be considered for the treatment of relapsed PPB.  相似文献   

17.
显微外科为主的综合治疗对小儿髓母细胞瘤预后的影响   总被引:2,自引:0,他引:2  
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  相似文献   

18.
BACKGROUND: In children with nephroblastoma, recurrence with metastases in the central nervous system is rare. Recently, previous reports (NWTSG and UKCCSG) reported brain metastases with an incidence of respectively 0.5% and 0.6% in Wilms tumor (WT) patients (respectively n = 30/5,852 and n = 7/1,249). PROCEDURE: We retrospectively investigated the incidence and survival of patients with central nervous system relapse in WT patients, treated according to the consecutive SIOP protocols 1, 2, 5, 6, 9, and 93-01. All children with WT from 1971 until 2000 were enrolled in the study (3,040 eligible patients). Specimens at diagnosis and if possible at relapse were centrally reviewed. Patients with renal neoplasms other than WT were excluded. RESULTS: CNS relapse was documented in 14 patients (0.5%). Median time to CNS relapse was 16 months (3-69). The occurrence of relapse was not associated with specific histological subtypes. In seven patients intracranial metastases occurred at first relapse, of which two were isolated relapses. In five patients no treatment was started because of the poor condition of the patient, the other nine cases were treated with (a combination of) chemotherapy (n = 6), surgery (n = 4), and radiotherapy (n = 6). CONCLUSIONS: CNS relapse in WNT is rare. In contrast to reports of other Wilms tumor study groups, although four patients reached (local) CR, the SIOP registry showed that eventually none of the documented WT patients survived.  相似文献   

19.
Craniopharyngiomas are benign, partly cystic embryogenic malformations of the sellar region, presumably derived from Rathke pouch epithelium. With an overall incidence of 0.5–2 new cases per million population per year, 30–50% of all cases represent childhood craniopharyngioma. Typical manifestations at primary diagnosis are headache, visual disturbance, polyuria/polydipsia, growth retardation, and weight gain. The treatment of choice in patients with favorable tumor localization is total resection with the intention to maintain visual and hypothalamic–pituitary function. In patients with unfavorable tumor localization (hypothalamic involvement), a limited resection followed by radiation therapy is recommended. The appropriate time point for irradiation after incomplete resection is currently being investigated in a randomized trial (KRANIOPHARYNGEOM 2007). The overall survival rates are high (92%). However, quality of life is substantially reduced in many survivors due to sequelae. In particular, extreme obesity due to hypothalamic involvement has a major negative impact on quality of life.  相似文献   

20.
Craniopharyngiomas are embryogenic malformations which lead to eating disorders and morbid obesity due to hypothalamic involvement in about 50% of all patients with pediatric craniopharyngioma. The experience with laparoscopic adjustable gastric banding (LAGB) in obese craniopharyngioma patients is limited. We are reporting on four patients with childhood craniopharyngioma diagnosed at age 2, 11, 12, and 21 years. BMI-SDS at diagnosis was +0.9, +4.5, +4.7 and -0.1 SD. During follow-up, all patients developed morbid obesity (BMI-SDS: +13.9, +10.3, +11.4, +7.3) so that 11, 6, 9 and 3 years after diagnosis LAGB were performed. After a follow-up of 4.5, 1.5, 3.0 and 2.5 years BMI decreased or stabilized continuously in all patients (BMI-SDS at latest visit: +9.9, +9.7, +9.5, +5.9 SD). The eating behavior changed in all patients profoundly. The addiction to food and especially sweets significantly improved based on self-assessment. In two patients a dislocation of the LAGB occurred and resulted in weight gain. We conclude that LAGB could be effective in weight reduction of obese craniopharyngioma patients with hypothalamic syndrome. Close follow-up is necessary in order to analyze long-term effects and complications of LAGB in patients with childhood craniopharyngioma and morbid obesity.  相似文献   

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