儿童脊髓内肿瘤的显微外科治疗

目的总结儿童髓内肿瘤的病理特点、显微手术和治疗效果，并进行探讨。方法81例儿童脊髓内肿瘤均经手术切除和病理证实，对手术治疗的效果进行回顾性分析。结果儿童髓内肿瘤以星形细胞瘤24例（29．6％）、室管膜瘤13例（16．0％）和表皮样囊肿11例（13．6％）为多见，不同的病理类型的肿瘤，切除率不同。放疗用于高度恶性的肿瘤。椎板复位术可以有效地减少脊柱畸形的发生率。结论大多数的髓内肿瘤组织学上为低级别星形细胞瘤（Ⅰ～Ⅱ级）、室管膜瘤和胚胎残余组织肿瘤，积极手术切除能够获得满意的疗效。     

显微技术切除儿童侧脑室肿瘤

目的：探讨儿童侧脑室肿瘤显微外科手术的治疗方式和临床效果。方法：回顾性总结51例儿童侧脑室肿瘤的临床表现、诊断方式、手术入路的选择与疗效。根据肿瘤的部位和大小分别采用经额中回入路、经顶枕叶或顶上小叶入路、经胼胝体入路，对儿童侧脑室肿瘤进行显微外科手术治疗。结果：51例儿童侧脑室肿瘤中，位于侧脑室三角区27例，侧脑室前角12例，侧脑室体部9例，室间孔区3例，左侧脑室27例，右侧脑室24例。肿瘤直径2～6．5cm。所有肿瘤均经手术和病理证实，肿瘤全切除43例，近全切除6例，大部切除1例，部分切除加外减压1例。31例获得随访，28例生活基本上能自理或参加学习。结论对儿童侧脑室肿瘤选择适当的手术入路进行显微外科手术能有效地降低手术创伤，提高全切除率，减少致残率和病死率，对于无法手术切除的肿瘤可以采用术后放疗和立体定向放射外科治疗，可减少本病复发。     

带蒂椎板成形术治疗椎管内肿瘤9例

目的 探讨带蒂椎板成形术重建椎管后部结构治疗椎管内肿瘤的临床效果。方法 对9例经MR1诊断椎管内肿瘤，行显微切除椎管内肿瘤后，采用带蒂椎板棘突复合体，实行椎板成形术重建椎管。结果 9例患儿采用保留带蒂椎板棘突重建椎管后部，术中椎板切开1～4个，平均2．5个。随访3个月～5年，均在3个月复查X线见良好骨性愈合，脊椎序列正常，未见骨不愈合椎板棘突复合体移入椎管倾向，无1例发生脊柱畸形和椎体滑脱、6例复查MR1示肿瘤切除完全，未见椎管狭窄及神经根黏连。结论 带蒂椎板成形术符合脊柱的解剖生物特性，可替代现行的椎板切除术，可避免椎板切除术后并发症发生。     

儿童胰腺肿瘤8例报告

目的 介绍儿童胰腺肿瘤的常见类型,提高对这些疾病的诊断和治疗水平.方法 报告 8 例儿童胰腺肿瘤,男 3 例,女 5 例,年龄2岁至14岁8个月.7 例患儿行手术治疗,其中 1 例良性畸胎瘤;低度恶性者 3 例(囊实瘤 2 例,胰岛细胞瘤 1 例);高度恶性者 3 例(胰母细胞瘤 2 例,胰头癌1 例);未手术 1 例.结果 7 例手术者中良性和低度恶性病例术后随访至今未见复发及不适;3 例高度恶性病例患儿术后均放弃进一步治疗;1 例未手术者6个月后死亡.结论 儿童胰腺肿瘤在临床上相对少见,不同病理类型临床表现不同,治疗方法 各异,预后也相差很大.     

儿童脊柱肿瘤

目的 探讨儿童脊柱肿瘤的临床特点、诊断、治疗方法及预后。方法回顾性分析我院1991年1月-2003年10月治疗的16岁以下脊柱肿瘤及瘤样病变44例，男27例，女17例；平均9．6岁。良性肿瘤（包括瘤样病变）38例，恶性肿瘤3例，转移瘤3例。疼痛39例，肿块9例，侧弯或斜颈8例，不同程度神经损害14例。18例初诊不确定或与最后诊断不符。发病至确诊时间平均8．5个月。手术治疗17例，化疗10例，放疗8例，手术与化疗1例，手术与放疗2例，放弃治疗3例，观察3例。结果围手术并发症4例。35例获平均5．5年随访。良性肿瘤及瘤样病变患儿手术或放疗或化疗后疗效较满意，所有患儿的局部疼痛消失，除1例外，所有患儿神经功能均有改善，3例病变复发再次手术。4例出现后弯畸形。6例恶性肿瘤和转移瘤患儿全部死亡。2例嗜酸性肉芽肿患儿症状消失，病变得到控制。结论对于儿童脊柱肿瘤，根据肿瘤的性质选择恰当的治疗方法，良性肿瘤与肿瘤样病变预后良好，恶性肿瘤及转移瘤治疗效果欠佳。     

儿童原发性心脏肿瘤21例分析

目的 总结儿童原发性心脏肿瘤的病理和临床特点。方法 回顾性分析21例确诊为儿童原发性心脏肿瘤患儿的临床资料，其中经二维超声心动图及(或)MRI确诊11例，经病理检查确诊10例。结果 21例中，心房内肿瘤3例，其中左房粘液瘤2例，右房粘液瘤1例；心室肿瘤15例，其中单纯右心室肿瘤7例，单纯左心室肿瘤5例，左、右心室多发性肿瘤1例；心包肿瘤3例。病理检查确诊的10例中，8例为良性，2例为恶性。19例患儿中，以进行性心功能不全起病8例，心律失常起病6例，体检发现心脏杂音而无其他临床症状的4例，无任何症状体征1例例。结论 儿童心脏肿瘤以良性为多见，临床上常因心功能不全、心律失常或发现心脏杂音就诊发现。     

儿童鞍区肿瘤的手术治疗

目的 探索儿童鞍区肿瘤的有效手术治疗方法。方法 回顾性分析53例儿童鞍区肿瘤患者的临床资料，其中颅咽管瘤29例，生殖细胞瘤7例，垂体瘤11例，表皮囊肿2例，星形细胞瘤1例，恶性畸胎瘤1例，神经鞘瘤1例，蛛网膜囊肿1例。29例患儿行经额下硬脑膜入路，13例经改良翼点入路，11例经蝶窦入路，16例行脑室一腹腔分流术。结果 肿瘤全切除32例；次全切除16例；大部切除5例。术中22例可见垂体柄，均予保留。术后出现多饮多尿27例，其中20例在术后2周内恢复正常；出现高钠血症7例，低钠血症7例，均在2周内恢复正常，术后死亡2例。随访30例，其中3例术后1年内复发而再次手术，2例行γ-刀治疗，25例患儿术后可参加正常学习。结论 选择合适的手术入路，并注意保护下丘脑结构，防止其供血动脉损伤，是儿童鞍区肿瘤手术治疗的原则和取得较好效果的关键。     

儿童脊髓髓内占位病变的临床特征

目的 探讨儿童脊髓髓内占位病变(IMSCT)的临床特征.方法 对本院确诊为IMSCT的16例患儿性别、发病年龄、卉灯变部位、病理组织学类型、首发症状、主要临床表现、特殊阳性体征、辅助检查如X线、CT、MRI、直肠肛管、测压、冲经传导速度等临床资料进行回顾性分析.结果 16例中男11例,女5例;年龄15个月～14岁,平均(8.55±4.28)岁.从发病到确诊的时间为1 d～9 a,平均2.23 a.首发症状以疼痛最为常见.主要症状:肢体运动障碍14例,疼痛12例,二便障碍9例,步态异常8例,肢体萎缩3例.体格检查:腱反射异常14例,浅反射消失9例,肛门括约肌松弛6例,巴氏征阳性5例,脊柱畸形4例,局部包块3例,感觉异常2例,双下肢不等长、潜毛窦、色素沉着各1例.患儿均行脊髓MRI检查,均有病变段脊髓增粗和髓内异常信号区.病理类型:畸胎瘤5例,皮样囊肿、表皮样囊肿各2例.误诊为尿路感染2例,类风湿关节炎、腰椎问盘突出各1例.结论 儿童IMScT以先天性肿瘤为主,首发症状多为疼痛,MRI检查为其诊治提供了可靠依据.早期发脱、早期诊断、早期治疗对改善本病的预后至关重要.     

Cytogenetic Studies in 45 Pediatric Brain Tumors

Brain tumors are the most frequent childhood tumors. There have been few cytogenetic studies published on these tumors in children compared to the numerous studies on adult brain tumors. We examined chromosomes from 45 primary pediatric brain neoplasms including 14 medulloblastomas, 12 astrocytomas, 4 glioblastomas, 7 ependymomas, 5 craniopharyngiomas, 2 meningiomas, and 1 ganglioglioma. Chromosomal abnormalities were found in 10 medulloblastomas out of the 14 analyzed. The most frequently observed abnormalities were the total or partial loss of one chromosome 17: monosomy 17, i(17q), and a monosomy 22 in 4 cases of desmoplastic medulloblastoma. In glioblastoma, we observed the gain of chromosome 7, chromosome 3, a monosomy 10, and hyperdiploidy. The loss of chromosome X was observed in 2 cases of ependymoma as was a monosomy 22. Our observations show that from the cytogenetic point of view childhood brain tumors differ from adult brain tumors.     

Pediatric Neuroblastic Brain Tumors Containing Abundant Neuropil and True Rosettes

We have encountered a series of seven unusual neuroblastic pediatric central nervous system (CNS) neoplasms with a unique constellation of histologic, immunohistochemical, and ultrastructural features. The tumors presented in five girls and two boys, ages 1 to 3 years. In six cases the lesions involved the frontoparietal region, in one case the tectal plate. The tumors consisted of small to medium-sized, round to oval, hyperchromatic cells with poorly defined cytoplasmic borders. Cells were found in clusters and cords set in a paucicellular fibrillar neuropil matrix. Distinctive, virtually anuclear regions of neuropil were scattered throughout the lesions. True rosettes with well-formed central lumens often filled with granular debris were present, along with perivascular pseudorosettes and occasional Homer-Wright rosettes. Mitoses and apoptosis were frequent, but large regions of confluent necrosis were absent. Immunohistochemically, the neuropil-like areas as well as the perinuclear cytoplasm of many embryonal tumor cells were positive for synaptophysin and neurofilament protein. Ultrastructurally, the tumor cells showed microtubule-containing neuronal processes, some with neurosecretory granules. While the lesions were largely glial fibrillary acidic protein (GFAP) negative, there was focal GFAP positivity consistent with divergent differentiation in one case. The clinical outcome was poor, with five patients dead from their disease 5 to 14 months after initial presentation and one patient with recurrent disease 7 months after resection and chemotherapy. The final patient is alive without recurrent disease 30 months after initial presentation. These lesions present distinctive histological features within the group of primitive neuroectodermal tumors. Received November 2, 1998; accepted September 9, 1999.     

Fine Needle Aspiration Cytology in Advanced Pediatric Tumors

Eighty-one children with clinically suspected malignant tumors were subjected to percutaneous fine needle aspiration cytology (FNAC) at the Pathology Department of the National Institute of Child Health, Jinnah Postgraduate Medical Centre, Karachi, from August 1986 through July 1987. There were 47 malignant diagnoses including lymphoma, neuroblastoma, nephroblastoma, Ewing's sarcoma, and leukemia. Histological findings confirmed the FNAC diagnoses in 36 cases in which a subsequent incisional biopsy or surgically removed specimen was available. FNAC results were confirmed in all benign cases. In 10 advanced cases of NonHodgkin's lymphoma, surgery was not possible because of marked malnourishment. One false negative and no false positive result was encountered. Forty-eight were females and thirty-three males. FNAC can be a quick, effective, and inexpensive alternative to open biopsy, particularly in advanced cases of malignancy in undernourished children where anesthesia and immediate surgery are contraindicated.     

Treatment of Pediatric Malignant Tumors with VP-16

The West Japan Pediatric Oncology Group studied the treatment of pediatric malignant tumors with VP-16 from December 1984 to March 1988. Study subjects were divided into two groups. One group received only VP-16, while the other received VP-16 combined with other anti-tumor agents. VP-16 evaluation was possible in a total of 116 cases. The efficacy rate was calculated by considering both complete and partial remission as effective. The efficacy rate for VP-16 alone was 87.5% for primary cases of ANLL and 100% for primary cases of histiocytosis. The efficacy rates for combination therapy were as follows: 92.6% for primary cases of ANLL, 66.7% for primary cases of histiocytosis, 45.5% for relapsed cases of ANLL and 66.7% for relapsed cases of ALL. Bone marrow suppression was seen in the form of leukopenia and thrombocytopenia for 2 to 3 weeks after VP-16 administration. Alopecia, mucositis and gastrointestinal symptoms were also observed, but they presented no significant problem. From our results, we believe that chemotherapy including VP-16 is effective for remission induction therapy in primary cases of ANLL and for salvage therapy in relapsed leukemia. Additionally, VP-16 is considered to be effective for the treatment of histiocytosis.     

小儿实体肿瘤310例分析

目的 总结各种类型小儿实体肿瘤的诊治以及各年龄段的大致分布情况。方法 选择1996年1月-2006年1月,我院收治的310例小儿实体肿瘤患儿病例资料,对其年龄分布、诊断及治疗进行回顾性分析。结果 310例患儿中,良性肿瘤147例,占总病例数的47．4％;恶性肿瘤163例,占总病例数的52．6％。男：女=1．25：1。年龄在1岁以内126例,占总病例数的40．7％;1。3岁93例,占30％;7-14岁44例,占14.2％。根据肿瘤类型和病人的个体差异分别采取手术、化疗、放疗以及支持疗法等。治疗不同,疗效各异。结论 小儿恶性肿瘤除需采取积极的手术治疗外,尚需要化疗、放疗以及营养支持治疗等多种方法的紧密配合。     

Pediatric Germ Cell Tumors: Protocol Update for Pathologists

The therapy for pediatric germ cell tumors has historically been widely variable and institution dependent. The efforts to provide consistent, biology-driven therapy through Intergroup protocols has been a relatively recent phenomenon that has provided the framework for future protocol designs. The first Intergroup protocols confirmed that stage I malignant testicular germ cell tumors and immature teratomas at all sites in children could be treated with surgery alone followed by close observation. Future protocols currently in the planning stages may extend this low-risk category to stage I ovarian germ cell tumors of all histologic types. Low-stage extragonadal germ cell tumors may be placed in a new intermediate risk category. Particular pathologic issues that were raised and reported during the first protocols and that may impact on future protocol design include the presence and size of foci of endodermal sinus tumors within low-stage immature teratomas at all sites. Accurate staging will grow more critical in future protocols. The ability to recruit international cooperative groups will determine the success of chemotherapy tailored to specific subgroups that now must be lumped for statistical purposes. Lastly, companion biologic studies will be critical to defining the different subtypes of germ cell tumors and to determining predictors of biologic behavior.     

Connective Tissue Growth Factor Expression in Pediatric Myofibroblastic Tumors

Human connective tissue growth factor (CTGF) is a secreted cysteine-rich peptide and a member of the peptide family that includes serum-induced immediate gene products such as a v-src-induced peptide and a putative proto-oncogene, c-src. CTGF is secreted by endothelial cells, fibroblasts, smooth muscle cells, and myofibroblasts. Its expression is increased in various human and animal fibrotic diseases. We hypothesized that tumors with significant fibrous and vascular components would exhibit increased expression of CTGF. We examined the expression of CTGF mRNA by in situ hybridization in 12 pediatric tumors and tumor-like conditions, including angiofibroma, malignant fibrous histiocytoma, infantile myofibromatosis, and malignant hemangiopericytoma. All the tumors showed moderate to intense CTGF expression in tumor cells and/or endothelial cells of the associated vasculature. Angiofibromas expressed CTGF only in factor VIII–positive endothelial cells and vascular smooth muscle cells. In contrast, infantile myofibromatosis, malignant hemangiopericytomas, and fibrous histiocytomas expressed CTGF in both endothelial cells and in vimentin-positive tumor cells, particularly those around the blood vessels. CTGF mRNA was not detected in the inflammatory cells observed in many of the tumors. The presence of CTGF in the endothelial cells and tumor cells around blood vessels raises the possibility that CTGF is involved in the pathogenesis of these myofibroblastic tumors. Received December 13, 1999; accepted May 23, 2000.