Solid tumors of childhood

Objective : Cancer is one of the leading causes of death in children. There is the need to have the histologic review of malignancies in children from the Indian sub–continent.Methods : In the present study, malignant tumors received over 12 years were reviewed and re–classified according to classifications based on prognosis.Results : A total of 472 tumors were received over 12 years. Of these 318 were benign and 154 malignant. The commonest malignant solid tumor was lymphoma followed by pediatric renal tumors. The sarcomas included bone tumors, Rhabdomyosarcoma and synovial sarcoma. There were 13 germ cell tumors, 10 retinoblastomas and six neuroblastomas.Conclusion: The review revealed that a definite diagnosis or classification was not assigned in 21 cases in the original reporting. Of these 14 could be assigned a definite category on review and immuno-staining. These included five non-Wilms sarcomas, four Rhabdomyosarcomas, three Ewing’s sarcoma/ PNETs and two Synovial sarcomas. The study also revealed an unexpected high percentage (11%) of epithelial malignancies in children     

小儿恶性实体肿瘤的发病与治疗现状

目的 探讨我国小儿恶性实体肿瘤的发病特点和治疗现状。方法 总结报道小儿实体肿瘤协作组２０家医院近２年的临床资料,统计分析登记病例的性别,年龄和病理类型,常见恶性实体肿瘤的临床分期,治疗方法,手术切除率和转移情况。结果 ４２６例小儿恶性肿瘤,男女比例１．９６：１;３１０例在６岁之内发病,其中５０．７％在３岁之内发病;胚胎性恶性肿瘤占６１．０％,而上皮性癌肿仅占３．８％;常见的神经母细胞瘤,肾母细胞瘤     

High-Dose Cyclophosphamide, Adriamycin, And Vlncrlstlne (Hd-Cav) In Children with Recurrent Solid Tumor

A dose-intensive regimen of cyclophosphamide (140 mg/kg over 2 days), doxorubicin (Adriamycin, 75 mg/m² over 3 days), and vincristine (1 mg/m² on days 1,2, and 3 and 1.5 mg/m² on day 9) was tested in 18 children and adolescents with poor-prognosis recurrent or refractory solid tumors. Nine were affected by neuroblastoma, 3 by Ewing's tumors, 2 by rhabdomyosarcoma, 2 by synovial sarcoma, 1 by hepatocellular carcinoma, and 1 by osteogenic sarcoma. All enrolled patients were heavily pretreated, including 2 patients after bone marrow transplantation. Forty courses were applied (median, 2). The overall response rate was 33% (2 complete remissions and 4 partial remissions). Responses were obtained in children with neuroblastoma, Ewing's tumors, and hepatocellular carcinoma. Myelosuppression [World Health Organization (WHO) grade IV after all courses] and cardiac toxicity (3 WHO grade I,5 WHO grade III, and 3 WHO grade IV) were the main side effects. Nephrotoxicity and hepatoxicity were not obserued. With further therapy consisting of surgery, radiotherapy, and high-dose chemotherapy [cisplatin, carboplatin/etoposide (VP16), or ifosfaamide/VP16 with or without autologous stem cell reinfusion after conditioning with melphalan/VP16/carboplatin], 3 complete remissions and 5 very good partial remissions were obtained. Ten of 18 Patients are alive after a median follow-up of 16 months.     

High-Dose Cyclophosphamide,Adriamycin, And Vlncrlstlne (Hd-Cav) In Children with Recurrent Solid Tumor

A dose-intensive regimen of cyclophosphamide (140 mg/kg over 2 days), doxorubicin (Adriamycin, 75 mg/m² over 3 days), and vincristine (1 mg/m² on days 1,2, and 3 and 1.5 mg/m² on day 9) was tested in 18 children and adolescents with poor-prognosis recurrent or refractory solid tumors. Nine were affected by neuroblastoma, 3 by Ewing's tumors, 2 by rhabdomyosarcoma, 2 by synovial sarcoma, 1 by hepatocellular carcinoma, and 1 by osteogenic sarcoma. All enrolled patients were heavily pretreated, including 2 patients after bone marrow transplantation. Forty courses were applied (median, 2). The overall response rate was 33% (2 complete remissions and 4 partial remissions). Responses were obtained in children with neuroblastoma, Ewing's tumors, and hepatocellular carcinoma. Myelosuppression [World Health Organization (WHO) grade IV after all courses] and cardiac toxicity (3 WHO grade I,5 WHO grade III, and 3 WHO grade IV) were the main side effects. Nephrotoxicity and hepatoxicity were not obserued. With further therapy consisting of surgery, radiotherapy, and high-dose chemotherapy [cisplatin, carboplatin/etoposide (VP16), or ifosfaamide/VP16 with or without autologous stem cell reinfusion after conditioning with melphalan/VP16/carboplatin], 3 complete remissions and 5 very good partial remissions were obtained. Ten of 18 Patients are alive after a median follow-up of 16 months.     

Solid organ transplantation in HIV-infected recipients

HIV is generally regarded as an acceptable reason to exclude a potential recipient from consideration for transplantation. Most of the data in the literature regarding transplantation of HIV sero-positive individuals pertains to the time prior to the administration of Highly Active Anti-Retroviral Therapy (pre-HAART). This data, therefore, provides little guidance for the management of HIV-positive individuals in the current era. The development of HAART has resulted in a decreased mortality. With prolonged survival more HIV-infected individual are developing end stage organ disease from co-existing conditions such as HCV and HBV, and diseases common in the general population such as diabetes mellitus and hypertension. This has lead to clinicians, researchers and patients to actively investigate the role of solid organ transplantation in HIV-infected individuals. In this article We review the literature to date in liver and renal transplantation, including more recent data in patients receiving HAART.     

Solid tumors in the neonatal period

From 1972 to 2000, 123 patients with solid tumors whose complaints had started in the first 28 days of life were retrospectively evaluated. Fifty-five patients were diagnosed in the first 28 days and 68 patients were diagnosed after 28 days. In the former group, 85.5% of patients had symptoms in the first day of life. In the latter group, 77.9% had the onset of symptoms in the first day. Tumor subgroups in the neonatal period included teratoma (34), neuroblastoma (11), rhabdomyosarcoma (3), Wilms tumor (1), and retinoblastoma (3), and the others (3). Three patients had other, less common tumors. In the second group the numbers were the following: for teratoma (32), neuroblastoma (15), germ cell tumors other than teratomas (8), rhabdomyosarcomas (4), the other soft tissue sarcomas (3), Wilms tumor (1), retinoblastoma (1), and other, rare tumors (4). There were 22 malignant tumors in the first group, and 44 in the second group. Fourteen patients in the first group died in the early postoperative period or with progressive disease. Nineteen of 44 patients died in the second group. Overall survival rates were 24.9% and 51.6% in first and second groups, respectively (p = 0.015). Event-free survival rates were 14.7% and 47.7% in these groups, respectively (p = 0.0063). This is the first report comparing clinical features and prognosis of tumors diagnosed in the first 28 days of the life with those diagnosed after 28 days. The prognosis was worse in infants diagnosed in the first 28 days of life.     

Solid tumors preceding or following sarcoidosis

Eight cases of sarcoidosis diagnosed after a preceding malignant solid tumor are presented. An analysis of these eight cases along with four previously reported cases was unable to answer the question whether in some cases the development of sarcoidosis may be triggered or modified by a preceding treatment of a malignant tumor. However, in the reverse situation--sarcoidosis preceding cancer--an analysis of 23 such cases from the literature suggests that sarcoidosis preceding cancer appears to be of the chronic active variety and that lung and breast cancer may occur more often than expected after sarcoidosis. Thus, it is conceivable that the immunologic abnormalities associated with sarcoidosis may somehow promote the development of certain malignant tumors. True sarcoidosis may be confused with local sarcoid reactions in cancer patients. One such case is reported in which the finding of a regional sarcoid reaction to a solitary lung metastasis from a uterine adenocarcinoma led to insufficient treatment in the belief that the patient had systemic sarcoidosis.     

小儿常见恶性实体肿瘤耐药性分析

目的 通过对小儿常见的神经母细胞瘤、肾母细胞瘤、横纹肌肉瘤和恶性畸胎瘤的药敏检测,讨论各种常用化疗方案的治疗价值。方法 用17例肿瘤标本,其中神经母细胞瘤5例,化疗方案CCSG。肾母细胞瘤4例,化疗方案为DD方案。横纹肌肉瘤5例,CYVADIC:方案。恶性畸胎瘤3例,PVB方案。用流式细胞术荧光标记法,测定化疗药物敏感性。分析化疗疗效与耐药性。 结果 神经母细胞瘤对烷化剂类(CTX,IFO)、抗代谢药物(ACD)、VM26、TAL敏感性较高(P<0.05),化疗方案与药敏符合率约50％。肾母细胞瘤敏感的药物较多,化疗方案与药敏结果符合率约66.7％。以上两者疗效较好。横纹肌肉瘤耐药性较强。恶性畸胎瘤药敏对化疗方案符合率仪33_3％。两者疗效较差。结论 小儿实体恶性肿瘤现行化疗方案与肿瘤实际的药敏结果有一定的差距。用流式细胞仪测定肿瘤药物敏感性对预测化疗疗效及个体化化疗有指导意义     

Growth after Treatment of Solid Tumours in Childhood

ABSTRACT. We examined 90 survivors of childhood solid tumours diagnosed in our hospital between 1960 and 1976. Their ages at the time of this study ranged from 12.2 to 41.5 years. Adult standing height was usually normal. However, final standing height was less than expected in the females, and sitting height was below the normal mean in the males. The males who had received both chemotherapy and radiation therapy to the spine had a greater decrement in sitting height, but we did not find any association between radiation therapy to the spine without chemotherapy and subsequent total growth of the spine as measured by sitting height. We conclude that these children generally do not experience any major growth disturbances.     

小儿胰腺乳头状囊性实性肿瘤

目的：探讨小儿胰腺乳头状囊性实性肿瘤的发病率、临床病理、免疫组化、诊断和治疗特点。方法：手术及病理诊断11例。肿瘤标本进行HE、PAS染色及免疫组化染色，抗体选用神经元特异性烯醇化酶（NSE）、α-1抗胰蛋白酶（α-1AT）、S-100、嗜铬粒素A(CgA)、细胞角蛋白（CK）、上皮膜抗原（EMA）、胰岛素。结果：11例患儿中，男1例，女10例，年龄9-14岁，平均年龄11.1岁。该肿瘤占儿童胰腺实体瘤的33.3％。多以腹痛、腹块为首发症状，肿瘤多位于胰头，瘤体较大，平均直径8.7cm。6例行肿瘤切除术，2例行远端胰腺切除术，3例行胰十二指肠切除术。11例患儿生存至今，随访1-15年。镜下观察：11例肿瘤均以实性区、囊性区及乳头状突起为其特征。免疫组化：α-1抗胰蛋白酶标志阳性率100％，神经元特异性烯醇化酶66.7％、S-10028.6%,PAS染色均呈阳性。结论：胰腺乳头状囊性实性肿瘤位居儿童胰腺实体瘤第二位，多发生于女孩，具有良性临床病程，手术治愈率高，预后良好。胰腺囊实性肿瘤起源尚有争议，有等进一步研究。     

恶性实体瘤患儿血脂蛋白质及氨基酸代谢变化

目的：探讨恶性实体瘤患儿血脂、蛋白质及氨基酸代谢规律，方法：２０例未经手术及化、放疗的恶性实体瘤患儿，取外周静脉血，以常规生化方法测定血脂、血清蛋白、氨基酸自动分析仪测定氨基酸谱。结果：血清高密度脂蛋白浓度降低；血清白蛋白、前白蛋白均降低。结论：恶性实体瘤患儿存在蛋白质营养不良，应予营养支持，因高密度脂蛋白水平低，脂肪乳剂的应用宜慎重。谷氨酰胺－谷氨酸、亮氨酸、组氨酸及牛磺酸浓度的降低反映了小儿恶