N-myc gene copies in children with neuroblastoma and its clinical significance / 中国实验血液学杂志

This study was purposed to analyze the relation of N-myc gene copy number with clinical staging, pathological types and tumor biological factors in children with neuroblastoma (NB), and to investigate the influence of chemotherapy on N-myc gene expression and explore the relationship of N-myc gene copies with prognosis of NB children. The newly diagnosed children with NB from 1 March 2007 to 31 January 2011 were enrolled in this study. The treatment was carried out by BCH-NB-2007 based on Hongkong NB-07 protocol, and the patients were follow up to 31 January 2012. The N-myc gene in NB children was detected by FISH. According to number of N-myc gene copies, the NB children were divided into 3 groups. A group (N-myc gene negative) had less than 2 copies, B group (N-myc gene gains) had 3 to 9 copies, and C group (N-myc amplification) had more than 10 copies. The results showed that the N-myc gene expression in 58 cases of NB was observed. There were 36 males and 22 females. NB children aged from 6.5 to 138 months (median age 47.5 months), all patients were followed up for 11 - 57 months with an average of 31.5 months. INSS stages I-IV were 1, 5, 8 and 44 cases, respectively. Twenty-five cases had primary post mediastinal tumor, thirty-three cases had retroperitoneal and pelvic tumor, three of which also companied with post mediastinal tumor. Thirty-five cases had bone metastasis (60.3%), thirty-two cases had bone marrow metastasis (55%). Of the 54 patients with fully known biologic features, seventeen cases had ganglioneuroblastoma, thirty-seven cases had neuroblastoma (15 displayed differentiated, 7 poorly differentiated or undifferentiated, 15 with pathological changes after chemotherapy), four cases had bone marrow metastasis only detected by bone marrow biopsy. Eleven cases had N-myc gene negative, forty-three had N-myc gains, four had N-myc amplification. The average copy number of N-myc gene copies in 58 cases was 5.96 ± 7.81 in which 28 children were non chemotherapy cases, their average copy number was 4.00 ± 1.88, thirty cases out of 58 cases received preoperation chemotherapy (chemotherapy group), and their average copy number was 7.80 ± 10.46, the difference is significant (P = 0.064). The clinic stage, the location of primary tumor, pathological classification, urine VMA and serum neurogenic specific enolase had no effects on the N-myc gene expression, but the serum LDH level had influence (P < 0.01). Single factor Kaplan-Meier analysis showed that the number of N-myc gene copies in NB patients were closely related with the poor prognosis. The more copies of N-myc gene, the more poor prognosis, the difference is statistically significant (P < 0.05). It is concluded that the number of N-myc gene copies correlates with the rapid growth of NB and its poor prognosis, detecting the N-myc amplification can help to estimate the prognosis and decide the program of treatment. Serum LDH, which correlated with the rapid growth of NB, had effect on the N-myc gene expression and is closely related with the poor prognosis of NB.

Lymphangioma of the gallbladder in childhood: a case report and review of the literature / 中华医学杂志(英文版)

Lymphangiomas are malformations of the lymphatic system, which is a common disease in children, usually superficial in body, but seldom seen in visceral organs. We present the case of a child with lymphangioma of the gallbladder. Also we reviewed the literatures. A 2-year-6-month-old boy complained of progressing difficulty in walking for 6 months. Cerebral MRI showed abnormal signals in the white matter suggesting leukodystrophy. Ultrasound of abdomen showed a mixed-echoic mass in the site of gallbladder. CT scan showed an enlarged gallbladder with increased density. Bloodcounting, liver function, and alpha fetal protein were within normal range. Exploratory laparotomy was done in order to rule out malignancy. The liver was found normal at surgery, and the gallbladder looked enlarged and deformed. The gallbladder wall was thick and edematous, and adherent with liver. The gallbladder was excised. Pathological examination gave the diagnosis as lymphangioma of the gallbladder wall. The postoperative recovery was uneventful. There was no evidence of recurrence. Searching in literatures, three cases of lymphangioma of gallbladder in adults were found. According to the pathology of lymphangioma and the anatomy of gallbladder, the outcome of this disease should be benign and may be symptomless throughout the life. No death was reported due to lymphangioma of gall bladder, nor severe complications endangering life at any age. The only indication for surgery in the reported cases was to rule out the risk of being malignancy. If MRI or other means can make a definite preoperative diagnosis of lymphangioma, being a stable lesion, surgery would be unnecessary unless there is a progressive organic obstruction causing repeated infection or persistent abdominal pain.

Effect of Chinese drugs combining with chemotherapy on quality of life in 146 children with solid tumor / 中国结合医学杂志

<p><b>OBJECTIVE</b>To explore the effect of Chinese drugs for supporting essence and strengthening Pi ( SESP) combining with chemotherapy on the quality of life (QOL) in the children with solid tumor.</p><p><b>METHODS</b>Using a digital table, 146 children with solid tumor were randomized into two groups, 77 in the control group and 69 in the treated group. They received conventional chemotherapy, but to the patients in the treated group, SESP were administered additionally. The patients' scores of QOL and adverse reaction occurred were compared.</p><p><b>RESULTS</b>The states in the treated group were superior to those in the control group. Statistical analysis showed that in the first year, the two groups were different in terms of somatic function, systemic symptoms, and general status (P<0.05) and also in the occurrence of adverse reaction (P<0.01). In the second year, the difference was shown in aspects of somatic function and adverse reaction occurrence (P<0.05).</p><p><b>CONCLUSION</b>Chinese drugs for SESP combining with chemotherapy could raise tumor patients' QOL by improving their somatic function and clinical symptoms.</p>

Application of ATP based bioluminescence tumor chemosensitivity assay in the chemotherapy of pediatric solid tumor / 中华儿科杂志

<p><b>OBJECTIVE</b>To explore the clinical significance of ATP based bioluminescence in vitro tumor chemosensitivity assay (ATP-TCA) in the chemotherapy of pediatric solid tumor.</p><p><b>METHODS</b>The cell culture technique and ATP-TCA were used to study chemosensitivity assay in specimens from 50 cases who underwent resection surgery for solid tumor (15 malignant neurogenic tumor, 8 malignant germ cell tumors, 10 Wilms' tumors, 10 hepatoblastomas, 6 rhabdomyosarcomas, 1 adrenocortical carcinoma), 8 chemotherapeutic drugs and 8 drug combination schedules were applied in every specimen.</p><p><b>RESULTS</b>(1) Specimens of 46 of 50 pediatric patients with solid tumors were suitable for evaluation and were evaluated, the overall evaluation rate was 92% (46/50). (2) There was the heterogeneity in the chemosensitivity of the solid tumors in vitro. (3) The drug combination schedules of high sensitivity rate of every kind of pediatric solid tumor are as follows: the malignant neurogenic tumor: CBP + EPI + IFO (12/15, 80.0%), VCR + CTX + DDP + DTIC (11/15, 73.3%); malignant germ cell tumor: DDP + VCR + BLM(8/8, 100%), TPTN + ACTD + IFO(8/8, 100%), As2O3 (7/8, 87.5%); Wilms' tumor: VCR + ACTD(6/7, 85.7%), CBP + VP16 (6/8, 75.0%); hepatoblastoma: VCR + CTX + DDP + VP16 (8/9, 88.9%), CBP +IFO + VM26 (7/9, 77.8%), DDP + VP16 + TPTN(7/9, 77.8%); rhabdomyosarcoma: VCR + CTX + DDP + VP16 (5/5, 100%); adrenocortical carcinoma: VCR + CTX + ADM. (4) As2O3 reached a high in vitro sensitive rate of 87.5% (7/8) and 46.7% (7/15) in malignant germ cell tumor and the malignant neurogenic tumor respectively, PTX was sensitive to the malignant neurogenic tumor and rhabdomyosarcoma (40.0% (6/15), 60.0% (3/5)), GEM was sensitive to pediatric malignant germ cell tumor and rhabdomyosarcoma (50.0% (4/8), 60.0% (3/5)).</p><p><b>CONCLUSIONS</b>ATP-TCA is a sensitive method for the chemotherapeutic agents screening of pediatric malignant solid tumor, and ATP-TCA assay results correlated well with clinical response. It appears to be useful in screening new drugs for pediatric solid tumor, exploring the possible combination plots and principles, evaluating the efficacy of existing chemotherapy, and optimize chemotherapy on an individual basis.</p>

Effect of supporting the healthy energy and strengthening Pi principle of TCM combined with chemotherapy in treating children with solid tumors / 中国中西医结合杂志

<p><b>OBJECTIVE</b>To study the clinical validity in improving quality of life (QOL) of patients and alleviating adverse reaction of chemotherapy in treating children with solid tumors by Chinese herbal medicine for supporting healthy energy and strengthening Pi.</p><p><b>METHODS</b>A prospective cohort study was conducted in 60 children with solid tumor in stage II-IV, who were assigned to two groups, 30 in each group. All children received chemotherapy and those in the observed group were given Chinese herbs according to syndrome differentiation additionally. The conditions of the two groups were compared after 6-month treatment.</p><p><b>RESULTS</b>Compared with before treatment, the white blood cells (WBC), hemoglobin (Hb), and platelet (PLT) all increased in the observed group after treatment (P < 0.01, P < 0.01, P < 0.05), while in the control group, the WBC and Hb have no significant difference (P > 0.05) and PLT decreased (P < 0.05) after treatment. Comparison between the two groups, the clinical symptom score in the observed group has significant difference after treatment (P < 0.05).</p><p><b>CONCLUSION</b>Chinese herbal medicine for supporting healthy energy, strengthening Pi and supplementing qi-blood is good for alleviating the adverse reaction and improving patients' peripheral blood picture in children with solid tumor undergoing chemotherapy.</p>

Effect of Preoperative Chemotherapy on Malignant Extragonadal Germinoma in Children / 实用儿科临床杂志

Objective To explore the effect of preoperative chemotherapy for children with malignant extragonadial germinoma. Methods Twenty patients with malignant extrogonadal germinoma had been treated with preoperative chemotherapy from Mar.2003 to Mar.2007.Protocol 1 of chemotherapy was to improve PEB:(cisplatin plus etoposide plus bleomycin A5);Protocol 2 was VAC(vincristine plus actinomycin D plus cyclophosphamide) the average time span of the chemotherapy was 16 weeks.Upon completion of chemotherapy,tumor resection was performed.The content of alpha fetoprotein(AFP) was determined before operation and compared with the content before chemotherapy.Re-gular follow-up procedure was taken after operation. Results All 20 patients received chemotherapy and underwent operation.The removal rate of resection operation was 100%,the process of operation was smooth.The volume of tumors in 14 patients decreased over 50% compared with those before chemotherapy.The response rate was 70%.The content of AFP decreased significantly in 15 patients(75%).Fifteen patients had been living without tumor for up to 2 years,for 5 cases the survival time went beyond 5 years. Conclusions Malignant extrogonadal ger-minoma in children grow infiltratively,bring detrimental effects to neighboring organs,and bring obstacles to resection operation.Chemotherapy for children with malignant extragonadal germinoma before operation can reduce volume of tumor and bleeding during operation,raise the resectability rate and lower the content of AFP.It will enhance the effectiveness of operation and improve prognosis.Therefore,it can be used as a conventional clinical method.