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1.
Medicine (Baltimore) ; 100(2): e23717, 2021 Jan 15.
Article in English | MEDLINE | ID: mdl-33466124

ABSTRACT

ABSTRACT: Renal cell carcinoma (RCC) is infrequent in the pediatric population. In addition, till date, only a few reports have summarized the characteristics of pediatric RCC and differences between pediatric and adult RCC. Therefore, the current study aimed to investigate the clinical characteristics of RCC in children and adolescents, and identify the differences between children and adolescent patients and adult patients through literature retrieval.The data of 13 pediatric patients diagnosed with RCC at the Children's Hospital of Zhejiang University School of Medicine between 2005 and 2019 were retrospectively analyzed.Three patients were aged <5 years, 2 were aged 6 to 10 years, and 8 were aged 11 to 18 years. Among the 13 patients, common clinical manifestations included abdominal pain in 5 patients, gross hematuria in 4, and an abdominal mass in 1, while the other 3 patients were incidentally detected after an abdominal contusion. The pathological types were microphthalmia family translocation RCC in 9 patients, clear-cell RCC in 2, papillary RCC in 1, and unclassified in 1. All the children underwent radical nephrectomy, including 2 patients with advanced disease who underwent preoperative transcatheter arterial chemoembolization. The mean follow-up time was 58.6 months. Two patients died after 4 and 17 months of follow-up, respectively.In conclusion, microphthalmia family translocation renal cell carcinoma is the predominant type of pediatric RCC associated with advanced tumor stage. The early diagnosis and treatment of pediatric patients is important for improving prognosis. Nevertheless, future studies are urgently needed to determine the treatment for pediatric advanced RCC to increase the survival rate.


Subject(s)
Carcinoma, Renal Cell/pathology , Kidney Neoplasms/pathology , Adolescent , Carcinoma, Renal Cell/surgery , Child , Child, Preschool , Female , Humans , Kidney Neoplasms/surgery , Male , Nephrectomy/methods , Retrospective Studies
2.
World J Clin Cases ; 8(1): 194-199, 2020 Jan 06.
Article in English | MEDLINE | ID: mdl-31970187

ABSTRACT

BACKGROUND: Neuroblastoma is an extracranial malignant tumor in children that is most often located in the adrenal gland and sympathetic ganglion. Here, we present a rare case of neuroblastoma originating from the urinary bladder. CASE SUMMARY: A 3-year-old girl presented with lower abdominal pain with micturition. Ultrasound revealed a lower abdominal mass. Abdominal computed tomography scan displayed a solitary mass at the top of the urinary bladder. Blood levels of neuron-specific enolase and lactate dehydrogenase were elevated. We treated the child with partial cystectomy and six courses of chemotherapy, and the outcome at 4-year follow-up was unremarkable. CONCLUSION: Neuroblastoma should be considered when tumors are located in the urinary bladder, especially in the dome; although this presentation is rare, the prognosis is very good.

3.
J Pediatr Surg ; 54(3): 550-556, 2019 Mar.
Article in English | MEDLINE | ID: mdl-30318310

ABSTRACT

BACKGROUND: Clear cell sarcoma of the kidney (CCSK) is a rare and aggressive malignant renal tumor. We describe our experience with neoadjuvant transcatheter arterial chemoembolization (TACE) and systematic chemotherapy for the treatment of advanced CCSK in children. METHODS: Between January 2010 and December 2016, seven patients (3 boys and 4 girls; median 2.2 years) with advanced CCSK received preoperative TACE of renal artery and systemic chemotherapy. The chemoembolic emulsion for TACE consisted of cisplatin, pirarubicin, vindesine, and iodized oil. Preoperative systemic chemotherapy with vindesine, ifosfamide, and etoposide was administered three weeks after TACE. Nephrectomy was performed three weeks after systemic chemotherapy. After surgery, patients received radiotherapy and postoperative chemotherapy. RESULTS: No cardiotoxicity, renal insufficiency, or hepatic dysfunction was found in any patients. Grade II-III marrow suppression developed in four patients. One patient with tumor progress during neoadjuvant therapy failed to successfully undergo surgery and died. Six patients underwent nephrectomy after neoadjuvant therapy. Median follow-up period was 49.5 months (range, 11-83 months). Five patients have recurrence-free survival. One patient is still in postoperative chemotherapy after nephrectomy, radiotherapy and thoracoscopic resection of lung metastases. CONCLUSIONS: Neoadjuvant TACE and systemic chemotherapy appeared to be feasible in the treatment of advanced CCSK in this pilot study. THE TYPE OF STUDY: A case series with no comparison group. LEVEL OF EVIDENCE: Level IV.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Chemoembolization, Therapeutic/methods , Kidney Neoplasms/therapy , Nephrectomy/methods , Sarcoma, Clear Cell/therapy , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Chemoembolization, Therapeutic/adverse effects , Child , Child, Preschool , Cisplatin/administration & dosage , Cisplatin/adverse effects , Combined Modality Therapy , Doxorubicin/administration & dosage , Doxorubicin/adverse effects , Doxorubicin/analogs & derivatives , Female , Humans , Infant , Iodized Oil/administration & dosage , Iodized Oil/adverse effects , Kidney/pathology , Kidney Neoplasms/pathology , Male , Neoadjuvant Therapy/methods , Nephrectomy/adverse effects , Pilot Projects , Retrospective Studies , Treatment Outcome , Vindesine/administration & dosage , Vindesine/adverse effects
4.
Curr Cancer Drug Targets ; 18(3): 295-303, 2018.
Article in English | MEDLINE | ID: mdl-28359249

ABSTRACT

BACKGROUND: Autophagy is a physiological pathway characterized by lysosomedependent self-digestion to recycle damaged or superfluous cellular content. Deregulation of autophagy hampers the maintenance of cellular homeostasis and contributes to tumorigenesis. However, during anticancer therapy, autophagy activation contributes to development of resistance. Thus autophagy has been recognized as an important pathway and a therapeutic target in cancer. Nephroblastoma (Wilm's tumor) is a common childhood malignancy. The role of autophagy in nephroblastoma is largely uninvestigated. OBJECTIVE: This study is to investigate the change of autophagy level in nephroblastoma, and whether autophagy could be a therapeutic target in anaplastic nephroblastoma. METHOD: In clinical samples of childhood nephroblastoma, autophagy activity was evaluated by the expressions of selected autophagy markers as well as the presence of autophagosome ultrastructure. Use of autophagy inhibitors alone and in combination with conventional chemotherapeutics, was studied both in vivo and in vitro. RESULTS: In nephroblastoma, there was decrease in the Beclin 1 level and the number of autophagosomes, suggesting autophagy inhibition. Furthermore, in two anaplastic nephroblastoma cell lines, G401 and SK-NEP1, autophagy inhibitors further enhanced the efficacy of conventional chemotherapeutics including vincristine and cisplatin. In G401 tumor model established in nude mice, combinational use of chloroquine, an inhibitor of autophagy degradation, further decreased the tumor mass compared with single use of the chemotherapeutics vindesine, although no statistical significance was achieved. CONCLUSION: Our results suggest that autophagy deregulation is involved in nephroblastoma, and targeting autophagy can serve as a potential adjuvant strategy for the highly malignant cases.


Subject(s)
Autophagy , Chloroquine/pharmacology , Cisplatin/pharmacology , Kidney Neoplasms/drug therapy , Vincristine/pharmacology , Wilms Tumor/drug therapy , Animals , Antimalarials/pharmacology , Antineoplastic Agents/pharmacology , Antineoplastic Agents, Phytogenic/pharmacology , Apoptosis , Cell Proliferation , Child , Child, Preschool , Drug Therapy, Combination , Female , Humans , Infant , Kidney Neoplasms/pathology , Male , Mice , Mice, Nude , Tumor Cells, Cultured , Wilms Tumor/pathology , Xenograft Model Antitumor Assays
5.
Pediatr Surg Int ; 33(6): 705-711, 2017 Jun.
Article in English | MEDLINE | ID: mdl-28260193

ABSTRACT

BACKGROUND/PURPOSE: Adrenocortical tumors (ACTs) are rare in childhood. There are no recognized criteria to exactly distinguish between benign and malignant forms, or predict prognosis. The incidence of tumor varies across geographic regions or ethnicities, as well as malignant proportion. The aim of this study is to examine a single institution's experience with pediatric ACTs and to validate the prognostic value of the biologic/pathologic criteria of Wienecke. METHODS: Records of 26 pediatric ACTs between 1994 and 2016 in our center were reviewed retrospectively. The data recorded of each patient included clinical characteristics, treatment, pathologic findings, disease stating, and outcome. Tumors were categorized according to the Wienecke criteria. RESULTS: All patients underwent primary surgical excision, including negative margins in 20 cases. Stage distribution at diagnosis was: ST I 12, ST II 8, ST III 5, and ST IV 1. According to Wienecke scoring system, 13 cases were <3 criteria, 6 cases were =3 criteria, and 7 cases were >3 criteria. At median follow-up of 34.5 months, 18 patients survived without evidence of disease and 8 patients had lethal outcome. There was a strong association between high Wienecke score and both high stage and adverse outcome. CONCLUSIONS: Wienecke criteria can be an appropriate points-scoring system to predict prognosis for adrenocortical tumors in children. Complete surgical resection with negative margins is optimal for survival.


Subject(s)
Adrenal Cortex Neoplasms/pathology , Adrenal Cortex Neoplasms/surgery , Child , Child, Preschool , Female , Humans , Infant , Male , Neoplasm Staging/classification , Prognosis , Retrospective Studies
6.
Clin Chim Acta ; 466: 127-132, 2017 Mar.
Article in English | MEDLINE | ID: mdl-28111273

ABSTRACT

BACKGROUND: Ureteropelvic junction obstruction (UPJO) is the most common cause of hydronephrosis in children. This study was to assess the relationship between serum thioredoxin (S-Trx) and urinary thioredoxin (U-Trx) concentrations and severity of children hydronephrosis caused by UPJO. METHODS: This study included 156 hydronephrosis children with unilateral UPJO and 80 healthy children. S-Trx and U-Trx concentrations were measured using enzyme-linked immunosorbent assay. U-Trx/creatinine (cr) ratio was calculated. RESULTS: S-Trx and U-Trx concentrations and U-Trx/cr ratio were significantly higher in hydronephrosis children than in healthy children. They were significantly correlated with split renal function, anterior-posterior diameter and Society for Fetal Urology classification, as well as were independently related to the split renal function <39.2%, anterior-posterior diameter>30mm and Society for Fetal Urology grade IV. Under receiver operating characteristic curves, U-Trx/cr ratio showed the higher predictive value compared to S-Trx and U-Trx concentrations. CONCLUSION: Increased S-Trx and U-Trx concentrations, especially U-Trx/cr ratio, are closely associated with the severity of children hydronephrosis, substantializing Trx as a promising biomarker for the progression of children hydronephrosis.


Subject(s)
Hydronephrosis/diagnosis , Severity of Illness Index , Thioredoxins/analysis , Biomarkers/analysis , Case-Control Studies , Child , Child, Preschool , Creatinine/analysis , Disease Progression , Humans , Hydronephrosis/blood , Hydronephrosis/etiology , Hydronephrosis/urine , Thioredoxins/blood , Thioredoxins/urine , Ureteral Obstruction/complications
7.
J Vasc Interv Radiol ; 27(7): 996-1000, 2016 Jul.
Article in English | MEDLINE | ID: mdl-27338497

ABSTRACT

Six patients (aged 3-36 mo) with vaginal tumors (rhabdomyosarcoma and endodermal sinus tumor [EST]; n = 3 each) received intraarterial chemotherapy (IAC) and intravenous chemotherapy. Patients underwent internal iliac artery infusion with cisplatin, pirarubicin, and vindesine. Intravenous chemotherapy with vindesine, ifosfamide, and etoposide was administered after 3 weeks. Vaginal tumors disappeared in all patients after 2 or 3 cycles of alternating therapy. Two patients underwent resection of pelvic metastases. Intravenous consolidation chemotherapy was applied. Four patients were disease-free at a median follow-up of 5.8 years. One patient had pelvic recurrence treated with "salvage" therapy with IAC and surgery and was disease-free for 2.5 years.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Endodermal Sinus Tumor/drug therapy , Neoadjuvant Therapy , Rhabdomyosarcoma, Embryonal/drug therapy , Vaginal Neoplasms/drug therapy , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Biopsy , Child, Preschool , China , Cisplatin/administration & dosage , Disease-Free Survival , Doxorubicin/administration & dosage , Doxorubicin/analogs & derivatives , Drug Administration Schedule , Endodermal Sinus Tumor/diagnostic imaging , Endodermal Sinus Tumor/secondary , Endodermal Sinus Tumor/surgery , Etoposide/administration & dosage , Female , Humans , Ifosfamide/administration & dosage , Iliac Artery , Infant , Infusions, Intra-Arterial , Infusions, Intravenous , Metastasectomy , Pelvic Neoplasms/drug therapy , Pelvic Neoplasms/secondary , Pelvic Neoplasms/surgery , Retrospective Studies , Rhabdomyosarcoma, Embryonal/diagnostic imaging , Rhabdomyosarcoma, Embryonal/secondary , Rhabdomyosarcoma, Embryonal/surgery , Time Factors , Tomography, X-Ray Computed , Treatment Outcome , Vaginal Neoplasms/diagnostic imaging , Vaginal Neoplasms/pathology , Vindesine/administration & dosage
8.
European J Pediatr Surg Rep ; 3(2): 90-3, 2015 Dec.
Article in English | MEDLINE | ID: mdl-26788456

ABSTRACT

Background Undifferentiated embryonal liver sarcoma (UELS) accounts for only 9 to 15% of all malignant liver tumors in children. Typically, UELS occurs in older children and presents as an abdominal mass. Most UELS are unresectable because of the later diagnosis. The outcome of UELS is very poor, with a 5-year overall survival of < 37.5%. Transarterial chemoembolization (TACE) has been reported to be an effective modality for unresectable liver tumors. To investigate the effects of TACE on UELS in children, we present two cases of children with UELS who underwent TACE and surgical resection in our center within the past 10 years. Methods In this study, two children with UELS were treated using TACE with cisplatin, doxorubicin, and iodized oil. The size of the tumors was measured before and after TACE using ultrasonography. Routine was also given before and after surgical resection. Side effects were recorded. Both patients had follow-up. Results After interventional therapy, both patients presented with vomiting, fever, and transient liver dysfunction without cardiac or renal dysfunction. One patient had bone marrow depression. The size of the tumors was reduced by 23% to 31% after TACE. The tumors were completely removed by surgical procedures after 4 weeks of TACE in both patients. One patient survived free of disease for 1 year, and the other survived free of disease for 9 years. Conclusion TACE yielded satisfactory results for unresectable UELS in children, with lower dosage of chemotherapy and fewer side effects. It may be applied as a preoperative therapy for children with unresectable UELS.

9.
World J Pediatr ; 10(3): 238-44, 2014 Aug.
Article in English | MEDLINE | ID: mdl-25124975

ABSTRACT

BACKGROUND: Congenital duodenal obstruction (CDO) is one of the most common anomalies in newborns, and accounting for nearly half of all cases of neonatal intestinal obstruction. This study aimed to review our single-center experience in managing congenital duodenal obstruction while evaluate the outcomes. METHODS: We conducted a retrospective analysis of the records of all neonates dianogsed with congenital duodenal obstruction admitted to our center between January 2003 and December 2012. We analyzed demographic criteria, clinical manifestations, associated anomalies, radiologic findings, surgical methods, postoperative complications, and final outcomes. RESULTS: The study comprised 287 newborns (193 boys and 94 girls). Birth weight ranged from 950 g to 4850 g. Fifty-three patients were born prematurely between 28 and 36 weeks' gestation. Malrotation was diagnosed in 174 patients, annular pancreas in 66, duodenal web in 55, duodenal atresia or stenosis in 9, preduodenal portal vein in 2, and congenital band compression in 1. Twenty patients had various combinations of these conditions. Presenting symptoms included bilious vomiting, dehydration, and weight loss. X-rays of the upper abdomen demonstrated the presence of a typical double-bubble sign or air-fluid levels in 68.64% of patients, and confirmatory upper and/or lower gastrointestinal contrast studies were obtained in 64.11%. Multiple associated abnormalities were observed in 50.52% of the patients. Various surgical approaches were used, including Ladd's procedure, duodenoplasty, duodenoduodenostomy, duodenojejunostomy, or a combination of these. Seventeen patients died postoperatively and 14 required re-operation. CONCLUSIONS: Congenital duodenal obstruction is a complex entity with various etiologies and often includes multiple concomitant disorders. Timely diagnosis and aggressive surgery are key to improving prognosis. Care should be taken to address all of the causes of duodenal obstruction and/or associated alimentary tract anomalies during surgery.


Subject(s)
Abnormalities, Multiple , Duodenal Obstruction/diagnosis , Duodenal Obstruction/surgery , Infant, Premature, Diseases/diagnosis , Infant, Premature, Diseases/surgery , Ultrasonography, Prenatal , Duodenal Obstruction/congenital , Duodenal Obstruction/mortality , Female , Follow-Up Studies , Humans , Infant, Newborn , Infant, Premature , Infant, Premature, Diseases/mortality , Male , Retrospective Studies , Risk Assessment , Risk Factors , Survival Analysis , Treatment Outcome
10.
Pediatr Surg Int ; 28(11): 1147-50, 2012 Nov.
Article in English | MEDLINE | ID: mdl-22922948

ABSTRACT

Primary splenic angiosarcoma in children is extremely rare and has a very poor prognosis. We reported a 2.5-year-old boy who had this rare entity and hepatic metastasis. The patient presented with left upper quadrant abdominal mass and anemia. The patient received multidisciplinary treatment and died 32 months after splenectomy.


Subject(s)
Hemangiosarcoma/secondary , Liver Neoplasms/secondary , Splenic Neoplasms/pathology , Child, Preschool , Humans , Male
11.
J Pediatr Surg ; 47(8): 1576-80, 2012 Aug.
Article in English | MEDLINE | ID: mdl-22901920

ABSTRACT

OBJECTIVE: Prepubertal testicular tumors are rare and fundamentally distinct from adult testicular tumors. We reviewed our 11-year experience in a single medical center of China. MATERIAL AND METHODS: This study reports the clinical characteristics, histopathologic diagnosis, treatment methods, and outcome in a series of 63 prepubertal boys who were treated between 1997 and 2008. RESULTS: A total of 63 primary prepubertal testicular and paratesticular tumors were identified. The median age at presentation was 11 months. Of these tumors, 27 (42.9%) were mature teratomas, 5 (7.9%) were immature teratomas, 21 (33.3%) were yolk sac tumors, 4 (6.3%) were epidermoid cyst, 2 (3.2%) were Leydig cell tumors, 1 (1.6%) was a mixed malignant germ cell tumor, and 3 (4.8%) were paratesticular tumors. The most common clinical presentation (95.2%) was a painless scrotal mass or swelling. Forty-eight tumors were treated with radical inguinal orchiectomy, and 15, with a testis-sparing procedure. Follow-up was available in 59 cases, range from 4 to 128 months (median, 50 months). One patient with yolk sac tumor had recurrence and progression to metastasis at the end of 4 months after surgery. Other patients were disease free at last follow-up. CONCLUSIONS: Most of the prepubertal testicular lesions were benign, and the most common histologic subtype was teratoma. Our experience with testis-sparing procedures supports the current trends that less invasive treatment should be performed for benign lesions. This study confirms the excellent cure rates obtained in children with prepubertal testicular tumors.


Subject(s)
Orchiectomy/methods , Testicular Neoplasms/epidemiology , Age of Onset , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biomarkers, Tumor , Child , Child, Preschool , China/epidemiology , Combined Modality Therapy , Endodermal Sinus Tumor/blood , Endodermal Sinus Tumor/drug therapy , Endodermal Sinus Tumor/epidemiology , Endodermal Sinus Tumor/surgery , Epidermal Cyst/epidemiology , Epidermal Cyst/surgery , Genital Neoplasms, Male/diagnostic imaging , Genital Neoplasms, Male/epidemiology , Genital Neoplasms, Male/surgery , Humans , Incidence , Infant , Leydig Cell Tumor/epidemiology , Leydig Cell Tumor/surgery , Male , Neoplasms, Germ Cell and Embryonal/epidemiology , Neoplasms, Germ Cell and Embryonal/surgery , Organ Sparing Treatments , Retrospective Studies , Teratoma/blood , Teratoma/diagnostic imaging , Teratoma/epidemiology , Teratoma/surgery , Testicular Neoplasms/blood , Testicular Neoplasms/diagnostic imaging , Testicular Neoplasms/drug therapy , Testicular Neoplasms/surgery , Treatment Outcome , Ultrasonography , alpha-Fetoproteins/analysis
12.
J Vasc Interv Radiol ; 22(3): 279-86, 2011 Mar.
Article in English | MEDLINE | ID: mdl-21353980

ABSTRACT

PURPOSE: To evaluate the therapeutic effect of preoperative transcatheter arterial chemoembolization (TACE) combined with short-term systematic chemotherapy in the treatment of advanced Wilms tumor. MATERIALS AND METHODS: This was a retrospective study on 66 patients with unilateral advanced Wilms tumor, age 5 months to 11 years (median, 2.9 years; 30 boys and 36 girls), treated at our institution between 1995 and 2007. Characteristics of the patient population were maximal tumor diameter > 10 cm, or involvement of periaortic lymph nodes, or inferior vena cava invasion, or distal metastasis, or tumor with anaplastic histology. Patients were divided into three groups. Twenty patients were treated with conventional preoperative chemotherapy (PC group) using vindesine, actinomycin D, and pirarubicin for 4 weeks; 21 patients were treated in the TACE group with preoperative renal arterial chemoembolization using Lipiodol-pirarubicin-vindesine emulsion; and 25 patients were treated with preoperative chemoembolization combined with short-term systematic chemotherapy (T+S) for 2 weeks. RESULTS: No drug-induced cardiotoxicity, nephrotoxicity, or hepatic dysfunction was observed. Complete surgical removal of the tumor was achieved in 12 (65.0%), 17 (80.9%), and 22 (88.0%) patients in the PC, TACE, and T+S groups, respectively (T+S group vs PC group, P = .030). The 2-year relapse-free survival rates were 65.0%, 80.9%, and 100.0% in the PC, TACE, and T+S groups, respectively (T+S vs PC, P = .001). CONCLUSIONS: From our experience, preoperative chemoembolization combined with short-term systematic chemotherapy is able to achieve higher rates of complete tumor resection and relapse-free survival in the treatment of advanced Wilms tumor.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Chemoembolization, Therapeutic , Kidney Neoplasms/drug therapy , Nephrectomy , Wilms Tumor/drug therapy , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Chemoembolization, Therapeutic/adverse effects , Chemotherapy, Adjuvant , Child , Child, Preschool , China , Dactinomycin/administration & dosage , Disease-Free Survival , Doxorubicin/administration & dosage , Doxorubicin/analogs & derivatives , Ethiodized Oil/administration & dosage , Female , Humans , Infant , Kaplan-Meier Estimate , Kidney Neoplasms/mortality , Kidney Neoplasms/secondary , Kidney Neoplasms/surgery , Male , Neoadjuvant Therapy , Neoplasm Invasiveness , Neoplasm Staging , Retrospective Studies , Survival Rate , Time Factors , Tomography, X-Ray Computed , Treatment Outcome , Vindesine/administration & dosage , Wilms Tumor/mortality , Wilms Tumor/pathology , Wilms Tumor/surgery
13.
Pediatr Radiol ; 38(9): 1021-3, 2008 Sep.
Article in English | MEDLINE | ID: mdl-18535824

ABSTRACT

Radiofrequency ablation (RFA) has been widely reported as a minimally invasive treatment for liver tumours in adults, but has not been documented as a treatment for hepatoblastoma in a child. We report a 2-year-old boy with local recurrence of hepatoblastoma after partial hepatectomy. Percutaneous RFA was performed under real-time sonographic guidance. There was no imaging evidence of recurrence after a follow-up of 2 years. We consider this a promising technique in children.


Subject(s)
Catheter Ablation/methods , Hepatoblastoma/surgery , Liver Neoplasms/surgery , Neoplasm Recurrence, Local/surgery , Humans , Infant , Male , Ultrasonography, Interventional
14.
Zhejiang Da Xue Xue Bao Yi Xue Ban ; 37(1): 83-7, 2008 01.
Article in Chinese | MEDLINE | ID: mdl-18275125

ABSTRACT

OBJECTIVE: To investigate the effect of preoperative transcatherter arterial chemo-embolization (TACE) on the cell proliferation in Wilms; tumor. METHODS: Forty-one cases of Wilms; tumor diagnosed by histopathology were divided into two groups: in TACE group, 23 patients received TACE first and were operated 2 weeks later; in control group, 18 patients were operated alone. A comparative analysis of the pathological finding was made in two groups, and the expression of PCNA and VEGF in tumor tissue was detected by immunohistochemistry. RESULT: The degeneration of tumor tissue such as tumor cell necrotic, broken, disappearance occurred in 17 cases of TACE group and in 4 cases of control group, respectively (P <0.01). The expression of PCNA in TACE group and in control group was 1/23 (4.3 %) and 9/18(50.0 %), respectively (P <0.01). VEGF was expressed in 7/23 (30.4 %) of TACE group and 9/18 (50.0 %) of control group (P=0.283). CONCLUSION: TACE can significantly inhibit proliferation and enhance degeneration of Wilms; tumor cells.


Subject(s)
Cell Proliferation , Chemoembolization, Therapeutic/adverse effects , Kidney Neoplasms/therapy , Wilms Tumor/complications , Wilms Tumor/pathology , Child , Child, Preschool , Female , Humans , Infant , Male , Preoperative Care , Proliferating Cell Nuclear Antigen/metabolism , Vascular Endothelial Growth Factor A/metabolism , Wilms Tumor/surgery , Wilms Tumor/therapy
15.
Zhonghua Nan Ke Xue ; 12(7): 622-4, 2006 Jul.
Article in Chinese | MEDLINE | ID: mdl-16894941

ABSTRACT

OBJECTIVE: To evaluate some currently used surgical approaches to the correction of congenital penile curvature. METHODS: Seventy-six patients with congenital penile curvature underwent surgical correction, of whom 67 were accompanied with hypospadias, 5 with epispadias and 4 with normal urethral meatus. The methods for straightening the phallus included 5-week preoperative hCG treatment, complete degloving of penile skin, release of periurethral fibrous bands extending proximally to the meatus, plication of dorsal or ventral tunica albuginea, and embedding of dermis and tunica vaginalis grafts. RESULTS: All the cases were followed up for 2 months to 2 years, and the mean follow-up time was 9.3 months. Satisfactory phallus straightening was achieved in 67 cases (88%), mild residual chordee remained in 6 (8%), which needed no reoperation for the time being unless warranted by follow-up, and relapse occurred in 3 (4%), which needed further operation. CONCLUSION: Most cases of congenital penile curvature can be corrected sufficiently with the above methods.


Subject(s)
Penis/abnormalities , Urogenital Abnormalities/surgery , Child , Child, Preschool , Follow-Up Studies , Humans , Infant , Male , Urologic Surgical Procedures, Male/methods
16.
Zhonghua Wai Ke Za Zhi ; 44(7): 463-6, 2006 Apr 01.
Article in Chinese | MEDLINE | ID: mdl-16772081

ABSTRACT

OBJECTIVE: To investigate the relationship between the morphological features of different types of neuronal intestinal malformations (NIM) and their postoperative complications. METHODS: The data of morphological and clinical features of 324 cases with NIM were analyzed retrospectively. RESULTS: In all 324 patients, 210 cases were Hirschsprung's disease (HD), 38 intestinal neuronal dysplasia (IND), 45 mixed HD/IND, 8 hypoganglionosis, 22 combined HD/hypoganglionosis and 1 immaturity of ganglion cells. The percentages of normal neuron in bowel of different NIM were 88.1%, 24.4%, 18.4%, 4/8, 27.7% and 0/1 in HD, HD/IND, IND, hypoganglionosis, HD/hypoganglionosis and immaturity of ganglion cells respectively. There were totally 46 cases complicated with recurrent postoperative enterocolitis (EC). Incidence of recurrent postoperative EC in HD patients was 6.7% while in IND/HD and IND patients was 35.6% and 28.9%, respectively. Incidences of EC in cases with the residual IND margins and with the normal margins were 38.2% and 8.7%, respectively. Incidence of EC in cases with transanal endorectal pull-through procedure and with transabdominal procedure was 18.0% and 8.3%, respectively. Nine cases underwent another procedure because of severe persistent constipation or EC after operation, including 4 cases HD/IND, 1 case IND, 3 cases HD and 1 case HD/hypoganglionosis. CONCLUSIONS: Neuron distribution is inconsistent with pathology of NIM. Postoperative EC are rare in the patients only with isolated HD. Furthermore, margins with residual IND and transanal endorectal pull-through procedure are risk factors to recurrent EC. However, the extension of excision about IND is uncertain and need further study.


Subject(s)
Digestive System Abnormalities/pathology , Digestive System Abnormalities/surgery , Enteric Nervous System/abnormalities , Postoperative Complications/etiology , Adolescent , Child , Child, Preschool , Digestive System Abnormalities/complications , Enteric Nervous System/pathology , Female , Hirschsprung Disease/complications , Hirschsprung Disease/pathology , Hirschsprung Disease/surgery , Humans , Infant , Male , Retrospective Studies
17.
World J Gastroenterol ; 12(7): 1136-9, 2006 Feb 21.
Article in English | MEDLINE | ID: mdl-16534860

ABSTRACT

AIM: To investigate the genetic relationship between Hirschsprung's disease (HD) and intestinal neuronal dysplasia (IND) in Chinese population. METHODS: Peripheral blood samples were obtained from 30 HD patients, 20 IND patients, 18 HD/IND combined patients and 20 normal individuals as control. Genomic DNA was extracted according to standard procedure. Exons 11,13,15,17 of RET proto-oncogene were amplified by polymerase chain reaction (PCR). The mutations of RET proto-oncogene were analyzed by single strand conformational polymorphism (SSCP) and sequencing of the positive amplified products was performed. RESULTS: Eight germline sequence variants were detected. In HD patients, 2 missense mutations in exon 11 at nucleotide 15165 G-->A (G667S), 2 frameshift mutations in exon 13 at nucleotide 18974 (18974insG), 1 missense mutation in exon 13 at nucleotide 18919 A-->G (K756E) and 1 silent mutation in exon 15 at nucleotide 20692 G-->A(Q916Q) were detected. In HD/IND combined patients, 1 missense mutation in exon 11 at nucleotide 15165 G-->A and 1 silent mutation in exon 13 at nucleotide 18888 T-->G (L745L) were detected. No mutation was found in IND patients and controls. CONCLUSION: Mutation of RET proto-oncogene is involved in the etiopathogenesis of HD. The frequency of RET proto-oncogene mutation is quite different between IND and HD in Chinese population. IND is a distinct clinical entity genetically different from HD.


Subject(s)
Enteric Nervous System/abnormalities , Germ-Line Mutation , Hirschsprung Disease/genetics , Intestinal Diseases/genetics , Mutation, Missense , Proto-Oncogene Proteins c-ret/genetics , Asian People/genetics , China , DNA/genetics , Exons , Hirschsprung Disease/etiology , Humans , Intestinal Diseases/etiology , Intestines/innervation , Neurons/pathology , Polymerase Chain Reaction , Polymorphism, Single-Stranded Conformational , Proto-Oncogene Mas , Proto-Oncogene Proteins c-ret/physiology
18.
Zhonghua Zhong Liu Za Zhi ; 28(10): 791-5, 2006 Oct.
Article in Chinese | MEDLINE | ID: mdl-17366799

ABSTRACT

OBJECTIVE: To improve prognosis of the patients with advanced Wilms' tumor, the authors compared different therapeutic strategies including preoperative transcatheter arterial chemoembolization (TACE), conventional preoperative chemotherapy and initial surgery. METHODS: Sixty-two patients aged from 5 months to 10 years (mean 3.2 years) were identified from medical records to have histologically confirmed advanced Wilms' tumor during the period from January 1993 to December 2002. The criteria for choice were huge tumor size with a volume more than 550 ml or the mass extending beyond the midline, involvement of vital structures, inferior vena cava invasion, distal metastasis or bilateral Wilms' tumor judged by imaging examination. All cases were divided into 3 groups according to the treatment received: 31 cases in group TACE received preoperative transcatheter arterial chemoembolization with Lipiodol-Epirubicin (EPI)-Vincristine emulsion. One week after TACE, systemic chemotherapy with Actinomycin D (ACTD) was administered and tumor resected at two weeks after TACE. 20 cases in group PC received conventional preoperative chemotherapy with VCR, ACTD plus EPI for 4-5 weeks, and 11 cases in group IS underwent initial surgery. Postoperative treatment for all patients was based on the postoperative staging and tumor histology. RESULTS: In the patients treated with TACE, no drug-induced complications such as cardiotoxicity, nephrotoxicity, hepatic dysfunction or bone marrow suppression were observed except for mild fever due to tumor necrosis. The percentages of tumor size shrinkage were 32.4% and 20.3% in group TACE and group PC, respectively (P < 0.05). Complete surgical removal of the tumor was achieved in 27 patients (87.1%) in group TACE, significantly higher in comparison with 14 in group PC (70.0%, P < 0.05) and 2 in group IS (18.2%, P < 0.01). Event-free survival (EFS) at 2 years was 87.1% (27/ 31), 60.0% (12/20) and 18.2% (2/11), respectivrely. EFS at 4 years was 84.6% (11/13), 56.3% (9/16 ) and 18.2% (2/11) in groups TACE, PC and IS, respectively. CONCLUSION: The present study has shown that both preoperative TACE and conventional preoperative chemotherapy can be applied to the patients with advanced Wilms' tumor who are not candidates for immediately surgical resection. The survival is significantly increased in the patients undergoing preoperativeTACE when compared with conventional preoperative chemotherapy and initial surgery.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Chemoembolization, Therapeutic , Kidney Neoplasms/therapy , Nephrectomy , Wilms Tumor/therapy , Child , Child, Preschool , Combined Modality Therapy , Dactinomycin/administration & dosage , Disease-Free Survival , Epirubicin/administration & dosage , Female , Follow-Up Studies , Humans , Infant , Iodized Oil/administration & dosage , Kidney Neoplasms/mortality , Kidney Neoplasms/pathology , Male , Neoplasm Staging , Preoperative Care , Survival Rate , Treatment Outcome , Vincristine/administration & dosage , Wilms Tumor/pathology
19.
Pediatr Surg Int ; 21(8): 635-40, 2005 Aug.
Article in English | MEDLINE | ID: mdl-16041609

ABSTRACT

Gastric outlet obstruction (GOO) presenting beyond the newborn period is a relatively rare condition, when infantile hypertrophic pyloric stenosis (IHPS) is excluded. This report describes the clinical features, evaluation and management of 18 patients with GOO which was not caused by IHPS. The sex, age, and main presenting features were recorded on admission. Hemoglobin estimation, serum chemistry and blood gas analysis were also assessed in these patients. The diagnosis was confirmed with gastrointestinal barium, B ultrasound, gastroscopy, or at surgery. Some of them underwent gastroscopy with biopsy sampling for rapid urease test or histologic examination with Giemsa stains for Helicobacter pylori. Thirteen patients had peptic ulcer disease, of which six were successfully treated with medications. While the other seven needed operative management which included Heineke-Mikulicz pyloroplasty, gastroduodenostomy or gastrojejunostomy, respectively. All the patients who had Heineke-Mikulicz pyloroplasty or gastrojejunostomy developed bile reflux gastritis that could be alleviated with omeprazol. None of the patients developed dumping syndrome, failing to thrive, or anemia. Three children with antral diaphragm recovered without any complications by diaphragm excision with Heineke-Mikulicz pyloroplasty. The pylorus was compressed by fibrotic band in one 30-month boy, and he recovered uneventfully with Heineke-Mikulicz pyloroplasty during 10-year follow-up. Gastric outlet obstruction secondary to ingestion of sulfuric acid was noted in one patient, and he was successfully treated with gastroduodenostomy. Our data suggests that the satisfactory results could be expected in children with GOO with different therapeutic modalities based on the specific cause and degree of obstruction.


Subject(s)
Gastric Outlet Obstruction/etiology , Gastric Outlet Obstruction/therapy , Adolescent , Child , Child, Preschool , Female , Gastric Outlet Obstruction/diagnosis , Humans , Infant , Male , Retrospective Studies , Treatment Outcome
20.
Zhonghua Nan Ke Xue ; 11(5): 368-70, 2005 May.
Article in Chinese | MEDLINE | ID: mdl-15934463

ABSTRACT

OBJECTIVE: To evaluate a new method of urethroplasty. METHODS: A total of 212 patients with hypospadias aged from 4 months to 9 years (glans type, n = 8; penis type, n = 181, penoscrotal type, n = 23) underwent tubularized incised plate urethroplasty. RESULTS: Followed up for 2 approximately 34 months, 188 cases obtained optimal functional and cosmetic results, 22 developed small fistulae and 16 were cured after a second operation 6 months later, 6 waited to be operated again, 2 cases of neourethra dehiscence were cured by second tubularized incised plate urethroplasty one year later. CONCLUSION: With its simplicity, low complication rate and reliable creation of a normal-appearing glanular meatus, tubularized incised plate urethroplasty can particularly be applied to boys with proximal hypospadias absent of severe penile curvature.


Subject(s)
Hypospadias/surgery , Urethra/surgery , Child , Child, Preschool , Follow-Up Studies , Humans , Infant , Male , Penis/surgery , Treatment Outcome
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