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1.
Eur J Surg Oncol ; 48(1): 283-291, 2022 Jan.
Article in English | MEDLINE | ID: mdl-34489122

ABSTRACT

INTRODUCTION: Surgery plays a key role in the management of Neuroblastic tumours (NB), where the standard approach is open surgery, while minimally invasive surgery (MIS) may be considered an option in selected cases. The indication(s) and morbidity of MIS remain undetermined due to small number of reported studies. The aim of this study was to critically address the contemporary indications, morbidity and overall survival (OS) and propose guidelines exploring the utility of MIS for NB. MATERIALS & METHODS: A SIOPEN study where data of patients with NB who underwent MIS between 2005 and 2018, including demographics, tumour features, imaging, complications, follow up and survival, were extracted and then analysed. RESULTS: A total of 222 patients from 16 centres were identified. The majority were adrenal gland origin (54%) compared to abdominal non-adrenal and pelvic (16%) and thoracic (30%). Complete and near complete macroscopic resection (>95%) was achieved in 95%, with 10% of cases having conversion to open surgery. Complications were reported in 10% within 30 days of surgery. The presence of IDRF (30%) and/or tumour volume >75 ml were risk factors for conversion and complications in multivariate analysis. Overall mortality was 8.5%. CONCLUSIONS: MIS for NB showed that it is a secure approach allowing more than 95% resection. The presence of IDRFs was not an absolute contraindication for MIS. Conversion to open surgery and overall complication rates were low, however they become significant if tumour volume >75 mL. Based on these data, we propose new MIS guidelines for neuroblastic tumours.


Subject(s)
Abdominal Neoplasms/surgery , Adrenal Gland Neoplasms/surgery , Ganglioneuroblastoma/surgery , Ganglioneuroma/surgery , Minimally Invasive Surgical Procedures/methods , Neuroblastoma/surgery , Pelvic Neoplasms/surgery , Thoracic Neoplasms/surgery , Abdominal Neoplasms/pathology , Adrenal Gland Neoplasms/pathology , Child , Child, Preschool , Conversion to Open Surgery , Female , Ganglioneuroblastoma/pathology , Ganglioneuroma/pathology , Humans , Infant , Male , Neuroblastoma/pathology , Pelvic Neoplasms/pathology , Practice Guidelines as Topic , Thoracic Neoplasms/pathology , Tumor Burden
2.
Radiologe ; 61(7): 619-628, 2021 Jul.
Article in German | MEDLINE | ID: mdl-34143242

ABSTRACT

CLINICAL/METHODOLOGICAL ISSUE: Renal tumors in children are treated according to the guidelines of the Renal Tumor Study Group of the International Society of Pediatric Oncology (SIOP-RTSG). Nephroblastoma is the most frequent renal tumor in children. STANDARD RADIOLOGICAL METHODS: After sonography, magnetic resonance imaging (MRI) is the preferred imaging modality. The task of imaging includes differential diagnosis with the help of morphological and epidemiological criteria. Thorax computed tomography (CT) is introduced for initial staging. METHODOLOGICAL INNOVATIONS: Current studies of diffusion-weighted imaging (DWI)-MRI with analysis of the apparent diffusion coefficient (ADC) histogram indicate the potential to differentiate blastemal or anaplastic high-risk histology nephroblatoma subtypes. Imaging criteria for nephron-sparing surgery are defined and allow an individual therapy option in unilateral and especially in bilateral renal nephroblastoma. PERFORMANCE: In addition to nephroblastoma, the differential diagnosis includes congenital mesoblastic nephroma, malignant rhabdoid tumor of the kidney, clear cell sarcoma and renal cell carcinoma. The diagnosis of nephrogenic rests and nephroblastomatosis is challenging. ACHIEVEMENTS: Diagnostic standardization improves diagnosis and therapy of renal childhood tumors, and new prognostic markers may be introduced in the near future.


Subject(s)
Carcinoma, Renal Cell , Kidney Neoplasms , Wilms Tumor , Child , Humans , Kidney , Kidney Neoplasms/diagnostic imaging , Kidney Neoplasms/surgery , Nephrectomy , Wilms Tumor/diagnostic imaging , Wilms Tumor/surgery
3.
Semin Pediatr Surg ; 29(4): 150941, 2020 Aug.
Article in English | MEDLINE | ID: mdl-32861445

ABSTRACT

Benign liver tumors in children are far less frequent than their malignant counterparts. Recently, there have been advances of diagnostic procedures and novel treatments with improved classification as a result. While malignant pediatric liver tumors have been comprehensively addressed by multicenter international tumor trials, benign tumors have more usually relied upon individualised workup and treatment. Due to the rarity and heterogeneity of these different entities, large studies are lacking. In this article the authors highlight the spectrum of benign liver tumors with special focus on specific clinical features, pathology, and treatment.


Subject(s)
Adenoma/therapy , Focal Nodular Hyperplasia/therapy , Hamartoma/therapy , Hemangioma/therapy , Liver Neoplasms/therapy , Adenoma/pathology , Adenoma/physiopathology , Child , Focal Nodular Hyperplasia/pathology , Focal Nodular Hyperplasia/physiopathology , Hamartoma/pathology , Hamartoma/physiopathology , Hemangioma/pathology , Hemangioma/physiopathology , Humans , Liver Neoplasms/pathology , Liver Neoplasms/physiopathology
4.
Cancer Biomark ; 27(1): 129-137, 2020.
Article in English | MEDLINE | ID: mdl-31771043

ABSTRACT

OBJECTIVE: The EDIM (Epitope detection in monocytes) blood test is based on two biomarkers Apo10 and TKTL1. Apo10 is responsible for cell proliferation and resistance to apoptosis. TKTL1 plays a major role in anaerobic glycolysis of tumor cells, leading to destruction of the basal membrane and metastasis as well as in controlling cell cycle. For the first time we analyzed Apo10 and TKLT1 in patients with cholangiocellular (CCC), pancreatic (PC), and colorectal carcinoma (CRC). METHODS: Blood samples of 62 patients with CCC, PC, and CRC were measured and compared to 29 control patients. We also investigated 13 patients with inflammatory conditions, because elevated TKTL1 and Apo10 have been previously described in affected individuals. Flow cytometry was used to detect surface antigens CD14+/CD16+ (activated monocytes/macrophages). Percentages of macrophages harboring TKTL1 and Apo10 were determined. A combined EDIM score (EDIM-CS: TKTL1 plus Apo10) was calculated. Results were correlated with serum tumor markers CEA and CA19-9. RESULTS: Patients with CCC had 100% positive EDIM-CS but CEA and CA19-9 were positive in only 22.2% and 70%, respectively. Patients with PC had 100% positive EDIM-CS but positive tumor markers in only 37.5% (CEA) and 72.7% (CA19-9). Patients with CRC had 100% positive EDIM-CS but only 50% positive CEA. EDIM-CS was positive in 100% (62/62) of all cancer patients and in 0% of healthy individuals. Of the individuals with inflammation, 7.7% had a positive EDIM-CS. CONCLUSION: The sensitivity of the EDIM blood test and the comparison with traditional tumor markers indicate that this new test might improve the detection of carcinomas (CCC, PC and, CRC) and might be relevant for the diagnosis of all tumor entities.


Subject(s)
Biomarkers, Tumor/blood , Cholangiocarcinoma/blood , Colorectal Neoplasms/blood , Intracellular Signaling Peptides and Proteins/metabolism , Pancreatic Neoplasms/blood , Transketolase/blood , Aged , Biomarkers, Tumor/immunology , CA-19-9 Antigen/blood , Carcinoembryonic Antigen/blood , Cholangiocarcinoma/pathology , Colorectal Neoplasms/pathology , Epitopes/blood , Epitopes/immunology , Female , Flow Cytometry , Humans , Macrophages/immunology , Male , Middle Aged , Monocytes/immunology , Pancreatic Neoplasms/pathology , Transketolase/immunology , Pancreatic Neoplasms
5.
Chirurg ; 89(3): 205-211, 2018 03.
Article in German | MEDLINE | ID: mdl-29318365

ABSTRACT

The overall survival of children with solid tumors has shown a substantial increase in the past decades due to progress in all of the disciplines involved in the treatment. The poor prognosis for advanced stages of disease and the morbidity related to therapeutic procedures are still a challenge. Innovations in the surgical treatment of solid tumors can contribute to increase the survival rate of affected children and to decrease the treatment-related morbidity. Considering these aspects, the successful implementation of innovations is described based on four examples. (1) Tumor nephrectomy has long been the standard surgical procedure for Wilms tumor/nephroblastoma. Modifications of the surgical technique allow a nephron-sparing resection of the tumor and thereby reducing the long-term effects of nephrectomy. (2) According to the protocols of the International Society of Pediatric Oncology (SIOP) liver transplantation should be used for high-risk hepatoblastoma. Virtual imaging methods based on new software processors enable a more accurate and individual planning of the surgical procedure and an organ-saving extended tumor resection which avoids a transplantation. (3) Mutilating surgical procedures are sometimes necessary for a curative treatment of rhabdomyosarcoma. By combining surgery and brachytherapy mutilating surgical procedures for urogenital rhabdomyosarcomas can be avoided. (4) Pulmonary metastatic nodules can become accessible to minimally invasive resection if CT-guided marking by coil wire is preoperatively performed. In selected cases thoracotomy will be avoided and due to a shorter postoperative course, the time to the subsequent adjuvant chemotherapy will be reduced.


Subject(s)
Kidney Neoplasms , Nephrectomy , Rhabdomyosarcoma , Urogenital Neoplasms , Wilms Tumor , Chemotherapy, Adjuvant , Child , Humans , Kidney Neoplasms/surgery , Nephrectomy/methods , Rhabdomyosarcoma/surgery , Survival Rate , Treatment Outcome , Urogenital Neoplasms/surgery , Wilms Tumor/surgery
6.
World J Urol ; 34(4): 577-83, 2016 Apr.
Article in English | MEDLINE | ID: mdl-26219514

ABSTRACT

PURPOSE: In children, ureteropelvic junction obstruction (UPJO) is mostly caused by intrinsic factors (IUPJO); extrinsic UPJO are rare and often due to crossing vessels (CVs). METHODS: We retrospectively reviewed all data of children with UPJO that underwent surgery in our institution from 2004 to 2011. Analyses included age at surgery, gender, preoperative and postoperative results of ultrasound and renal scans [differential renal function (DRF); signs of obstruction], and pathology reports. Available histological specimens of cases with CV were compared to a random selection of intrinsic cases in a blinded fashion. After additional Masson's trichrome staining, the specimens were scored for fibrosis, muscular hypertrophy, and chronic inflammation. RESULTS: Out of 139 patients with UPJO, 39 cases were associated with CV. Median age at surgery was 68 months (range 2-194) in the CV group and 11.5 months (range 0-188) in IUPJO group. Laparoscopic dismembered pyeloplasty (LDMP) was carried out in 134 and open DMP in five patients. Preoperative ultrasound identified 28/39 cases with CV. DRF below 40 % was more frequently seen in CV patients (p = 0.020). Histological analyses revealed no differences between the CV and IUPJO specimens in total. CV patients with higher grades of muscular hypertrophy had lower preoperative DRF, compared to those with higher preoperative DRF (p = 0.026). Functional recovery after (L)DMP was excellent in both groups. CONCLUSION: We could not find any significant histological differences between CV and IUPJO in children. To obtain excellent functional recovery, surgical procedures with a definite correction of the UPJ should be preferred in paediatric patients with CV.


Subject(s)
Diagnostic Imaging/methods , Kidney Pelvis/blood supply , Recovery of Function , Ureter/blood supply , Ureteral Obstruction/diagnosis , Urodynamics/physiology , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Infant, Newborn , Kidney Pelvis/diagnostic imaging , Male , Prognosis , Retrospective Studies , Severity of Illness Index , Time Factors , Ureter/diagnostic imaging , Ureteral Obstruction/physiopathology
7.
Zentralbl Chir ; 139(6): 607-12, 2014 Dec.
Article in German | MEDLINE | ID: mdl-25531634

ABSTRACT

Peritoneal tumour dissemination represents an advanced tumour stage and survival rates are usually low. In the past, cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) has been established in adults leading to increased survival rates in comparison to chemotherapy alone. CRS and HIPEC are indicated in primary peritoneal tumours such as mesothelioma and pseudomyxoma peritonei, and in peritoneal metastases from gastrointestinal and ovarian cancers in adults. The incidence of peritoneal surface malignancies in children seems to be lower than in adults, but the incidence is unknown. Nevertheless, peritoneal carcinomatosis/sarcomatosis may occur in patients suffering from desmoplastic small round cell tumour (DSRCT), soft tissue sarcoma (rhabdomyosarcoma, leiomyosarcoma, GIST or liposarcoma), as well as in patients with gastrointestinal cancers. CRS and HIPEC have been established as a novel treatment option in children suffering from peritoneal carcinomatosis/sarcomatosis in very few centres worldwide. This paper reviews the indications, treatment regimens, and pitfalls of this approach in children.


Subject(s)
Chemotherapy, Cancer, Regional Perfusion/methods , Cytoreduction Surgical Procedures/methods , Hyperthermia, Induced/methods , Neoadjuvant Therapy , Peritoneal Neoplasms/secondary , Peritoneal Neoplasms/therapy , Child , Combined Modality Therapy , Humans , Peritoneal Neoplasms/mortality , Survival Rate
8.
Pediatr Surg Int ; 29(2): 141-9, 2013 Feb.
Article in English | MEDLINE | ID: mdl-23266718

ABSTRACT

PURPOSE: The proto-oncogene beta-catenin is linked to an abnormal activation of the Wnt/beta-catenin-pathway and shows mutations in 50-90 % of hepatoblastoma (HB). Corresponding, the recently published murine orthotopic HB model differs from the former subcutaneous model by nuclear beta-catenin distribution. As the nuclear localization of beta-catenin is considered to reflect a more aggressive tumor growth, the influence of beta-catenin inhibition on cell viability and drug-efficiency in HB cells was analyzed. METHODS: Beta-catenin distribution in HB cells was analyzed by immunofluorescence. The influence of beta-catenin inhibitors Celecoxib, Etodolac, ICG001, and MET kinase inhibitor (SU11274) alone and in combination with cisplatin (CDDP) on HB cell lines (HuH6, HepT1) was evaluated by cell viability assays and BrdU incorporation. RESULTS: Celecoxib and ICG001 reduced dose-dependently HB cell viability and decreased nuclear beta-catenin in cultivated HB cells. Etodolac was without influence at concentrations up to 100 µM. Combinations of Celecoxib or ICG001 with MET kinase inhibitor or CDDP resulted in additive reduction of cell viability. CONCLUSION: Pharmaceutical beta-catenin inhibitors can modulate the nuclear localization of beta-catenin and reduce cell viability of HB cells in vitro. These promising effects might optimize the outcome of high-risk HB. The orthotopic HB model is a suitable basis for further in vivo studies.


Subject(s)
Antineoplastic Agents/pharmacology , Hepatoblastoma/drug therapy , Liver Neoplasms/drug therapy , beta Catenin/antagonists & inhibitors , Bridged Bicyclo Compounds, Heterocyclic/pharmacology , Celecoxib , Cell Line, Tumor , Cell Proliferation/drug effects , Cell Survival/drug effects , Cisplatin/pharmacology , Etodolac/pharmacology , Hepatoblastoma/metabolism , Hepatoblastoma/pathology , Humans , Liver Neoplasms/metabolism , Liver Neoplasms/pathology , Protein Kinase Inhibitors/pharmacology , Proto-Oncogene Mas , Pyrazoles/pharmacology , Pyrimidinones/pharmacology , Sulfonamides/pharmacology , Tissue Distribution , Tumor Cells, Cultured , beta Catenin/metabolism
9.
Br J Cancer ; 108(2): 334-41, 2013 Feb 05.
Article in English | MEDLINE | ID: mdl-23257893

ABSTRACT

BACKGROUND: Sorafenib has recently been shown to reduce tumour growth in hepatoblastoma (HB) xenografts. The effect of a combined administration with cytostatic agents was now investigated. METHODS: Cell viability after treatment with sorafenib and different cytostatic agents was evaluated in two HB cell lines (HUH6 and HepT1) using MTT assay. ERK signalling was investigated by western blot, NOXA expression by rt-PCR, and formation of DNA adducts using immunocytology. NMRI mice bearing subcutaneous HUH6-derived tumours were treated with sorafenib alone or in combination with cisplatin. Tumour progression, viability, apoptosis, and vascularisation were monitored by tumour volume, AFP levels, TUNEL assay, and CD31 immunostaining, respectively. RESULTS: The combination of sorafenib and cisplatin led to a remarkable decrease in cell viability. The cisplatin-induced enhanced ERK1/2 activation, but not NOXA expression and the formation of DNA adducts was partly abrogated by sorafenib. In HB xenografts, both, sorafenib and alternated application of sorafenib and cisplatin significantly reduced tumour growth (P<0.05). Levels of AFP were lower in both treated groups (P=0.08). Relative apoptotic areas were increased (P=0.003). Mean vascular density was the lowest in the sorafenib/CDDP group (P=0.02). CONCLUSION: The combination of sorafenib with cisplatin might be a promising treatment option for high risk or recurrent HB.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Cisplatin/therapeutic use , Cytostatic Agents/therapeutic use , Hepatoblastoma/drug therapy , Hepatoblastoma/pathology , Niacinamide/analogs & derivatives , Phenylurea Compounds/therapeutic use , Animals , Apoptosis/drug effects , Cell Line, Tumor , Cell Survival/drug effects , Cisplatin/pharmacology , DNA Adducts/metabolism , Enzyme Activation/drug effects , Female , Humans , Liver Neoplasms/drug therapy , Mice , Mice, Nude , Mitogen-Activated Protein Kinase 1/metabolism , Mitogen-Activated Protein Kinase 3/metabolism , Mitogen-Activated Protein Kinase 3/pharmacology , Neovascularization, Pathologic , Niacinamide/pharmacology , Niacinamide/therapeutic use , Phenylurea Compounds/pharmacology , Protein Kinase Inhibitors/pharmacology , Protein Kinase Inhibitors/therapeutic use , Proto-Oncogene Proteins c-bcl-2/biosynthesis , Sorafenib , Xenograft Model Antitumor Assays
10.
Klin Padiatr ; 224(3): 148-52, 2012 Apr.
Article in English | MEDLINE | ID: mdl-22513793

ABSTRACT

Malignant non-Wilms renal tumors (NWRT) are a small but relevant subgroup of renal neoplasms in children. In this study we analyzed corresponding data from the trials SIOP 93-01/GPOH and SIOP 2001/GPOH of the Society of Pediatric Oncology and Hematology.Data of 22 patients with NWRT and primary lung metastases were retrospectively reviewed. Analyses included epidemiology, tumor characteristics, chemotherapy, local treatment, and outcome.The following diagnoses were registered: Malignant Rhabdoid tumor of the kidney (MRTK, n=15), Renal-cell carcinoma (RCC, n=3), Clear-cell sarcoma of the kidney (CCSK, n=3), and primitive neuro ectodermal tumor (PNET, n=1). Median age of patients at diagnosis was 14 months. Overall survival was 36.36% (8/22). Of the 15 children with MRTK 3 survived, 3/3 patients with RCC, 1/3 patients with CCSK, and 1/1 patient with PNET survived. Lung metastases disappeared in 6 patients after initial chemotherapy, 6/8 patients undergoing local treatment of lung metastases (surgery, irradiation, or both) achieved complete remission. Only patients with complete clearance of lung lesions, either through neoadjuvant chemotherapy or subsequent local treatment, survived. Mean Follow up was 31 months (1-137).Survival of patients with stage IV NWRT is dismal. Complete removal of lung metastases seems mandatory for survival. An aggressive diagnostic and therapeutic approach seems justified in affected children.


Subject(s)
Carcinoma, Renal Cell/secondary , Kidney Neoplasms/drug therapy , Lung Neoplasms/secondary , Rhabdoid Tumor/secondary , Sarcoma, Clear Cell/secondary , Adolescent , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Carcinoma, Renal Cell/diagnosis , Carcinoma, Renal Cell/drug therapy , Carcinoma, Renal Cell/pathology , Child , Child, Preschool , Clinical Trials as Topic , Combined Modality Therapy , Disease Progression , Europe , Female , Humans , Infant , Kidney Neoplasms/diagnosis , Kidney Neoplasms/pathology , Lung Neoplasms/diagnosis , Lung Neoplasms/drug therapy , Lung Neoplasms/pathology , Male , Neoadjuvant Therapy , Neoplasm Staging , Neuroectodermal Tumors, Primitive/diagnosis , Neuroectodermal Tumors, Primitive/drug therapy , Neuroectodermal Tumors, Primitive/pathology , Neuroectodermal Tumors, Primitive/secondary , Pneumonectomy , Prognosis , Rhabdoid Tumor/diagnosis , Rhabdoid Tumor/drug therapy , Rhabdoid Tumor/pathology , Sarcoma, Clear Cell/diagnosis , Sarcoma, Clear Cell/drug therapy , Sarcoma, Clear Cell/pathology , Survival Rate
11.
J Urol ; 186(4): 1430-6, 2011 Oct.
Article in English | MEDLINE | ID: mdl-21855944

ABSTRACT

PURPOSE: Bilateral nephroblastoma involving the renal hilus represents a significant surgical challenge. Different operative strategies have been proposed for this condition. We analyzed the outcome of simultaneous bilateral partial nephrectomy for complex stage V nephroblastoma. MATERIALS AND METHODS: We retrospectively analyzed patients with bilateral nephroblastoma involving the renal hilus operated on at our institution between 2002 and 2008. We assessed patient data and surgical and oncologic outcomes. RESULTS: We analyzed data from 5 patients with a median age of 27 months at surgery (range 13 to 58). Two children had additional pulmonary metastases. Patients were treated according to the International Society of Pediatric Oncology 2001/German Society of Pediatric Oncology and Hematology protocol. All children underwent synchronous bilateral nephron sparing surgery (longitudinal partial nephrectomy or enucleation) of the central tumors. Median operating time was 182 minutes (range 129 to 291), with vascular exclusion performed in 4 patients (7 to 25 minutes). Complications consisted of urinary leakage in 2 cases. Postoperative renal function was unimpaired in 4 patients and 1 patient had Wilms tumor/aniridia/genitourinary malformations/retardation syndrome with glomerulopathy. One patient with pulmonary metastases and blastemal histology had multiple pulmonary relapses and died due to pulmonary progressive disease. Four patients are alive without evidence of disease at a median followup of 45 months (range 44 to 73). CONCLUSIONS: Surgery for central stage V nephroblastoma is possible with good functional and oncologic outcomes. The single stage approach is safe, provided that operating and treating physicians have sufficient expertise with such conditions. The ultimate goal is to avoid tumor nephrectomy.


Subject(s)
Kidney Neoplasms/surgery , Neoplasms, Multiple Primary/surgery , Nephrectomy/methods , Wilms Tumor/surgery , Chemotherapy, Adjuvant , Child , Child, Preschool , Female , Humans , Kidney Neoplasms/pathology , Male , Neoadjuvant Therapy , Neoplasms, Multiple Primary/pathology , Wilms Tumor/pathology
12.
Zentralbl Chir ; 134(6): 537-41, 2009 Dec.
Article in German | MEDLINE | ID: mdl-20020386

ABSTRACT

Distant metastases regularly occur in cases of paediatric solid tumours. Overall the most common localisation of metastases is the lung. The correct treatment of lung metastases represents a prognostic factor in most paediatric solid tumours. Guidelines for the therapy of lung metastases are commonly included in the protocols of the different multicentric treatment trials. However, specific surgical aspects are usually not addressed in these protocols. The aim of this article is to present the principles of surgery for lung metastases in paediatric solid tumours. The principles of surgery for metastases of the most common extracranial paediatric solid tumours are presented including aspects of the diagnostic work-up, surgical preparations, and surgical procedures. Special points of interest are discussed focusing on the different entities. Surgery for lung metastases in paediatric solid tumours can be performed with a good surgical outcome regardless of the patients' age. Large numbers of sequelae can be surgically excised. Depending on the localisation sternotomy is an option concerning the surgical approach. Surgery for lung metastases in paediatric solid tumours is a safe method when respecting the principles for the procedure. It has a positive influence on the patients' prognosis. An aggressive surgical procedure may be justified depending on the tumour entity. An interdisciplinary approach including paediatric oncology and radiology is mandatory in any case.


Subject(s)
Lung Neoplasms/secondary , Lung Neoplasms/surgery , Pneumonectomy/methods , Child , Combined Modality Therapy , Cooperative Behavior , Humans , Interdisciplinary Communication , Lung Neoplasms/mortality , Lung Neoplasms/pathology , Multicenter Studies as Topic , Neoplasm Staging , Patient Care Team , Practice Guidelines as Topic , Survival Rate , Thoracoscopy/methods , Thoracotomy/methods
13.
Fetal Diagn Ther ; 26(3): 170-2, 2009.
Article in English | MEDLINE | ID: mdl-19887767

ABSTRACT

We report on a case of a large intra-abdominal teratoma diagnosed antenatally and managed successfully after birth. Intra-abdominal teratomas are rare in prenatal life. Ultrasound examination shows a heterogenic tumor with cystic and solid components. After postpartum surgical removal, the prognosis is generally good.


Subject(s)
Abdominal Neoplasms/diagnostic imaging , Fetal Diseases/diagnostic imaging , Prenatal Diagnosis , Teratoma/diagnostic imaging , Ultrasonography, Prenatal , Abdominal Neoplasms/pathology , Abdominal Neoplasms/surgery , Adult , Cesarean Section , Female , Fetal Diseases/surgery , Humans , Infant, Newborn , Laparotomy , Male , Pregnancy , Prognosis , Teratoma/pathology , Teratoma/surgery
14.
J Urol ; 182(4): 1561-8, 2009 Oct.
Article in English | MEDLINE | ID: mdl-19683760

ABSTRACT

PURPOSE: Minimally invasive procedures are increasingly important in pediatric urology. However, experience is still limited with minimally invasive operations on the upper urinary tract in infants. We analyzed 3 minimally invasive procedures (pyeloplasty, heminephroureterectomy and nephrectomy) in children younger than 1 year. MATERIALS AND METHODS: We analyzed 67 children (mean +/- SD age 5.1 +/- 2.9 months) undergoing minimally invasive pyeloplasty in 26 patients (group 1), heminephroureterectomy in 18 (group 2) or nephrectomy in 23 (group 3) with regard to technical aspects, surgical outcome and complications. Preoperative and postoperative ultrasound and mercaptoacetyltriglycine renal scan were statistically evaluated in groups 1 and 2. RESULTS: Mean +/- SD patient weight was 6.4 +/- 1.8 kg and mean +/- SD operative time was 113.2 +/- 41.6 minutes. Conversion to open surgery was necessary in 1 pyeloplasty. One complication (missed intraoperative bowel perforation) occurred. No blood transfusion was required in any child. After pyeloplasty there were improved tracer clearances (mercaptoacetyltriglycine scan) and improved morphologies of the pyelon (ultrasound) in all patients. In groups 1 and 2 there was no statistical difference between preoperative and postoperative partial function of the affected kidney. Mean +/- SD followup was 32.5 +/- 19.8 months. CONCLUSIONS: Minimally invasive procedures on the upper urinary tract in children younger than 1 year are technically challenging, and require expertise of the surgeon and the entire team. Given these assumptions, such procedures can be safely performed with excellent functional outcomes.


Subject(s)
Kidney Pelvis/surgery , Laparoscopy , Nephrectomy/methods , Ureter/surgery , Humans , Infant , Retrospective Studies , Treatment Outcome , Urologic Surgical Procedures/methods
15.
Ultraschall Med ; 29(4): 383-7, 2008 Aug.
Article in English | MEDLINE | ID: mdl-18528809

ABSTRACT

PURPOSE: To evaluate the usefulness of transperineal sonography of the anal sphincter complex for differentiating between an anteriorly displaced anus, which is a normal anatomical variant, and a low-type imperforate anus with perineal fistula, which is a pathological developmental abnormality requiring surgical repair. MATERIALS AND METHODS: Transperineal sonography was performed with a 13-MHz linear-array transducer on 8 infants (1 day-5.3 months old) who were considered on clinical grounds to have an anteriorly displaced anus and on 9 infants (0-8 months old) with a low-type imperforate anus and perineal fistula confirmed at surgery. The anal sphincter complex was identified and the relationship between the anal canal and the anal sphincter complex was evaluated. RESULTS: Transperineal sonography was feasible for all children without any specific preparation. An anal canal running within an intact sphincter complex was identified in all infants with an anteriorly displaced anus (n = 8). In 8 of 9 infants with a low-type imperforate anus, a perineal fistula running outside the anal sphincter complex was correctly diagnosed by transperineal sonography. In one infant with a low-type imperforate anus, transperineal sonography revealed a deficient anal sphincter complex. CONCLUSION: Transperineal sonography appears to be a useful non-invasive imaging technique for assessing congenital anorectal abnormalities in neonates and infants, allowing the surgeon to select infants who would benefit from surgical repair.


Subject(s)
Anal Canal/diagnostic imaging , Anus, Imperforate/diagnostic imaging , Endosonography , Perineum/diagnostic imaging , Rectal Fistula/diagnostic imaging , Anal Canal/abnormalities , Anal Canal/surgery , Anus, Imperforate/surgery , Diagnosis, Differential , Endosonography/instrumentation , Equipment Design , Feasibility Studies , Female , Humans , Infant , Infant, Newborn , Male , Perineum/surgery , Rectal Fistula/surgery , Reference Values , Sensitivity and Specificity , Transducers
16.
Cell Prolif ; 41(2): 365-74, 2008 Apr.
Article in English | MEDLINE | ID: mdl-18336479

ABSTRACT

OBJECTIVE: The aim of this study was to establish a preclinical mouse model to study metastases of paediatric rhabdomyosarcoma at the macroscopic and cellular levels, with different imaging methods. EXPERIMENTAL DESIGN: The alveolar rhabdomyosarcoma cell line Rh30 was stably transfected with the red fluorescent protein (DsRed2) then was xenotransplanted (intravenous injection [n = 8], and footpad injection [n = 8]) into nude mice (NMRI nu/nu). Macroscopic imaging of metastases was performed using DsRed2-fluorescence and flat-panel volumetric computed tomography scan. In a further series of animals (n = 8), in vivo cell trafficking of rhabdomyosarcoma cells using cellular imaging with an Olympus OV100 variable-magnification small-animal imaging system was used. RESULTS: Metastases in the pelvis, thoracic wall and skin were visualized by fluorescence imaging. Pelvic metastases were found after tail vein injection and at other metastatic sites after footpad injection. Flat-panel volumetric computed tomography scan data allowed highly specific analysis of contrast between tumour and surrounding tissue. Correlation between fluorescence and flat-panel volumetric computed tomography scan imaging data was observed. Single-cell imaging visualized tumour cells in the vessels and demonstrated the arrest of tumour cells at vessel junctions followed by extravasation of the tumour cells. CONCLUSION: We established a model for visualization of experimental metastatic invasion and describe relevant tools for imaging childhood rhabdomyosarcoma metastases at the macroscopic and cellular levels. Imaging of cell trafficking visualized the behaviour of tumour cells and development of metastases by accumulation and extravasation of rhabdomyosarcoma cells.


Subject(s)
Image Interpretation, Computer-Assisted/methods , Imaging, Three-Dimensional/methods , Rhabdomyosarcoma/pathology , Rhabdomyosarcoma/secondary , X-Ray Intensifying Screens , Animals , Cell Movement , Cell Proliferation , Disease Models, Animal , Humans , Image Interpretation, Computer-Assisted/instrumentation , Imaging, Three-Dimensional/instrumentation , Luminescent Proteins/chemistry , Mice , Mice, Nude , Microscopy, Fluorescence , Neoplasm Invasiveness , Neoplasm Metastasis , Neovascularization, Pathologic/pathology , Sensitivity and Specificity , Tumor Cells, Cultured , Red Fluorescent Protein
17.
Zentralbl Chir ; 132(3): 247-50, 2007 Jun.
Article in German | MEDLINE | ID: mdl-17610198

ABSTRACT

There are only few pediatric surgical centers across the world with expertise for minimally invasive anatomical lung resections in children. Between September 2003 and September 2005, 67 children underwent thoracoscopic surgery at the Department of Pediatric Surgery, University Hospital of Tuebingen, Germany. In 19 of these cases a lung resection was carried out, 8 of them had an anatomical lung resection. All patients underwent general anesthesia without selective intubation for the procedure. Intrathoracic pressure with insufflation of carbon dioxide of 1.5 l / min was held at 3-5 mmHg. Two 5 mm ports for video and instruments and one 12 mm port for a stapling device were used. Resected lung specimens were removed from the thorax through an additional 2-3 cm long incision. A bronchoscopy was carried out during surgery in all patients. Median age at operation was 5.6 years (range 3 months-20 years). Median operation time was 150 minutes (range 94-250 min). Conversion to open surgery was performed in 3 cases. This was due to bleeding in one child, due to a stiff lung in another patient with cystic fibrosis and due to a vascular and bronchial malformation in a third child suffering from middle lobe syndrome. There were no postoperative complications. Our preliminary results show, that thoracoscopic lung resections in children can be performed without major complications and excellent cosmetic results. For the necessity of a conversion to open surgery possible reasons may be insufficient intrathoracic overview as well as congenital anomalies of the vascular and / or the bronchial tract. Co-morbidities such as rib-fusion, deformities of the thorax or scoliosis can be avoided using thoracoscopic procedures.


Subject(s)
Lung Diseases/surgery , Pneumonectomy/instrumentation , Thoracic Surgery, Video-Assisted/instrumentation , Adolescent , Adult , Child , Child, Preschool , Female , Germany , Humans , Infant , Intraoperative Complications/surgery , Male , Postoperative Care , Treatment Outcome
18.
Curr Pharm Biotechnol ; 8(2): 93-7, 2007 Apr.
Article in English | MEDLINE | ID: mdl-17430157

ABSTRACT

Treatment results of human hepatoblastoma (HB) have been improved remarkably during recent years, mainly through the establishment of integrated regimens controlled and coordinated by multicentric treatment studies. Today, neoadjuvant and adjuvant chemotherapy is combined with surgical resection of the tumors. The main therapeutic goal is a complete surgical removal of tumor masses, which is also essential for the survival of the patients. Despite improved overall survival rates, treatment results of advanced tumors are still far from being satisfying. Multidrug resistance has been identified as a major factor responsible for the bad prognosis of children suffering from advanced staged hepatoblastoma. During recent years investigations focused on factors contributing to drug resistance in hepatoblastoma and on possible approaches towards overcoming this therapeutical challenge. Alternative approaches that are currently evaluated in experimental and clinical settings comprise new cytotoxic agents, chemosensitizers, gene directed applications but also surgical techniques and an expansion of indication for liver transplantation.


Subject(s)
Antineoplastic Agents/therapeutic use , Drug Resistance, Neoplasm , Hepatoblastoma/drug therapy , Liver Neoplasms/drug therapy , Neoplasm Recurrence, Local/drug therapy , Neoplasm Recurrence, Local/prevention & control , Hepatoblastoma/prevention & control , Humans , Liver Neoplasms/prevention & control
19.
Ultraschall Med ; 27(5): 462-6, 2006 Oct.
Article in English | MEDLINE | ID: mdl-16894508

ABSTRACT

PURPOSE: To describe the sonographic appearance of cervical lymph nodes in twelve children with histopathologically confirmed atypical mycobacterial lymphadenitis. MATERIALS AND METHODS: Sonography of cervical lymph nodes was performed with a real-time linear scanner using a 13 MHz probe. Sonographic findings were retrospectively reviewed in 9 girls and 3 boys age 1.7 to 10.7 years (median age, 3.7 years), with cervical lymphadenopathy at initial presentation. RESULTS: Strong echoes within the lymph nodes were found in 4 (33 %) of the children, corresponding to calcifications on histopathological examination. Loss of regular nodal shape with circumscript polypoid spread of nodal masses into the subcutaneous tissue was found in 6 (50 %) patients; at least 1 of these 2 features was noted in 10 (83 %) of our 12 patients. At surgery, abscess formation was found in 8 (67 %) children. CONCLUSION: When nodal calcifications and spread of nodal masses into the subcutaneous tissue are found in patients with cervical lymphadenopathy, the examiner should maintain a high level of clinical suspicion for atypical mycobacterial lymphadenitis. Contrary to other causes of cervical lymphadenopathy, total surgical excision is the treatment of choice. Identification of these patients prior to surgery is therefore of utmost importance. Our data suggest that sonography could serve as an easy non-invasive method for this purpose.


Subject(s)
Mycobacterium Infections, Nontuberculous/diagnostic imaging , Tuberculosis, Lymph Node/diagnostic imaging , Uterine Cervical Diseases/microbiology , Child , Child, Preschool , Female , Humans , Infant , Lymph Node Excision , Lymph Nodes/diagnostic imaging , Lymph Nodes/microbiology , Male , Mycobacterium Infections, Nontuberculous/surgery , Tuberculosis, Lymph Node/surgery , Ultrasonography , Uterine Cervical Diseases/diagnostic imaging , Uterine Cervical Diseases/surgery
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