Minimally invasive surgery for neuroblastic tumours: A SIOPEN multicentre study: Proposal for guidelines.

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.

[Imaging of renal tumors in children]. / Bildgebung renaler Tumoren im Kindesalter.

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.

Fatty acid composition of adipose tissue at term indicates deficiency of arachidonic and docosahexaenoic acid and excessive linoleic acid supply in preterm infants.

BACKGROUND: Arachidonic (ARA) and docosahexaenoic acid (DHA) are constitutive to membrane phospholipids, and essential for brain and overall development. ARA/DHA pools in term infants (TI) are built during the third trimester, stored as adipose tissue triglycerides and predominantly distributed via plasma phosphatidylcholine (PC). In preterm infants (PTI), placental ARA/DHA supply is replaced by linoleic-acid (LA)-enriched nutrition. This study aimed to investigate the impact of PTI nutrition, compared to placental supply, on fatty acid composition in adipose tissue and blood. METHODS: Prospective observational study (4/2017-3/2019) in 12 PTI and 3 PTI with enterostomy (PTI/E) (gestational age (GA) < 32 weeks) with surgical intervention at term (± 6 weeks) and 14 TI (GA ≥ 34 weeks, surgical intervention < 2 weeks postnatally). PTI/E were analyzed descriptively only. PC and triglyceride fatty acids were analyzed with tandem mass spectrometry and gas chromatography, respectively. Results were compared between TI and PTI with Wilcoxon Test and shown as median [25th percentile-75th percentile] mol%. RESULTS: PTI had less ARA in adipose tissue TG (0.77[0.67-0.87]% vs. 1.04[0.95-1.14]%, p = 0.0003) and plasma PC (20.7[18.7-22.8]% vs. 28.3[22.7-33.5]%, p = 0.011) than TI. PTI also had less DHA in adipose tissue TG (0.6[0.4-0.8]% vs. 1.1[0.8-1.4]%, p = 0.006) and plasma PC (6.4[5.6-7.1]% vs. 8.4[7.8-13.1]%, p = 0.002). LA was increased in PTI's adipose tissue TG (10.0[8.8-12.3]% vs. 3.0[2.5-3.6]%, p < 0.0001) and plasma PC (48.4[44.6-49.6]% vs. 30.6[24.9-35.6]%, p = 0.0002). Similar differences were observed in erythrocyte PC. CONCLUSION: In PTI, LA is increased and ARA/DHA decreased in adipose tissue, plasma and erythrocyte lipids as proxies for other tissues, likely caused by PTI nutrition. This may contribute to impaired PTI development.

Management of benign liver tumors.

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.

Biomarkers Apo10 and TKTL1: Epitope-detection in monocytes (EDIM) as a new diagnostic approach for cholangiocellular, pancreatic and colorectal carcinoma.

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.

[Innovations in surgical treatment of pediatric solid tumors]. / Innovationen in der chirurgischen Behandlung solider Tumoren im Kindesalter.

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.

Differences between intrinsic and extrinsic ureteropelvic junction obstruction related to crossing vessels: histology and functional analyses.

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.

[Cytoreductive surgery and HIPEC for peritoneal malignancies in children]. / Zytoreduktive Chirurgie und HIPEC bei peritoneal-metastasierten Tumoren im Kindesalter.

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.

High-pitch computed tomography of the lung in pediatric patients: an intraindividual comparison of image quality and radiation dose to conventional 64-MDCT.

PURPOSE: The aim of this study was to investigate frequencies of typical artifacts in low-dose pediatric lung examinations using high-pitch computed tomography (HPCT) compared to MDCT, and to estimate the effective radiation dose (Eeff). MATERIALS AND METHODS: Institutional review board approval for this retrospective study was obtained. 35 patients (17 boys, 18 girls; mean age 112 ±â€Š69 months) were included and underwent MDCT and follow-up scan by HPCT or vice versa (mean follow-up time 87 days), using the same tube voltage and current. The total artifact score (0 - 8) was defined as the sum of artifacts arising from movement, breathing or pulsation of the heart or pulmonary vessels (0 - no; 1 - moderate; 2 - severe artifacts). Eeff was estimated according to the European Guidelines on Quality Criteria for Multislice Computed Tomography. The Mann-Whitney U test was used to analyze differences between the patient groups. The Spearman's rank correlation coefficient was used for correlation of ordinal variables. RESULTS: The scan time was significantly lower for HPCT compared to MDCT (0.72 ±â€Š0.13 s vs. 3.65 ±â€Š0.81s; p < 0.0001). In 28 of 35 (80 %) HPCT examinations no artifacts were visible, whereas in MDCT artifacts occurred in all examinations. The frequency of pulsation artifacts and breathing artifacts was higher in MDCT compared to HPCT (100 % vs. 17 % and 31 % vs. 6 %). The total artifact score significantly correlated with the patient's age in MDCT (r = - 0.42; p = 0.01), but not in HPCT (r = - 0.32; p = 0.07). The estimated Eeff was significantly lower in HPCT than in MDCT (1.29 ±â€Š0.31 vs. 1.47 ±â€Š0.37 mSv; p < 0.0001). CONCLUSION: Our study indicates that the use of HPCT has advantages for pediatric lung imaging with a reduction of breathing and pulsation artifacts. Moreover, the estimated Eeff was lower. In addition, examinations can be performed without sedation or breath-hold without losing image quality. KEY POINTS: • Fewer artifacts in pediatric lung imaging with HPCT• Reduced Eeff in HPCT• HPCT without sedation or breath-hold without loss of image quality.

Pharmacological inhibition of beta-catenin in hepatoblastoma cells.

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.

Effect of sorafenib combined with cytostatic agents on hepatoblastoma cell lines and xenografts.

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.

Fast intraoperative testosterone assay confirms the location of an ovarian virilizing tumor in a young girl.

The detection of testosterone-producing ovarian tumors in childhood and adolescence by imaging techniques only can be difficult because of the tumors' radiological structure and sometimes diminutive size. We describe an 11.5-year-old girl with a 9-month history of voice deepening, mild hirsutism, minor acne, increased growth velocity, weight gain, and clitoromegaly. Laboratory investigation revealed an extremely elevated serum testosterone level without any additional endocrine abnormalities. Abdominal ultrasound and MRI showed a sparsely noticeable solid mass in the center of the right ovary. At laparotomy, blood was selectively drawn from the right and the left ovarian veins. Rapid testosterone measurement revealed a 70-fold higher testosterone concentration in the right ovarian vein within 45 min. Based on this finding, a right salpingo-oophorectomy was performed. The patient's postoperative testosterone level declined within 24 h. The histopathological diagnosis was Leydig cell tumor. In conclusion, the implementation of a fast intraoperative testosterone assay enabled the localization and curative therapy of a Leydig cell tumor. This technique seems to be a good alternative to preoperative selective venous blood sampling when body imaging does not unveil the tumor's site.

Primary lung metastases in pediatric malignant non-Wilms renal tumors: data from SIOP 93-01/GPOH and SIOP 2001/GPOH.

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.

Nephron sparing surgery for synchronous bilateral nephroblastoma involving the renal hilus.

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.

[Principles of oncological surgery for lung metastases in paediatric solid tumours]. / Prinzipien der onkologischen Chirurgie bei Lungenmetastasen kindlicher solider Tumoren.

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.

Prenatal diagnosis and management of a fetal intra-abdominal teratoma: a case report and review of the literature.

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.

Laparoscopic surgery on upper urinary tract in children younger than 1 year: technical aspects and functional outcome.

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.

Transperineal sonography of the anal sphincter complex in neonates and infants: differentiation of anteriorly displaced anus from low-type imperforate anus with perineal fistula.

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.

Imaging of cell trafficking and metastases of paediatric rhabdomyosarcoma.

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.

[Thoracoscopic anatomical lung resection in children]. / Die thorakoskopische anatomiegerechte Lungenresektion im Kindesalter.

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.