Vascular encasement as element of risk stratification in abdominal neuroblastoma.

BACKGROUND: Vascular encasement of major vessels has been introduced as element of image defined risk factors (IDRF) for stratification of abdominal neuroblastoma. Some subgroups of this tumor entity are still subject of discussion regarding surgical approach and radicality. Aim of this study was to analyse a cohort of related patients. PATIENTS AND METHODS: Children operated on for neuroblastoma with encasement of major abdominal vessels (April 2002-April 2009) were retrospectively evaluated regarding surgical procedures, intra- and postoperative complications, and outcome. RESULTS: There were 18 patients with abdominal NB and encasement of major vessels. Mean age at operation was 43.5 months (2.5-113), mean operation time was 228 minutes (157-428). Complete macroscopic tumor resection was realised in 14 children. Vascular reconstruction was necessary in 5 patients. Tumor progression/relapses requiring further operation occurred in 3 patients. Major postoperative complications were 1 loss of unilateral renal function with subsequent nephrectomy, 1 renal vein thrombosis (operative revision), 1 renal artery embolism (operative revision), and 1 ureteral obstruction (stenting). Mean follow up was 34.8 months (2-78). CONCLUSIONS: Vascular encasement as part of IDRF is a valuable tool for stratification of abdominal NB. Surgery of NB with vascular encasement includes divers and complex procedures. Children seem to benefit from complete tumor resection or at least relevant tumor reduction although operations can mean a relevant strain for the patients.

Giant mullerian duct cyst with malignant transformation in 15-year-old boy.

We present a case of a giant mullerian duct cyst with components of clear cell adenocarcinoma in a 15-year-old boy. Complete tumor resection was achieved in two steps. The presented case with a combination that has never been described before may change the perspective of treatment approaches for this rare entity.

A complicated case of bilateral cystic nephroma in a 16-month-old boy.

We describe a case of a progressive bilateral cystic nephroma (BCN) in a child undergoing a multistaged surgical procedure. After partial resection of the left sided tumor, a progress occurred on that side and the left kidney had to be removed 10 weeks later. After 35 months a tumor progression was observed on the right side together with an ureteral obstruction leading to a decreased renal function. In a third operation a complete tumor resection on the right side was achieved through longitudinal partial nephrectomy, reconstruction of the renal pelvis, and reanastomosis of the reconstructed pelvis and ureter. The patient showed no evidence of the disease at 28 months of follow-up. The presented case provides an evidence that in BCN a tumor progress may occur after multistaged surgical approaches. A single-staged complete tumor resection with renal salvage techniques seems indicated.

Salvage therapy of progressive and recurrent Hodgkin's disease: results from a multicenter study of the pediatric DAL/GPOH-HD study group.

PURPOSE: To evaluate a salvage therapy (ST-HD-86) for patients with progressive and relapsed Hodgkin's disease after primary treatment in the pediatric DAL/GPOH studies. The essential chemotherapeutic regimens were ifosfamide, etoposide, and prednisone (IEP) and doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD). METHODS: One hundred seventy-six patients with progression (n = 51) or first relapse (n = 125) were enrolled by 67 centers. The median time from initial diagnosis to progression/relapse was 1.1 year (range, 0.1 to 15.3 years), and the patients' median age was 14.7 years (range, 4.3 to 24.5 years). Salvage chemotherapy consisted of two to three cycles of IEP alternating with one to two cycles of ABVD supplemented in part by one to two cycles of cyclophosphamide, vincristine, procarbazine, and prednisone or lomustine (CCNU), etoposide, and prednimustine. Radiotherapy was given to involved areas using individualized doses. In the 1990s, additional high-dose chemotherapy with autologous stem-cell transplantation (SCT) was introduced for patients with unfavorable prognosis. RESULTS: Disease-free survival (DFS) and overall survival (OS) after 10 years are 62% and 75%, respectively (SE, 4% each). Of 176 patients, 73 suffered second events. The risk-factor analysis revealed the time to progression/relapse as the strongest prognostic factor (P = .0001). Patients with progression have an inferior outcome (DFS, 41%; OS, 51%), whereas patients with late relapse (> 12 months after end of therapy) do well (DFS, 86%; OS, 90%), although none of them received SCT in second remission. CONCLUSION: The result can be considered favorable. Whereas the salvage strategy for progressive disease has to be optimized further, it is possible to reduce intensity and avoid SCT in late relapses after Hodgkin's disease in childhood/adolescence.

Three-dimensional visualization and virtual simulation of resections in pediatric solid tumors.

PURPOSE: Three-dimensional visualization of solid tumors is possible because of high-resolution computed tomography and magnetic resonance imaging scans. However, additional preoperative information is often desirable in complex malignancies. For the first time, the authors present a model of preoperative 3-dimensional visualization and virtual resections in pediatric solid tumors. METHODS: Image analysis of various pediatric tumors was performed using the research software HepaVision2 (MeVis, Bremen). Organs, tumors, and the vascular system were extracted from multislice computed tomography scans. After hierarchical analysis of the vascular system, territories supplied or drained by the major vascular branches were calculated. Results were explored and virtual resections of organs were carried out using the research software InterventionPlanner (MeVis, Bremen). Data were correlated to intraoperative findings. RESULTS: Four hepatic malignancies, 4 renal tumors, and 3 other neoplasms were analyzed. The technique of 3-dimensional visualization was feasible for all investigated children (mean age 5 years and 9 months). Spatial relations between physiological and pathological structures were identified, and anatomical structures (vessels, tumor tissue, and organ parenchyma) were determined using colorimetric encoding. Virtual simulations of tumor resection were used successfully for planning of surgical procedures in the hepatic and renal tumors. CONCLUSIONS: The technique of 3-dimensional tumor visualization and virtual simulation of tumor resections provides the basis for a successful planning of complex tumor resections in children. The efficiency of these techniques should be further analyzed in series with higher numbers and differentiations of tumors.