Age-Dependent Presentation and Clinical Course of 1465 Patients Aged 0 to Less than 18 Years with Ovarian or Testicular Germ Cell Tumors; Data of the MAKEI 96 Protocol Revisited in the Light of Prenatal Germ Cell Biology.

Objective: To evaluate prognostic factors in pediatric patients with gonadal germ cell tumors (GCT).

Methods: Patients <18 years with ovarian and testicular GCT (respectively OGCT and TGCT) were prospectively registered according to the guidelines of MAKEI 96. After resection of the primary tumor, patients staged ≥II received risk-stratified cisplatin-based combination chemotherapy.

Leukotriene receptor blockade as a life-saving treatment in severe bronchopulmonary dysplasia.

Background: Bronchopulmonary dysplasia (BPD) is a major cause of mortality and morbidity in infants with an extremely low birth weight. Because there is no effective therapy, the mortality of this condition in severely affected patients is high. Therapeutic blocking of the leukotriene system seems to be a logical approach due to the known pathophysiology of BPD.

Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults.

Purpose: To improve risk-adapted therapy for localized childhood soft tissue sarcoma within an international multicenter setting.

Patients And Methods: Four hundred forty-one patients younger than 21 years with localized rhabdomyosarcoma and rhabdomyosarcoma-like tumors (ie, extraosseous tumors of the Ewing family, synovial sarcoma, and undifferentiated sarcoma) were eligible. Therapy was stratified according to postsurgical stage, histology, and tumor site.

Germ cell tumors of the head and neck: report from the MAKEI Study Group.

Background: Germ cell tumors (GCTs) of the head and neck region are rare but may pose significant problems for perinatal management as well as surgical and adjuvant therapy.

Procedure: Thirty-two prospectively reported patients from the German MAKEI studies (Maligne Keimzelltumoren) were analyzed with regard to perinatal management and long-term survival.

Results: Twenty-three tumors were diagnosed around birth and four during the first 3 months of life.

Nonviable tumor tissue should not upstage Wilms' tumor from stage I to stage II: a report from the SIOP 93-01 nephroblastoma trial and study.

In SIOP trials, Wilms' tumors were labeled as stage II by the presence of nonviable and/or viable tumor in the renal sinus and/or perirenal fat. The aim of this study was to determine if this approach was justified. Stage II Wilms' tumors were reviewed to establish whether staging was due to viable or nonviable tumor, and this was related to clinical outcome.

KIT, PDGFRalpha and EGFR analysis in nephroblastoma.

Nephroblastoma prognosis has dramatically improved, but an unfavourable prognostic subgroup warrants development of novel therapeutic strategies. Selective KIT, PDGFRalpha and epidermal growth factor receptor (EGFR) tyrosine kinase inhibition evolved as powerful targeted therapy for gastrointestinal stromal tumours and non-small-cell lung cancer. To investigate a potential role for tyrosine kinase inhibition, we analyzed 209 nephroblastomas for immunohistochemical KIT and EGFR expression, 63 nephroblastomas for mutations in KIT exons 9, 11, 13, EGFR exons 18, 19, 20 and 21, and all 209 nephroblastomas for PDGFRalpha exons 12, 14 and 18.

Wnt signaling pathway analysis in renal cell carcinoma in young patients.

Renal cell carcinomas in young patients constitute a morphologically and genetically heterogeneous group. Twenty percent belong to the newly recognized Xp11.2 translocation-associated family and rare tumors arise from nephroblastoma.

Ovarian small cell carcinoma of the hypercalcemic type in children and adolescents: a prognostically unfavorable but curable disease.

Background: Ovarian small cell carcinoma of the hypercalcemic type is a rare neoplasm that is associated with a poor prognosis. The objective of the current study was to investigate the clinicopathologic features of this tumor and to develop preliminary diagnostic and therapeutic guidelines.

Methods: Between 1994 and 2005, 11 girls (ages 9-22 years) who were registered on the German Maligne Keimzelltumoren studies and the Kiel Pediatric Tumor Registry were analyzed.

Imbalances of chromosome arm 1p in pediatric and adult germ cell tumors are caused by true allelic loss: a combined comparative genomic hybridization and microsatellite analysis.

Previous studies on childhood germ cell tumors (GCTs) report highly variable frequencies of losses at chromosome arm 1p. Since deletions at 1p portend a poor prognosis in other embryonal tumors, this study aims to clarify the question of the frequency of true allelic loss at 1p and whether it constitutes a prognostic parameter. We analyzed 13 GCTs from different gonadal and extragonadal sites of children (4 teratomas, 9 malignant GCTs) and 18 GCTs of adolescents and adults (3 teratomas; 15 malignant GCTs) using automated microsatellite analysis with 23 polymorphic markers and chromosomal "high resolution" comparative genomic hybridization (HR-CGH).

Genetic clonality is a feature unifying nephroblastomas regardless of the variety of morphological subtypes.

Nephroblastomas are embryonal tumors exhibiting a wide variety of different morphological features and genetic changes. Some of the genetic aberrations were associated with a certain histological subtype. It is generally assumed that nephroblastomas develop as subclonal proliferations from nephrogenic rests.

Treatment of severe disseminated juvenile systemic xanthogranuloma with multiple lesions in the central nervous system.

Juvenile xanthogranuloma (JXG), one of the most common forms of Langerhans-dendritic cell proliferation in young children, usually presents as spontaneously regressing cutaneous lesions. JXG with systemic (extracutaneous) involvement is a rare histiocytic disorder in which significant morbidity and death may occur. The systemic type, especially combined with multiple central nervous system lesions in young children, has a very poor prognosis.

Ovarian sex cord-stromal tumors in children and adolescents.

Ovarian sex cord-stromal tumors (OSCSTs) are a heterogeneous group of tumors that develop from the gonadal non-germ-cell component. Despite recent advances in the clinical and histopathologic diagnosis of OSCSTs, a high degree of uncertainty remains with regard to adequate therapy, particularly in patients presenting with microscopic or macroscopic tumor spread. We review the currently available data on the biology and histology of OSCST in children and adolescents.

Abdominal inflammatory masses mimicking neoplasia in children-experience of two centers.

Despite progress in modern imaging, some inflammatory masses are difficult to distinguish clinically from neoplastic processes. In such cases the pathology report has a great distinctive value, but even then the final diagnosis may be difficult to reach. Eight patients with abdominal tumors of inflammatory origin were treated in two institutions, the Department of Pediatric Surgery of the Medical University of Gdansk, Poland, and Helios Center of Pediatric Surgery in Berlin, Germany, during the last 10 years.

Low occurrence of familial neuroblastomas and ganglioneuromas in five consecutive GPOH neuroblastoma treatment studies.

Familial neuroblastoma is of special interest in view of the oncogenesis of this tumour with its early manifestation in childhood. The inheritance seems to follow an autosomal-dominant mendelian trait with incomplete penetrance. Familial neuroblastomas and ganglioneuromas have not been reported in detail within large treatment studies.

Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the kiel pediatric tumor registry.

Juvenile xanthogranuloma (JXG) is an uncommon non-Langerhans cell histiocytosis. We investigated 148 biopsy specimens from 129 patients collected in the Kiel Pediatric Tumor Registry (KPTR) between 1965 and 2001. The clinical, histologic, and immunohistochemical characteristics of JXG were evaluated to gain more and deeper insights into the morphology and clinical behavior of JXG.

Morphologic and molecular characterization of renal cell carcinoma in children and young adults.

A new WHO classification of renal cell carcinoma has been introduced in 2004. This classification includes the recently described renal cell carcinomas with the ASPL-TFE3 gene fusion and carcinomas with a PRCC-TFE3 gene fusion. Collectively, these tumors have been termed Xp11.

Epidemiologic analysis of 1,442 children and adolescents registered in the German germ cell tumor protocols.

Background: Germ cell tumors (GCTs) constitute a heterogeneous group of tumors that significantly vary with respect to their clinical presentation and biology. The objective of this analysis was to analyze a large population-based pediatric cohort of GCTs and to evaluate the parameters age, sex, site of the tumor, histology, and potential correlations between these parameters.

Procedure: Between 1981 and 2000, 1,442 patients were prospectively enrolled onto the German protocols for testicular and non-testicular GCTs.

Expression of the c-kit receptor characterizes a subset of neuroblastomas with favorable prognosis.

Expression of the c-kit proto-oncogene product in neuroblastomas has been reported, but its clinical relevance is unclear. We determined the expression of c-kit by immunohistochemistry in a series of 155 neuroblastomas with long-term follow-up. The specificity of the reaction was verified by Western blot analysis and quantitative RT-PCR, and exon 11 of the kit gene was screened for mutations by PCR and capillary electrophoresis.

Group II rhabdomyosarcoma and rhabdomyosarcomalike tumors: is radiotherapy necessary?

Purpose: In the prospective Cooperative Soft Tissue Sarcoma Study Group (CWS) 81, 86, 91, and 96 trials, radiotherapy was omitted in some patients with rhabdomyosarcoma and rhabdomyosarcoma-like tumors within Intergroup Rhabdomyosarcoma Study (IRS) group II. This analysis evaluates whether subgroups can be defined for which radiotherapy is not necessary.

Patients And Methods: Two hundred three patients who were registered between January 1981 and December 1998 were eligible for evaluation.

Partial nephrectomy for unilateral Wilms tumor: results of study SIOP 93-01/GPOH.

Purpose: We evaluate results and long-term outcome after partial nephrectomy (PN) for unilateral Wilms tumor (ulWT) in relation to different histological features, performed as initial surgery or after induction chemotherapy (ChT).

Materials And Methods: Data from patients with ulWT who had undergone PN in the German Study SIOP 93-01/GPOH were analyzed for time of surgery in the treatment schedule, postoperative stage, histological features, surgical radicality and outcome. The results were correlated with overall survival and relapse-free survival, and compared with those of patients who had undergone total tumor nephrectomy (TN).

Ovarian sex cord-stromal tumors--a clinicopathological study of 72 cases from the Kiel Pediatric Tumor Registry.

We analyzed 72 patients with ovarian sex cord-stromal tumors (OSCST) registered at the German Pediatric Tumor Registry in Kiel over a 20-year period. Juvenile granulosa cell tumors (JGCT, n=48) were the most frequent histological subtype. In addition, there were 14 Sertoli-Leydig cell tumors, 5 sclerosing stromal tumors, 2 sex cord tumors with annular tubules, 2 thecomas and 1 steroid cell tumor.

Ulcerated epithelioid hemangioendothelioma of the foot in childhood.

Epithelioid hemangioendothelioma is an uncommon malignant vascular tumor usually involving soft tissue and, in rare cases, the skin. Histologically and biologically it is considered to be a borderline neoplasm between an angiolymphoid hyperplasia with eosinophilia and an epithelioid angiosarcoma. Here we describe an 11-year-old girl with a 1-year history of an isolated, spontaneously appearing, painful ulceration on the instep of the right foot.

Two molecular subgroups of Wilms' tumors with or without WT1 mutations.

Purpose: Wilms' tumors (WTs) exhibit more than one pattern of differentiation, each of which is associated with distinctive clinical features and treatment responses. Mutations in the WT1 gene are found predominantly in WTs with stromal histology. To better understand the biological and clinical features in different WTs, we have analyzed WTs with and without WT1 mutations for a set of parameters.

Repp86 expression and outcome in patients with neuroblastoma.

Purpose: Given the well-known challenges of neuroblastoma prognosis, we investigated whether the expression of restrictedly expressed proliferation-associated protein of 86 kDa theoretical molecular mass (repp86), a proliferation-associated protein expressed in S, G2, and M phases of the cell cycle, correlates with the clinical outcome in patients with neuroblastoma.

Patients And Methods: 161 children with different stages of neuroblastoma were studied; the median follow-up time was 72.8 months.