Cerebrospinal Fluid Protein Concentrations in Hydrocephalus.

CSF protein levels are altered in neurological disorders, such as hydrocephalus of different etiologies. In this retrospective observational study, we analyzed cerebrospinal fluid (CSF) samples in hydrocephalic diseases such as aqueductal stenosis (AQS, n = 27), normal pressure hydrocephalus (NPH, n = 24), hydrocephalus communicans (commHC, n = 25) and idiopathic intracranial hypertension (IIH)/pseudotumor cerebri (PC, n = 7) in comparison with neurological patients without hydrocephalic configuration (control, n = 95). CSF was obtained through CSF diversion procedures and lumbar punction and analyzed for protein concentrations according to the institution's laboratory standards. We found significantly decreased CSF protein levels in patients suffering from AQS (0.13 mg/dL [0.1-0.16 mg/dL] p = 2.28 × 10-8) and from PC (0.18 mg/dL [0.12-0.24 mg/dL] p = 0.01) compared with controls (0.34 mg/dL [0.33-0.35 mg/dL]). Protein levels were not altered in patients suffering from commHC and NPH compared with neurologically healthy individuals. We propose that a decrease in CSF protein levels is part of an active counterregulatory mechanism to lower CSF volume and, subsequently, intracranial pressure in specific diseases. Research regarding said mechanism and more specific proteomic research on a cellular level must still be performed to prove this hypothesis. Differences in protein levels between different diseases point to different etiologies and mechanisms in different hydrocephalic pathologies.

Chest examinations in children with real-time magnetic resonance imaging: first clinical experience.

BACKGROUND: Real-time magnetic resonance imaging (MRI) based on a fast low-angle shot technique 2.0 (FLASH 2.0) is highly effective against artifacts caused due to the bulk and pulmonary and cardiac motions of the patient. However, to date, there are no reports on the application of this innovative technique to pediatric lung MRI. OBJECTIVE: This study aimed to identify the limits of resolution and image quality of real-time lung MRI in children and to assess the types and minimal size of lesions with these new sequences. MATERIALS AND METHODS: In this retrospective study, pathological lung findings in 87 children were classified into 6 subgroups, as detected on conventional MRI: metastases and tumors, consolidation, scars, hyperinflation, interstitial pathology and bronchiectasis. Subsequently, the findings were grouped according to size (4-6 mm, 7-9 mm and ≥ 10 mm) and evaluated for visual delineation of the findings (0 = not visible, 1 = hardly visible and 2 = well visualized). RESULTS: Real-time MRI allows for diagnostic, artifact-free thorax images to be obtained, regardless of patient movements. The delineation of findings strongly correlates with the size of the pathology. Metastases, consolidation and scars were visible at 100% when larger than 9 mm. In the 7-9 mm subgroup, the visibility was 83% for metastases, 88% for consolidation and 100% for scars in T2/T1 weighting. Though often visible, smaller pathological lesions of 4-6 mm in size did not regularly meet the expected diagnostic confidence: The visibility of metastases was 18%, consolidation was 64% and scars was 71%. Diffuse interstitial lung changes and hyperinflation, known as "MR-minus pathologies," were not accessible to real-time MRI. CONCLUSION: The method provides motion robust images of the lung and thorax. However, the lower sensitivity for small lung lesions is a major limitation for routine use of this technique. Currently, the method is adequate for diagnosing inflammatory lung diseases, atelectasis, effusions and lung scarring in children with irregular breathing patterns or bulk motion on sedation-free MRI. A medium-term goal is to improve the diagnostic accuracy of small nodules and interstitial lesions.

Resection of an Extraovarian Noniuteinized Thecoma in a 14-Year-Old Girl.

Thecomas are rare benign sex cord-stromal tumors that account for less than 1% of all ovarian tumors. They usually affect postmenopausal women and become symptomatic with abnormal bleeding. In adolescents, less than 10 cases have been reported so far, mainly with symptoms of hormonal disbalance. Extraovarian thecomas represent an even rarer entity, with only two cases described so far, none of them in the pediatric population. We report the case of a 14-year-old girl who presented with sudden-onset abdominal pain, dysuria, and fever, as well as highly elevated serum inflammation parameters. Ultrasound and magnetic resonance imaging (MRI) revealed a large, inhomogeneous pelvic mass (16 cm × 9 cm × 13 cm) with indistinct margins, suggestive of an infiltrative malignant teratoma or sarcoma. Laparoscopy confirmed a large mass of unknown origin. In contrast to the infiltrative character seen on preoperative MRI, the tumor could be easily exteriorized and resected after conversion to laparotomy. Ovaries, fallopian tubes, and uterus remained unaffected. Histopathology revealed a benign nonluteinized thecoma. The postoperative course and 19-month follow-up were uneventful.

Treatment of Ruptured Giant Omphalocele and Gastroschisis with Liver Herniation using a Wound Retractor as a Novel Approach.

Ruptured giant omphaloceles (GO) and gastroschisis with total liver herniation are rare cases of exceptionally large abdominal wall defects. Many of these children have lethal outcome. The surgical and postsurgical management are complex. We report on two cases treated with staged surgical repair using a wound retractor as a silo. With this technique, the liver and intestines could be reduced into the abdomen with secondary closure of the abdominal cavity within the first 1 to 2 weeks of life.

CHL1 and NrCAM are Primarily Expressed in Low Grade Pediatric Neuroblastoma.

BACKGROUND: Neural cell adhesion molecules like close homolog of L1 protein (CHL1) and neuronal glia related cell adhesion molecule (NrCAM) play an important role in development and regeneration of the central nervous system. However, they are also associated with cancerogenesis and progression in adult malignancies, thus gain increasing importance in cancer research. We therefore studied the expression of CHL1 and NrCAM according to the course of disease in children with neuroblastoma. METHODS: CHL1 and NrCAM expression levels were histologically assessed by tissue microarrays from surgically resected neuroblastoma specimens of 56 children. Expression of both markers was correlated to demographics as well as clinical data including metastatic dissemination and survival. RESULTS: CHL1 was expressed in 9% and NrCAM in 51% of neuroblastoma tissue samples. Expression of CHL1 was higher in patients with low Hughes grade 1a/b (p=0.01). NrCAM was more often detected in patients with a low International Staging System (INSS) score 1/2 (p=0.04). CONCLUSION: CHL1 and NrCAM expression was associated with low-grade pediatric neuroblastoma. These adhesion molecules may play a role in early tumor development of neuroblastoma.

Increased L1CAM (CD171) levels are associated with glioblastoma and metastatic brain tumors.

L1 cell adhesion molecule (L1CAM) is a member of the immunoglobulin-like cell-adhesion molecule family that was shown to be associated with a worse prognosis in several human cancers. L1 ectodomain shedding via vesicles or exosomes has been detected in extracellular fluids after cleavage from the cell surface by metalloproteases. We evaluated the presence of L1CAM in cyst fluid and tissue from glioblastomas or brain metastases.The amount of L1CAM in cyst fluid of 9 glioblastomas and 11 brain metastases was assessed using enzyme-linked immunosorbent assay (ELISA). Corresponding tumor tissue slices were stained immunohistochemically for L1CAM. Cerebrospinal fluid of 20 non-tumor patients served as controls.Mean levels of L1CAM in tumor cyst fluid were significantly higher in glioblastoma (6118 ±â€Š4095 ng/mL) and metastasis patients (8001 ±â€Š6535 ng/mL) than in CSF of control patients (714 ±â€Š22 ng/mL). The immunohistochemical expression of L1CAM in corresponding tissue was significantly higher in metastases than in glioblastomas.The present study demonstrates high levels of L1CAM in cyst fluid of glioblastoma and metastatic brain tumors. Soluble L1CAM may represent a motility promoting molecule in cancer progression, a co-factor for development of tumor cysts and a target for new treatment strategies.

Prognostic Impact of Activated Leucocyte Cell Adhesion Molecule (ALCAM/CD166) in Infantile Neuroblastoma.

BACKGROUND/AIM: Activated leukocyte cell adhesion molecule (ALCAM/CD166) as a member of the 'immunoglobulin superfamily' is known to be involved in cancer cell proliferation and migration. The aim of this study was to investigate the expression of ALCAM in neuroblastoma tissues. MATERIALS AND METHODS: ALCAM expression was analyzed in primary neuroblastoma specimens by immunohistochemistry on microarray sections. Histopathological and clinical data were correlated with ALCAM expression and survival analysis was performed. RESULTS: Sixty-six children were included in the study. Strong expression of ALCAM was detected in 52 (79%) of the samples. Weak expression was significantly correlated with the International Neuroblastoma Staging System (INSS) stage (p=0.024) and positive n-MYC amplification (p=0.019). Recurrence-free survival (RFS) and overall survival (OS) were significantly shorter if ALCAM was expressed weakly (p=0.032 and p=0.001). CONCLUSION: Weak ALCAM expression was significantly correlated with established markers for poor prognosis, as well as shorter RFS and OS. ALCAM might be considered as a prognostic marker for infantile neuroblastoma.

Hydrocephalus in aqueductal stenosis--a retrospective outcome analysis and proposal of subtype classification.

UNLABELLED: Treatment of aqueductal stenosis (AQS) has undergone several paradigm shifts during the past decades. Currently, endoscopic ventriculostomy (ETV) is recommended as treatment of choice. Several authors have addressed the issue of variable ETV success rates depending on age and pathogenetic factors. However, success rates have usually been defined as "ETV non-failure." The aim of the study was a retrospective analysis of radiological and neurological treatment response after ETV or VP-shunting (VPS) in age-dependent subtypes of AQS. PATIENTS AND METHODS: Eighty patients (median age 12.0 years, range 0-79 years) have been treated for MRI-proven aqueductal stenosis. Neurological treatment success was defined by neurological improvement and, in childhood, head circumference. Radiological response was measured as Evan's index in follow-up MRI. Initial signs and symptoms, type of surgery, and complications were analyzed. RESULTS: Four types of AQS have been defined with distinct age ranges and symptomatology: congenital type I (n = 24), chronic progressive (tectal tumor-like) type II (n = 23), acute type III (n = 10), and adult chronic (normal-pressure hydrocephalus-like) type IV (n = 23). Retrospective analysis of neurological and radiological outcome suggested that congenital type I (<1 years of age) may be more successfully treated with VPS than with ETV (81 vs. 50 %). Treatment of chronic juvenile type II (age 2-15) by ETV 19 % compared to 57 % after VP-shunt, but similar neurological improvement (>80 %). There has been no influence of persistent ventriculomegaly in type II after ETV in contrast to VPS therapy for neurological outcome. Adult acute type III (age > 15 years) responded excellent to ETV. Chronic type IV (iNPH-like) patients (age > 21) responded neurologically in 70 % after ETV and VPS, but radiological response was low (5 %). CONCLUSION: AQS can be divided into four distinct age groups and types in regards of clinical course and symptomatology. Depending on the AQS type, ETV cannot be unequivocally recommended. Congenital type I AQS may have a better neurological outcome with VP-shunt whereas acute type III offers excellent ETV results. Chronic progressive type II still requires prospective investigation of long-term ETV outcome, especially when ventriculomegaly persists. Late chronic type IV seems to result in similar outcome after VP-shunt and ETV.

The Foker technique (FT) and Kimura advancement (KA) for the treatment of children with long-gap esophageal atresia (LGEA): lessons learned at two European centers.

INTRODUCTION: We present the experiences from two European centers performing the Foker technique (FT) of esophageal lengthening by axial traction and the Kimura advancement (KA) method of lengthening the upper pouch by extrathoracic resiting a spit fistula (SF) in children with long-gap esophageal atresia (LGEA, gap length > 5 cm). MATERIALS AND METHODS: A total of 15 children were treated (8 pure EA, 6 lower tracheoesophageal fistula [TEF], and 1 upper TEF). Gaps ranged from 5 to 14 cm. Nine children already had a SF. Patients were grouped according to the presence of a SF and the subsequent surgical strategy: Group A (no SF, n = 6) received FT on both pouches. Group B (with SF, n = 6) received KA of SF and FT of the lower pouch. Group C (with SF, n = 3) received closure of the SF and subsequent Foker traction (CSFT) on both pouches. RESULTS: Group A: Primary repairs for all six children (mean age 3 months, gap length 6.5 cm) after a mean traction time of 3 weeks and a mean of 2.1 thoracotomies (range 2 to 3). Dilations were required in three out of six for anastomotic strictures with one perforation during the second dilation. Group B: All six children (mean age 16.4 months, gap length 9.5 cm) had a primary anastomosis, although for two it was significantly delayed (48 and 143 weeks traction time) because of infections. The number of thoracotomies ranged from 2 to 8 (mean 3.6). Leaks occurred in five out of six anastomoses (responsive to conservative management). Two children developed severe strictures, which required the anastomosis to be redone. In group C (mean age 10.6 months, gap length 6.5 cm), several major complications occurred. The three SF closures leaked (one iatrogenic) causing severe mediastinitis. CSFT was successful in only one case and the other two children had an esophageal replacement (stomach, jejunum). No deaths occurred in the series. CONCLUSION: FT of both pouches (group A) resulted in primary repairs of all six LGEA patients. The combination of KA and FT (group B) resulted in an equivalent rate of primary repairs, but with an increased number of thoracotomies and rate of complications compared with group A. CSFT (group C) resulted in a high failure rate. More data are needed (we propose a multicenter registry) to elucidate the safety and efficacy of each elongation technique and to establish an algorithm with clearer inclusion and exclusion criteria.

Oesophageal elongation with traction sutures (FOKER procedure) in a newborn baby with long-gap oesophageal atresia (LGEA): maybe too early, maybe too dangerous?

In children with long gap oesophageal atresia (LGEA), the FOKER technique (oesophageal elongation with traction sutures) has been criticized for its high complication rate. We advocate analysing such problems to increase the safety in the future. The present case report will focus on timing. A female newborn (3000 g) with LGEA (gap of 5 cm) was delivered in an outward hospital. On day two of life, she received traction sutures on both pouches. By day five, all sutures had torn out, and a primary anastomosis was attempted. However, it leaked severely. Thus, on day ten, the oesophagus was approached from the neck converting the proximal end into a spit fistula and closing the distal end blindly. Furthermore, the gastro-oesophageal (GE-) junction was wrapped with a Teflon sling. When the baby arrived in our institution, she suffered from cavernous oesophageal masses extending from the thoracic inlet down to the diaphragm and fistulas draining them into the neck as well as into the right lung. Moreover, the Teflon sling had dislodged allowing for GE-reflux. In several stages, the oesophageal remnants were resected without any complications. Finally, Prof. Alaa Hamza performed a colonic interposition, which is working well today. In conclusion, the present case aims to caution paediatric surgeons to apply traction sutures for oesophageal elongation in newborns with LGEA.

Laparoscopic versus robot-assisted Nissen fundoplication in an infant pig model.

PURPOSE: Surgical robots are designed to facilitate dissection and suturing, although objective data on their superiority are lacking. This study compares conventional laparoscopic Nissen fundoplication (CLNF) to robot-assisted Nissen fundoplication (RANF) using computer-based workflow analysis in an infant pig model. METHODS: CLNF and RANF were performed in 12 pigs. Surgical workflow was segmented into phases. Time required to perform specific actions was compared by t test. The quality of knot-tying was evaluated by a skill scoring system. Cardia yield pressure (CYP) was determined to test the efficacy of the fundoplications, and the incidence of complications was compared. RESULTS: There was no difference in average times to complete the various phases, despite faster robotic knot-tying (p = 0.001). Suturing quality was superior in CLNF (p = 0.02). CYP increased similarly in both groups. Workflow-interrupting hemorrhage and pneumothorax occurred more frequently during CLNF (p = 0.040 and 0.044, respectively), while more sutures broke during RANF (p = 0.001). CONCLUSION: The robot provides no clear temporal advantage compared to conventional laparoscopy for fundoplication, although suturing was faster in RANF. Fewer complications were noted using the robot. RANF and CLNF were equally efficient anti-reflux procedures. For robotic surgery to manifest its full potential, more complex operations may have to be evaluated.

Single-incision multiport laparoscopy does not cause more pain than conventional laparoscopy: a prospective evaluation in children undergoing appendectomy.

BACKGROUND: The benefit of single-incision multiport laparoscopy (SIMPL) remains a matter of vivid discussion. For good reason it has been speculated that SIMPL causes more postoperative pain, because a minilaparotomy is required to place the multiport system. We prospectively evaluated postoperative pain scores and requirement of analgesic medication following conventional laparoscopic (CL) versus SIMPL appendectomy in children. METHODS: The access for laparoscopic appendectomy was decided upon the surgeon's preference. Between April and October 2010, individual abdominal pain scores at 8, 16, 24, 48, and 72 hours postoperatively as well as the incidence of umbilical or shoulder pain and the total amount of peri- and postoperative analgesics, operative time, length of hospital stay, and demographics were assessed. Analgesics (paracetamol and/or metamizole, 15 mg/kg body weight) were administered regularly or on inquiry of the patient. Data are presented as means±standard deviation tested at a significance level of P<.05. RESULTS: All operations were laparoscopically completed without conversion or addition of extra ports. Thirty-nine patients (8 SIMPL appendectomy) at a mean age of 12.3±2.4 years and a mean body mass index of 19.16±3.2 kg/m(2) were included. Equal operation times were observed (SIMPL: 68.5±19.9 minutes versus CL: 66.2±19.5 minutes). There were no significant differences for the individual pain scores or the incidence of umbilical and shoulder pain between study groups. The total amount of required analgesic medication was significantly lower after SIMPL appendectomy (SIMPL: 65.73±43.8 mg/kg versus CL: 106.39±46.4 mg/kg, P=.04). CONCLUSION: In summary, the present study substantiates the evidence that SIMPL appendectomy in children and adolescents is not only feasible but also beneficial for the patient without translation into increased postoperative pain. Presently, we are conducting a randomized, blinded study to validate these findings.

Embryology of the early foregut.

In embryology, no agreement exists how the early foregut differentiates into the respiratory tract and the intestinal tract. In particular, the formation of the early lung anlage as well as the process of separation of trachea and esophagus remains unclear. This process is explained in a rather schematic way and aims more to explain pathologic findings, whereas true embryologic investigations are extremely rare in this field. Here, scanning electron microscopy of the normal foregut development illustrates the steps, which finally leads to the development of larynx and trachea on the one hand, and pharynx and esophagus on the other hand. This study was performed in chicken embryos in accordance to the developmental stages described. As the main results from these illustrations show, we found no evidence for lateral foregut ridges inside the undivided foregut chamber and no fusion of lateral foregut components to form a trachea-esophageal septum.

Residual lymph node metastasis in stage 4 neuroblastoma--advantage of radio-guided surgery?

Log-term prognosis of children suffering from high-risk neuroblastomas is characterized by a shortened event-free survival, especially if metastases remain after chemotherapy. We report the case of a 3-year-old boy afflicted with a stage 4 neuroblastoma and persistent residual lymph node metastases despite the administration of a various number of treatment modalities. The insertion of a MIBG (metaiodobenzylguanidine) single-photon emission computed tomography (SPECT)-CT and radio-guided surgery implementing a hand held gamma probe finally allowed the exact localization and resection of the suspected lymphatic tissue. As a consequence, the child has been under event-free remission for 20 months. Because study-based knowledge is missing due to the small number of affected patients, individual case reports are helpful to improve future treatment strategies.

Y-box-binding protein-1 is a potential novel tumour marker for neuroblastoma.

BACKGROUND: The Y-box-binding protein-1 (YB-1) is a member of a family of DNA-binding proteins and an oncogenic transcription factor that is highly expressed in cancers of the breast, lung and prostate. To date, no data are available on its role in neuroblastoma. The aim of the present study was to evaluate the YB-1 expression in neuroblastoma. MATERIALS AND METHODS: A tumour tissue microarray (TMA) was constructed from 36 neuroblastoma samples which were analysed by immunohistochemistry for YB-1 expression. RESULTS: Expression of YB-1 was detected in 35 of 37 (94.6%) neuroblastoma cases examined. Nevertheless, no correlation of YB-1 expression with survival, risk factors or stage of the disease was observed. CONCLUSION: As the majority of neuroblastomas express YB-1, this protein may play an important role in tumour pathogenesis. The results of this study suggest that YB-1 may serve as a novel immune marker for neuroblastoma and may be potentially useful as a therapeutic target.

Workflow analysis of laparoscopic Nissen fundoplication in infant pigs- a model for surgical feedback and training.

BACKGROUND: Many fields use workflow analysis to assess and improve performance of complex tasks. In pediatric endosurgery, workflow analysis may help optimize operative planning and motor skills by breaking down the procedure into particular phases, evaluating these steps individually, and supplying feedback to the surgeon. OBJECTIVE: To develop a module of computer-based surgical workflow analysis for laparoscopic Nissen fundoplication(LNF) and to evaluate its applicability in an infant pig model. METHODS: LNF was performed in 12 pigs (weight, 7-10 kg) by a single surgeon. Based on synchronized intra and extracorporal movie recordings, the surgical workflow was segmented into temporal operative phases(preparation, dissection, reconstruction and conclusion). During each stage, all actions were recorded in a virtual timeline using a customized workflow editor. Specific tasks, such as knot-tying, were evaluated in detail.Time necessary to perform these actions was compared throughout the study. RESULTS: While time required for the preparation decreased by more than 70% from 4577 to 1379 seconds,and the dissection phase decreased from 2359 to 399 seconds (pig 1 and 12, respectively), the other two phases remained relatively stable. Mean time to perform the entire suture and a 5-throw knot remained constant as well. CONCLUSION: Our workflow analysis model allows the quantitative evaluation of dynamic actions related to LNF.This data can be used to define average benchmark criteria for the procedures that comprise this operation. It thereby permits task-oriented refinement of surgical technique as well as monitoring the efficacy of training.Although preoperative preparation time decreased substantially, and dissection became faster, time required for the reconstruction and conclusion phases remained relatively constant for a surgeon with moderate experience.Likewise, knot-tying did not accelerate in this setting.S-117

Cardia yield pressure measurement in an infant porcine model: a novel technique to evaluate the quality of laparoscopic fundoplication.

BACKGROUND: Cardia yield pressure (CYP) has been described as a measure of the combined effect of all antireflux mechanisms and not simply as another test of lower esophageal sphincter pressure. In this paper, we present a simple technique for the measurement of CYP before and after fundoplication through laparoscopic gastrostomy in an experimental pig model. MATERIALS AND METHODS: Twelve 8-week-old female pigs with a mean weight of 8.7 +/- 0.7 kg underwent laparoscopic gastrostomy placement and Nissen fundoplication under general anesthesia. CYP was determined before and after the fundoplication by filling the stomach with water until reaching the pressure at which the cardia opened and became incompetent. Pre- and postoperative CYP was compared by using the Student's t-test for paired samples. RESULTS: Laparoscopic Nissen fundoplication and gastrostomy was completed in all pigs. CYP increased in all subjects after fundoplication, from a mean of 20 +/- 8 to a mean of 63 +/- 13 cm of H(2)O (p < 0.001). The lowest increase in yield pressure of 17.5 cm was recorded after the first operation. Work-flow analysis revealed that this particular procedure took the longest, that bleeding from the liver was encountered, and shorter sutures than those used on all subsequent fundoplications may have compromised knot tying. CONCLUSIONS: CYP increases consistently after laparoscopic Nissen Fundoplication in young pigs. This parameter may be a good indicator of antireflux efficacy and functional quality of the result. Yield pressure measured through laparoscopic gastrostomy offers a new, feasible, and effective technique for the evaluation of antireflux surgery in an experimental setting. Moreover, this minimally invasive technique may become a simple investigative tool for other antireflux procedures.

Midkine is highly expressed in neuroblastoma tissues.

PURPOSE: Neuroblastoma (NBL) is a tumor from neural crest cells, and is the most frequent solid tumor in children. Midkine (MK) is a pleiotropin analogon, which is frequently expressed in neuronal and epithelial tumors and is a marker for a poor clinical outcome. The aims of this study were to assess MK expression in NBL and investigate the correlation with clinical outcome. METHODS: Fifty-six specimens of NBL were stained for MK on a tissue microarray by immunohistochemistry (IHC). Fresh frozen tumor tissues were used for RNA isolation, and RT-PCR analysis for MK-mRNA expression was performed. Survival data, risk factors and disease stages were correlated with MK status assessed by IHC and RT-PCR analysis. RESULTS: MK-mRNA expression was found in the majority of the tumor tissues (75%), whereas MK protein could be detected only in 46% of the NBL by IHC. No correlation of MK status with survival, risk factors or disease stage was observed. CONCLUSION: A majority of NBL express MK-mRNA, whereas not all MK mRNA positive tumors showed also a positive MK IHC staining. The high expression of MK-mRNA expression might present a promising target for new adenovirus-based gene therapeutic approaches for the treatment of NBL.

Universal expression of cell adhesion molecule NCAM in neuroblastoma in contrast to L1: implications for different roles in tumor biology of neuroblastoma?

PURPOSE: Neuroblastoma is a biological, genetic and morphological heterogeneous tumor with a variable clinical course. NCAM is a cell adhesion molecule belonging to the immunoglobulin superfamily with structural similarities to cell adhesion molecule L1. The aim of this study was to determine the expression of NCAM in neuroblastoma and to compare the results to the findings of a previous study which examined L1 expression in the same group of patients. MATERIALS AND METHODS: NCAM expression was investigated on a tissue array with 66 surgically resected neuroblastoma samples by immunohistochemistry with a monoclonal antibody clone 1B6 and peroxidase method. RESULTS: Strong expression of NCAM was detected in all of the 66 (100%) neuroblastoma tumors in contrast to L1 which was not expressed in all tumors. CONCLUSION: In contrast to L1, which was found to predict favorable outcome, NCAM is universally expressed in neuroblastoma. Therefore NCAM represents a marker for neuroblastomas irrespectively of their stages whereas L1 as an indicator for developing neuronal cells seems to identify more mature stages of this tumor. The high grade of NCAM expression might present a prerequisite for establishment of antibody-based therapies.