Fabian Pohl

Wound healing after radiation therapy: Review of the literature

Radiation therapy is an established modality in the treatment of head and neck cancer patients. Compromised wound healing in irradiated tissues is a common and challenging clinical problem. The pathophysiology and underlying cellular mechanisms including the complex interaction of cytokines and growth factors are still not understood completely. In this review, the current state of research regarding the pathomechanisms of compromised wound healing in irradiated tissues is presented. Current and possible future treatment strategies are critically reviewed.

Xanthogranuloma, Rathke's Cyst, and Childhood Craniopharyngioma: Results of Prospective Multinational Studies of Children and Adolescents with Rare Sellar Malformations

CONTEXT Craniopharyngioma (CP), Rathkes cyst (RC), and xanthogranuloma (XG) are closely related rare sellar masses. Treatment strategies in children lack consensus. OBJECTIVE Our objective was to study clinical manifestations and treatment-related outcome in RC, XG, and CP patients. DESIGN We conducted a multicenter surveillance trial. PATIENTS Inclusion criteria were 1) histological diagnosis of CP, XG, or RC and 2) diagnosis at age of 18 yr or less. A total of 117 CP, 14 XG (2001-2006), and 14 RC (1996-2006) were recruited. MAIN OUTCOME Overall survival (OS), event-free survival (EFS), and quality of life (QoL) were evaluated. RESULTS The 5-yr OS rates were 1.00 ± 0.00 in RC and XG and 0.97 ± 0.02 in CP. The 5-yr EFS rates were 0.85 ± 0.10 in RC, 1.00 ± 0.00 in XG, and 0.50 ± 0.05 in CP. Surgical resection of XG results in complete remission without recurrence. Recurrences occur in RC (14%) and CR (59%) but can be efficiently treated by irradiation, reoperation, and/or intracystic treatment. Severe hypothalamic sequelae such as obesity and others affecting QoL are predominant in CP due to presurgical involvement (59%) and postsurgical lesions (44%) of posterior hypothalamic structures. Centers with lower neurosurgery patient load use more radical surgical approaches to treat CP, resulting in higher rates of obesity and reduced QoL. Despite 46% anterior hypothalamic involvement, severe obesity is not encountered in XG. CONCLUSIONS Treatment of choice in XG and RC is radical surgery. In CP involving hypothalamic structures, less radical surgical approaches preserving hypothalamic integrity are recommended. Due to frequent relapses, regular imaging during follow-up is recommended for CP and RC. Treatment of patients with sellar masses should be confined to experienced multidisciplinary teams.

Analyses of treatment variables for patients with childhood craniopharyngioma--results of the multicenter prospective trial KRANIOPHARYNGEOM 2000 after three years of follow-up.

Background: Controversies surround various treatment variables for patients with childhood craniopharyngioma such as growth hormone (GH) replacement, which some believe can exacerbate recurrence/progression. We prospectively assessed the risk of tumor recurrence/progression in survivors of childhood craniopharyngioma. Methods: Multivariable analyses of risk factors (age at diagnosis, degree of resection, irradiation, GH treatment and gender) and descriptive analyses of overall survival (OS) and event-free survival (EFS) rates were performed in 117 patients, recruited prospectively and evaluated after 3 years of follow-up in the German, Austrian and Swiss multicenter trial KRANIOPHARYNGEOM 2000. Results: We observed a 3-year OS of 0.97 and a 3-year EFS of 0.46, indicating high recurrence rates after complete resection (CR) (n = 47; 3-year-EFS: 0.64) and high progression rates after incomplete resection (IR) (n = 64; 3-year EFS: 0.31). The risk of an event decreased by 80% after CR compared to IR (hazard ratio = 0.20; p < 0.001). Irradiation had protective effects on EFS: irradiated patients had an 88% lower risk of recurrence/progression compared to patients without/before irradiation (hazard ratio = 0.12; p < 0.001). GH treatment had no impact on 3-year EFS rates. Conclusions: Tumor recurrences/progressions are frequent and occur early after initial treatment of childhood craniopharyngioma. A radical resection preserving the integrity of hypothalamic structures appears optimal at original diagnosis. Irradiation was efficient in preventing recurrences/progressions. GH treatment had no impact on the low 3-year EFS observed in our study. However, further conclusions on the influence of GH on recurrence rates have to be refined to long-term follow-up studies of patients with childhood craniopharyngioma.

Function of carbonic anhydrase IX in glioblastoma multiforme.

Carbonic anhydrase (CA) IX is over-expressed in glioblastoma; however, its functions in this context are unknown. Metabolically, glioblastomas are highly glycolytic, leading to a significant lactic acid load. Paradoxically, the intracellular pH is alkaline. We hypothesized that CAIX contributes to the extrusion of hydrogen ions into the extracellular space, thereby moderating intra- and extracellular pH and creating an environment conductive to enhanced invasion. We investigated the role of CAIX as a prognostic marker in patients with glioblastoma and its biological function in vitro. CAIX expression was analyzed in 59 patients with glioblastoma by immunohistochemistry. The expression levels were correlated to overall survival. In vitro, U251 and Ln 18 glioblastoma cells were incubated under hypoxia to induce CAIX expression, and RNA interference (RNAi) was used to examine the function of CAIX on cell attachment, invasion, intracellular energy transfer, and susceptibility to adjuvant treatment. High CAIX expression was identified as an independent factor for poor survival in patients with glioblastoma. In vitro, cell attachment and invasion were strongly reduced after knockdown of CAIX. Finally, the effects of radiation and chemotherapy were strongly augmented after CAIX interference and were accompanied by a higher rate of apoptotic cell death. CAIX is an independent prognostic factor for poor outcome in patients with glioblastoma. Cell attachment, invasion, and survival during adjuvant treatment are significantly influenced by high CAIX expression. These results indicate that inhibition of CAIX is a potential metabolic target for the treatment of patients with glioblastoma.

The influence of heterotopic ossification on functional status of hip joint following total hip arthroplasty

Purpose:The functional failure induced by heterotopic ossification (HO) following total hip arthroplasty (THA) was analyzed and correlated to the radiologic failure.Patients and Methods:From July 1997 to July 2001, 315 patients (345 hips) received THA indicated by a hypertrophic osteoarthritis of higher degree (Kellgren grade III, IV). All patients were irradiated prophylactically for prevention of HO on the evening before surgery with a 7-Gy single fraction. The patients’ median age was 66.3 years. Radiologic failure was assessed by comparison of pre- and postoperative hip X-rays (immediately and 6 months after surgery). Analysis of radiographs was performed according to the Brooker Score. Clinical failure was appraised by measurement of passive range of motion (ROM) of the hip joint with a standard goniometer. The t-test was used for statistical analysis.Results:281 patients (81.5%) did not develop HO. HO of Brooker grade I or II was found in 58 patients (16.8%). Six patients (1.7%) developed HO Brooker grade III or IV. There was a significant negative correlation between the degree of radiologic and clinical failure. ROM differed significantly between patients with HO Brooker grade 0, I, II and patients with HO Brooker grade III, IV. Comparing the pre- and postoperative ROM, all patients with Brooker grade 0, I and II showed a significant improvement of flexion, internal and external rotation, abduction and adduction movement. Patients with HO Brooker grade III and IV showed no improvement of ROM in the postoperative follow-up.Conclusion:The development of HO following THA influences the physical function of the hip joint dependent on the degree of ossification. HO of lower degree (Brooker I, II) does not influence the clinical outcome, whereas HO of higher degree (Brooker III, IV) reduces the function of hip arthroplasty. Therefore, the purpose of a prophylactic therapy must be to reduce HO of higher degree.Ziel:Der Einfluss heterotoper Ossifikationen (HO) nach Totalendoprothese des Hüftgelenks (TEP) auf die Gelenkbeweglichkeit wird untersucht.Patienten und Methodik:Von Juli 1997 bis Juli 2001 erhielten 315 Patienten (345 Hüften) eine TEP. Als Operationsindikation lag bei allen Patienten eine höhergradige Koxarthrose vor (Kellgren III, IV). Die Patienten wurden am Vorabend der Operation mit einer Einzeldosis von 7 Gy als Ossifikationsprophylaxe bestrahlt. Das mediane Alter der Patienten betrug 66,3 Jahre. Die Klassifikation der HO wurde anhand prä- und postoperativer Röntgenaufnahmen (am Operationstag bzw. 6 Monate nach Operation) nach dem Brooker-Score durchgeführt. Folgende präoperative und in der Nachsorge erhobene Beweglichkeitsparameter (ROM) wurden in Abhängigkeit von der HO-Entwicklung miteinander verglichen: Flexion, Außen- und Innenrotation, Ab- und Adduktion. Der t-Test für gepaarte Stichproben wurde für die statistische Analyse verwendet.Ergebnisse:281 Patienten (81,5%) entwickelten keine HO, 58 Patienten (16,8%) HO Grad I/II und sechs Patienten (1,7%) HO Grad III/IV. Die erhobenen ROM unterschieden sich signifikant bei den Patienten, die keine oder niedriggradige HO (Brooker I, II) entwickelt hatten, und denen, die höhergradige HO (Brooker III, IV) aufwiesen. Die Patienten mit HO Brooker 0, I und II zeigten im Gegensatz zu den Patienten mit HO Brooker III und IV postoperativ eine signifikante Verbesserung der Werte für Flexion, Innen- und Außenrotation, Ab- und Adduktion.Schlussfolgerung:Die Entstehung von HO nach TEP beeinflusst in Abhängigkeit von deren Ausmaß die postoperative Hüftbeweglichkeit. Dabei sind HO Grad I und II nicht, Grad III und IV von großer klinischer Relevanz. Daher muss eine prophylaktische Therapie insbesondere darauf abzielen, die Inzidenz höhergradiger HO zu reduzieren.

Preoperative irradiation for prevention of heterotopic ossification following prosthetic total hip replacement results of a prospective study in 462 hips.

Background:The effectiveness of pre- or postoperative radiotherapy for prevention of heterotopic ossification (HO) following total hip replacement (THR) has already been demonstrated in the past. Thereby, in most studies using preoperative radiotherapy patients were irradiated < 6 h before surgery. The purpose of this prospective study was to analyze the effectiveness of preoperative irradiation on the evening before surgery and to identify risk factors for HO in a homogeneous collective of patients.Patients and Methods:From July 1997 to July 2001, 416 patients (462 hips; 235 males, 227 females) received preoperative radiotherapy of the hip on the evening before surgery with a 7-Gy single fraction. The patients’ median age was 67.1 years. The most frequent indication for radiotherapy was hypertrophic osteoarthritis (383 hips, 82.9%). Treatment results were assessed by comparison of pre- and postoperative hip X-rays (immediately and 6 months after surgery). The analysis of radiographs was performed according to the Brooker score.Results:The overall incidence of HO was 18.1% (n = 84), Brooker score 1 12.3% (n = 57), score 2 3.9% (n = 18), score 3 1.5% (n = 7), and score 4 0.4% (n = 2). Sex, body height, hypertrophic osteoarthritis of higher degree, size of the femoral component of the prosthesis, previous ipsi- or contralateral HO, and short course of nonsteroidal anti-inflammatory drug (diclofenac) therapy significantly influenced the HO rate in univariate analysis. In multivariate analysis, an interdependence of prosthesis size, sex and patient’s height was found. From these three variables, only prosthesis size was statistically significant in multivariate analysis. The cumulative dose of diclofenac (≤ 300 mg or > 300 mg) within the first 7 postoperative days and previous ipsi- or contralateral HO influenced the incidence of HO in multivariate analysis.Conclusion:Preoperative radiotherapy on the evening before surgery is an effective treatment modality to reduce overall (Brooker 1–4) and clinically relevant, severe HOs (Brooker 3–4), and includes several advantages compared to postoperative irradiation. Previous ipsi- and contralateral HOs were identified as high risk factors for HO in this study. In patients with these risk factors, the incidence of HO increased.Hintergrund:Die Wirksamkeit einer prä- oder postoperativen Radiatio zur Vermeidung heterotoper Ossifikationen (HO) nach totalendoprothetischem Hüftgelenkersatz (TEP) wurde in der Vergangenheit bereits gezeigt. Dabei wurde in den meisten Studien einer präoperativen Radiatio die Bestrahlung im Zeitraum von 6 h vor der Operation durchgeführt. Ziel dieser Arbeit war es, die Wirksamkeit einer präoperativen Bestrahlung am Abend vor dem Operationstag in einem homogenen Patientenkollektiv zu überprüfen.Patienten und Methodik:Von 07/1997 bis 07/2001 wurde bei 416 Patienten (462 Hüften; 235 Männer, 227 Frauen) eine präoperative Einzeitbestrahlung der Hüfte mit einer Dosis von 7 Gy durchgeführt. Das mediane Alter der Patienten betrug 67,1 Jahre. Die häufigste Indikation für die prophylaktische Bestrahlung war eine hypertrophe Osteoarthritis (383 Hüften, 82,9%). Das Therapieergebnis wurde anhand von Röntgenbildern unmittelbar postoperativ und 6 Monate nach Operation gemäß dem Brooker- Score beurteilt.Ergebnisse:Die HO-Inzidenz betrug 18,1% (n = 84), Brooker-Grad 1 12,3% (n = 57), Grad 2 3,9% (n = 18), Grad 3 1,5% (n = 7) and Grad 4 0,4% (n = 2). Geschlecht, Körpergröße, Ausmaß der Osteoarthritis, Größe des Prothesenschafts, vorherige ipsi- oder kontralaterale HO und die zusätzliche Gabe von nichtsteroidalen Antiphlogistika (Diclofenac) hatten in der univariaten Analyse signifikanten Einfluss auf die HO-Rate. Es fand sich eine Korrelation von Geschlecht, Körpergröße und Schaftgröße, so dass in der multivariaten Analyse nur noch die Schaftgröße eine Signifikanz aufwies. Die kumulative Diclofenacdosis (≤ 300 mg bzw. > 300 mg) und vorherige ipsi- oder kontralaterale HO behielten auch in der multivariaten Analyse ihren signifikanten Einfluss auf die HO-Rate.Schlussfolgerung:Eine präoperative Bestrahlung am Vorabend der Operation stellt eine effektive Behandlung zur Vermeidung von HO nach TEP dar und hat gegenüber einer postoperativen Bestrahlung Vorteile. Insbesondere vorherige ipsi- und kontralaterale HO stellen Hochrisikofaktoren für die Entstehung von HO auch nach Bestrahlung dar.

Influence of rectum delineation (rectal volume vs. rectal wall) on IMRT treatment planning of the prostate.

Purpose:To evaluate the delineation of either the rectal volume (RV) or the rectal wall (RW) in intensity-modulated radiotherapy (IMRT) for prostate cancer: influence on dose distribution to the targets and organs at risk (OARs) was investigated.Material and Methods:For ten patients with localized prostate cancer IMRT treatment plans were generated with the RV, wall including the filling, and the RW without the lumen as OAR (plan-RV and plan-RW), respectively. Two different IMRT treatment- planning systems (TPS) were utilized. The influence on target coverage and sparing of OARs was investigated.Results:No influence was seen on target coverage and sparing of the bladder and femoral heads. Doses to the RV were significantly reduced in plan-RV for all evaluated dose levels: maximum 26% and 17%, respectively, in both TPS. The dose distribution to the RW was not significantly different between plan-RV and plan-RW.Conclusion:The different delineation of the OAR rectum significantly affected the inverse IMRT treatment-planning process. The use of the RV as OAR resulted in improved dose distributions to the RV. Therefore, it is suggested using the RV as OAR in IMRT treatment planning of the prostate.Ziel:Untersucht wurde, ob die unterschiedliche Konturierung des Rektums—Rektumvolumen (RV) als Wand einschließlich Lumen oder Rektumwand allein (RW)—den inversen Bestrahlungsplanungsprozess der intensitätsmodulierten Strahlentherapie (IMRT) beeinflusst.Material und Methodik:Für zehn Patienten mit lokal begrenztem Prostatakarzinom wurden jeweils zwei IMRT-Behandlungspläne erstellt, 1. mit dem RV (Plan-RV) und 2. mit der RW als Risikoorgan (Plan-RW). Diese Pläne wurden jeweils mit zwei verschiedenen IMRT-Bestrahlungsplanungssystemen (BPS) berechnet. Der Einfluss der unterschiedlichen Konturierung des Rektums auf die Dosisverteilung im Zielvolumen und in den Risikoorganen wurde untersucht.Ergebnisse:Die Konturierung des Rektums hatte keinen Einfluss auf die Dosisverteilungen im Zielvolumen oder in den Risikoorganen Blase und Hüftköpfe. Die Belastung des RV war in Plan-RV in allen untersuchten Dosisbereichen signifikant reduziert verglichen mit Plan-RW: maximal 26% und 17% in den beiden verwendeten BPS. Die Dosisverteilung im RW wurde durch die Konturierung des Rektums nicht beeinflusst.Schlussfolgerung:Die unterschiedliche Konturierung des Rektums führte zu einer signifikanten Beeinflussung des inversen Planungsprozesses der IMRT. Es wird empfohlen, bei der IMRT-Planung des Prostatakarzinoms das RV als Risikoorgan zu konturieren.

Preoperative Irradiation for Prevention of Heterotopic Ossification Following Prosthetic Total Hip Replacement

Background:The effectiveness of pre- or postoperative radiotherapy for prevention of heterotopic ossification (HO) following total hip replacement (THR) has already been demonstrated in the past. Thereby, in most studies using preoperative radiotherapy patients were irradiated < 6 h before surgery. The purpose of this prospective study was to analyze the effectiveness of preoperative irradiation on the evening before surgery and to identify risk factors for HO in a homogeneous collective of patients.Patients and Methods:From July 1997 to July 2001, 416 patients (462 hips; 235 males, 227 females) received preoperative radiotherapy of the hip on the evening before surgery with a 7-Gy single fraction. The patients’ median age was 67.1 years. The most frequent indication for radiotherapy was hypertrophic osteoarthritis (383 hips, 82.9%). Treatment results were assessed by comparison of pre- and postoperative hip X-rays (immediately and 6 months after surgery). The analysis of radiographs was performed according to the Brooker score.Results:The overall incidence of HO was 18.1% (n = 84), Brooker score 1 12.3% (n = 57), score 2 3.9% (n = 18), score 3 1.5% (n = 7), and score 4 0.4% (n = 2). Sex, body height, hypertrophic osteoarthritis of higher degree, size of the femoral component of the prosthesis, previous ipsi- or contralateral HO, and short course of nonsteroidal anti-inflammatory drug (diclofenac) therapy significantly influenced the HO rate in univariate analysis. In multivariate analysis, an interdependence of prosthesis size, sex and patient’s height was found. From these three variables, only prosthesis size was statistically significant in multivariate analysis. The cumulative dose of diclofenac (≤ 300 mg or > 300 mg) within the first 7 postoperative days and previous ipsi- or contralateral HO influenced the incidence of HO in multivariate analysis.Conclusion:Preoperative radiotherapy on the evening before surgery is an effective treatment modality to reduce overall (Brooker 1–4) and clinically relevant, severe HOs (Brooker 3–4), and includes several advantages compared to postoperative irradiation. Previous ipsi- and contralateral HOs were identified as high risk factors for HO in this study. In patients with these risk factors, the incidence of HO increased.Hintergrund:Die Wirksamkeit einer prä- oder postoperativen Radiatio zur Vermeidung heterotoper Ossifikationen (HO) nach totalendoprothetischem Hüftgelenkersatz (TEP) wurde in der Vergangenheit bereits gezeigt. Dabei wurde in den meisten Studien einer präoperativen Radiatio die Bestrahlung im Zeitraum von 6 h vor der Operation durchgeführt. Ziel dieser Arbeit war es, die Wirksamkeit einer präoperativen Bestrahlung am Abend vor dem Operationstag in einem homogenen Patientenkollektiv zu überprüfen.Patienten und Methodik:Von 07/1997 bis 07/2001 wurde bei 416 Patienten (462 Hüften; 235 Männer, 227 Frauen) eine präoperative Einzeitbestrahlung der Hüfte mit einer Dosis von 7 Gy durchgeführt. Das mediane Alter der Patienten betrug 67,1 Jahre. Die häufigste Indikation für die prophylaktische Bestrahlung war eine hypertrophe Osteoarthritis (383 Hüften, 82,9%). Das Therapieergebnis wurde anhand von Röntgenbildern unmittelbar postoperativ und 6 Monate nach Operation gemäß dem Brooker- Score beurteilt.Ergebnisse:Die HO-Inzidenz betrug 18,1% (n = 84), Brooker-Grad 1 12,3% (n = 57), Grad 2 3,9% (n = 18), Grad 3 1,5% (n = 7) and Grad 4 0,4% (n = 2). Geschlecht, Körpergröße, Ausmaß der Osteoarthritis, Größe des Prothesenschafts, vorherige ipsi- oder kontralaterale HO und die zusätzliche Gabe von nichtsteroidalen Antiphlogistika (Diclofenac) hatten in der univariaten Analyse signifikanten Einfluss auf die HO-Rate. Es fand sich eine Korrelation von Geschlecht, Körpergröße und Schaftgröße, so dass in der multivariaten Analyse nur noch die Schaftgröße eine Signifikanz aufwies. Die kumulative Diclofenacdosis (≤ 300 mg bzw. > 300 mg) und vorherige ipsi- oder kontralaterale HO behielten auch in der multivariaten Analyse ihren signifikanten Einfluss auf die HO-Rate.Schlussfolgerung:Eine präoperative Bestrahlung am Vorabend der Operation stellt eine effektive Behandlung zur Vermeidung von HO nach TEP dar und hat gegenüber einer postoperativen Bestrahlung Vorteile. Insbesondere vorherige ipsi- und kontralaterale HO stellen Hochrisikofaktoren für die Entstehung von HO auch nach Bestrahlung dar.

Radiolabeled annexin V for imaging apoptosis in radiated human follicular thyroid carcinomas — is an individualized protocol necessary?

INTRODUCTION Induction of apoptosis is a widely used strategy for cancer therapy, but evaluating the degree and success of this therapy still poses a problem. Radiolabeled annexin V has been proposed to be a promising candidate for detecting apoptotic cells in tumors following chemotherapy in vivo. In order to see whether radiolabeled annexin V could be a suitable substance for the noninvasive in vivo detection of apoptosis in thyroid tissue and to establish an optimized study protocol, we investigated two poorly differentiated thyroid carcinoma cell lines: ML-1 and FTC-133. METHODS Apoptosis was evaluated before as well as 2 and 4 days after in vitro irradiation with 30 Gy X-rays. In this study, binding of FITC- and of (125)I-labeled annexin V was measured in comparison to other apoptosis markers such as Bax, caspase-3 and Fas, which were determined by flow cytometry and Western blot analysis with densitometric evaluation. RESULTS ML-1 and FTC-133 cells showed a significant increase in annexin V binding 48 h after irradiation. Ninety-six hours after irradiation, the annexin V absorption capability of ML-1 cells was still maximal, while the living fraction of FTC-133 increased significantly. The amount of caspase-3 and Bax was clearly increased 48 h after irradiation and had normalized after 96 h in both cell lines. Fas protein concentrations remained unchanged in ML-1 cells but were significantly enhanced in FTC-133 cells. CONCLUSION The binding of FITC- and (125)I-labeled annexin V showed a significant accordance. A reliable evaluation of apoptosis induced by radiotherapy in thyroid tumors was possible 48 h after irradiation, when binding of radiolabeled annexin V is most significantly enhanced. Using two poorly differentiated cell lines of thyroid carcinoma, one may expect to find a nearly similar response to external irradiation. In contrast, the cell lines showed a completely contrary response. However, an individualized study protocol for each type of tumor and probably within each type is necessary.

Kraniopharyngeom im Kindes- und Jugendalter

ZusammenfassungKraniopharyngeome sind dysontogenetische Mittellinienfehlbildungen mit Raum forderndem Charakter. 30–50% der Fälle werden im Kindes- und Jugendalter manifest und repräsentieren damit 5–10% aller intrakraniellen Tumoren im Kindesalter. Der Versuch einer kompletten Resektion unter Wahrung der optischen und hypothalamisch-hypophysären Funktion stellt die Therapie der ersten Wahl bei günstiger Lokalisation dar. Bei ungünstiger Lokalisation ist diese Therapieoption gegen eine begrenzte Resektion mit anschließender Strahlentherapie abzuwägen. Bei insgesamt hoher Überlebensrate wird die Lebensqualität der Patienten durch ophthalmologische, neuropsychiatrische und endokrine Spätfolgen beeinträchtigt. Der Adipositas infolge hypothalamischer Essstörungen kommt hierbei besondere Bedeutung zu. In einer multizentrischen Querschnittuntersuchung (n=138) zeigte die Lebensqualität keine signifikante Abhängigkeit vom intendierten oder realisierten neurochirurgischen Resektionsgrad. Im Rahmen einer multizentrischen Beobachtungsstudie (Kraniopharyngeom 2000; www.kraniopharyngeom.com) wird der Einfluss unterschiedlicher Therapiestrategien auf die Überlebensrate und die Lebensqualität von Kindern und Jugendlichen mit Kraniopharyngeom prospektiv untersucht. Aufgrund der hohen Meldebereitschaft der beteiligten Zentren ist zu hoffen, dass auch in Zukunft die Vollständigkeit der Erfassung deutlich verbessert wird.AbstractCraniopharyngiomas are benign, partly cystic epithelial tumors of the sellar region. Between 30% and 50% of the cases manifest during childhood and adolescence and represent 5–10% of all cases of intracranial tumors during childhood. The therapy of choice in patients with favourable tumor location is total microscopic resection while maintaining the visual and hypothalamic-pituitary function. In patients with unfavourable tumor location, this therapeutic strategy is subject to controversy, and consideration should be given to limited resection followed by radiation therapy. The overall survival rates are high. However, the quality of life (QoL) is substantially reduced in many survivors due to neuropsychological, ophthalmologic, and endocrine sequelae. Extreme obesity caused by eating disorders of hypothalamic origin has a major negative impact on QoL. In a cross-sectional multicenter study (n=138), QoL was not related to intended or realized degree of surgical resection. A multicenter study (Kraniopharyngeom 2000; English-language study protocol available at: www.kraniopharyngeom.com) was initiated to analyze prospectively the survival rate and QoL in newly diagnosed patients with childhood craniopharyngioma in relation to different therapeutic strategies. Based on the high rate of compliance of participating centers in Germany, Austria, and Switzerland, we hope to substantially improve the extent of epidemiologic registration of this rare disease in the near future.