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1. Combination of nivolumab with standard induction chemotherapy in children and adults with EBV-positive nasopharyngeal carcinoma: Protocol of a prospective multicenter phase 2 trial

Author

Römer, Tristan, Vokuhl, Christian, Staatz, Gundula, Mottaghy, Felix M., Christiansen, Hans, Eble, Michael J., Timmermann, Beate, Klussmann, Jens Peter, Elbracht, Miriam, Calaminus, Gabriele, Zimmermann, Martin, Brümmendorf, Tim H., Feuchtinger, Tobias, Kerp, Helena, and Kontny, Udo

Published
2024
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10. Refractory and relapsed paediatric ACC in the MET studies – A challenging situation necessitating novel diagnostic and therapeutic concepts

Author

Kuhlen, Michaela, Kunstreich, Marina, Lessel, Lienhard, Wudy, Stefan A., Holterhus, Paul-Martin, Vokuhl, Christian, Juettner, Eva, Roecken, Christoph, Seitz, Guido, Slavetinsky, Christoph, Fuchs, Jörg, Schewe, Denis M., Claus, Rainer, Johann, Pascal D., Frühwald, Michael C., Vorwerk, Peter, and Redlich, Antje

Published
2023
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11. Congenital spindle cell rhabdomyosarcoma: An international cooperative analysis

Author

Whittle, Sarah, Venkatramani, Rajkumar, Schönstein, Anton, Pack, Svetlana D., Alaggio, Rita, Vokuhl, Christian, Rudzinski, Erin R., Wulf, Anna–Lena, Zin, Angelica, Gruver, Juliana R., Arnold, Michael A., Merks, Johannes H.M., Hettmer, Simone, Koscielniak, Ewa, Barr, Frederic G., Hawkins, Douglas S., Bisogno, Gianni, and Sparber-Sauer, Monika

Published
2022
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12. Placental Sonomorphologic Appearance and Fetomaternal Outcome in Fontan Circulation.

Author

Jost, Elena, Gembruch, Ulrich, Schneider, Martin, Gieselmann, Andrea, La Rosée, Karl, Momcilovic, Diana, Vokuhl, Christian, Kosian, Philipp, Ayub, Tiyasha H., and Merz, Waltraut M.

Subjects
FETAL growth retardation, HIGH-risk pregnancy, PREGNANCY complications, FETAL diseases, PREGNANCY outcomes
Abstract

Objectives: Pregnancies in women with Fontan circulation are on the rise, and they are known to imply high maternal and fetal complication rates. The altered hemodynamic profile of univentricular circulation affects placental development and function. This study describes placental sonomorphologic appearance and Doppler examinations and correlates these to histopathologic findings and pregnancy outcomes in women with Fontan circulation. Methods: A single-center retrospective analysis of pregnancies in women with Fontan circulation was conducted between 2018 and 2023. Maternal characteristics and obstetric and neonatal outcomes were recorded. Serial ultrasound examinations including placental sonomorphologic appearance and Doppler studies were assessed. Macroscopic and histopathologic findings of the placentas were reviewed. Results: Six live births from six women with Fontan physiology were available for analysis. Prematurity occurred in 83% (5/6 cases) and fetal growth restriction and bleeding events in 66% (4/6 cases) each. All but one placenta showed similar sonomorphologic abnormalities starting during the late second trimester, such as thickened globular shape, inhomogeneous echotexture, and hypoechoic lakes, resulting in a jelly-like appearance. Uteroplacental blood flow indices were within normal range in all women. The corresponding histopathologic findings were non-specific and consisted of intervillous and subchorionic fibrin deposition, villous atrophy, hypoplasia, or fibrosis. Conclusions: Obstetric and perinatal complication rates in pregnancies of women with Fontan circulation are high. Thus, predictors are urgently needed. Our results suggest that serial ultrasound examinations with increased awareness of the placental appearance and its development, linked to the Doppler sonographic results of the uteroplacental and fetomaternal circulation, may be suitable for the early identification of cases prone to complications. [ABSTRACT FROM AUTHOR]

Published
2024
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18. Supratentorial ependymoma in childhood: more than just RELA or YAP

Author

Zschernack, Valentina, Jünger, Stephanie T., Mynarek, Martin, Rutkowski, Stefan, Garre, Maria Luisa, Ebinger, Martin, Neu, Marie, Faber, Jörg, Erdlenbruch, Bernhard, Claviez, Alexander, Bielack, Stefan, Brozou, Triantafyllia, Frühwald, Michael C., Dörner, Evelyn, Dreschmann, Verena, Stock, Annika, Solymosi, Laszlo, Hench, Jürgen, Frank, Stephan, Vokuhl, Christian, Waha, Andreas, Andreiuolo, Felipe, and Pietsch, Torsten

Published
2021
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19. Non-Syndromic and Syndromic Defects in Children with Extracranial Germ Cell Tumors: Data of 2610 Children Registered with the German MAKEI 96/MAHO 98 Registry Compared to the General Population.

Author

Schultewolter, Judit H., Rissmann, Anke, von Schweinitz, Dietrich, Frühwald, Michael, Blattmann, Claudia, Fischer, Lars, Lange, Björn Sönke, Wessalowski, Rüdiger, Fröhlich, Birgit, Behnisch, Wolfgang, Schmid, Irene, Reinhard, Harald, Dürken, Matthias, Hundsdörfer, Patrick, Heimbrodt, Martin, Vokuhl, Christian, Schönberger, Stefan, Schneider, Dominik T., Seitz, Guido, and Looijenga, Leendert

Subjects
SYNDROMES, RISK assessment, TUMORS in children, HUMAN abnormalities, DATA analysis, FISHER exact test, DESCRIPTIVE statistics, LONGITUDINAL method, ODDS ratio, STATISTICS, DATA analysis software, CONFIDENCE intervals, GERM cell tumors, DISEASE complications, CHILDREN
Abstract

Simple Summary: Our findings confirm the role of developmental aspects in extracranial germ cell tumors (eGCTs). Strong associations between syndromes or congenital anomalies and eGCTs may hint at the need for research to identify potential causes and mechanisms. Strong effects were observed for Swyer and Currarino syndrome. The strengths of the associations emphasize the need for further elaboration on presently discussed pathophysiological mechanisms. For other suggested risk factors such as Klinefelter, Turner, and Down syndrome, the strength of risk appears to be substantially low and may be missed because of diagnostic bias for the first two or lack of power for the latter. A number of further syndromes were identified in single cases. Most of these pertain to girls. GCTs are developmental tumors and are likely to reflect ontogenetic and teratogenetic determinants. The objective of this study was to identify syndromes with or without congenital anomalies and non-syndromic defects as potential risk factors. Patients with extracranial GCTs (eGCTs) registered in MAKEI 96/MAHO 98 between 1996 and 2017 were included. According to Teilum's holistic concept, malignant and benign teratomas were registered. We used a case–control study design with Orphanet as a reference group for syndromic defects and the Mainz birth registry (EUROCAT) for congenital anomalies at birth. Co-occurring genetic syndromes and/or congenital anomalies were assessed accordingly. Odds ratios and 95% confidence intervals were calculated and p-values for Fisher's exact test with Bonferroni correction if needed. A strong association was confirmed for Swyer (OR 338.6, 95% CI 43.7–2623.6) and Currarino syndrome (OR 34.2, 95% CI 13.2–88.6). We additionally found 16 isolated cases of eGCT with a wide range of syndromes. However, these were not found to be significantly associated following Bonferroni correction. Most of these cases pertained to girls. Regarding non-syndromic defects, no association with eGCTs could be identified. In our study, we confirmed a strong association for Swyer and Currarino syndromes with additional congenital anomalies. [ABSTRACT FROM AUTHOR]

Published
2024
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20. How to improve initial diagnostic accuracy of kidney tumours in childhood?—A non‐invasive approach.

Author

Welter, Nils, Metternich, Gregor, Furtwängler, Rhoikos, Bayoumi, Ahmed, Mergen, Marvin, Kager, Leo, Vokuhl, Christian, Warmann, Steven W., Fuchs, Jörg, Meier, Clemens‐Magnus, Melchior, Patrick, Gessler, Manfred, Wagenpfeil, Stefan, Schenk, Jens‐Peter, and Graf, Norbert

Subjects
RECEIVER operating characteristic curves, RENAL cell carcinoma, DIFFUSION magnetic resonance imaging, NEOADJUVANT chemotherapy, TUMORS
Abstract

Non‐invasive differentiation of paediatric kidney tumours is particularly important in the SIOP‐RTSG protocols, which recommend pre‐operative chemotherapy without histological confirmation. The identification of clinical and tumour‐related parameters may enhance diagnostic accuracy. Age, metastases, and tumour volume (TV) were retrospectively analysed in 3306 patients enrolled in SIOP/GPOH 9, 93‐01, and 2001 including Wilms tumour (WT), congenital mesoblastic nephroma (CMN), clear cell sarcoma (CCSK), malignant rhabdoid tumour of the kidney (MRTK), and renal cell carcinoma (RCC). WT was diagnosed in 2927 (88.5%) patients followed by CMN 138 (4.2%), CCSK 126 (3.8%), MRTK 58 (1.8%) and RCC 57 (1.7%). CMN, the most common localized tumour (71.6%) in patients younger than 3 months of age, was diagnosed earliest and RCC the latest (median age [months]: 0 and 154, respectively) both associated with significantly smaller TV (median TV [mL]: 67.2 and 45.0, respectively). RCC occurred in >14% of patients older than 120 months or older than 84 months with TV <100 mL. Receiver operating characteristic analyses discriminated WT from CMN, RCC and MRTK regarding age (AUC = 0.976, 0.929 and 0.791) and TV (AUC = 0.768, 0.813 and 0.622). MRTK had the highest risk of metastasis (37.9%) despite young age, whereas the risk of metastasis increased significantly with age in WT. Age and TV at diagnosis can differentiate WT from CMN and RCC. MRTK must be considered for metastatic tumours at young age. Identification of CCSK without histology remains challenging. Combined with MRI‐characteristics, including diffusion‐weighted imaging, and radiomics and liquid biopsies in the future, our approach allows optimization of biopsy recommendations and prevention of misdiagnosis‐based neoadjuvant treatment. [ABSTRACT FROM AUTHOR]

Published
2024
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21. The impact of the route to diagnosis in nephroblastoma.

Author

Mergen, Marvin, Welter, Nils, Furtwängler, Rhoikos, Melchior, Patrick, Vokuhl, Christian, Gessler, Manfred, Meier, Clemens‐Magnus, Kager, Leo, Schenk, Jens‐Peter, and Graf, Norbert

Subjects
NEPHROBLASTOMA, DIAGNOSIS, TUMOR diagnosis, NEOADJUVANT chemotherapy, RENAL cancer
Abstract

Introduction: Wilms tumor (WT) is the most common childhood kidney cancer. It is a rapid growing embryonal tumor in young children and can be diagnosed with and without tumor related symptoms. Methods: We retrospectively analyzed the route to diagnosis of WT treated prospectively according to the SIOP 93‐01/GPOH and 2001/GPOH in Germany between 1993 and 2022. Four routes were defined: diagnosis due to tumor‐related symptoms, incidental diagnosis during another disease, diagnosis by preventive examinations, and diagnosis within a surveillance program. For these groups we compared clinical and tumor characteristics and outcome. Results: Of 2549 patients with WT 1822 (71.5%) were diagnosed by tumor‐related symptoms, 472 (18.5%) incidentally, 213 (8.4%) by preventive medical examinations, and 42 (1.6%) by surveillance. Age, general health status, tumor volume, and local and overall stage varied significantly between these groups. The youngest patients were those diagnosed by preventive medical examination (mean: 1.70 years). These patients also showed the best general health status. Tumor volume at diagnosis (549 mL) and after preoperative chemotherapy (255 mL) was significantly higher for children with tumor‐related symptoms. The highest percentage of local stage I (78.6%) and the lowest percentage of metastatic disease (4.8%) was found in the surveillance group. The outcome of patients was not significantly different, with up to 19.0% relapses in the surveillance group and 3.0% deaths in the group with tumor‐related symptoms. Conclusion: The route to diagnosis of WT correlates with age, general health status, tumor volume, and stage distribution, but does not impact the outcome of patients. Nonetheless, diagnosis without tumor related symptoms results in lower treatment burden and thus improved quality of life. [ABSTRACT FROM AUTHOR]

Published
2024
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24. International Comparisons of Clinical Demographics and Outcomes in the International Society of Pediatric Oncology Wilms Tumor 2001 Trial and Study

Author

de Aguirre-Neto, Joaquim Caetano, de Camargo, Beatriz, van Tinteren, Harm, Bergeron, Christophe, Brok, Jesper, Ramírez-Villar, Gema, Verschuur, Arnauld, Furtwängler, Rhoikos, Howell, Lisa, Saunders, Daniel, Olsen, Oystein, Coulomb, Aurore, Vokuhl, Christian, Godzinski, Jan, Smets, Anne M., Vujanic, Gordan M., van den Heuvel-Eibrink, Marry M., Graf, Norbert, and Pritchard-Jones, Kathy

Published
2022
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26. A mechanistic classification of clinical phenotypes in neuroblastoma

Author

Ackermann, Sandra, Cartolano, Maria, Hero, Barbara, Welte, Anne, Kahlert, Yvonne, Roderwieser, Andrea, Bartenhagen, Christoph, Walter, Esther, Gecht, Judith, Kerschke, Laura, Volland, Ruth, Menon, Roopika, Heuckmann, Johannes M., Gartlgruber, Moritz, Hartlieb, Sabine, Henrich, Kai-Oliver, Okonechnikov, Konstantin, Altmüller, Janine, Nürnberg, Peter, Lefever, Steve, de Wilde, Bram, Sand, Frederik, Ikram, Fakhera, Rosswog, Carolina, Fischer, Janina, Theissen, Jessica, Hertwig, Falk, Singhi, Aatur D., Simon, Thorsten, Vogel, Wenzel, Perner, Sven, Krug, Barbara, Schmidt, Matthias, Rahmann, Sven, Achter, Viktor, Lang, Ulrich, Vokuhl, Christian, Ortmann, Monika, Büttner, Reinhard, Eggert, Angelika, Speleman, Frank, O’Sullivan, Roderick J., Thomas, Roman K., Berthold, Frank, Vandesompele, Jo, Schramm, Alexander, Westermann, Frank, Schulte, Johannes H., Peifer, Martin, and Fischer, Matthias

Published
2018

27. Sarcoma classification by DNA methylation profiling

Author

Koelsche, Christian, Schrimpf, Daniel, Stichel, Damian, Sill, Martin, Sahm, Felix, Reuss, David E., Blattner, Mirjam, Worst, Barbara, Heilig, Christoph E., Beck, Katja, Horak, Peter, Kreutzfeldt, Simon, Paff, Elke, Stark, Sebastian, Johann, Pascal, Selt, Florian, Ecker, Jonas, Sturm, Dominik, Pajtler, Kristian W., Reinhardt, Annekathrin, Wefers, Annika K., Sievers, Philipp, Ebrahimi, Azadeh, Suwala, Abigail, Fernández-Klett, Francisco, Casalini, Belén, Korshunov, Andrey, Hovestadt, Volker, Kommoss, Felix K. F., Kriegsmann, Mark, Schick, Matthias, Bewerunge-Hudler, Melanie, Milde, Till, Witt, Olaf, Kulozik, Andreas E., Kool, Marcel, Romero-Pérez, Laura, Grünewald, Thomas G. P., Kirchner, Thomas, Wick, Wolfgang, Platten, Michael, Unterberg, Andreas, Uhl, Matthias, Abdollahi, Amir, Debus, Jürgen, Lehner, Burkhard, Thomas, Christian, Hasselblatt, Martin, Paulus, Werner, Hartmann, Christian, Staszewski, Ori, Prinz, Marco, Hench, Jürgen, Frank, Stephan, Versleijen-Jonkers, Yvonne M. H., Weidema, Marije E., Mentzel, Thomas, Griewank, Klaus, de Álava, Enrique, Martín, Juan Díaz, Gastearena, Miguel A. Idoate, Chang, Kenneth Tou-En, Low, Sharon Yin Yee, Cuevas-Bourdier, Adrian, Mittelbronn, Michel, Mynarek, Martin, Rutkowski, Stefan, Schüller, Ulrich, Mautner, Viktor F., Schittenhelm, Jens, Serrano, Jonathan, Snuderl, Matija, Büttner, Reinhard, Klingebiel, Thomas, Buslei, Rolf, Gessler, Manfred, Wesseling, Pieter, Dinjens, Winand N. M., Brandner, Sebastian, Jaunmuktane, Zane, Lyskjær, Iben, Schirmacher, Peter, Stenzinger, Albrecht, Brors, Benedikt, Glimm, Hanno, Heining, Christoph, Tirado, Oscar M., Sáinz-Jaspeado, Miguel, Mora, Jaume, Alonso, Javier, del Muro, Xavier Garcia, Moran, Sebastian, Esteller, Manel, Benhamida, Jamal K., Ladanyi, Marc, Wardelmann, Eva, Antonescu, Cristina, Flanagan, Adrienne, Dirksen, Uta, Hohenberger, Peter, Baumhoer, Daniel, Hartmann, Wolfgang, Vokuhl, Christian, Flucke, Uta, Petersen, Iver, Mechtersheimer, Gunhild, Capper, David, Jones, David T. W., Fröhling, Stefan, Pfister, Stefan M., and von Deimling, Andreas

Published
2021
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28. CD79a promotes CNS-infiltration and leukemia engraftment in pediatric B-cell precursor acute lymphoblastic leukemia

Author

Lenk, Lennart, Carlet, Michela, Vogiatzi, Fotini, Spory, Lea, Winterberg, Dorothee, Cousins, Antony, Vossen-Gajcy, Michaela, Ibruli, Olta, Vokuhl, Christian, Cario, Gunnar, El Ayoubi, Omar, Kramer, Lisa, Ritgen, Matthias, Brüggemann, Monika, Häsler, Robert, Schrappe, Martin, Fuhrmann, Stephan, Halsey, Christina, Jeremias, Irmela, Hobeika, Elias, Jumaa, Hassan, Alsadeq, Ameera, and Schewe, Denis M.

Published
2021
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32. Array-based DNA-methylation profiling in sarcomas with small blue round cell histology provides valuable diagnostic information

Author

Koelsche, Christian, Hartmann, Wolfgang, Schrimpf, Daniel, Stichel, Damian, Jabar, Susanne, Ranft, Andreas, Reuss, David E., Sahm, Felix, Jones, David T.W., Bewerunge-Hudler, Melanie, Trautmann, Marcel, Klingebiel, Thomas, Vokuhl, Christian, Gessler, Manfred, Wardelmann, Eva, Petersen, Iver, Baumhoer, Daniel, Flucke, Uta, Antonescu, Cristina, Esteller, Manel, Fröhling, Stefan, Kool, Marcel, Pfister, Stefan M., Mechtersheimer, Gunhild, Dirksen, Uta, and von Deimling, Andreas

Published
2018
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33. DNA methylation profiling distinguishes Ewing-like sarcoma with EWSR1–NFATc2 fusion from Ewing sarcoma

Author

Koelsche, Christian, Kriegsmann, Mark, Kommoss, Felix K. F., Stichel, Damian, Kriegsmann, Katharina, Vokuhl, Christian, Grünewald, Thomas G. P., Romero-Pérez, Laura, Kirchner, Thomas, de Alava, Enrique, Diaz-Martin, Juan, Hartmann, Wolfgang, Baumhoer, Daniel, Antonescu, Cristina R., Szuhai, Karoly, Flucke, Uta, Dirksen, Uta, Pfister, Stefan M., Jones, David T. W., Mechtersheimer, Gunhild, and von Deimling, Andreas

Published
2019
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35. Radiotherapy and long-term sequelae in pediatric patients with parameningeal rhabdomyosarcoma: Results of two Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry.

Author

Sparber-Sauer, Monika, Dietzschold, Maximilian, Amadeus Heinz, Anton Schönstein4, Vokuhl, Christian, Pajtler, Kristian W., Harrabi, Semi, Lin, Yi-Lan, Kalle, Thekla von, Hagen, Rudolf, Ladenstein, Ruth, Kazanowska, Bernarda, Ljungman, Gustaf, Klingebiel, Thomas, Ebinger, Martin, Koscielniak, Ewa, Münter, Marc, and Timmermann, Beate

Published
2024
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36. Significance of fusion status, Oberlin risk factors, local and maintenance treatment in pediatric and adolescent patients with metastatic rhabdomyosarcoma: Data of the European Soft Tissue Sarcoma Registry SoTiSaR.

Author

Heinz, Amadeus T., Schönstein, Anton, Ebinger, Martin, Fuchs, Jörg, Timmermann, Beate, Seitz, Guido, Vokuhl, Christian, Münter, Marc, Pajtler, Kristian W., Stegmaier, Sabine, Kalle, Thekla von, Kratz, Christian P., Ljungman, Gustaf, Juntti, Hanna, Klingebiel, Thomas, Koscielniak, Ewa, and Sparber-Sauer, Monika

Published
2024
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37. TRIM28 inactivation in epithelial nephroblastoma is frequent and often associated with predisposing TRIM28 germline variants.

Author

Wegert, Jenny, Fischer, Anne Kristin, Palhazi, Balazs, Treger, Taryn D, Hilgers, Cäcilia, Ziegler, Barbara, Jung, Hyunchul, Jüttner, Eva, Waha, Andreas, Fuchs, Jörg, Warmann, Steven W, Frühwald, Michael C, Hubertus, Jochen, Pritchard‐Jones, Kathy, Graf, Norbert, Behjati, Sam, Furtwängler, Rhoikos, Gessler, Manfred, and Vokuhl, Christian

Subjects
NEPHROBLASTOMA, GERM cells, IMMUNOSTAINING, CANCER genes, DELETION mutation
Abstract

Wilms tumors (WTs) are histologically diverse childhood cancers with variable contributions of blastema, stroma, and epithelia. A variety of cancer genes operate in WTs, including the tripartite‐motif‐containing‐28 gene (TRIM28). Case reports and small case series suggest that TRIM28 mutations are associated with epithelial morphology and WT predisposition. Here, we systematically investigated the prevalence of TRIM28 inactivation and predisposing mutations in a cohort of 126 WTs with >2/3 epithelial cells, spanning 20 years of biobanking in the German SIOP93‐01/GPOH and SIOP2001/GPOH studies. Overall, 44.4% (56/126) cases exhibited loss of TRIM28 by immunohistochemical staining. Of these, 48 could be further analyzed molecularly, revealing TRIM28 sequence variants in each case – either homozygous (~2/3) or heterozygous with epigenetic silencing of the second allele (~1/3). The majority (80%) of the mutations resulted in premature stops and frameshifts. In addition, we detected missense mutations and small deletions predicted to destabilize the protein through interference with folding of key structural elements such as the zinc‐binding clusters of the RING, B‐box‐2, and PHD domains or the central coiled‐coil region. TRIM28‐mutant tumors otherwise lacked WT‐typical IGF2 alterations or driver events, except for rare TP53 progression events that occurred with expected frequency. Expression profiling identified TRIM28‐mutant tumors as a homogeneous subset of epithelial WTs that mostly present with stage I disease. There was a high prevalence of perilobar nephrogenic rests, putative precursor lesions, that carried the same biallelic TRIM28 alterations in 7/7 cases tested. Importantly, 46% of the TRIM28 mutations were present in blood cells or normal kidney tissue, suggesting germline events or somatic mosaicism, partly supported by family history. Given the high prevalence of predisposing variants in TRIM28‐driven WT, we suggest that immunohistochemical testing of TRIM28 be integrated into diagnostic practice as the management of WT in predisposed children differs from that with sporadic tumors. © 2023 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland. [ABSTRACT FROM AUTHOR]

Published
2024
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39. Second-line treatment of pediatric patients with relapsed rhabdomyosarcoma adapted to initial risk stratification: Data of the European Soft Tissue Sarcoma Registry (SoTiSaR)

Author

Heinz, Amadeus T, Ebinger, Martin, Schönstein, Anton, Fuchs, Jörg, Timmermann, Beate, Seitz, Guido, Vokuhl, Christian, Münter, Marc W, Pajtler, Kristian W, Stegmaier, Sabine, von Kalle, Thekla, Kratz, Christian P, Rößler, Jochen, Ljungman, Gustaf, Klingebiel, Thomas, Koscielniak, Ewa, and Sparber-Sauer, Monika

Subjects
Medizin, 610 Medicine & health
Abstract

BACKGROUND Outcome of relapsed disease of localized rhabdomyosarcoma remains poor. An individual treatment approach considering the initial systemic treatment and risk group was included in the Cooperative Weichteilsarkom Studiengruppe (CWS) Guidance. METHODS Second-line chemotherapy (sCHT) ACCTTIVE based on anthracyclines (adriamycin, carboplatin, cyclophosphamide, topotecan, vincristine, etoposide) was recommended for patients with initial low- (LR), standard- (SR), and high-risk (HR) group after initial treatment without anthracyclines. TECC (topotecan, etoposide, carboplatin, cyclophosphamide) was recommended after initial anthracycline-based regimen in the very high-risk (VHR) group. Data of patients with relapse (n = 68) registered in the European Soft Tissue Sarcoma Registry SoTiSaR (2009-2018) were retrospectively analyzed. RESULTS Patients of initial LR (n = 2), SR (n = 16), HR (n = 41), and VHR (n = 9) group relapsed. sCHT consisted of ACCTTIVE (n = 36), TECC (n = 12), or other (n = 15). Resection was performed in 40/68 (59%) patients and/or radiotherapy in 47/68 (69%). Initial risk stratification, pattern/time to relapse, and achievement of second complete remission were significant prognostic factors. Microscopically incomplete resection with additional radiotherapy was not inferior to microscopically complete resection (p = .17). The 5-year event-free survival (EFS) and overall survival (OS) were 26% (±12%) and 31% (±14%). The 5-year OS of patients with relapse of SR, HR, and VHR groups was 80% (±21%), 20% (±16%), and 13% (±23%, p = .008), respectively. CONCLUSION Adapted systemic treatment of relapsed disease considering the initial risk group and initial treatment is reasonable. New treatment options are needed for patients of initial HR and VHR groups.

Published
2023

40. Primary intracranial spindle cell sarcoma with rhabdomyosarcoma-like features share a highly distinct methylation profile and DICER1 mutations

Author

Koelsche, Christian, Mynarek, Martin, Schrimpf, Daniel, Bertero, Luca, Serrano, Jonathan, Sahm, Felix, Reuss, David E., Hou, Yanghao, Baumhoer, Daniel, Vokuhl, Christian, Flucke, Uta, Petersen, Iver, Brück, Wolfgang, Rutkowski, Stefan, Zambrano, Sandro Casavilca, Garcia Leon, Juan Luis, Diaz Coronado, Rosdali Yesenia, Gessler, Manfred, Tirado, Oscar M., Mora, Jaume, Alonso, Javier, Garcia del Muro, Xavier, Esteller, Manel, Sturm, Dominik, Ecker, Jonas, Milde, Till, Pfister, Stefan M., Korshunov, Andrey, Snuderl, Matija, Mechtersheimer, Gunhild, Schüller, Ulrich, Jones, David T. W., and von Deimling, Andreas

Published
2018
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45. Genome-wide methylation profiling and copy number analysis in atypical fibroxanthomas and pleomorphic dermal sarcomas indicate a similar molecular phenotype

Author

Koelsche, Christian, Stichel, Damian, Griewank, Klaus G., Schrimpf, Daniel, Reuss, David E., Bewerunge-Hudler, Melanie, Vokuhl, Christian, Dinjens, Winand N. M., Petersen, Iver, Mittelbronn, Michel, Cuevas-Bourdier, Adrian, Buslei, Rolf, Pfister, Stefan M., Flucke, Uta, Mechtersheimer, Gunhild, Mentzel, Thomas, and von Deimling, Andreas

Published
2019
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46. Locally Advanced Adrenocortical Carcinoma in Children and Adolescents—Enigmatic and Challenging Cases.

Author

Kuhlen, Michaela, Mier, Pascal, Kunstreich, Marina, Lessel, Lienhard, Slavetinsky, Christoph, Fuchs, Jörg, Seitz, Guido, Holterhus, Paul-Martin, Wudy, Stefan A., Vokuhl, Christian, Frühwald, Michael C., Vorwerk, Peter, and Redlich, Antje

Subjects
ADENOCARCINOMA, MICROSCOPY, RETROSPECTIVE studies, TREATMENT effectiveness, CANCER patients, SYMPTOMS, RESEARCH funding, DESCRIPTIVE statistics, ADRENAL tumors, PROGRESSION-free survival, PREDICTION models, LONGITUDINAL method, CHILDREN, ADOLESCENCE
Abstract

Simple Summary: Pediatric COG stage II and III adrenocortical carcinoma (ACC) is one of the most unpredictable and challenging tumors in terms of prognosis and management. The EXPeRT/PARTNER recommendation suggested inclusion of the five-item microscopic score to guide management in these patients. In this study, we report on 18 pediatric ACC stage II and 37 stage III patients registered with the German MET studies. We explored the five-item microscopic and the pS-GRAS score to predict outcome. Three-year event-free survival estimates were 76.5% in stage II and 49.8% in stage III patients. In COG stage III patients, EFS was impaired for patients with unfavorable histology according to the five-item score. No differences were observed in stage II patients and in the pS-GRAS groups. Further research including molecular studies is needed to identify high-risk features in locally advanced pediatric ACC tumors. Background: Locally advanced tumors account for approximately 50% of children and adolescents with adrenocortical carcinoma (ACC), and of these, up to 50% relapse. We explored the five-item microscopic score and the pS-GRAS score for guiding management. Methods: Data from children and adolescents with COG stage II and III ACC registered in the MET studies were included. The five-item and pS-GRAS score were retrospectively calculated. Results: By December 2021, 55 patients with stage II and III (stage II n = 18, stage III n = 37) had been reported. Median age was 4.3 years [0.1–17.8], median duration of follow-up 6.0 years [0–16.7]. 3-year event-free survival (EFS) rate was 76.5% and 49.8% (p = 0.088), respectively. In stage II tumors, neither the five-item score (p = 0.872) nor pS-GRAS grouping (p = 0.218) had any effect as prognostic factors. In stage III patients, EFS was impaired in tumors with unfavorable histology according to the five-item score (100% vs. 30.8%, p = 0.018). No difference was observed for pS-GRAS groups (p = 0.798). Conclusions: In patients with COG stage III, but not stage II, the five-item score affected EFS. Further studies are needed to identify patients at risk in COG stage II. [ABSTRACT FROM AUTHOR]

Published
2023
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47. Consensus classification of pediatric hepatocellular tumors: A report from the Children's Hepatic tumors International Collaboration (CHIC).

Author

Cho, Soo‐Jin, Ranganathan, Sarangarajan, Alaggio, Rita, Maibach, Rudolf, Tanaka, Yukichi, Inoue, Takeshi, Leuschner, Ivo, de Krijger, Ronald, Vokuhl, Christian, Krailo, Mark, Malogolowkin, Marcio, Meyers, Rebecka, Czauderna, Piotr, Hiyama, Eiso, Ansari, Marc, Morland, Bruce, Trobaugh‐Lotrario, Angela, O'Neill, Allison F., Rangaswami, Arun, and Häberle, Beate

Published
2023
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48. Pathology of Hepatocellular Carcinoma and Tumor-Bearing Liver Tissue in Association with hTERT Promoter Mutation.

Author

Fischer, Anne Kristin, Semaan, Alexander, Wulf, Anna-Lena, Vokuhl, Christian, Goltz, Diane, and Fischer, Hans-Peter

Abstract

Background. The hTERT promoter mutation represents a common and early event in hepatocarcinogenesis, but its linkage to the morphological status of the underlying liver tissue is poorly understood. We analyzed the connection between the histopathological changes in tumor-bearing liver tissue and the occurrence of the hTERT promoter mutation in hepatocellular carcinoma (HCC), correlated with clinical data. Methods. The study cohort comprised 160 histologically confirmed HCC in patients with or without cirrhosis that were investigated for the hTERT promoter mutation. We evaluated the frequency of the hTERT promoter mutation in patients with HCC with or without cirrhosis and correlated it with potential clinical and histopathological drivers. In particular, we examined tumor-bearing noncirrhotic liver tissue regarding inflammation; the modified histological activity index (mHAI), fibrosis, and steatosis; and its correlation with the frequency of the hTERT promoter mutation in HCC. We evaluated overall survival with multivariate Cox regression. Furthermore, we compared hTERT antibody immunohistochemistry and molecular hTERT promoter mutation analysis of both HCC and background liver tissue. Results. The hTERT promoter mutation was especially related to HCC in cirrhotic compared with noncirrhotic liver (p < 0.001) and independently of cirrhosis in patients ≥ 60 years (p = 0.005). Furthermore, the hTERT promoter mutation was associated with cirrhosis caused by alcohol toxicity and hepatitis C virus infection. In noncirrhotic liver tissue, the frequency of hTERT-promoter-mutated HCC increased with the degree of inflammation and fibrosis. Nevertheless, 25% of the hTERT-promoter-mutated HCC developed in normal liver tissue without HCC risk factors. Multivariate Cox regression analysis did not reveal an influence of the hTERT promoter mutation in HCC on overall survival at 3, 5, and 16 years. Immunohistochemical analysis with the hTERT antibodies LS-B95 and 2D8 in hTERT-promoter-mutated HCC and hTERT-wildtype HCC showed a mildly stronger immunoreaction compared with the tumor-bearing liver tissue (LS-B95: p < 0.01 , 2D8: p < 0.01). Conclusions. Our study reveals a connection between pathological changes in tumor-bearing liver tissue and the hTERT promoter mutation in most HCC, even in noncirrhotic liver tissue. Immunohistochemical hTERT antibodies do not discriminate between hTERT-promoter-mutated and wildtype HCC. [ABSTRACT FROM AUTHOR]

Published
2023
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50. Central nervous system acute lymphoblastic leukemia: role of natural killer cells

Author

Frishman-Levy, Liron, Shemesh, Avishai, Bar-Sinai, Allan, Ma, Chao, Ni, Zhenya, Frenkel, Shahar, Muench, Vera, Bruckmueller, Hilke, Vokuhl, Christian, Debatin, Klaus-Michael, Eckert, Cornelia, Stanulla, Martin, Schrappe, Martin, Campbell, Kerry S., Loewenthal, Ron, Schewe, Denis M., Hochman, Jacob, Meyer, Lueder H., Kaufman, Dan, Cario, Gunnar, Porgador, Angel, and Izraeli, Shai

Published
2015
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