tbuckley/cabot-search huggingface dataset:
MDB-05. PERSONALIZED, RISK ADAPTED THERAPY FOR PEDIATRIC PATIENTS WITH MEDULLOBLASTOMA – A SINGLE CENTER 8-YEAR RETROSPECTIVE REVIEW
Authors: Devin Antonio Martinez, Ruben Fragoso, Marike Zwienenberg, Julia Sharma, Cameron Sadegh, Han Sung Lee, Vihar Patel, Melinda Beckham, Anita Rochelle Johnson, Reuben Antony
Journal: Neuro-Oncology (2024)

Abstract BACKGROUND Clinically relevant risk stratification and optimal therapies for pediatric medulloblastoma molecular subgroups are still being investigated. METHODS We reviewed host/disease characteristics, patient/tumor specific risk adapted therapy and outcomes of patients treated for medulloblastoma at UC Davis since 2016. RESULTS Fifteen patients (age 1-19 years, 12 male, 3 with Li Fraumeni Syndrome (LFS), 2 with spinal cord disease) were included in our review. Tumor histologies included: Classic (n=8), Desmoplastic-Nodular (n=6), Anaplastic (n=1). Tumor genomic profiles were SHHP53WT(n=5), SHHP53 mutated (n=4), Group4 (n=3), NWNSHH (n=2), WNT (n=1). Gross total resection was achieved in 9 patients. Patients with post resection residual tumor, high risk genomic features or disseminated disease were stratified as high risk. We treated all radiation eligible patients (n=13) per COG ACNS0332 maintenance chemotherapy regardless of risk group with 12/13 receiving radiosensitization (Vincristine/Carboplatin) and either 23.4 Gy/36 Gy CSI (two patients treated per Headstart3D2 and ACNS0334 respectively). 2 patients with spinal cord disease were treated with intrathecal topotecan and liposomal cytarabine respectively. During ACNS0332 maintenance bacteremia, weight loss and hypomagnesemia were frequent complications. 5 patients required chemotherapy dose reductions (4 Vincristine/1 Cisplatin). Two patients with LFS developed glioblastoma/undifferentiated pleomorphic sarcoma following ACNS0332 therapy. Two patients (with WNT and NWNSHH subgroup tumors) experienced medulloblastoma relapse. These patients were treated using LITT/Intrathecal chemotherapy and ACNS0821 chemotherapy. Both are alive and one patient is NED off therapy. All patients who have not developed secondary malignancies (n=13) are alive. (follow up 7-72 months). All attend school with support, 10 have hearing impairment and 5 receive endocrine supplements. CONCLUSIONS Utilizing patient specific risk adapted therapy we have achieved favorable clinical outcomes in our patient cohort. Therapy deintensification for patients with WNT medulloblastomas should be approached with caution. Patients with LFS might require radiation sparing therapeutic protocols for longer term benefit.
EPEN-19. IMPACT OF MOLECULAR SUBTYPING ON PROGNOSIS IN CHILDREN AND ADOLESCENTS WITH SPINAL EPENDYMOMA
Authors: Lara Engertsberger, Martin Benesch, Martin Mynarek, Svenja Tonn, Denise Obrecht-Sturm, Thomas Perwein, Martina Stickan-Verfürth, Angela Funk, Beate Timmermann, Alexander Claviez, Rolf-Dieter Kortmann, Markus J Riemenschneider, Torsten Pietsch, Brigitte Bison, Monika Warmuth-Metz, Kristian W Pajtler, Stefan Rutkowski, Ulrich Schüller
Journal: Neuro-Oncology (2024)

Abstract PURPOSE Ependymomas of the spinal cord are rare among children and adolescents, and the individual risk of disease progression is difficult to predict. Recent advances in subtyping the main molecular groups, myxopapillary ependymoma (MPE) and spinal ependymoma (SP-EPN), have successfully identified distinct risk groups among adult patients. This study aims at evaluating the prognostic impact of MPE subtypes A and B in a pediatric cohort. METHODS Eighty-three patients with spinal ependymoma ≤22 years registered in the HIT-MED database between 1992 and 2022 were included. Forty-seven tumors were analyzed regarding global DNA methylation. In six cases, antibodies against HOXB13 and MYCN were used as surrogate markers for the methylation group. Twenty-eight MPE were further classified into subtypes by t-distributed stochastic neighbor embedding (t-SNE) analysis. RESULTS MPE (n=32, 63%) was the most common molecular type followed by SP-EPN (n=17, 33%) and MYCN-amplified ependymoma (n=2, 4%). One case remained molecularly unclear, and one was excluded due to reclassification as anaplastic pilocytic astrocytoma. MPE subtyping identified 18 MPE-A and 9 MPE-B (inconclusive: n=1). 5-year progression-free survival (5y-PFS) did not significantly differ between MPE and SP-EPN (65% vs. 78%, p=0.64). MYCN-amplification was associated with early relapses and with death in one patient. Patients with MPE-B may have a tendency towards superior 5y-PFS compared to MPE-A (86% vs. 56%, p=0.15), irrespective of the use of adjuvant treatment. MPE-A was more frequently disseminated at time of diagnosis (n=6/18) than MPE-B (n=1/8, not evaluable: n=1; p=0.28). CONCLUSION Molecular subtyping of spinal ependymoma in children does not allow to separate patients into distinct risk groups, yet. However, the potentially favorable PFS among patients with MPE-B prompts the consideration of treatment de-intensification for this group. Larger cohorts and further insights into the molecular heterogeneity of these tumors are needed to complete the basis for future clinical decision-making.
DIPG-26. H3.3K27M ENHANCES SENSITIVITY TO ATR INHIBITION IN PEDIATRIC GLIOMA
Authors: Heng Wang, Jakub P Kaplan, Dipanjan Chowdhury, Sharmistha Pal, Daphne Haas-Kogan
Journal: Neuro-Oncology (2024)

Abstract BACKGROUND Diffuse midline glioma (DMG) is an aggressive, uniformly fatal childhood brain tumor. Radiation remains the standard of care but provides limited survival benefit. The most prevalent driver mutation in DMG is lysine to methionine mutation at position 27 (K27M) of histone H3.3, resulting in global loss of H3K27 trimethylation and remodeled chromatin, thus promoting DMG tumorigenesis. We have previously shown that inhibition of de novo pyrimidine synthesis induces replication stress that enhances DMG dependency on ATR. In this study, we sought to explore the contribution of H3K27M to basal replication stress in DMGs and vulnerability to ATR inhibition. METHODS To investigate ATR sensitivity of DMG, we employed ART0380, a potent and selective ATR inhibitor currently in Phase 2 clinical trials in adults. Using isogenic models and a panel of patient tumor-derived DMG cell lines we perform viability assays, immunoblotting, microscopy and flow cytometry-based assays to determine various endpoints/measures of ATR inhibition in DMGs. RESULTS Our data show that H3K27M histone oncoproteins enhance dependency on ATR and ATR inhibition synergizes with radiation to induce DMG cell death. After ART0380 treatment, H3K27M oncoproteins result in greater induction in apoptosis and accumulation of DNA damage (as measured by g-H2AX) compared with their histone H3 wild-type isogenic counterparts. Inhibition of ATR by ART0380 is confirmed by effects on phosphorylation of bona fide ATR targets-CHK1 and RPA. To gain mechanistic insights into how H3K27M modulates dependency on ATR, we have evaluated accumulation of chromatin-bound RPA, R-loops, and frequency of transcription and replication collisions (TRC) in isogenic cells. CONCLUSIONS Our data indicate a heightened sensitivity of H3K27M mutated DMG to ATR inhibition thus identifying a therapeutic window for DMG treatment with ATR inhibitor as monotherapy or in combination with radiotherapy.
DIPG-73. FOCUSED ULTRASOUND FOR TREATMENT OF CHILDREN DIAGNOSED WITH DIFFUSE MIDLINE GLIOMAS
Authors: Ayda Woldegerima, Hong-Jian Wei, Chunchao Zhang, Sridevi Yadavillli, Roger Packer, Cheng-Chia Wu, Javad Nazarian
Journal: Neuro-Oncology (2024)

Abstract BACKGROUND Diffuse midline glioma (DMG) is a highly aggressive and fatal brain tumor that predominantly impacts children and young adults. The infiltrative pattern and rapid growth characteristics of DMG causes a significant challenge in finding an effective treatment. Additionally, the blood-brain barrier (BBB) poses a significant challenge by restricting the passage of therapeutic drugs to tumor bed. Currently the established standard of care is radiotherapy (RT), which is transitory. Thus there is a need for improved treatment options. OBJECTIVE Focused ultrasound (FUS) is an advancing non-invasive technology that has been used to open BBB and enhance drug delivery. We hypothesize that a combination therapy of FUS with pharmacological agents will be an effective and non-invasive treatment option for children diagnosed with DMG. METHOD Syngeneic DMG murine models were used to study the combination therapy of FUS with ONC201, FUS with anti-PD1 and FUS with RT. FUS mediated BBB opening was monitored with contrast-enhanced T1-weighted MRI. FUS-mediated drug delivery was validated with western blot, assessment of ROS, and optical label imaging. Kaplan Meier analysis was used to assess survival benefits. Single cell RNA-sequencing (scRNA-Seq) analysis is being done to assess the treatment effects of FUS and RT combination therapy. RESULTS/CONCLUSION We found that FUS and ONC201 combination therapy resulted in a decrease in NDUFA12 protein (a biomarker of ONC201 response) and an increase in ROS production compared to ONC201 monotherapy. For FUS and anti-PD1 combination therapy, an increased anti-PD1 delivery to the brainstem was detected. The overall survival of mice treated with FUS and anti-PD1 combination therapy increased when compared to anti-PD1 alone. Our preliminary scRNA-Seq analysis showed that combination therapy of FUS and RT induces innate immune response compared to the untreated group. Combination therapy with focused ultrasound shows a promising outcome by increasing the efficacy of therapeutic drugs and immunotherapy.
MDB-03. MEDULLOBLASTOMA AT RELAPSE: FOR WHICH PATIENTS AND WHICH TUMORS REIRRADIATION IS THE BETTER CHOICE
Authors: Maura Massimino, Sabina Vennarini, Francesca Romana Buttarelli, Manila Antonelli, Simone Minasi, Emilia Pecori, Carlo Giussani, Andrea Trezza, Elisabetta Schiavello, Veronica Biassoni, Alessandra Erbetta, Luisa Chiapparini, Olga Nigro, Francesca Gianno, Evelina Miele, Piergiorgio Modena, Chiara Dossena, Loris De Cecco, Bianca Pollo, Francesco Barretta
Journal: Neuro-Oncology (2024)

Abstract BACKGROUND First-line therapies for medulloblastoma(MBL) are obtaining higher survival-rates while decreasing late-effects, but treatment at relapse is not yet standardized. We report the experience with MBL re-irradiation(re-RT), its timing and outcome in different clinical settings and tumor groups. METHODS Patient’s staging/treatment at diagnosis, histotypes/molecular subgroups, relapse site/s, re-treatments outcome are reported. RESULTS Between 2002 and 2019, relapsing patients were 25, median age at diagnosis 11.4 years, 8 had metastases, three LC/A histotype. According to 2016-2021 WHO-classification, 14 had SHH subgroup tumors (6 TP53 mutated,1 + MYC and 1 + NMYC amplification), 11 non-WNT/non-SHH (2 with MYC/MYCN amplification). Thirteen had received HART-CSI, 11 standard-CSI, one HFRT; all post-radiation chemotherapy(CT), 16 also pre-RT. Median time to relapse (local-LR in 9, distant-DR in 14, LR+DR in two) was 26 months. Fourteen patients were re-operated, in 5 excising single DR-sites, thereafter 3 received CT, two after re-RT; out of 11 not re-operated patients, 4 had re-RT as first treatment and 7 after CT. Re-RT was administered at median 32 months after first RT: focally in 20 cases, CSI in 5, never resulting in radionecrosis. Median post-relapse-PFS/after re-RT were 16.7/8.2 months, while overall survival-OS were 35.1/23.9 months, respectively. Metastatic status both at diagnosis/relapse negatively affected outcome and re-surgery was prognostically favorable. MYC,MYCN,P53 status and molecular subgroups, RT extension/fractionation, gender and age were not statistically prognostic; in the multivariable model, OSs were positively influenced by longer intervals before re-RT, re-surgery and not-SHH subgroups (P=0.019 from recurrence and 0.004 from second RT). CONCLUSIONS Re-surgery + reRT can prolong survival; a substantial fraction of patients with worse outcome belonged to SHH-subgroup.
GCT-19. SIOP CNS GCT II: PRESENTATION AND OUTCOME OF BIFOCAL NON-GERMINOMATOUS GERM CELL TUMOURS (NGGCTS)
Authors: Gabriele Calaminus, Rolf Dieter Kortmann, Claire Alapetite, Brigitte Bison, Didier Frappaz, Thankamma Ajithkumar, Nicolas Gerber, Andreas Peyrl, Giovanni Morana, Matthew Murray, Stefan Schoenberger, Herve Brisse, Martin Zimmermann, James Nicholson, Cecile Faure Conter
Journal: Neuro-Oncology (2024)

Abstract BACKGROUND Between 2/2012 and 7/2018, 112 patients with non-germinoma were registered as intend-to-treat patients within the SIOP CNS GCT II trial: 88 patients were male and 24 female. Median age was 13 years (range: 0,1-25,1 years). 95 patients had localised and 17 metastastic disease. 21 patients with bifocal tumors were reported, defined as a synchronous suprasellar and pineal tumour at diagnosis. 6/21 had metastases. PATIENTS/METHODS All Patients presented with several main clinical symptoms: diabetes insipidus (DI) (18), vision impairment (16), raised intracranial pressure (10), headache (10), cognitive impairments (7), somnolence (5). Diagnosis was confirmed by tumor markers in all, two with additional resection and 4 with biopsy. All patients received upfront PEI (cisplatin/etoposide/ifosfamide) chemotherapy, with dose-intensification (HD-PEI) for those with diagnostic serum or CSF AFP &amp;gt;1000 ng/ml (high-risk), before RT. Patients without dissemination received local radiotherapy (RT) of 54 Gy to both sites. Patients with dissemination received CSI 30 Gy plus boost to primary sites of 24 Gy and RT to visible metastases. RESULTS Among 12 standard-risk patients with localised disease 3/12 achieved CR, 1/12 PR and 8/12 relapsed. Of the 8 relapses, 3 achieved a 2nd CR. Three patients with localised disease were high risk. Among them only one patient achieved CR after treatment, but all 3 -pts relapsed and 2 died. Among 6 patients with dissemination and standard risk 5/6 achieved CR, 1/6 PR, no relapses occurred. Bifocal localised non-Germinoma are not sufficiently controlled by chemotherapy and focal tumor boost. Other treatment concepts including upfront chemo-intensification in standard risk patients and whole-ventricular RT need to be explored. For the SIOP CNS GCT group, supported in part by German Childhood Cancer Aid
DIPG-59. RADIATION-INDUCED DIPG IN A CHILD PREVIOUSLY IRRADIATED FOR CRANIOPHARYNGIOMA: CASE REPORT AND LITERATURE REVIEW
Authors: Rahat Ul Ain, Laeeq Ur Rehman, Rabia Aqeel Qaiser, Amber Goraya, Zeena Salman, Ibrahim Qaddoumi, Naureen Mushtaq, Eric Bouffet
Journal: Neuro-Oncology (2024)

Abstract BACKGROUND Radiation-induced second malignancies occur in a subset of patients following cranial radiation therapy (RT). In Craniopharyngioma (CP), adjuvant RT is commonly recommended for residual tumor or recurrence, and radiation-induced malignant gliomas (RIMGs) are rarely reported. METHODS We report a case of radiation-induced diffuse intrinsic pontine glioma (DIPG) in pediatric Craniopharyngioma and conducted a review of previously reported cases. RESULTS A 14-year-old boy presented with complaints of headaches and vomiting for 1 month; difficulty in walking, and slurred speech for 1 week, with Lansky Performance Score (LPS) of 20. Eight years before this admission, he underwent surgical excision and 3D-conformal RT for Craniopharyngioma. His recent MRI brain was suggestive of DIPG and after a multidisciplinary discussion re-irradiation (30Gy in 10 fractions) was given. He has been alive for 5 months since diagnosis with improved symptoms and LPS (70). Including the index case, 19 cases of RIMGs in CP were identified. All except one were reported from high-income countries. Only 4 involved the pons/brainstem. The median age at CP diagnosis was 7.0 years (2.0-22.0 years), and with RIMG was 18 (6.8-41.0 years). Median latency period was 10.0 years (4.5-25.0 years). The female-to-male ratio was 1.8:1. The median previous RT dose was 54.8 Gy (49.3-60.1 Gy) with 37% cases treated with 3D conformal and 37% with conventional RT (not specified in 26%). Cerebral cortex (66.6%) was the most common site for the RIMG with temporal lobe predisposition. Chemotherapy was given in 56%, surgical excision in 50% and repeat RT in 31% cases with a median survival of 4 months (0-13 months). CONCLUSION We report the first case of radiation-induced DIPG in pediatric Craniopharyngioma in an LMIC. RIMG appears to be a rare occurrence in CP and doesn’t seem to be associated with the RT type. The overall prognosis is extremely poor.
DIPG-06. FUNCTIONAL MULTI-OMIC PROFILING OF THE LANDSCAPE OF DIFFUSE MIDLINE GLIOMAS TO IDENTIFY THERAPEUTIC VULNERABILITIES
Authors: Izac J Findlay, Dilana Staudt, Ryan J Duchatel, Evangeline R Jackson, Padraic Kearny, Mika L Persson, Holly McEwen, Nathan D Smith, Nicholas A Vitanza, Jason E Cain, Sebastian M Waszak, Mitchell Hansen, Frank Alvaro, Matthew D Dun
Journal: Neuro-Oncology (2024)

Abstract BACKGROUND Diffuse midline glioma (DMG) is a devastating pediatric high-grade glioma (pHGG) located along the midline structures of the brain and spine. Palliative radiotherapy is the only approved treatment outside of clinical trials, with patients given 9–11-months survival post-diagnosis. Over the last 10-years, it has become common for patients to be prescribed precision therapies targeting genetic alterations, however largely, DMG harbor genetic alterations that are untargetable. METHODS To improve DMG patient outcomes, we are performing multi-omic analysis on 210 DMG, normal controls and other pHGG samples to functionally characterize the genomic landscape of these tumors and understand the protein-controlled functional consequences of recurring somatic alterations. RESULTS Genomic sequencing has been performed on 40/210 pHGG samples, identifying 290 unique mutations and several recurrent chromosomal alterations. Additionally, proteomic/phosphoproteomic profiling identified both unique and similar features of post-translational landscapes across genotypes. Examining H3.3K27M DMG cell-lines, SU-DIPG-XIII and UON-VIBE5, genomic sequencing identified 11 and 6 mutations respectively, however, only UON-VIBE5 possessed a genomically-predicted target, a PDGFRA-D842V mutation, potentially targetable by Avapritinib. Conversely, investigation of proteomes/phosphoproteomes revealed several potential therapeutic vulnerabilities. SU-DIPG-XIII possessed high enrichment of HDAC/BRD and CDK pathways and UON-VIBE5, ATM-centric pathways. In vitro proliferation assays of Avapritinib treated UON-VIBE5 failed to elicit a response at clinically relevant doses, while all proteomically-predicted therapies (HDAC – Fimepinostat, BRD – JQ1, CDK – Ribociclib and ATM – WSD-0628) successfully influenced tumor cell growth whilst leaving microglia controls cells unaffected. Avapritinib and WSD-0628 (CNS-active ATM inhibitor) drug predictions were further validated in the RA055 DMG model, which possesses similar genomic and proteomic features to UON-VIBE5. Avapritinib alone failed to influence tumor growth, while WSD-0628 notably reduced cell proliferation. The combination of these drugs, however, synergistically inhibited RA055 proliferation. CONCLUSIONS These findings thus provide preliminary evidence supporting the need to take a multi-omic approach in treating DMGs.
CP-11. BODY MASS INDEX INCREASE FOLLOWING CHILDHOOD CRANIOPHARYNGIOMA SURGERY IS MOST SEVERE IN THE FIRST THREE MONTHS; TIME TO ACT!
Authors: Sanne C Hulsmann, Eelco W Hoving, Evelien de Vos-Kerkhof, Geert O Janssens, Boudewijn Bakker, Hanneke M van Santen
Journal: Neuro-Oncology (2024)

Abstract BACKGROUND Centralization of care for childhood craniopharyngioma (cCP) in the Netherlands (June 2018), including the involvement of a multidisciplinary team, has resulted in a decreased prevalence of hypothalamic obesity. Unfortunately, in certain patients, increasing body mass index standard deviation scores (BMI SDS) are still encountered after tumor resection. The aim of this analysis is to identify timing of BMI change, incidence/prevalence and predictive factors for BMI changes enabling early preventive strategies. METHODS A retrospective cohort study was performed of all newly diagnosed cCP patients since centralization of care in the Netherlands (June 2018). Data on anthropometrics, radiology, tumor treatment (surgery, radiotherapy, interferon and tocilizumab), hypothalamic-pituitary function, medication and visual function, were collected. Uni- and multivariate analyses were done with the ‘mean’ change in BMI SDS at 3, 6 and 12 months after resection as primary endpoint. Body mass index trends were assessed as secondary outcome. RESULTS Of 34 patients, median age 8.93 (range 0.79-17.36), mean ∆BMI SDS was most pronounced at 3 months (+1.09 SDS (SD 1.17), p&amp;lt;0.01), followed by a further mean increase of +0.25 SDS from 3 to 6 months (SD 0.50, p=0.01). In 17 patients, BMI SDS increased with ≥1.0 SDS in the first 3 months after resection (50%). A Muller grade 2 at diagnosis was associated with ∆BMI SDS at 3 months. Children with the highest BMI at diagnosis had the highest BMI at three months, but not the highest ∆BMI SDS. CONCLUSION Changes in BMI SDS ≥1.0 are observed in half of cCP patients following surgical resection despite expert multidisciplinary care, with most pronounced BMI changes during the first 3 months. These observations are a plea for more early interventions to prevent (morbid) hypothalamic obesity.
ETMR-17. CLINICAL DESCRIPTION OF PEDIATRIC CHOROID PLEXUS PAPILLOMAS FOR IDENTIFICATION AND VALIDATION OF HIGH-RISK CHARACTERISTICS
Authors: Christian Fiedler, Uwe Kordes, Christian Thomas, Martin Hasselblatt, Brigitte Bison, Lars Behrens, Rolf-Dieter Kortmann, Beate Timmermann, Torsten Pietsch, Stefan Rutkowski, Denise Obrecht-Sturm
Journal: Neuro-Oncology (2024)

Abstract BACKGROUND Choroid plexus papilloma (CPP) and atypical CPP (aCPP) have an excellent prognosis. However, some CPP/aCPP relapse and may qualify for postoperative treatment. METHODS Patients from the International CPT-SIOP Registry with CPP/aCPP recruited in Germany between 2011–2023 were included and analysed according to initial staging (postoperative residual tumor [R+], meningeosis [M+]), biology, postoperative treatment and outcome. RESULTS Ninety-three patients were identified (male: n=53, female: n=40). Median age at diagnosis was 1.9(0.1–17.6) years. Initial staging was R0/M0 in n=63, R+/M0 in n=21, R0/M+ in n=5 and R+/M+ in n=4. aCPP was diagnosed in n=38. Methylation subtype was available for n=34:”adult” n=3, “pediatric-A” n=19 and “pediatric-B” n=12 (6/12 aCPP). Median follow-up was 5.5(±0.99) years. Twelve tumors relapsed: R0/M0 n=4, R+/M0 n=7, R+/M+ n=1. Two relapsed patients died. Eleven patients initially received ≥1 surgery. Most patients did not receive postoperative treatment (watchful waiting, n=86). Seven patients (R0/M0 n=3, R+/M0 n= 1, R0/M+ n=2, R+/M+ n=1) received chemotherapy. None was irradiated during first-line treatment. Histology and biology had no impact on PFS (5y-PFS: CPP 88.6±4.9%, aCPP 82.7±6.5%, pPFS=0.3; “adult” 33.3±27.2%, “pediatric-A” 93.8±6.1%, “pediatric-B” 72.9±13.5%, pPFS=0.08). Patients with R0/M0-status had better 5y-PFS than those with R+ and/or M+ (90.4±4.1% vs. 77.7±8.1%, p=0.05). Neither incomplete resection alone, nor meningeosis alone did relate to inferior PFS in CPP/aCPP separately analysed (R+: p=0.07/p=0.07, M+: p=0.5/p=0.4). In the whole cohort, R+ was associated with inferior survival (5y-PFS R+ 72.2±9.8% vs. R0 91.3±3.7%, p=0.009). It remains unclear if R+-patients benefit from chemotherapy (5y-PFS watch-and-wait vs. chemotherapy 75.1±9.8% vs. 50.0±35.4% pPFS=0.7). CONCLUSION Incomplete resection appears to have impact on the risk for relapse in CPP/aCPP. It remains unclear whether those patients benefit from postoperative chemotherapy.