Critical evaluation of molecular tumour board outcomes following 2 years of clinical practice in a Comprehensive Cancer Centre.

BACKGROUND: Recently, molecular tumour boards (MTBs) have been integrated into the clinical routine. Since their benefit remains debated, we assessed MTB outcomes in the Comprehensive Cancer Center Ostbayern (CCCO) from 2019 to 2021. METHODS AND RESULTS: In total, 251 patients were included. Targeted sequencing was performed with PCR MSI-evaluation and immunohistochemistry for PD-L1, Her2, and mismatch repair enzymes. 125 treatment recommendations were given (49.8%). High-recommendation rates were achieved for intrahepatic cholangiocarcinoma (20/30, 66.7%) and gastric adenocarcinoma (10/16, 62.5%) as opposed to colorectal cancer (9/36, 25.0%) and pancreatic cancer (3/18, 16.7%). MTB therapies were administered in 47 (18.7%) patients, while 53 (21.1%) received alternative treatment regimens. Thus 37.6% of recommended MTB therapies were implemented (47/125 recommendations). The clinical benefit rate (complete + partial + mixed response + stable disease) was 50.0% for MTB and 63.8% for alternative treatments. PFS2/1 ratios were 34.6% and 16.1%, respectively. Significantly improved PFS could be achieved for m1A-tier-evidence-based MTB therapies (median 6.30 months) compared to alternative treatments (median 2.83 months; P = 0.0278). CONCLUSION: The CCCO MTB yielded a considerable recommendation rate, particularly in cholangiocarcinoma patients. The discrepancy between the low-recommendation rates in colorectal and pancreatic cancer suggests the necessity of a weighted prioritisation of entities. High-tier recommendations should be implemented predominantly.

A German-Wide Systematic Study on Mobilization and Collection of Hematopoietic Stem Cells in Poor Mobilizer Patients with Multiple Myeloma prior to Autologous Stem Cell Transplantation.

Introduction: In patients with a clinical indication for autologous hematopoietic stem cell transplantation (ASCT), sufficient mobilization of CD34+ precursor cells into peripheral blood is essential to ensure adequate hematopoietic stem cell (HSC) collection prior to intensive therapy. However, with standard granulocyte-colony stimulating factor (G-CSF)-based mobilization schemes, an important minority of patients fail to mobilize sufficient (e.g., >10/µL) CD34+ cell counts into the peripheral blood and are considered as poor mobilizers (PM). Because failure to achieve sufficient CD34+ cell mobilization can negatively affect important clinical treatment endpoints, the use of plerixafor (PLX) was approved to increase CD34+ mobilization in PM patients. Methods: The German non-interventional, multicenter, open-label, prospective OPTIMOB study evaluated HSC mobilization strategies prior to planned ASCT in adult patients with hematologic malignancies (lymphomas or multiple myeloma [MM]) focusing on PM patients. PM patients were defined as follows: (1) never achieving ≥20 CD34+ cells/µL before 1st apheresis, (2) receiving PLX at any timepoint of mobilization, (3) their initially planned stem cell yield had to be reduced, or (4) they had not received apheresis due to low CD34+ count in peripheral blood. Results: 168 of 475 MM patients (35%) participating in the OPTIMOB study were classified as PM, and 155 of them (92%) received PLX (PM+PLX) during the study. PM patients were 40-78 years old, slightly more often male (n = 97, 58%), mostly newly diagnosed (n = 146, 87%) and received highly individualized previous treatments. Ninety-four of the PMs underwent chemotherapy mobilization (65%), and 51 patients (35%) received steady-state mobilization with G-CSF only during 1st mobilization attempt. 92% of the total PM population (n = 155) underwent apheresis, 78% of them (n = 117) achieved >2.0 × 106 CD34+ cells/kg body weight on the 1st day of apheresis. PM+PLX had a higher median total collection result than those PM patients without PLX support (7.2 vs. 5.7 × 106 CD34+ cells/kg body weight). In total, ASCT was performed in 136 PM+PLX (88%) versus 8 PM-PLX patients (62%). Conclusion: The OPTIMOB study showed that a considerable proportion of adult MM patients in Germany are PMs. Even though most of PMs were supported with PLX in the OPTIMOB study, PM-PLX also successfully mobilized HSCs, allowing ASCT in majority of all PMs. However, further analyses are required for treatment optimization in PMs.

Mobilization and Hematopoietic Stem Cell Collection in Poor Mobilizing Patients with Lymphoma: Final Results of the German OPTIMOB Study.

Introduction: Successful mobilization and collection of peripheral hematopoietic stem cells (HSCs) are necessary for lymphoma patients eligible for myeloablative chemotherapy with subsequent autologous stem cell transplantation (ASCT). Albeit G-CSF alone or combined with chemotherapy is well-established methods for HSC mobilization, up to 40% of the patients fail to mobilize (poor mobilizer, PM). Plerixafor (PLX) is commonly used in PM patients resulting in increased migration of HSCs into peripheral blood and thus improves the collection outcome. Methods: The prospective, multicenter, open-label, non-interventional OPTIMOB study assessed mobilization and collection parameter of patients with lymphoma or multiple myeloma to get deep insights in the treatment of those patients in clinical routine focusing on PM patients. PM was defined as follows: (1) no achievement of ≥20 CD34+ progenitor cells/µL before first apheresis, (2) PLX administration at any time point during the observational period, (3) reduction of the initially planned CD34+ progenitor cell yield as necessity due to failed mobilization or HSC collection, and (4) no performance of apheresis due to low CD34+ progenitor level. Primary objective of the study was to assess mobilization success by the proportion of PM patients achieving >2 × 106 CD34+ progenitor cells/kg body weight on the first day of apheresis. Here, the data of the lymphoma cohort are presented. Results: Out of 238 patients with lymphoma documented in the study, 32% were classified as PM. 87% of them received PLX. Demographic data revealed no obvious differences between PM and good mobilizing (GM) patients. All patients were treated highly individualized prior to mobilization. Majority of all PM patients were able to undergo apheresis (95%) and reached their individual requested CD34+ progenitor cell target (72%). 57% of the PM patients achieved >2.0 × 106 CD34+ progenitor cells/kg body weight on day 1 of apheresis and nearby 70% of them underwent ASCT. Median time to engraftment was similar in PM and GM patients of the lymphoma cohort. Conclusions: Majority of PM patients with lymphoma were successfully mobilized and underwent ASCT. Most of them received PLX during the study.

Distinct Mechanisms of IgM Antibody-Mediated Acquired von Willebrand Syndrome and Successful Treatment with Recombinant von Willebrand Factor in One Patient.

Acquired von Willebrand Syndrome (AVWS) is a rare coagulation disorder which can be associated with IgM paraproteinaemia. Recently, recombinant von Willebrand factor (rVWF) has become available for the treatment of bleedings in patients with inherited von Willebrand disease, but experience in patients with AVWS is limited. We report on 2 patients with AVWS with underlying IgM paraproteinaemia with distinct underlying pathomechanisms. In 1 patient, the paraprotein built unspecific complexes with von Willebrand factor (VWF). In the other patient, we were able to detect an IgM antibody against VWF. Bleeding in this patient was successfully treated with rVWF. To our knowledge, this is the first report about the successful use of rVWF in a patient with AVWS with the detection of a VWF-specific antibody.

Six versus eight doses of rituximab in patients with aggressive B cell lymphoma receiving six cycles of CHOP: results from the "Positron Emission Tomography-Guided Therapy of Aggressive Non-Hodgkin Lymphomas" (PETAL) trial.

Standard first-line treatment of aggressive B cell lymphoma comprises six or eight cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) plus eight doses of rituximab (R). Whether adding two doses of rituximab to six cycles of R-CHOP is of therapeutic benefit has not been systematically investigated. The Positron Emission Tomography-Guided Therapy of Aggressive Non-Hodgkin Lymphomas (PETAL) trial investigated the ability of [18F]-fluorodesoxyglucose PET scanning to guide treatment in aggressive non-Hodgkin lymphomas. Patients with B cell lymphomas and a negative interim scan received six cycles of R-CHOP with or without two extra doses of rituximab. For reasons related to trial design, only about a third underwent randomization between the two options. Combining randomized and non-randomized patients enabled subgroup analyses for diffuse large B cell lymphoma (DLBCL; n = 544), primary mediastinal B cell lymphoma (PMBCL; n = 37), and follicular lymphoma (FL) grade 3 (n = 35). With a median follow-up of 52 months, increasing the number of rituximab administrations failed to improve outcome. A non-significant trend for improved event-free survival was seen in DLBCL high-risk patients, as defined by the International Prognostic Index, while inferior survival was observed in female patients below the age of 60 years. Long-term outcome in PMBCL was excellent. Differences between FL grade 3a and FL grade 3b were not apparent. The results were confirmed in a Cox proportional hazard regression model and a propensity score matching analysis. In conclusion, adding two doses of rituximab to six cycles of R-CHOP did not improve outcome in patients with aggressive B cell lymphomas and a fast metabolic treatment response.

Genetic PTX3 deficiency and aspergillosis in stem-cell transplantation.

BACKGROUND: The soluble pattern-recognition receptor known as long pentraxin 3 (PTX3) has a nonredundant role in antifungal immunity. The contribution of single-nucleotide polymorphisms (SNPs) in PTX3 to the development of invasive aspergillosis is unknown. METHODS: We screened an initial cohort of 268 patients undergoing hematopoietic stem-cell transplantation (HSCT) and their donors for PTX3 SNPs modifying the risk of invasive aspergillosis. The analysis was also performed in a multicenter study involving 107 patients with invasive aspergillosis and 223 matched controls. The functional consequences of PTX3 SNPs were investigated in vitro and in lung specimens from transplant recipients. RESULTS: Receipt of a transplant from a donor with a homozygous haplotype (h2/h2) in PTX3 was associated with an increased risk of infection, in both the discovery study (cumulative incidence, 37% vs. 15%; adjusted hazard ratio, 3.08; P=0.003) and the confirmation study (adjusted odds ratio, 2.78; P=0.03), as well as with defective expression of PTX3. Functionally, PTX3 deficiency in h2/h2 neutrophils, presumably due to messenger RNA instability, led to impaired phagocytosis and clearance of the fungus. CONCLUSIONS: Genetic deficiency of PTX3 affects the antifungal capacity of neutrophils and may contribute to the risk of invasive aspergillosis in patients treated with HSCT. (Funded by the European Society of Clinical Microbiology and Infectious Diseases and others.).

Recruitment strategies in a prospective longitudinal family study on parents with obesity and their toddlers.

BACKGROUND: Recruitment of participants with obesity is a real challenge. To reduce time and costs in similar projects, we investigated various recruiting strategies used in a longitudinal family study with respect to their enrolment yield and cost effectiveness. Results may help other research groups to optimize their recruitment strategies. METHODS: We applied different recruitment strategies to acquire families with children aged 6 to 47 months and at least one parent with obesity (risk group) or two parents of normal weight (control group) for a longitudinal non-interventional study. Based on four main strategies-via media, kindergartens, health professionals and focusing on the community-we examined 15 different subcategories of strategies. Based on enrolment yield and relative costs (e.g., material expenses, staff time) we analyzed the effectiveness of each recruitment strategy. RESULTS: Following different recruitment approaches, 685 families contacted us; 26% (n = 178) of these met the inclusion criteria. Of the four main strategies, the community-focused strategy was the most successful one (accounting for 36.5% of the sample) followed by contacts with kindergartens (accounting for 28.1% of the sample). Of the subcategories, two strategies were outstanding: Posters (community-focused strategies), and recruitment via kindergartens using phone contacts rather than emailing. Only a small number of participants were recruited via announcements in newspapers (lower cost strategy), advertisements on public transport or face-to-face recruitment at various places (higher cost strategies). CONCLUSIONS: Results revealed that only a combination of different active and passive methods and approaches led to a sufficient sample size. In this study, recruitment via posters and contacting kindergartens on the phone produced the highest numbers of participants (high enrolment yield) at moderate costs.

Risk Factors and Outcome of Chronic Graft-versus-Host Disease after Allogeneic Stem Cell Transplantation-Results from a Single-Center Observational Study.

Chronic graft versus host disease (cGVHD) is the most frequent long-term complication after allogeneic stem cell transplantation (allo-SCT) and results in impaired quality of life and increased long-term morbidity and mortality. We analyzed 243 patients with cGVHD, documented according to the 2005 revised National Institutes of Health consensus criteria, to identify risk factors for the occurrence of cGVHD and outcomes for the patients with cGVHD. Patients without evidence of cGVHD (n = 147) were used as controls. Performing univariate and multivariate Cox regression analyses, we identified prior acute GVHD grades III or IV (hazard ratio [HR], 2.01; P = .005), use of peripheral blood stem cell graft (HR, 2.10; P = .03), and HLA-mismatched allo-SCT from unrelated donor (HR, 1.57; P = .02) as independent risk factors for cGVHD. Performing Kaplan-Meier analyses, progressive compared with de novo and quiescent onset of cGVHD and a platelet count of less than 100/nL compared with more than 100/nL at the time of cGVHD onset were associated with a significantly increased cumulative incidence of transplantation-related mortality (TRM) and significantly decreased overall survival. Furthermore, we found a significantly higher incidence of TRM in patients with severe cGVHD compared with patients without cGVHD (58% versus 11%, P < .0001). However, in subgroup analysis, patients with severe cGVHD and involvement of the lung, liver, or gastrointestinal (GI) tract had a 6.5-fold significantly higher incidence of TRM (72%), whereas patients with severe cGVHD lacking lung, liver, or GI involvement had only a 2.8-fold significantly higher incidence of TRM (31%) compared with patients without cGVHD (11%; P < .0001 and P = .03). Patients without lung, liver, or GI involvement did not have a significantly different TRM compared with patients with moderate cGVHD (31% versus 25%, P = .52). In conclusion, we confirm prior known risk factors for the occurrence of cGVHD and subsequent mortality and we provide evidence that the presence of lung, liver, or GI involvement in patients with severe cGVHD defines a subgroup with high mortality after allo-SCT; however, in the absence of these risk factors, the outcome appears not to be impaired compared with moderate cGVHD.

ABO blood group antigen mismatch has an impact on outcome after allogeneic peripheral blood stem cell transplantation.

ABO blood group antigen incompatibility (ABO mismatch) is not an obstacle to allogeneic stem cell transplantation (allo-SCT). However, the impact on clinical outcome after allo-SCT remains controversial. We analyzed 512 patients after allogeneic peripheral blood SCT (allo-PBSCT) for an association of ABO mismatch with transfusion requirements, myeloid and platelet engraftment, the incidence of GvHD, relapse, transplant-related mortality (TRM), and overall survival (OS). A total of 260 patients underwent ABO-mismatched transplantation and the control group consisted of 252 patients with ABO-matched allo-PBSCT. We found a significant association between major-0 ABO mismatch (group 0 recipient/group A, B, or AB donor) and increased red blood cell (RBC) and platelet transfusion requirements (both P<.001) as well as delayed platelet engraftment (P<.001). Minor-A (group A recipient/group 0 donor) and minor-AB (group AB recipient/group 0, A, or B donor) ABO mismatch was significantly associated with an increased TRM after allo-PBSCT (P=.001 and P=.02). In multivariate analysis performed using Cox regression, minor ABO mismatch appeared as independent risk factor for TRM after allo-PBSCT. No association was found for ABO mismatch with the incidence of GvHD, relapse, and OS. Our results suggest that ABO blood group mismatch has a significant impact on the outcome and that minor-A and minor-AB ABO mismatch represents a risk factor for increased TRM after allo-PBSCT.

Donor Nucleotide-Binding Oligomerization-Containing Protein 2 (NOD2) Single Nucleotide Polymorphism 13 Is Associated with Septic Shock after Allogeneic Stem Cell Transplantation.

Single nucleotide polymorphisms (SNPs) within nucleotide-binding oligomerization domain-containing protein 2 (NOD2) and toll-like receptor (TLR) 5 genes have been recently associated with the incidence and outcome of infections. In this study, we analyzed 38 patients with septic shock after allogeneic stem cell transplantation (allo-SCT) for an association of SNPs within NOD2 and TLR5 genes, with susceptibility to septic shock. One hundred twenty-seven transplant recipients unaffected by any infectious complications were used as controls. We found a significant association between the presence of donor NOD2 SNP13 (3016_3017insC) and the incidence of septic shock (P = .002). In multivariate analysis, donor NOD2 SNP13 appeared as an independent risk factor for the incidence of septic shock after allo-SCT. No association was found for recipient SNPs (NOD2 and TLR5) and donor NOD2 SNP8, SNP12, and TLR5-Stop SNP. Our results suggest that NOD2 SNP13 has an impact on the pathophysiology of severe infectious complications and is an independent risk factor for the development of septic shock after allo-SCT.

Invasive mole in a perimenopausal woman: a case report and systematic review.

OBJECTIVE: Gestational trophoblastic disease (GTD) is a term used for a group of pregnancy-related tumors. We present a case of a perimenopausal woman with invasive mole. A systematic review was performed to identify reports on GTD in older women and to determine adequate treatment options. CASE: A 51-year-old perimenopausal woman was admitted to hospital with abdominal feeling of pressure and nausea. Diagnostic curettage revealed hydatidiform mole. She also presented symptomatic hyperthyroidism with hypertensive blood pressure and uneasiness. After treatment with beta blockers and carbimazole, the patient underwent abdominal hysterectomy and bilateral oophorosalpingectomy. Histopathological examination confirmed an invasive hydatidiform mole (IHM). Serum ß-hCG has decreased from initially 300,000-100 unit/L after 4 weeks. DATA SOURCES: A systematic review was performed to identify all prior cases of GTD in women over 50. We searched in Medline, The Cochrane Library and Embase, to identify any articles published in the English language after 1970 and before Oct 31, 2013 pertaining to GTD in older woman (50 years or older). TABULATION, INTEGRATION, AND RESULTS: Ten records were included in the systematic review, involving 203 cases of trophoblastic disease in older women. Although the diagnosis of GTD in older women is rare, it should be considered especially in patients with suspicious intrauterine findings in transvaginal ultrasound examinations. Different treatments were performed. In a limited number of reports, older women with GTD underwent initial hysterectomy. Benefits are avoidance of chemotherapy-induced toxicity and reduced risk of recurrence. Hysterectomy should be performed by an experienced surgeon. CONCLUSION: It is concluded that GTD is very rare in peri- or postmenopausal women. Treatment has to be individualized, and hysterectomy can be considered as an appropriate option.

Insatiable insecurity: maternal obesity as a risk factor for mother-child attachment and child weight.

Childhood obesity has become a rising health problem, and because parental obesity is a basic risk factor for childhood obesity, biological factors have been especially considered in the complex etiology. Aspects of the family interaction, e.g., mother-child attachment, have not been the main focus. Our study tried to fill this gap by investigating whether there is a difference between children of obese and normal weight mothers in terms of mother-child attachment, and whether mother-child attachment predicts child's weight, in a sample of 31 obese and 31 normal weight mothers with children aged 19 to 58 months. Mother-child attachment was measured with the Attachment Q-Set. We found that (1) children of obese mothers showed a lower quality of mother-child attachment than children of normal weight mothers, which indicates that they are less likely to use their mothers as a secure base; (2) the attachment quality predicted child`s BMI percentile; and (3) the mother-child attachment adds incremental validity to the prediction of child's BMI beyond biological parameters (child's BMI birth percentile, BMI of the parents) and mother's relationship status. Implications of our findings are discussed.

Retrospective analysis of the incidence and outcome of late acute and chronic graft-versus-host disease-an analysis from transplant centers across Europe.

Introduction: Chronic graft-versus-host disease (cGvHD) is a serious late complication of allogeneic hematopoietic stem cell transplantation (allo-HSCT). Methods: This multicenter analysis determined the cumulative incidence (CI) of cGvHD and late acute GvHD (laGvHD) and its impact on transplantation-related mortality (TRM), relapse (R), and overall survival (OS) in 317 patients [296 adults, 21 pediatrics (<12 years of age)] who underwent their first allo-HSCT in 2017. Results: The CI of laGvHD was 10.5% in adults and 4.8% in pediatrics, and the CI of cGvHD was 43.0% in all adult transplant patients and 50.2% in the adult at-risk cohort at the study end. The onset of cGvHD was de novo in 42.0% of patients, quiescent in 52.1%, and progressive in 5.9%. In adults, prophylactic use of antithymocyte globulin or posttransplant cyclophosphamide was associated with a significantly lower incidence of cGvHD (28.7%) vs. standard prophylaxis with calcineurin inhibitors (30.6%) and methotrexate/mycophenolate mofetil (58.4%) (all p < 0.01). TRM was significantly higher in patients with aGvHD (31.8%) vs. cGvHD (12.6%) and no GvHD (6.3%) (all p = 0.0001). OS in the adult at-risk cohort was significantly higher in patients with cGvHD (78.9%) vs. without (66.2%; p = 0.0022; HR 0.48) due to a significantly lower relapse rate (cGvHD: 14.5%; without cGvHD: 27.2%; p = 0.00016, HR 0.41). OS was also significantly higher in patients with mild (80.0%) and moderate (79.2%) cGvHD vs. without cGvHD (66.2%), excluding severe cGvHD (72.7%) (all p = 0.0214). Discussion: The negative impact of severe cGvHD on OS suggests a focus on prevention of severe forms is warranted to improve survival and quality of life.

TLR5 stop codon polymorphism is associated with invasive aspergillosis after allogeneic stem cell transplantation.

Single nucleotide polymorphisms (SNPs) have been associated with an increased incidence of invasive aspergillosis (IA) after allogeneic stem cell transplantation (allo-SCT). We analyzed 41 patients with proven/probable IA after allo-SCT for an association of SNPs, within the TLR2, TLR4, TLR5, TLR9, and NOD2/CARD15 genes, with susceptibility to IA. The control group consisted of 130 patients who had allo-SCT but did not develop IA. While no association was found for donor SNPs and the recipients' risk of IA, analysis of recipient SNPs showed a significant association between the presence of recipient TLR5-Stop SNP (1174C> T) and the incidence of IA (P = 0.004). Multivariate analysis demonstrated that the recipient TLR5-Stop SNP appeared as an independent risk factor for IA after allo-SCT. Our study suggests that TLR5 is involved in host defense against Aspergillus fumigatus, and that the recipient TLR5-Stop SNP represents a risk factor for the development of IA after allo-SCT.

Obese parents--obese children? Psychological-psychiatric risk factors of parental behavior and experience for the development of obesity in children aged 0-3: study protocol.

BACKGROUND: The incidences of childhood overweight and obesity have increased substantially and with them the prevalence of associated somatic and psychiatric health problems. Therefore, it is important to identify modifiable risk factors for early childhood overweight in order to develop effective prevention or intervention programs. Besides biological factors, familial interactions and parental behavioral patterns may influence children's weight development. Longitudinal investigation of children at overweight risk could help to detect significant risk and protective factors. We aim to describe infants' weight development over time and identify risk and protective factors for the incidence of childhood obesity. Based on our findings we will draw up a risk model that will lay the foundation for an intervention/prevention program. METHODS/DESIGN: We present the protocol of a prospective longitudinal study in which we investigate families with children aged from 6 months to 47 months. In half of the families at least one parent is obese (risk group), in the other half both parents are normal weight (control group). Based on developmental and health-psychological models, we consider measurements at three levels: the child, the parents and parent-child-relationship. Three assessment points are approximately one year apart. At each assessment point we evaluate the psychological, social, and behavioral situation of the parents as well as the physical and psychosocial development of the child. Parents are interviewed, fill in questionnaires, and take part in standardized interaction tasks with their child in a feeding and in a playing context in our research laboratory. The quality of these video-taped parent-child interactions is assessed by analyzing them with standardized, validated instruments according to scientific standards. DISCUSSION: Strengths of the presented study are the prospective longitudinal design, the multi-informant approach, including the fathers, and the observation of parent-child interaction. A limitation is the variation in children's age.

Osteosarcomas in older adults: A report from the Cooperative Osteosarcoma Study Group.

INTRODUCTION: Osteosarcoma is typically a disease of the young, but may affect any age. Little is known about the disease in older patients beyond retirement age. We aim to describe the characteristics, treatment, and outcomes of older adult patients registered with our cooperative group. MATERIALS AND METHODS: The database of the Cooperative Osteosarcoma Study Group (COSS) was searched for osteosarcoma patients diagnosed from 1980 to 2020 who were aged 65 years or older at diagnosis. Affected individuals were analyzed for presenting factors, treatments employed, and outcomes. RESULTS: Fifty-five eligible patients were detected (median age 68 [range: 65-84] years; male:female = 25:30). Among these patients, 15/55 (27%) tumors were secondary malignancies, 41/55 (75%) were high-grade central, 4/55 (7%) surface, and 10/55 (18%) extraosseous malignancies, and all but three high-grade. Primary metastases were present in 15/55 (27%) patients. Surgery was reported for 46/55 (84%) patients, radiotherapy for 6/54 (11%, 1 unknown), chemotherapy for 42/50 (84%, 5 unknown). A complete surgical remission was achieved in 31/55 (56%). There were two toxic deaths. With a median follow-up of 1.7 (range: 0.1-18.0) years for all 55 patients and 2.2 (0.1-12.4) years for 24 survivors, event-free and overall survival at 2/5 years were 39.6% (standard error: 6.8%) / 24.5% (6.5%) and 62.0% (7.1%) / 32.7% (7.5%), respectively. Tumor site, metastatic status, surgery, and a complete surgical remission were prognostic for event-free and/or overall survival. DISCUSSION: Osteosarcomas can occur in older individuals. It is more often secondary, axially located, or extraosseous than in younger patients. However, the same treatment principles seem to apply, and selected patients may be cured. Multi-center cooperation is encouraged, thereby gathering expertise for such a rare disease presentation.