WorldCat Identities

Maki, Robert G.

Works: 10 works in 30 publications in 4 languages and 534 library holdings
Roles: Editor, Author
Publication Timeline
Most widely held works by Robert G Maki
Management of soft tissue sarcoma by Murray F Brennan( )

20 editions published between 2012 and 2018 in 3 languages and held by 523 WorldCat member libraries worldwide

Management of Soft Tissue Sarcoma addresses the diagnosis and best current management of adult soft tissue sarcomas. Edited by world renowned experts, this book delineates and discusses each different sarcoma subtype individually. Both clinical and molecular diagnoses are addressed, and tumor histopathology is employed as the basis of treatment recommendations including surgery, radiation therapy, systemic therapy and novel therapeutics
Sarcomas and gastrointestinal stromal tumors : presentations from the 2008 European Society for Medical Oncology international symposium( Book )

2 editions published in 2009 in English and held by 3 WorldCat member libraries worldwide

Monogenic and polygenic determinants of sarcoma risk: an international genetic study( )

1 edition published in 2016 in English and held by 1 WorldCat member library worldwide

Background: Sarcomas are rare, phenotypically heterogeneous cancers that disproportionately affect the young. Outside rare syndromes, the nature, extent, and clinical significance of their genetic origins are not known. We aimed to investigate the genetic basis for bone and soft-tissue sarcoma seen in routine clinical practice. Methods: In this genetic study, we included 1162 patients with sarcoma from four cohorts (the International Sarcoma Kindred Study [ISKS], 966 probands;Project GENESIS, 48 probands; Asan Bio-Resource Center, 138 probands; and kConFab, ten probands), who were older than 15 years at the time of consent and had a histologically confirmed diagnosis of sarcoma, recruited from specialist sarcoma clinics without regard to family history. Detailed clinical, pathological, and pedigree information was collected, and cancer diagnoses in probands and relatives were independently verified. Targeted exon sequencing using blood (n=1114) or saliva (n=48) samples was done on 72 genes (selected due to associations with increased cancer risk) and rare variants were stratified into classes approximating the International Agency for Research on Cancer (IARC) clinical classification for genetic variation. We did a case-control rare variant burden analysis using 6545 Caucasian controls included from three cohorts (ISKS, 235 controls; LifePool, 2010 controls; and National Heart, Lung, and Blood Institute Exome Sequencing Project [ESP], 4300 controls). Findings: The median age at cancer diagnosis in 1162 sarcoma probands was 46 years (IQR 29–58), 170 (15%) of 1162 probands had multiple primary cancers, and 155 (17%) of 911 families with informative pedigrees fitted recognisable cancer syndromes. Using a case-control rare variant burden analysis, 638 (55%) of 1162 sarcoma probands bore an excess of pathogenic germline variants (combined odds ratio [OR] 1·43, 95% CI 1·24–1·64, p<0·0001), with 227 known or expected pathogenic variants occurring in 217 individuals. All classes of pathogenic variants (known, expected, or predicted) were associated with earlier age of cancer onset. In addition to TP53, ATM, ATR, and BRCA2, an unexpected excess of functionally pathogenic variants was seen in ERCC2 . Probands were more likely than controls to have multiple pathogenic variants compared with the combined control cohort group and the LifePool control cohort (OR 2·22, 95% CI 1·57–3·14, p=1·2 × 10 −6 ) and the cumulative burden of multiple variants correlated with earlier age at cancer diagnosis (Mantel-Cox log-rank test for trend, p=0·0032). 66 of 1162 probands carried notifiable variants following expert clinical review (those recognised to be clinically significant to health and about which patients should be advised), whereas 293 (25%) probands carried variants with potential therapeutic significance. Interpretation: About half of patients with sarcoma have putatively pathogenic monogenic and polygenic variation in known and novel cancer genes, with implications for risk management and treatment. Funding: Rainbows for Kate Foundation, Johanna Sewell Research Foundation, Australian National Health and Medical Research Council, Cancer Australia, Sarcoma UK, National Cancer Institute, Liddy Shriver Sarcoma Initiative
Malignant Peripheral Nerve Sheath Tumors( )

1 edition published in 2014 in English and held by 1 WorldCat member library worldwide

Learning Objectives: Explain the characteristics and treatment of malignant peripheral nerve sheath tumors, both in relation to neurofibromatosis type I and otherwise. Cite the unique challenges in optimal management of malignant peripheral nerve sheath tumors. Appraise the large amount of new data surrounding the potential molecular drivers, possible targets for therapy in this disease. Abstract : Malignant peripheral nerve sheath tumors (MPNST) are uncommon, biologically aggressive soft tissue sarcomas of neural origin that pose tremendous challenges to effective therapy. In 50% of cases, they occur in the context of neurofibromatosis type I, characterized by loss of function mutations to the tumor suppressor neurofibromin; the remainder arise sporadically or following radiation therapy. Prognosis is generally poor, with high rates of relapse following multimodality therapy in early disease, low response rates to cytotoxic chemotherapy in advanced disease, and propensity for rapid disease progression and high mortality. The last few years have seen an explosion in data surrounding the potential molecular drivers and targets for therapy above and beyond neurofibromin loss. These data span multiple nodes at various levels of cellular control, including major signal transduction pathways, angiogenesis, apoptosis, mitosis, and epigenetics. These include classical cancer‐driving genetic aberrations such as TP53 and phosphatase and tensin homolog ( PTEN ) loss of function, and upregulation of mitogen‐activated protein kinase (MAPK) and (mechanistic) target of rapamycin (TOR) pathways, as well as less ubiquitous molecular abnormalities involving inhibitors of apoptosis proteins, aurora kinases, and the Wingless/int (Wnt) signaling pathway. We review the current understanding of MPNST biology, current best practices of management, and recent research developments in this disease, with a view to informing future advancements in patient care. Abstract : Malignant peripheral nerve sheath tumors are amongst the most challenging mesenchymal malignancies to treat. Their frequent association with a seemingly simple genetic aberration—the loss of the tumor suppressor gene neurofibromin—belies genomic complexity that has rendered effective therapy elusive to date. This review aims to detail elements of current optimal clinical management and summarize recent data on potential molecular drivers and targets, with a view to charting the course for future progress in patient care
Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial( )

1 edition published in 2017 in English and held by 1 WorldCat member library worldwide

Background: Patients with advanced sarcomas have a poor prognosis and few treatment options that improve overall survival. Chemotherapy and targeted therapies offer short-lived disease control. We assessed pembrolizumab, an anti-PD-1 antibody, for safety and activity in patients with advanced soft-tissue sarcoma or bone sarcoma. Methods: In this two-cohort, single-arm, open-label, phase 2 study, we enrolled patients with soft-tissue sarcoma or bone sarcoma from 12 academic centres in the USA that were members of the Sarcoma Alliance for Research through Collaboration (SARC). Patients with soft-tissue sarcoma had to be aged 18 years or older to enrol; patients with bone sarcoma could enrol if they were aged 12 years or older. Patients had histological evidence of metastatic or surgically unresectable locally advanced sarcoma, had received up to three previous lines of systemic anticancer therapy, had at least one measurable lesion according to the Response Evaluation Criteria In Solid Tumors version 1.1, and had at least one lesion accessible for biopsy. All patients were treated with 200 mg intravenous pembrolizumab every 3 weeks. The primary endpoint was investigator-assessed objective response. Patients who received at least one dose of pembrolizumab were included in the safety analysis and patients who progressed or reached at least one scan assessment were included in the activity analysis. Accrual is ongoing in some disease cohorts. This trial is registered, numberNCT02301039 . Findings: Between March 13, 2015, and Feb 18, 2016, we enrolled 86 patients, 84 of whom received pembrolizumab (42 in each disease cohort) and 80 of whom were evaluable for response (40 in each disease cohort). Median follow-up was 17·8 months (IQR 12·3–19·3). Seven (18%) of 40 patients with soft-tissue sarcoma had an objective response, including four (40%) of ten patients with undifferentiated pleomorphic sarcoma, two (20%) of ten patients with liposarcoma, and one (10%) of ten patients with synovial sarcoma. No patients with leiomyosarcoma (n=10) had an objective response. Two (5%) of 40 patients with bone sarcoma had an objective response, including one (5%) of 22 patients with osteosarcoma and one (20%) of five patients with chondrosarcoma. None of the 13 patients with Ewing's sarcoma had an objective response. The most frequent grade 3 or worse adverse events were anaemia (six [14%]), decreased lymphocyte count (five [12%]), prolonged activated partial thromboplastin time (four [10%]), and decreased platelet count (three [7%]) in the bone sarcoma group, and anaemia, decreased lymphocyte count, and prolonged activated partial thromboplastin time in the soft-tissue sarcoma group (three [7%] each). Nine (11%) patients (five [12%] in the bone sarcoma group and four [10%] in the soft-tissue sarcoma group) had treatment-emergent serious adverse events (SAEs), five of whom had immune-related SAEs, including two with adrenal insufficiency, two with pneumonitis, and one with nephritis. Interpretation: Pembrolizumab has meaningful clinical activity in patients with undifferentiated pleomorphic sarcoma or dedifferentiated liposarcoma. Enrolment to expanded cohorts of those subtypes is ongoing to confirm and characterise the activity of pembrolizumab. Funding: Merck, SARC, Sarcoma Foundation of America, QuadW Foundation, Pittsburgh Cure Sarcoma, and Ewan McGregor
An application of statistical process control measures for maintaining optimal quality from dry kiln operations by Robert G Maki( Book )

1 edition published in 1991 in English and held by 1 WorldCat member library worldwide

Statistical Process Control techniques have been successfully implemented in the mechanical fabrication and chemical manufacturing industries. The techniques consist of the application of statistical theory in conjunction with quality control procedures. To date the Forest Products industry has yet to apply these techniques toward lumber drying. This thesis focuses on the application of Statistical Process Controls to evaluate the final moisture content of dried lumber and the accuracy of sticker placement by stackers. Computer models, based on real data, were used to produce the necessary quantitative information required to construct X-bar, Range, group, and p charts. These models were modified to reproduce drying and stickering processes that were under control or out of control. From the computer, simulated data control charts were drawn to graphically prove that such charts can be used for quality control purposes when drying or stickering lumber
Activity of Pazopanib and Trabectedin in Advanced Alveolar Soft Part Sarcoma( )

1 edition published in 2017 in English and held by 1 WorldCat member library worldwide

Abstract: Background: Alveolar soft part sarcoma (ASPS) is an exceedingly rare and orphan disease, without active drugs approved in the front line. Pazopanib and trabectedin are licensed for sarcoma treatment from second‐line, but very little and contradictory data are available on their activity in ASPS. Lacking ongoing and/or planned clinical trials, we conducted a multi‐institutional study involving the reference sites for sarcoma in Europe, U.S., and Japan, within the World Sarcoma Network, to investigate the efficacy of pazopanib and trabectedin. Materials and Methods: From May 2007, 14 of the 27 centers that were asked to retrospectively review their databases had identified 44 advanced ASPS patients treated with pazopanib and/or trabectedin. Response was evaluated by Response Evaluation Criteria in Solid Tumors 1.1. Progression‐free survival (PFS) and overall survival (OS) were computed by Kaplan‐Meier method. Results: Among 30 patients who received pazopanib, 18 were pretreated (13 with other antiangiogenics). Response was evaluable in 29/30 patients. Best responses were 1 complete response, 7 partial response (PR), 17 stable disease (SD), and 4 progressions. At a 19‐month median follow‐up, median PFS was 13.6 months (range: 1.6–32.2+), with 59% of patients progression‐free at 1 year. Median OS was not reached. Among 23 patients treated with trabectedin, all were pretreated and evaluable for response. Best responses were 1 PR, 13 SD, and 9 progressions. At a 27‐month median follow‐up, median PFS was 3.7 months (range: 0.7–109), with 13% of patients progression‐free at 1 year. Median OS was 9.1 months. Conclusion: The value of pazopanib in advanced ASPS is confirmed, with durable responses, whereas the value of trabectedin appears limited. These results are relevant to defining the best approach to advanced ASPS. Implications for Practice: This retrospective study, conducted among the world reference centers for treatment of sarcoma, confirms the value of pazopanib in patients with advanced alveolar soft part sarcoma (ASPS), with dimensional and durable responses, whereas trabectedin shows a limited activity. Alveolar soft part sarcoma is resistant to conventional cytotoxic chemotherapy. Pazopanib and trabectedin are licensed for treatment of sarcoma from second line; in the lack of prospective clinical trials, these results are relevant to defining ASPS best management and strongly support initiatives aimed at obtaining the approval of pazopanib in the front line of the disease. Abstract : Because of the rarity of alveolar soft part sarcoma and the lack of prospective trials, a retrospective, multi‐institutional, caseseries analysis was conducted among reference centers for treatment of soft tissue sarcoma.With the support of the World Sarcoma Network, the activity and efficacy of trabectedin and pazopanib in advanced alveolar soft part sarcoma patients was investigated. The results are presented here
Los sarcomas, arquetipos de cancer by Igor Matushansky( )

1 edition published in 2006 in Spanish and held by 1 WorldCat member library worldwide

Age‐Stratified Risk of Unexpected Uterine Sarcoma Following Surgery for Presumed Benign Leiomyoma( )

1 edition published in 2015 in English and held by 1 WorldCat member library worldwide

Abstract : Background: Estimates of unexpected uterine sarcoma following surgery for presumed benign leiomyoma that use age‐stratification are lacking. Patients and Methods: A retrospective cohort of 2, 075 patients that had undergone myomectomy was evaluated to determine the case incidence of unexpected uterine sarcoma. An aggregate risk estimate was generated using a meta‐analysis of similar studies plus our data. Database‐derived age distributions of the incidence rates of uterine sarcoma and uterine leiomyoma surgery were used to stratify risk by age. Results: Of 2, 075 patients in our retrospective cohort, 6 were diagnosed with uterine sarcoma. Our meta‐analysis revealed 8 studies from 1980 to 2014. Combined with our study, 18 cases of leiomyosarcoma are reported in 10, 120 patients, for an aggregate risk of 1.78 per 1, 000 (95% confidence interval [CI]: 1.1–2.8) or 1 in 562. Eight cases of other uterine sarcomas were reported in 6, 889 patients, for an aggregate risk of 1.16 per 1, 000 (95% CI: 0.5–4.9) or 1 in 861. The summation of these risks gives an overall risk of uterine sarcoma of 2.94 per 1, 000 (95% CI: 1.8–4.1) or 1 in 340. After stratification by age, we predict the risk of uterine sarcoma to range from a peak of 10.1 cases per 1, 000, or 1 in 98, for patients aged 75–79 years to <1 case per 500 for patients aged <30 years. Conclusion: The risk of unexpected uterine sarcoma varies significantly across age groups. Our age‐stratified predictive model should be incorporated to more accurately counsel patients and to assist in providing guidelines for the surgical technique for leiomyoma. Abstract : Significant controversy about the use of minimally invasive procedures for leiomyoma removal currently exists because of the potential risk of dissemination of unexpected sarcoma. The results of this study show that the risk of unexpected uterine sarcoma is projected to vary significantly by age. The proposed predictive model should be used by clinicians to counsel patients and to assist in providing guidelines for the surgical technique for leiomyoma
Eribulin versus dacarbazine in previously treated patients with advanced liposarcoma or leiomyosarcoma: a randomised, open-label, multicentre, phase 3 trial( )

1 edition published in 2016 in English and held by 1 WorldCat member library worldwide

Background: A non-randomised, phase 2 study showed activity and tolerability of eribulin in advanced or metastatic soft-tissue sarcoma. In this phase 3 study, we aimed to compare overall survival in patients with advanced or metastatic soft-tissue sarcoma who received eribulin with that in patients who received dacarbazine (an active control). Methods: We did this randomised, open-label, phase 3 study across 110 study sites in 22 countries. We enrolled patients aged 18 years or older with intermediate-grade or high-grade advanced liposarcoma or leiomyosarcoma who had received at least two previous systemic regimens for advanced disease (including an anthracycline). Using an interactive voice and web response system, an independent statistician randomly assigned (1:1) patients to receive eribulin mesilate (1·4 mg/m 2 intravenously on days 1 and 8) or dacarbazine (850 mg/m 2, 1000 mg/m 2, or 1200 mg/m 2 [dose dependent on centre and clinician] intravenously on day 1) every 21 days until disease progression. Randomisation was stratified by disease type, geographical region, and number of previous regimens for advanced soft-tissue sarcoma and in blocks of six. Patients and investigators were not masked to treatment assignment. The primary endpoint was overall survival in the intention-to-treat population. The study is registered, numberNCT01327885, and is closed to recruitment, but treatment and follow-up continue. Findings: Between March 10, 2011 and May 22, 2013, we randomly assigned patients to eribulin (n=228) or dacarbazine (n=224). Overall survival was significantly improved in patients assigned to eribulin compared with those assigned to dacarbazine (median 13·5 months [95% CI 10·9–15·6] vs 11·5 months [9·6–13·0]; hazard ratio 0·77 [95% CI 0·62–0·95]; p=0·0169). Treatment-emergent adverse events occurred in 224 (99%) of 226 patients who received eribulin and 218 (97%) of 224 who received dacarbazine. Grade 3 or higher adverse events were more common in patients who received eribulin (152 [67%]) than in those who received dacarbazine (126 [56%]), as were deaths (10 [4%] vs 3 [1%]); one death (in the eribulin group) was considered treatment-related by the investigators. Interpretation: Overall survival was improved in patients assigned to eribulin compared with those assigned to an active control, suggesting that eribulin could be a treatment option for advanced soft-tissue sarcoma. Funding: Eisai
Audience Level
Audience Level
  Kids General Special  
Audience level: 0.50 (from 0.49 for Management ... to 0.91 for Sarcomas a ...)