<i>Purpose</i>. A retrospective study of prognostic factors and treatment outcome of osteosarcoma (OS) during modern chemotherapy era with focus on patients with primary metastatic disease, nonextremity localisation, or age &#x3E;40 years (nonclassical OS). <i>Methods</i>. A nationwide cohort, comprising 424 high-grade Norwegian bone OS patients, was based on registry sources supplemented with clinical records from hospitals involved in sarcoma management between 1975 and 2009. <i>Results</i>. Only 48&#x25; were younger patients with tumour in the extremities and without metastasis at diagnosis (classical OS). A considerable discrepancy in survival between classical and nonclassical OS was observed: 61&#x25; versus 26&#x25;  10-year sarcoma specific survival. Twice as many of the former received both adequate surgery and chemotherapy compared to the latter. This could only partly explain the differences in survival due to inherent chemoresistance in primary metastatic disease and a higher rate of local relapse among patients with axial tumours. Metastasis at diagnosis, increased lactate dehydrogenase, age &#x3E; 40 years, and tumour size above median value were all adverse prognostic factors for overall survival. <i>Conclusion</i>. We confirm a dramatic difference in outcome between classical and nonclassical high-grade OS patients, but treatment variables could only partly explain the dismal outcome of the latter.

Kjetil Berner

Kirsten Sundby Hall

Odd R. Monge

Harald Weedon-Fekj&#xe6;r

Olga Zaikova

&#xd8;yvind S. Bruland

Prognostic Factors and Treatment Results of High-Grade Osteosarcoma in Norway: A Scope Beyond the &#x201C;Classical&#x201D; Patient

<i>Introduction</i>. Although several intercalary reconstructions after resection of a lower extremity malignant bone tumor are reported, there are no optimal methods which can provide a long-term reconstruction with fewest complications. We present the outcome of reconstruction using a devitalized autograft and a vascularized fibula graft composite.<i> Materials and Methods.</i> We conducted a retrospective review of 11 patients (7 males, 4 females; median age 27 years) undergoing reconstruction using a devitalized autograft (pasteurization (<svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" style="vertical-align:-0.2130003pt" id="M1" height="8.91176pt" version="1.1" viewBox="-0.0498162 -8.69876 29.0065 8.91176" width="29.0065pt"><g transform="matrix(.0135,0,0,-0.0135,0,0)"><path id="g113-111" d="M495 86L479 114C446 82 419 66 409 66C401 66 401 72 406 97C420 166 436 231 453 297C489 435 454 448 428 448C406 448 384 439 354 422C305 394 222 327 161 247H159L183 345C200 415 194 448 173 448C143 448 82 410 23 351L38 325C64 349 95 371 105 371C111 371 116 365 109 336L25 -4L31 -12C50 -4 77 3 107 9C119 69 132 122 145 168C197 254 321 381 370 381C387 381 393 374 378 305L329 95C309 17 320 -12 345 -12C372 -12 430 19 495 86Z"/></g><g transform="matrix(.0135,0,0,-0.0135,10.586,0)"><path id="g117-34" d="M535 323V373H52V323H535ZM535 138V188H52V138H535Z"/></g><g transform="matrix(.0135,0,0,-0.0135,22.324,0)"><path id="g113-55" d="M137 343C167 482 260 545 321 574C357 591 397 603 429 609L423 641C382 634 335 622 295 608C189 570 37 457 37 238C37 84 125 -12 242 -12C362 -12 447 89 447 209C447 311 374 393 267 393C247 393 226 386 204 376L137 343ZM227 337C318 337 361 256 361 173C361 105 336 22 258 22C176 22 126 120 126 240C126 266 127 291 132 310C155 323 189 337 227 337Z"/></g></svg>), deep freezing (<svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" style="vertical-align:-0.2129993pt" id="M2" height="8.66698pt" version="1.1" viewBox="-0.0498162 -8.45398 29.0065 8.66698" width="29.0065pt"><g transform="matrix(.0135,0,0,-0.0135,0,0)"><use xlink:href="#g113-111"/></g><g transform="matrix(.0135,0,0,-0.0135,10.586,0)"><use xlink:href="#g117-34"/></g><g transform="matrix(.0135,0,0,-0.0135,22.324,0)"><path id="g113-54" d="M153 550H386L412 615L406 623H120L82 318C104 327 142 338 184 338C294 338 347 275 347 187C347 112 305 39 221 39C160 39 119 71 97 89C88 97 80 96 71 90C59 80 50 67 49 57C48 45 52 36 66 23C80 9 123 -12 169 -12C221 -11 288 15 342 59C403 109 431 165 431 225C431 308 366 395 238 395C212 395 165 379 127 364L153 550Z"/></g></svg>)) and a vascularized fibula graft composite for lower extremity malignant bone tumors (femur (<svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" style="vertical-align:-0.2130003pt" id="M3" height="8.91176pt" version="1.1" viewBox="-0.0498162 -8.69876 35.534 8.91176" width="35.534pt"><g transform="matrix(.0135,0,0,-0.0135,0,0)"><use xlink:href="#g113-111"/></g><g transform="matrix(.0135,0,0,-0.0135,10.586,0)"><use xlink:href="#g117-34"/></g><g transform="matrix(.0135,0,0,-0.0135,22.324,0)"><path id="g113-50" d="M384 0V27C293 34 287 42 287 114V635C232 613 172 594 109 583V559L157 557C201 555 205 550 205 499V114C205 42 199 34 109 27V0H384Z"/></g><g transform="matrix(.0135,0,0,-0.0135,28.827,0)"><path id="g113-49" d="M241 635C89 635 35 457 35 312C35 153 89 -12 240 -12C390 -12 443 166 443 312C443 466 390 635 241 635ZM238 602C329 602 354 454 354 312C354 172 330 22 240 22C152 22 124 173 124 313S148 602 238 602Z"/></g></svg>), tibia (<svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" style="vertical-align:-0.2130003pt" id="M4" height="8.91176pt" version="1.1" viewBox="-0.0498162 -8.69876 29.0065 8.91176" width="29.0065pt"><g transform="matrix(.0135,0,0,-0.0135,0,0)"><use xlink:href="#g113-111"/></g><g transform="matrix(.0135,0,0,-0.0135,10.586,0)"><use xlink:href="#g117-34"/></g><g transform="matrix(.0135,0,0,-0.0135,22.324,0)"><use xlink:href="#g113-50"/></g></svg>)).<i> Results</i>. The mean period required for callus formation and bone union was 4.4 months and 9.9 months, respectively. Four postoperative complications occurred in 3 patients: 2 infections (1 pasteurized autograft, 1 frozen autograft) and 1 fracture and 1 implant failure (both in pasteurized autografts). Graft removal was required in 2 patients with infections. The mean MSTS score was 81&#x25; at last follow-up.<i> Conclusions.</i> Although some complications were noted in early cases involving a pasteurized autograft, our novel method involving a combination of a frozen autograft with a vascularized fibula graft and rigid fixation with a locking plate may offer better outcomes than previously reported allografts or devitalized autografts.

Koichi Ogura

Shimpei Miyamoto

Minoru Sakuraba

Tomohiro Fujiwara

Hirokazu Chuman

Akira Kawai

Intercalary Reconstruction after Wide Resection of Malignant Bone Tumors of the Lower Extremity Using a Composite Graft with a Devitalized Autograft and a Vascularized Fibula

In 2013 Australia introduced Wiki-based Clinical Practice Guidelines for the Management of Adult Onset Sarcoma. These guidelines utilized a customized MediaWiki software application for guideline development and are the first evidence-based guidelines for clinical management of sarcoma. This paper presents our experience with developing and implementing web-based interactive guidelines and reviews some of the challenges and lessons from adopting an evidence-based (rather than consensus-based) approach to clinical sarcoma guidelines. Digital guidelines can be easily updated with new evidence, continuously reviewed and widely disseminated. They provide an accessible method of enabling clinicians and consumers to access evidence-based clinical practice recommendations and, as evidenced by over 2000 views in the first four months after release, with 49&#x25; of those visits being from countries outside of Australia. The lessons learned have relevance to other rare cancers in addition to the international sarcoma community.

S. J. Neuhaus

D. Thomas

J. Desai

C. Vuletich

J. von Dincklage

I. Olver

Wiki-Based Clinical Practice Guidelines for the Management of Adult Onset Sarcoma: A New Paradigm in Sarcoma Evidence

<i>Objective</i>. Extremity sarcoma (ES) is a rare cancer that presents with unique challenges. This study was performed to identify the prevalence, trajectory, and determinants of distress and characterise sources of stress in this cohort. <i>Methods</i>. Consecutive patients with ES were prospectively recruited between May 2011 and December 2012. Questionnaires were administered during initial diagnosis and then six months and one year after surgery. <i>Results</i>. Distress was reported by about a third of our cohort and associated with poorer physical function, poorer quality of life, and pain. In addition to fears regarding mortality and life role changes, the most common sources of stress were centered on dissatisfaction with the healthcare system, such as frustrations with a lack of communication with the hospital regarding appointments and lack of education regarding management and outcomes. <i>Conclusions</i>. Psychological distress presents early in the cancer journey and persists up to one year after surgery. Distress is associated with negative outcomes. Active screening and effective interventions are necessary to improve outcomes. Sources of stress have been identified that may be amenable to targeted interventions.

Melissa H. Tang

David J. Castle

Peter F. M. Choong

Identifying the Prevalence, Trajectory, and Determinants of Psychological Distress in Extremity Sarcoma

Treatment options are limited in well differentiated (WD) and dedifferentiated (DD) retroperitoneal liposarcoma. We sought to study the intratumoral adaptive immune response and explore the potential feasibility of immunotherapy in this disease. Tumor-infiltrating lymphocytes (TILs) were isolated from fresh surgical specimens and analyzed by flow cytometry for surface marker expression. Previously reported immune cell aggregates known as tertiary lymphoid structures (TLS) were further characterized by immunohistochemistry. In all fresh tumors, TILs were found. The majority of TILs were CD4 T cells; however cytotoxic CD8 T cells were also seen (average: 20&#x25; of CD3 T cells). Among CD8 T cells, 65&#x25; expressed the immune checkpoint molecule PD-1. Intratumoral TLS may be sites of antigen presentation as DC-LAMP positive, mature dendritic cells were found juxtaposed next to CD4 T cells. Clinicopathologic correlation, however, demonstrated that presence of TLS was associated with worse recurrence-free survival in WD disease and worse overall survival in DD disease. Our data suggest that an adaptive immune response is present in WD/DD retroperitoneal liposarcoma but may be hindered by TLS, among other possible microenvironmental factors; further investigation is needed. Immunotherapy, including immune checkpoint blockade, should be evaluated as a treatment option in this disease.

William W. Tseng

Shruti Malu

Minying Zhang

Jieqing Chen

Geok Choo Sim

Wei Wei

Davis Ingram

Neeta Somaiah

Dina C. Lev

Raphael E. Pollock

Gregory Liz&#xe9;e

Laszlo Radvanyi

Patrick Hwu

Analysis of the Intratumoral Adaptive Immune Response in Well Differentiated and Dedifferentiated Retroperitoneal Liposarcoma

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<p><em>Sarcoma</em> covers all aspects of connective tissue oncology research. It brings together work from scientists and clinicians carrying out a broad range of research in this field, including the basic sciences, molecular biology and pathology etc.</p>


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Sarcoma covers all aspects of connective tissue oncology research. It brings together work from scientists and clinicians carrying out a broad range of research in this field, including the basic sciences, molecular biology and pathology etc.

Sarcoma