Outcome of Reoperation for Local Recurrence Following En Bloc Resection for Bone Giant Cell Tumor of the Extremity

En bloc resection is typically performed to treat giant cell tumors of bone (GCTB), particularly when curettage can be challenging owing to extensive bone cortex destruction with soft tissue extension. Few reports have addressed the clinical outcomes after reoperation for local recurrence in patients with GCTB who underwent en bloc resection. In this multicenter retrospective study, we investigated local recurrence, distant metastasis, malignant transformation, mortality, and limb function in patients treated for local recurrence following en bloc resection for GCTB. Among 205 patients who underwent en bloc resection for GCTB of the extremities between 1980 and 2021, we included 29 with local recurrence. En bloc resection was performed for large tumors with soft tissue extension, pathological fractures with joint invasion, complex fractures, and dispensable bones, such as the proximal fibula and distal ulna. Local re-recurrence, distant metastasis, malignant transformation, and mortality rates were 41.4% (12/29), 34.5% (10/29), 6.9% (2/29), and 6.9% (2/29), respectively. The median Musculoskeletal Tumor Society score was 26 (interquartile range, 23–28). The median follow-up period after surgery for local recurrence was 70.1 months (interquartile range, 40.5–123.8 months). Local recurrence following en bloc resection for GCTB could indicate an aggressive GCTB, necessitating careful follow-up

Evaluation der Expression von PDGFR-β, VEGF-ab1, Ki-67 und Bcl-2 zur Therapieprädiktion bei Riesenzelltumoren des Skelettsystems

Diese Studie untersucht die prognostische Relevanz von PDGFR-ß, VEGF-ab1, Ki-67 und Bcl-2 bei ossären Riesenzelltumoren zwecks zielgerichteter molekularer Therapien. MATERIAL UND METHODEN: Mittels TMA-Verfahren wurden 112 Biopsien auf die Expression der Faktoren untersucht und den klinischen Verläufen gegenübergestellt. SIGNIFIKANTE ERGEBNISSE: 87,2 % zeigten im Stroma keine Expression für Bcl-2. Der Anteil gering oder mittelstark angefärbter Proben war mit je 3,7% sehr gering. 70,3% der Gewebeproben zeigte keine oder geringe Expression von Ki-67. Für VEGF konnte bei je 13,8% eine leichte und mittelstarke Färbung nachgewiesen werden. 97,2% des Stromas zeigte eine Expression von PDGFR-ß. Es bestand keine Korrelation der Ergebnisse mit Rezidiven, Metastasen oder Malignität. DISKUSSION UND SCHLUSSFOLGERUNG: Die Expression der untersuchten Faktoren ist bei RZT kein prognostischer Parameter. PDGFR-ß und VEGF sind eventuell Ansatzpunkte für Angiogenese-Hemmstoffe in der RZT-Therapie

Total Spondylectomy for Upper Thoracic Spine Giant Cell Tumor: A Case Report

Introduction: Giant cell tumors (GCT) are benign lesions that are generally locally aggressive tumors with occasional malignant behavior. These tumors are most frequently encountered in long bones; however, they also occur rarely in the spine. GCT of the spine are rare pathological entities, and spinal involvement shows a sacral predilection, with only a few cases involving the supra-sacral segment (mobile spine). Only a few cases of thoracic spinal GCT are reported in the literature; these tumors are particularly uncommon in the thoracic segment. Presentation of Case: A 29-year-old man presented with a complaint of neck pain over the previous six months that radiated to his left hand. GCT of the upper thoracic spine was diagnosed, which was surgically managed using a 2-stage approach involving total resection of the tumor followed by spondylectomy and multilevel spinal fixation. Discussion: Accurate diagnosis of vertebral column lesions, and choosing an optimum management plan are crucial. In the majority of cases, En-bloc resection of GCTs is not feasible ought to the close contact of the lesion with the spinal cord. Larger studies are encouraged to ascertain the efficacy of variable management approaches, particularly compared with piecemeal resection techniques. Conclusion: Spinal GCT are a unique group of tumors with an uncommon and unexpected presentation. Although surgery is the mainstay of treatment for spinal GCT, the management of this tumor can be challenging. No clear management algorithm has been established, and the tumor displays an unpredictable course. Therefore, each case needs tailored treatment

Etablierung eines Modells zur Kultivierung von Riesenzelltumoren des Knochens auf der Chorio-Allantois-Membran (CAM) von Hühnereiern

Riesenzelltumoren des Knochens sind seltene Tumoren, mit lokal aggressivem Wachstum und hoher Rezidivgefahr. Bisher gab es zur Erforschung dieser Tumoren kein In-Vivo-Modell. Die CAM ist ein wichtiges Organ des Hühnerembryos und mit einfachen Mitteln kostengünstig nutzbar. Sie ermöglicht eine große Anzahl von Versuchen in relativ kurzer Zeit. Wir verwendeten Tumorproben von 10 verschiedenen Patienten, gaben diese als Suspension auf die CAM von Hühnerembryonen und dokumentierten die Veränderungen. Die Größe der Tumoren wurde abgeschätzt und Proben für verschiedene histologische und immunhistologische Untersuchungen fixiert. Von 125 beimpften Eiern zeigten 76% ein tumorartiges Gebilde mit Riesenzellen, Monozyten und Stromazellen. Weitere Untersuchungen konnten die Identität der Zellen weiter bestätigen. Die Ergebnisse zeigen, dass es gelungen ist, vollwertige Riesenzelltumoren auf der Hühner-CAM zu kultivieren

Treatment of Ewing Sarcoma, Paediatric Bone Sarcomas and Severe Paediatric Spinal Deformities in Finland

Background: Ewing sarcomas are rare highly malignant tumours. There are not many population-based studies including both bone and soft tissue tumours. Previous reports have suggested that the incidence of Ewing sarcoma in the paediatric population may be lower in Northern Europe compared to other parts of Europe. Ewing sarcomas frequently arise in the pelvis and other axial sites and rarely in the spine. Surgical techniques of vertebral column resection to treat spinal tumours or severe spinal deformities have evolved with techniques using only a posterior approach. Aims: Our aim was to find out how the treatment of Ewing sarcoma is carried out in Finland and what are the five-year survival rates and to reveal the surgical treatment and its complications. We also aimed at establishing the incidence rates of bone sarcomas in the paediatric and adolescent population in Finland and to establish the ten-year survival rates. Additionally, we aimed to report the results of a population-based material on paediatric vertebral column resection. Material and methods: We identified all Finnish patients with Ewing sarcoma diagnosed during 1990-2009, all paediatric bone sarcoma patients diagnosed during 1991-2005 and all paediatric patients who underwent vertebral column resection during 2005-2009. The medical records were reviewed for detailed information and these data were analysed. The study is retrospective, nationwide and population-based. Results: Seventy-six Ewing sarcoma patients were included in the final analyses. The five-year sarcoma-specific survival rates were 70% for those with localized and 33% for those with metastatic disease (p=0.001). Fifty-seven patients underwent surgical treatment of the primary tumour. The annual incidence rates of osteosarcoma, Ewing sarcoma and chondrosarcoma per million 0-18-year-old people in Finland during 1991-2005 were 3.6, 1.2 and 0.3, respectively. The ten-year overall survival was 74% for osteosarcoma, 76% for Ewing sarcoma and 80% for chondrosarcoma. Fourteen paediatric patients underwent vertebral column resection during 2005-2009. There were no differences between those operated via posterior-only approach (n=7) compared to those operated via antero-posterior approach (n=7) in the mean final radiological corrections or the quality-of-life results. Conclusions: We conclude that the outcomes of treatment of Ewing sarcoma and paediatric bone sarcomas in Finland are comparable to previously reported outcomes. The incidence of Ewing sarcoma in Finland seems to be a bit lower than reported elsewhere in Europe. Posterior-only approach in paediatric vertebral column resections carry comparable results to anteroposterior approach.Tausta: Ewingin sarkooma on harvinainen pahanlaatuinen kasvain. Sekä luun että pehmytkudoksen kasvaimia käsitteleviä, väestöön pohjautuvia tutkimuksia on julkaistu vain vähän. Aiemmat tutkimukset ovat viitanneet siihen, että Ewingin sarkooma olisi Pohjois-Euroopassa harvinaisempi kuin muissa osissa Eurooppaa. Ewingin sarkooma voi harvoin sijaita myös selkärangassa. Kirurgiset tekniikat rangan kasvainten ja vaikeiden epämuodostumien hoidossa ovat kehittyneet ja nykyisin leikkauksia voidaan tehdä yhtä selän puoleista avausta käyttäen. Tavoitteet: Tavoitteemme oli selvittää miten Ewingin sarkooman hoito, erityisesti kirurginen hoito, on Suomessa on toteutunut ja mitkä ovat potilaiden viiden vuoden elossaololuvut. Halusimme myös selvittää lasten ja nuorten luusarkoomien ilmaantuvuusluvut ja 10 vuoden elossaololuvut. Lisäksi tavoitteenamme selvittää lasten nikamanpoistojen tulokset Suomessa. Aineisto ja menetelmät: Tunnistimme kaikki potilaat, joilla oli diagnosoitu Suomessa vuosina Ewingin sarkooma 1990-2009 ja kaikki alle 18-vuotiaat, joilla oli diagnosoitu luusarkooma vuosina 1991-2005 ja kaikki alle 18-vuotiaat, joille oli 2005-2009 tehty nikamanpoistoleikkaus. Sairaskertomukset käytiin läpi yksityiskohtaisten tietojen saamiseksi ja kerätyt aineistot analysoitiin. Tutkimus on malliltaan takautuva, valtakunnallinen ja väestöön pohjautuva. Tulokset: Lopullisiin analyyseihin otettiin mukaan 76 Ewingin sarkooma sairastanutta potilasta. Viiden vuoden elossaololuvut olivat 70% paikallista tautia sairastaneilla ja 33% levinnyttä tautia sairastaneilla (p=0.001). Potilaista 57:llä primaarikasvain hoidettiin kirurgisesti. Osteosarkooman, Ewingin sarkooman ja kondrosarkooman vuosittaiset ilmaantuvuusluvut miljoonaa alle 18-vuotiasta kohden olivat 3.6, 1.2 ja 0.3. 10 vuoden elossaololuvut olivat vastaavasti 74%, 76% ja 80%. Suomessa tehtiin 2005-2009 nikamanpoistoleikkaus 14:lle alle 18-vuotiaalle potilaalle. Verrattaessa sekä selän- että etupuolelta suoritettuja toimenpiteitä (n=7) niihin, jotka suoritettiin pelkästä selän puoleisesta viillosta (n=7), eroja ei ollut radiologisen virheasennon korjautumisen tai elämänlaatua mittaavien kyselytulosten suhteen. Yhteenveto: Tutkimuksen perusteella voidaan todeta, että Ewingin sarkooman ja lasten luusarkoomien hoito Suomessa on tuloksiltaan hyvää kansainvälistä tasoa. Ewingin sarkooman ilmaantuvuus Suomessa alle 18-vuotiailla näyttäisi hieman pienemmältä kuin mitä muualla Euroopassa on raportoitu. Pelkästä selän puoleisesta viillosta suoritettujen nikamanpoistojen tulokset ovat hyvät, eivätkä eroa sekä selän- että etupuolelta tehtyjen toimenpiteiden tuloksista

SELNET clinical practice guidelines for bone sarcoma

Bone sarcoma; Clinical guidelines; Latin-America contextSarcoma óseo; Guías clínicas; Contexto latinoamericanoSarcoma ossi; Guies clíniques; Context llatinoamericàBone sarcoma are infrequent diseases, representing < 0.2% of all adult neoplasms. A multidisciplinary management within reference centers for sarcoma, with discussion of the diagnostic and therapeutic strategies within an expert multidisciplinary tumour board, is essential for these patients, given its heterogeneity and low frequency. This approach leads to an improvement in patient’s outcome, as demonstrated in several studies. The Sarcoma European Latin-American Network (SELNET), aims to improve clinical outcome in sarcoma care, with a special focus in Latin-American countries. These Clinical Practice Guidelines (CPG) have been developed and agreed by a multidisciplinary expert group (including medical and radiation oncologist, surgical oncologist, orthopaedic surgeons, radiologist, pathologist, molecular biologist and representatives of patients advocacy groups) of the SELNET consortium, and are conceived to provide the standard approach to diagnosis, treatment and follow-up of bone sarcoma patients in the Latin-American context.The authors would like to thank the SELNET project, which has received funding from the EU Horizon 2020 research and innovation programme (grant number 825806)

SELNET clinical practice guidelines for bone sarcoma

Bone sarcoma are infrequent diseases, representing < 0.2% of all adult neoplasms. A multidisciplinary management within reference centers for sarcoma, with discussion of the diagnostic and therapeutic strategies within an expert multidisciplinary tumour board, is essential for these patients, given its heterogeneity and low frequency. This approach leads to an improvement in patient's outcome, as demonstrated in several studies. The Sarcoma European Latin-American Network (SELNET), aims to improve clinical outcome in sarcoma care, with a special focus in Latin-American countries. These Clinical Practice Guidelines (CPG) have been developed and agreed by a multidisciplinary expert group (including medical and radiation oncologist, surgical oncologist, orthopaedic surgeons, radiologist, pathologist, molecular biologist and representatives of patients advocacy groups) of the SELNET consortium, and are conceived to provide the standard approach to diagnosis, treatment and follow-up of bone sarcoma patients in the Latin-American context

Novel therapeutic strategies for spinal osteosarcomas

At the dawn of the third millennium, cancer has become the bane of twenty-first century man, and remains a predominant public health burden, affecting welfare and life expectancy globally. Spinal osteogenic sarcoma, a primary spinal malignant tumor, is a rare and challenging neoplastic disease to treat. After the conventional therapeutic modalities of chemotherapy, radiation and surgery have been exhausted, there is currently no available alternative therapy in managing cases of spinal osteosarcoma. The defining signatures of tumor survival are characterised by cancer cell ability to stonewall immunogenic attrition and apoptosis by various means. Some of these biomarkers, namely immune-checkpoints, have recently been exploited as druggable targets in osteosarcoma and many other different cancers. These promising strides made by the use of reinvigorated immunotherapeutic approaches may lead to significant reduction in spinal osteosarcoma disease burden and corresponding reciprocity in increase of survival rates. In this review, we provide the background to spinal osteosarcoma, and proceed to elaborate on contribution of the complex ecology within tumor microenvironment giving arise to cancerous immune escape, which is currently receiving considerable attention. We follow this section on the tumor microenvironment by a brief history of cancer immunity. Also, we draw on the current knowledge of treatment gained from incidences of osteosarcoma at other locations of the skeleton (long bones of the extremities in close proximity to the metaphyseal growth plates) to make a case for application of immunity-based tools, such as immune-checkpoint inhibitors and vaccines, and draw attention to adverse upshots of immune-checkpoint blockers as well. Finally, we describe the novel biotechnique of CRISPR/Cas9 that will assist in treatment approaches for personalized medication.This work is funded by a grant (MPP 320133) from the American University of Beirut to Dr. Ali H. Eid

Treatment of pelvic chondrosarcomas: prognostic factors, reconstructive surgical techniques, oncologic and functional outcomes

Introduzione: Scopo dello studio è stato analizzare i risultati oncologici e funzionali di un’ampia casistica multicentrica di pazienti trattati chirurgicamente per condorsarcoma (CS) della pelvi. Materiali e metodi: La casistica comprende 309 pazienti trattati nel periodo 1975-2013: 193 uomini (63%) e 116 donne (37%) con un età media di 46,4 anni (15-81 anni). I CS erano centrali in 136 casi (34 grado 1; 83 grado 2; 19 grado 3), periferici in 109 (60 grado 1; 47 grado 2; 2 grado 3), dedifferenziati in 36, a cellule chiare in 4, mesenchimali in 2 e periostei in 3, mentre in 19 casi non era nota la variante istologica. Il tumore aveva una localizzazione di tipo I in 74 casi, I-IV in 13, I-II in 39, II-III in 57, III in 35, II in 42 e I-II-III in 49 casi. Un’amputazione interileoaddominale è stata effettuata in 49 pazienti (16%), mentre 260 (84%) sono stati trattati conservativamente: 144 resezioni senza ricostruzione e 116 resezioni con ricostruzione. Margini ampi in 212 casi, ampi contaminati in 23, marginali in 50 ed intralesionali in 24 casi. Risultati: La sopravvivenza a 10 e 15 anni è stata rispettivamente del 73% e 70%. Ad un follow-up medio di 9 anni (range 2-32 anni), 188 pazienti (61%) risultavano continuativamente liberi da malattia, 28 liberi dopo trattamento di recidiva locale (9%), 54 (17%) deceduti per malattia, 13 (4%) deceduti per altre cause e 26 (8%) vivi con malattia. L’alto grado istologico ed il sottotipo dedifferenziato correlano significativamente con peggior prognosi (p<0,0001). All’analisi multivariata sulla sopravvivenza, stadio e grado influenzano significativamente la prognosi. L’incidenza di recidiva locale è stata del 27,8% (86 pazienti). Conclusioni: La chirurgia conservativa è il trattamento “gold standard”. La localizzazione acetabolare pone importanti difficoltà tecniche per la ricostruzione. C’è una correlazione significativa tra grado tumorale e sopravvivenza.Introduction. Aim of this study was to review the long-term oncologic and functional outcome of surgical management in a large series of patients with pelvic chondrosarcoma (CS). Material and Methods. We analyzed 309 patients treated between 1975-2013: 193 males (63%) and 116 females (37%) with a mean age of 46.4 years (range, 15-81 years). There were 136 central CSs (34, 83 and 19 cases grade 1, 2 and 3 respectively), 109 peripheral CSs (60, 47 and 2 cases grade 1, 2 and 3), 36 dedifferentiated CSs, 4 clear cell CSs, 2 mesenchymal CSs and 3 periosteal CSs, 19 otherwise defined CSs. Tumor site was classified as type I in 74 cases, I-IV in 13, I-II in 39, II-III in 57, III in 35, II in 42 and I-II-III in 49 cases. Forty-nine patients had an external hemipelvectomy (16%), whereas 260 patients (84%) underwent a limb-salvage procedure: 144 resections without reconstruction and 116 resections with reconstruction. Margins were wide in 212 cases, wide but contaminated in 23, marginal in 50 and intralesional in 24 cases. Results. Survival on Kaplan Meier curve was 73% and 70% at 10 and 15 years respectively. At a mean of 9 years (1 to 32 years), 188 patients (61%) were continuously NED, 28 were NED after treatment of local recurrence (9%), 54 (17%) DWD, 13 (4%) died of other causes and 26 (8%) AWD. In central and peripheral CSs, high-grade tumors correlated with worse survival. Dedifferentiated CS had a significantly worst prognosis (p<0.0001). At multivariate analysis on survival, stage and grade statistically influenced prognosis. Overall incidence of local recurrence was 27.8% (86 patients). Conclusions. Surgery is the mainstay of treatment for pelvic CS. CSs with acetabular involvement offer challenging technical problems to reliable and lasting reconstruction. There was a significant correlation between histologic grade and survival