Andreas Krieg - Academia.edu (original) (raw)
Papers by Andreas Krieg
Sarcoma, 2013
We analyze the delay in diagnosis and tumor size of malignant bone tumors of the foot in a retros... more We analyze the delay in diagnosis and tumor size of malignant bone tumors of the foot in a retrospective study. We compared the oncological and surgical long-term results with identical tumor at other anatomical sites in order to analyze the biological behavior of sarcomas that are found in the foot. Thirty-two patients with a histologically proven malignant bone tumor (fifteen chondrosarcomas, nine osteosarcomas, and eight Ewing sarcomas) between the years 1969 and 2008 were included. The median follow-up was 11.9 years. The overall median time gap between the beginning of symptoms and diagnosis in the study group was 10 months. Ewing sarcoma presented with the longest delay in diagnosis (median of 18 months), followed by osteosarcoma (median of 15 months) and chondrosarcoma (median of 7.5 months). The delay in diagnosis of these tumors was significantly longer than that of equivalent tumors at other skeletal sites, but the 5-and 10-year survival rates and the occurrence of distant metastases were comparable. In contrast, the average size of foot tumors was 5-to 30-fold less than that of tumors analyzed at other skeletal sites. This study indicates that sarcomas of the foot demonstrate a distinct biological behavior compared to the same tumor types at other skeletal sites.
Acta Orthopaedica, May 11, 2011
Background and purpose In the last decade, intramedullary limb lengthening has become a viable al... more Background and purpose In the last decade, intramedullary limb lengthening has become a viable alternative to traditional external systems. We retrospectively analyzed the use of an intramedullary motorized nail (Fitbone) in a consecutive series of 32 patients. Patients and methods During the period September 2006 to December 2008, 32 consecutive patients with a median age of 17 (IQR: 15-19) years were treated with a fully implantable, motorized intramedullary lengthening device (Fitbone). The median leg length discrepancy was 35 (IQR: 30-44) mm at the femur (n = 21) and 28 (IQR: 25-30) mm at the tibia (n = 11). Results Leg lengthening was successful in 30 of 32 cases, with no residual relevant discrepancy (± 5 mm). No intraoperative complications were observed. The consolidation index was significantly different (p = 0.04) between femoral lengthening (mean 35 days/cm) and tibial lengthening (mean 48 days/cm) but did not depend on age older/younger than 16 or previous operations at the affected site. 3 problems, 3 obstacles, and 4 complications (3 minor, 1 major) were encountered in 8 patients, 5 of which were implant-associated. Interpretation This technique even allows correction in patients with multiplanar deformities. Compared to external devices, intramedullary systems provide comfort and reduce complication rates, give improved cosmetic results, and lead to fast rehabilitation since percutaneous, transmuscular fixation is prevented. This results in reasonable overall treatment costs despite the relatively high costs of implants.
Journal of Clinical Medicine
Congenital pseudarthrosis of forearm fractures is rare and is strongly associated with neurofibro... more Congenital pseudarthrosis of forearm fractures is rare and is strongly associated with neurofibromatosis type 1 (NF1). Our case report illustrates the progression of a non-union of the ulna after minor trauma in a twelve-year-old boy, newly diagnosed with NF1, and presents the technique of microsurgical bone reconstruction, including the growth plate. More than seven years after the first operation, follow-up presents a favorable outcome with a pain-free patient and unrestricted function of the forearm after a secondary correction of the remaining radial bowing. This treatment is discussed with a comprehensive review of the current literature on ulnar congenital pseudarthrosis in PubMed and Google Scholar and free fibular growth plate transfer in PubMed and Google Scholar. Nine publications reporting on 20 cases of congenital ulnar non-unions were identified. With this reconstructive option, favorable outcomes were achieved in all cases with the union after primary surgery and compl...
Acta chirurgiae orthopaedicae et traumatologiae Cechoslovaca
2008 as current state of research. All of these studies evaluated the efficacy of new chemotherap... more 2008 as current state of research. All of these studies evaluated the efficacy of new chemotherapeutic strategies in comparison to the best available standard therapy (16, 17). Prognostic factors are still under a partially controversial debate. Metastatic disease at diagnosis and the response to neo-adjuvant and adjuvant chemotherapy are significant indicators for the survival rate as reported in various multicenter studies (15, 16). However, age and site of the tumor did not prove to be independent prognostic factors (3, 5, 23). Tumor volume, has been shown to be prognostic factor in some studies (1, 8, 16, 22) but evidence is not consistent for all data (3, 14). Other factors such as lactate dehydrogenase (LDH) levels in
Anticancer Research, Nov 1, 2022
Modern Pathology, 2022
Rearrangements of the transcription factors FOS and FOSB have recently been identified as the gen... more Rearrangements of the transcription factors FOS and FOSB have recently been identified as the genetic driver event underlying osteoid osteoma and osteoblastoma. Nuclear overexpression of FOS and FOSB have since then emerged as a reliable surrogate marker despite limitations in specificity and sensitivity. Indeed, osteosarcoma can infrequently show nuclear FOS expression and a small fraction of osteoblastomas seem to arise independent of FOS/FOSB rearrangements. Acid decalcification and tissue preservation are additional factors that can negatively influence immunohistochemical testing and make diagnostic decision-making challenging in individual cases. Particularly aggressive appearing osteoblastomas, also referred to as epithelioid osteoblastomas, and osteoblastoma-like osteosarcoma can be difficult to distinguish, underlining the need for additional markers to support the diagnosis. Methylation and copy number profiling, a technique well established for the classification of brain...
Journal of Orthopaedic Case Reports, 2020
Introduction Osteomyelitis with multiresistant bacteria in non-union following fracture treated w... more Introduction Osteomyelitis with multiresistant bacteria in non-union following fracture treated with osteosynthesis requires complete removal of infected sequestrum and dead bone. For consecutive bone defects, it is frequently necessary to bridge with a fixator external. The treatment is not only challenging due to reduced bone stock but also characterized by decreased bioavailability of antibiotics. Case Report We report a two-step-surgery approach to preserve the bone stock using autologous cancellous bone in a bacterial infected non-union for subsequently leg length reconstruction. The 24-year-old male patient from Belarus was admitted to our department with persistent wound secretion and subsequent osteomyelitis of the right femur 3 years after initial surgery, several revisions, and several different antibiotic therapies. Biopsy revealed methicillin-resistant and borderline oxacillin-resistant Staphylococcus aureus. Firstly, the Ilizarov ring fixator was removed and a vigorous ...
Journal of Children's Orthopaedics, 2020
Introduction Simple bone cysts (SBCs) are common in children and adolescents. The risk of refract... more Introduction Simple bone cysts (SBCs) are common in children and adolescents. The risk of refracture and the probability of spontaneous healing in SBCs are mainly dependent on the activity of the cyst and can be quantified with the Cyst-Index. Avoiding pathological fractures is the primary goal. Our study presents a comparison of two different bioresorbable bone graft substitutes (BGSs) in the minimally-invasive treatment of SBC in the active stage by percutaneous cyst aspiration and injection. Methods Between 2006 and 2017, 38 patients (aged two to 37 years; mean age 12.4 (sd 5.6)) were treated with percutaneous cyst aspiration and refilled with bioresorbable BGSs in three hospitals. The cysts of 21 patients (11 humerus, five femur, four calcaneus, one fibula) were refilled with porous beta-tricalcium phosphate (PB-TP group) (ChronOS Inject) and of 17 patients (nine humerus, six femur, one calcaneus, one fibula) with hydroxyapatite/calcium sulphate (H/CS group) (CERAMENT|BONE VOID ...
Swiss Medical Weekly, 2019
PURPOSE: Extra-abdominal desmoid tumours are benign and rare, and lead to a persistent treatment ... more PURPOSE: Extra-abdominal desmoid tumours are benign and rare, and lead to a persistent treatment dilemma because of their high recurrence rate and their heterogeneous behaviour. The goal of this retrospective study was to evaluate the results of different treatment modalities for extra-abdominal desmoid tumours at four sarcoma treatment centres. METHODS: The mean follow-up time for the 96 patients included in the study (63.5% female; mean age 38.9 years) was 8.4 years (2.0-40.5 years). The initial treatments were surgery (n = 44), surgery with radiation (n = 16), watchful waiting (n = 15), radiation only (n = 9), or systemic treatment (n = 12). Patient demographics, tumour sites, and the follow-up status of all patients were reviewed and evaluated for each of the treatment modalities. RESULTS: The local recurrence rate was 45.5% in patients with primary surgical treatment and 37.5% following surgery combined with irradiation. Patients who were treated with radiation alone showed regressive (33.3%) or stable disease (66.6%). Systemic treatment alone resulted in disease progression in 41.7% of our patients. In the watchful waiting group, 73.3% showed stable disease, 20.0% showed spontaneous regression, and 6.7% showed progression after a mean follow-up of 4.1 years (2.0-11.5 years). CONCLUSIONS: Our results suggest that a watchful waiting approach should be the first line treatment in asymptomatic desmoid tumours. However, radiation can help improve local control rates in patients who have undergone surgery. Progression and local recurrence rates following systemic treatment were comparable to those observed in surgery combined with radiation.
Journal of Children's Orthopaedics, 2018
Purpose Intramedullary lengthening nails are an accepted alternative to external fixators but are... more Purpose Intramedullary lengthening nails are an accepted alternative to external fixators but are limited by anatomical preconditions. Therefore, to date the use of external fixators is sometimes inevitable. We report on a new technique for correction of combined limb length discrepancies and complex axis deformities using solely internal devices - a lengthening nail and a locking plate. Methods Between October 2008 and November 2011 five patients (two femora, three tibias) with a mean leg length discrepancy of 36 mm (25 to 50) and a complex angular deformity were treated with a fully implantable motorized lengthening nail (Fitbone) and a locking plate. All patients were evaluated with regards to the pre- and postoperative leg length as well as axis alignment, functional outcome, lengthening indices and complications. Results A successful leg length equalization was achieved in all cases and physiological joint orientation angles in all but one case. The mean distraction index was 1...
Swiss Medical Weekly, 2019
Nature Communications, 2015
Osteosarcomas are aggressive bone tumours with a high degree of genetic heterogeneity, which has ... more Osteosarcomas are aggressive bone tumours with a high degree of genetic heterogeneity, which has historically complicated driver gene discovery. Here we sequence exomes of 31 tumours and decipher their evolutionary landscape by inferring clonality of the individual mutation events. Exome findings are interpreted in the context of mutation and SNP array data from a replication set of 92 tumours. We identify 14 genes as the main drivers, of which some were formerly unknown in the context of osteosarcoma. None of the drivers is clearly responsible for the majority of tumours and even TP53 mutations are frequently mapped into subclones. However, >80% of osteosarcomas exhibit a specific combination of single-base substitutions, LOH, or large-scale genome instability signatures characteristic of BRCA1/2-deficient tumours. Our findings imply that multiple oncogenic pathways drive chromosomal instability during osteosarcoma evolution and result in the acquisition of BRCA-like traits, whi...
Sarcoma, 2013
We analyze the delay in diagnosis and tumor size of malignant bone tumors of the foot in a retros... more We analyze the delay in diagnosis and tumor size of malignant bone tumors of the foot in a retrospective study. We compared the oncological and surgical long-term results with identical tumor at other anatomical sites in order to analyze the biological behavior of sarcomas that are found in the foot. Thirty-two patients with a histologically proven malignant bone tumor (fifteen chondrosarcomas, nine osteosarcomas, and eight Ewing sarcomas) between the years 1969 and 2008 were included. The median follow-up was 11.9 years. The overall median time gap between the beginning of symptoms and diagnosis in the study group was 10 months. Ewing sarcoma presented with the longest delay in diagnosis (median of 18 months), followed by osteosarcoma (median of 15 months) and chondrosarcoma (median of 7.5 months). The delay in diagnosis of these tumors was significantly longer than that of equivalent tumors at other skeletal sites, but the 5- and 10-year survival rates and the occurrence of distan...
Journal of Children's Orthopaedics, 2012
Any lower limb discrepancy may be equalised by conservative means (insoles, prosthesis and orthos... more Any lower limb discrepancy may be equalised by conservative means (insoles, prosthesis and orthosis). However, their long-term acceptance is low in regard to function, costs, expenditure and appearance. Timely epiphysiodesis is the best option in uniplanar deformities with adequate remaining growth and for patients whose predicted final body height is above the 50th percentile. However, many patients present late or with multi-planar deformities, which warrant more sophisticated operative approaches. The history of surgical bone lengthening comprises 100 exciting years of struggling, development and ongoing learning. The initial strategy of acute or rapid incremental distraction had lasted almost half a century until Ilizarov recognised the benefits of biological periosteum-preserving osteotomies and incremental lengthening at slow rates (1 mm/day) at a 4 × 0.25-mm daily rhythm, well appreciated as callotasis. In parallel, ring and wire constructs made complex three-dimensional axia...
Journal of Children's Orthopaedics, 2011
Purpose Synovial sarcoma (SS) is an aggressive soft-tissue tumor noted for late local recurrence ... more Purpose Synovial sarcoma (SS) is an aggressive soft-tissue tumor noted for late local recurrence and metastasis. This study investigates the long-term outcome of SS in patients of pediatric age and evaluates potential prognostic factors for SS. Methods We performed a retrospective review of 13 SS cases in patients younger than 20 years at the time of diagnosis who had a minimum follow-up of 10 years. The mean follow-up for living patients ( n = 8) was 20.1 years (12.1–27.6) and for nonsurvivors ( n = 5) 4.9 years (range: 2.6–9.3). Nine patients had unplanned excisions (69%), of which 6 (67%) were performed prior to their referral. Re-excisions were necessary in all 13 patients. The factors sex, tumor site, tumor size, tumor grade, histological subtype, fusion type, and type of treatment were evaluated for their prognostic value. Results Only 2 patients (15%) met the criteria of adequate tumor treatment. Overall, the 5- and 10-year survival rates were 77 and 61%, respectively. The me...
The Journal of Bone and Joint Surgery. British volume, 2007
We evaluated 31 patients who were treated with a non-vascularised fibular graft after resection o... more We evaluated 31 patients who were treated with a non-vascularised fibular graft after resection of primary musculoskeletal tumours, with a median follow-up of 5.6 years (3 to 26.7 years). Primary union was achieved in 89% (41 of 46) of the grafts in a median period of 24 weeks. All 25 grafts in 18 patients without additional chemotheraphy and/or radiotherapy achieved primary union, compared with 16 of the 21 grafts (76%; 13 patients) with additional therapy (p = 0.017). Radiographs showed an increase in diameter in 70% (59) of the grafts. There were seven fatigue fractures in six patients, but only two needed treatment. Non-vascularised fibular transfer is a simpler, less expensive and a shorter procedure than the use of vascularised grafts and allows remodelling of the fibula at the donor site. It is a biological reconstruction with good long-term results, and a relatively low donor site complication rate of 16%.
The Journal of Bone and Joint Surgery. British volume, 2010
We retrospectively evaluated 18 patients with a mean age of 37.3 years (14 to 72) who had undergo... more We retrospectively evaluated 18 patients with a mean age of 37.3 years (14 to 72) who had undergone pelvic reconstruction stabilised with a non-vascularised fibular graft after resection of a primary bone tumour. The mean follow-up was 10.14 years (2.4 to 15.7). The mean Musculoskeletal Tumor Society Score was 76.5% (50% to 100%). Primary union was achieved in the majority of reconstructions within a mean of 22.9 weeks (7 to 60.6). The three patients with delayed or nonunion all received additional therapy (chemotherapy/radiation) (p = 0.0162). The complication rate was comparable to that of other techniques described in the literature. Non-vascularised fibular transfer to the pelvis is a simpler, cheaper and quicker procedure than other currently described techniques. It is a biological reconstruction with good results and a relatively low donor site complication rate. However, adjuvant therapy can negatively affect the outcome of such grafts.
Annals of Oncology, 2010
Background: Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis becaus... more Background: Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis because of late local recurrence and distant metastases. To our knowledge, no studies have minimum follow-up of 10 years that evaluate long-term outcomes for survivors. Patients and methods: Data on 62 patients who had been treated for SS from 1968 to 1999 were studied retrospectively in a multicenter study. Mean follow-up of living patients was 17.2 years and of dead patients 7.7 years. Results: Mean age at diagnosis was 35.4 years (range 6-82 years). Overall survival was 38.7%. The 5-year survival was 74.2%; 10-year survival was 61.2%; and 15-year survival was 46.5%. Fifteen patients (24%) died of disease after 10 years of follow-up. Local recurrence occurred after a mean of 3.6 years (range 0.5-14.9 years) and metastases at a mean of 5.7 years (range 0.5-16.3 years). Only four patients were treated technically correctly with a planned biopsy followed by a wide resection or amputation. Factors associated with significantly worse prognosis included larger tumor size, metastases at the time of diagnosis, high-grade histology, trunk-related disease, and lack of wide resection as primary surgical treatment. Conclusions: In SS, metastases develop late with high mortality. Patients with SS should be followed for >10 years.
BMC musculoskeletal disorders, Jan 5, 2017
The reconstruction of meta-/diaphyseal bone defects following bone tumour resection is challengin... more The reconstruction of meta-/diaphyseal bone defects following bone tumour resection is challenging, and biological treatment options should be applied whenever possible, especially in benign lesions and early stage sarcomas. We aimed to evaluate the results of segmental (SR) and hemicortical reconstructions (HR) at the extremities using non-vascularised fibula grafts. We retrospectively enrolled 36 patients who were treated with non-vascularised fibula reconstructions (15 SR, 21 HR) after bone tumour resection (15 malignant, 21 benign). All cases were evaluated regarding consolidation, hypertrophy at the graft-host junctions, and complications; moreover, the functional and oncological results were assessed. The mean follow-up was 8.3 years (2.1-26.6 years). Primary union was achieved in 94% (SR 87%, HR 100%) of patients, and 85% (SR 81%, HR 88%) showed hypertrophy at the graft-host junction. The overall complication rate was 36% with 4 patients (11%) developing local recurrence. The...
Sarcoma, 2013
We analyze the delay in diagnosis and tumor size of malignant bone tumors of the foot in a retros... more We analyze the delay in diagnosis and tumor size of malignant bone tumors of the foot in a retrospective study. We compared the oncological and surgical long-term results with identical tumor at other anatomical sites in order to analyze the biological behavior of sarcomas that are found in the foot. Thirty-two patients with a histologically proven malignant bone tumor (fifteen chondrosarcomas, nine osteosarcomas, and eight Ewing sarcomas) between the years 1969 and 2008 were included. The median follow-up was 11.9 years. The overall median time gap between the beginning of symptoms and diagnosis in the study group was 10 months. Ewing sarcoma presented with the longest delay in diagnosis (median of 18 months), followed by osteosarcoma (median of 15 months) and chondrosarcoma (median of 7.5 months). The delay in diagnosis of these tumors was significantly longer than that of equivalent tumors at other skeletal sites, but the 5-and 10-year survival rates and the occurrence of distant metastases were comparable. In contrast, the average size of foot tumors was 5-to 30-fold less than that of tumors analyzed at other skeletal sites. This study indicates that sarcomas of the foot demonstrate a distinct biological behavior compared to the same tumor types at other skeletal sites.
Acta Orthopaedica, May 11, 2011
Background and purpose In the last decade, intramedullary limb lengthening has become a viable al... more Background and purpose In the last decade, intramedullary limb lengthening has become a viable alternative to traditional external systems. We retrospectively analyzed the use of an intramedullary motorized nail (Fitbone) in a consecutive series of 32 patients. Patients and methods During the period September 2006 to December 2008, 32 consecutive patients with a median age of 17 (IQR: 15-19) years were treated with a fully implantable, motorized intramedullary lengthening device (Fitbone). The median leg length discrepancy was 35 (IQR: 30-44) mm at the femur (n = 21) and 28 (IQR: 25-30) mm at the tibia (n = 11). Results Leg lengthening was successful in 30 of 32 cases, with no residual relevant discrepancy (± 5 mm). No intraoperative complications were observed. The consolidation index was significantly different (p = 0.04) between femoral lengthening (mean 35 days/cm) and tibial lengthening (mean 48 days/cm) but did not depend on age older/younger than 16 or previous operations at the affected site. 3 problems, 3 obstacles, and 4 complications (3 minor, 1 major) were encountered in 8 patients, 5 of which were implant-associated. Interpretation This technique even allows correction in patients with multiplanar deformities. Compared to external devices, intramedullary systems provide comfort and reduce complication rates, give improved cosmetic results, and lead to fast rehabilitation since percutaneous, transmuscular fixation is prevented. This results in reasonable overall treatment costs despite the relatively high costs of implants.
Journal of Clinical Medicine
Congenital pseudarthrosis of forearm fractures is rare and is strongly associated with neurofibro... more Congenital pseudarthrosis of forearm fractures is rare and is strongly associated with neurofibromatosis type 1 (NF1). Our case report illustrates the progression of a non-union of the ulna after minor trauma in a twelve-year-old boy, newly diagnosed with NF1, and presents the technique of microsurgical bone reconstruction, including the growth plate. More than seven years after the first operation, follow-up presents a favorable outcome with a pain-free patient and unrestricted function of the forearm after a secondary correction of the remaining radial bowing. This treatment is discussed with a comprehensive review of the current literature on ulnar congenital pseudarthrosis in PubMed and Google Scholar and free fibular growth plate transfer in PubMed and Google Scholar. Nine publications reporting on 20 cases of congenital ulnar non-unions were identified. With this reconstructive option, favorable outcomes were achieved in all cases with the union after primary surgery and compl...
Acta chirurgiae orthopaedicae et traumatologiae Cechoslovaca
2008 as current state of research. All of these studies evaluated the efficacy of new chemotherap... more 2008 as current state of research. All of these studies evaluated the efficacy of new chemotherapeutic strategies in comparison to the best available standard therapy (16, 17). Prognostic factors are still under a partially controversial debate. Metastatic disease at diagnosis and the response to neo-adjuvant and adjuvant chemotherapy are significant indicators for the survival rate as reported in various multicenter studies (15, 16). However, age and site of the tumor did not prove to be independent prognostic factors (3, 5, 23). Tumor volume, has been shown to be prognostic factor in some studies (1, 8, 16, 22) but evidence is not consistent for all data (3, 14). Other factors such as lactate dehydrogenase (LDH) levels in
Anticancer Research, Nov 1, 2022
Modern Pathology, 2022
Rearrangements of the transcription factors FOS and FOSB have recently been identified as the gen... more Rearrangements of the transcription factors FOS and FOSB have recently been identified as the genetic driver event underlying osteoid osteoma and osteoblastoma. Nuclear overexpression of FOS and FOSB have since then emerged as a reliable surrogate marker despite limitations in specificity and sensitivity. Indeed, osteosarcoma can infrequently show nuclear FOS expression and a small fraction of osteoblastomas seem to arise independent of FOS/FOSB rearrangements. Acid decalcification and tissue preservation are additional factors that can negatively influence immunohistochemical testing and make diagnostic decision-making challenging in individual cases. Particularly aggressive appearing osteoblastomas, also referred to as epithelioid osteoblastomas, and osteoblastoma-like osteosarcoma can be difficult to distinguish, underlining the need for additional markers to support the diagnosis. Methylation and copy number profiling, a technique well established for the classification of brain...
Journal of Orthopaedic Case Reports, 2020
Introduction Osteomyelitis with multiresistant bacteria in non-union following fracture treated w... more Introduction Osteomyelitis with multiresistant bacteria in non-union following fracture treated with osteosynthesis requires complete removal of infected sequestrum and dead bone. For consecutive bone defects, it is frequently necessary to bridge with a fixator external. The treatment is not only challenging due to reduced bone stock but also characterized by decreased bioavailability of antibiotics. Case Report We report a two-step-surgery approach to preserve the bone stock using autologous cancellous bone in a bacterial infected non-union for subsequently leg length reconstruction. The 24-year-old male patient from Belarus was admitted to our department with persistent wound secretion and subsequent osteomyelitis of the right femur 3 years after initial surgery, several revisions, and several different antibiotic therapies. Biopsy revealed methicillin-resistant and borderline oxacillin-resistant Staphylococcus aureus. Firstly, the Ilizarov ring fixator was removed and a vigorous ...
Journal of Children's Orthopaedics, 2020
Introduction Simple bone cysts (SBCs) are common in children and adolescents. The risk of refract... more Introduction Simple bone cysts (SBCs) are common in children and adolescents. The risk of refracture and the probability of spontaneous healing in SBCs are mainly dependent on the activity of the cyst and can be quantified with the Cyst-Index. Avoiding pathological fractures is the primary goal. Our study presents a comparison of two different bioresorbable bone graft substitutes (BGSs) in the minimally-invasive treatment of SBC in the active stage by percutaneous cyst aspiration and injection. Methods Between 2006 and 2017, 38 patients (aged two to 37 years; mean age 12.4 (sd 5.6)) were treated with percutaneous cyst aspiration and refilled with bioresorbable BGSs in three hospitals. The cysts of 21 patients (11 humerus, five femur, four calcaneus, one fibula) were refilled with porous beta-tricalcium phosphate (PB-TP group) (ChronOS Inject) and of 17 patients (nine humerus, six femur, one calcaneus, one fibula) with hydroxyapatite/calcium sulphate (H/CS group) (CERAMENT|BONE VOID ...
Swiss Medical Weekly, 2019
PURPOSE: Extra-abdominal desmoid tumours are benign and rare, and lead to a persistent treatment ... more PURPOSE: Extra-abdominal desmoid tumours are benign and rare, and lead to a persistent treatment dilemma because of their high recurrence rate and their heterogeneous behaviour. The goal of this retrospective study was to evaluate the results of different treatment modalities for extra-abdominal desmoid tumours at four sarcoma treatment centres. METHODS: The mean follow-up time for the 96 patients included in the study (63.5% female; mean age 38.9 years) was 8.4 years (2.0-40.5 years). The initial treatments were surgery (n = 44), surgery with radiation (n = 16), watchful waiting (n = 15), radiation only (n = 9), or systemic treatment (n = 12). Patient demographics, tumour sites, and the follow-up status of all patients were reviewed and evaluated for each of the treatment modalities. RESULTS: The local recurrence rate was 45.5% in patients with primary surgical treatment and 37.5% following surgery combined with irradiation. Patients who were treated with radiation alone showed regressive (33.3%) or stable disease (66.6%). Systemic treatment alone resulted in disease progression in 41.7% of our patients. In the watchful waiting group, 73.3% showed stable disease, 20.0% showed spontaneous regression, and 6.7% showed progression after a mean follow-up of 4.1 years (2.0-11.5 years). CONCLUSIONS: Our results suggest that a watchful waiting approach should be the first line treatment in asymptomatic desmoid tumours. However, radiation can help improve local control rates in patients who have undergone surgery. Progression and local recurrence rates following systemic treatment were comparable to those observed in surgery combined with radiation.
Journal of Children's Orthopaedics, 2018
Purpose Intramedullary lengthening nails are an accepted alternative to external fixators but are... more Purpose Intramedullary lengthening nails are an accepted alternative to external fixators but are limited by anatomical preconditions. Therefore, to date the use of external fixators is sometimes inevitable. We report on a new technique for correction of combined limb length discrepancies and complex axis deformities using solely internal devices - a lengthening nail and a locking plate. Methods Between October 2008 and November 2011 five patients (two femora, three tibias) with a mean leg length discrepancy of 36 mm (25 to 50) and a complex angular deformity were treated with a fully implantable motorized lengthening nail (Fitbone) and a locking plate. All patients were evaluated with regards to the pre- and postoperative leg length as well as axis alignment, functional outcome, lengthening indices and complications. Results A successful leg length equalization was achieved in all cases and physiological joint orientation angles in all but one case. The mean distraction index was 1...
Swiss Medical Weekly, 2019
Nature Communications, 2015
Osteosarcomas are aggressive bone tumours with a high degree of genetic heterogeneity, which has ... more Osteosarcomas are aggressive bone tumours with a high degree of genetic heterogeneity, which has historically complicated driver gene discovery. Here we sequence exomes of 31 tumours and decipher their evolutionary landscape by inferring clonality of the individual mutation events. Exome findings are interpreted in the context of mutation and SNP array data from a replication set of 92 tumours. We identify 14 genes as the main drivers, of which some were formerly unknown in the context of osteosarcoma. None of the drivers is clearly responsible for the majority of tumours and even TP53 mutations are frequently mapped into subclones. However, >80% of osteosarcomas exhibit a specific combination of single-base substitutions, LOH, or large-scale genome instability signatures characteristic of BRCA1/2-deficient tumours. Our findings imply that multiple oncogenic pathways drive chromosomal instability during osteosarcoma evolution and result in the acquisition of BRCA-like traits, whi...
Sarcoma, 2013
We analyze the delay in diagnosis and tumor size of malignant bone tumors of the foot in a retros... more We analyze the delay in diagnosis and tumor size of malignant bone tumors of the foot in a retrospective study. We compared the oncological and surgical long-term results with identical tumor at other anatomical sites in order to analyze the biological behavior of sarcomas that are found in the foot. Thirty-two patients with a histologically proven malignant bone tumor (fifteen chondrosarcomas, nine osteosarcomas, and eight Ewing sarcomas) between the years 1969 and 2008 were included. The median follow-up was 11.9 years. The overall median time gap between the beginning of symptoms and diagnosis in the study group was 10 months. Ewing sarcoma presented with the longest delay in diagnosis (median of 18 months), followed by osteosarcoma (median of 15 months) and chondrosarcoma (median of 7.5 months). The delay in diagnosis of these tumors was significantly longer than that of equivalent tumors at other skeletal sites, but the 5- and 10-year survival rates and the occurrence of distan...
Journal of Children's Orthopaedics, 2012
Any lower limb discrepancy may be equalised by conservative means (insoles, prosthesis and orthos... more Any lower limb discrepancy may be equalised by conservative means (insoles, prosthesis and orthosis). However, their long-term acceptance is low in regard to function, costs, expenditure and appearance. Timely epiphysiodesis is the best option in uniplanar deformities with adequate remaining growth and for patients whose predicted final body height is above the 50th percentile. However, many patients present late or with multi-planar deformities, which warrant more sophisticated operative approaches. The history of surgical bone lengthening comprises 100 exciting years of struggling, development and ongoing learning. The initial strategy of acute or rapid incremental distraction had lasted almost half a century until Ilizarov recognised the benefits of biological periosteum-preserving osteotomies and incremental lengthening at slow rates (1 mm/day) at a 4 × 0.25-mm daily rhythm, well appreciated as callotasis. In parallel, ring and wire constructs made complex three-dimensional axia...
Journal of Children's Orthopaedics, 2011
Purpose Synovial sarcoma (SS) is an aggressive soft-tissue tumor noted for late local recurrence ... more Purpose Synovial sarcoma (SS) is an aggressive soft-tissue tumor noted for late local recurrence and metastasis. This study investigates the long-term outcome of SS in patients of pediatric age and evaluates potential prognostic factors for SS. Methods We performed a retrospective review of 13 SS cases in patients younger than 20 years at the time of diagnosis who had a minimum follow-up of 10 years. The mean follow-up for living patients ( n = 8) was 20.1 years (12.1–27.6) and for nonsurvivors ( n = 5) 4.9 years (range: 2.6–9.3). Nine patients had unplanned excisions (69%), of which 6 (67%) were performed prior to their referral. Re-excisions were necessary in all 13 patients. The factors sex, tumor site, tumor size, tumor grade, histological subtype, fusion type, and type of treatment were evaluated for their prognostic value. Results Only 2 patients (15%) met the criteria of adequate tumor treatment. Overall, the 5- and 10-year survival rates were 77 and 61%, respectively. The me...
The Journal of Bone and Joint Surgery. British volume, 2007
We evaluated 31 patients who were treated with a non-vascularised fibular graft after resection o... more We evaluated 31 patients who were treated with a non-vascularised fibular graft after resection of primary musculoskeletal tumours, with a median follow-up of 5.6 years (3 to 26.7 years). Primary union was achieved in 89% (41 of 46) of the grafts in a median period of 24 weeks. All 25 grafts in 18 patients without additional chemotheraphy and/or radiotherapy achieved primary union, compared with 16 of the 21 grafts (76%; 13 patients) with additional therapy (p = 0.017). Radiographs showed an increase in diameter in 70% (59) of the grafts. There were seven fatigue fractures in six patients, but only two needed treatment. Non-vascularised fibular transfer is a simpler, less expensive and a shorter procedure than the use of vascularised grafts and allows remodelling of the fibula at the donor site. It is a biological reconstruction with good long-term results, and a relatively low donor site complication rate of 16%.
The Journal of Bone and Joint Surgery. British volume, 2010
We retrospectively evaluated 18 patients with a mean age of 37.3 years (14 to 72) who had undergo... more We retrospectively evaluated 18 patients with a mean age of 37.3 years (14 to 72) who had undergone pelvic reconstruction stabilised with a non-vascularised fibular graft after resection of a primary bone tumour. The mean follow-up was 10.14 years (2.4 to 15.7). The mean Musculoskeletal Tumor Society Score was 76.5% (50% to 100%). Primary union was achieved in the majority of reconstructions within a mean of 22.9 weeks (7 to 60.6). The three patients with delayed or nonunion all received additional therapy (chemotherapy/radiation) (p = 0.0162). The complication rate was comparable to that of other techniques described in the literature. Non-vascularised fibular transfer to the pelvis is a simpler, cheaper and quicker procedure than other currently described techniques. It is a biological reconstruction with good results and a relatively low donor site complication rate. However, adjuvant therapy can negatively affect the outcome of such grafts.
Annals of Oncology, 2010
Background: Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis becaus... more Background: Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis because of late local recurrence and distant metastases. To our knowledge, no studies have minimum follow-up of 10 years that evaluate long-term outcomes for survivors. Patients and methods: Data on 62 patients who had been treated for SS from 1968 to 1999 were studied retrospectively in a multicenter study. Mean follow-up of living patients was 17.2 years and of dead patients 7.7 years. Results: Mean age at diagnosis was 35.4 years (range 6-82 years). Overall survival was 38.7%. The 5-year survival was 74.2%; 10-year survival was 61.2%; and 15-year survival was 46.5%. Fifteen patients (24%) died of disease after 10 years of follow-up. Local recurrence occurred after a mean of 3.6 years (range 0.5-14.9 years) and metastases at a mean of 5.7 years (range 0.5-16.3 years). Only four patients were treated technically correctly with a planned biopsy followed by a wide resection or amputation. Factors associated with significantly worse prognosis included larger tumor size, metastases at the time of diagnosis, high-grade histology, trunk-related disease, and lack of wide resection as primary surgical treatment. Conclusions: In SS, metastases develop late with high mortality. Patients with SS should be followed for >10 years.
BMC musculoskeletal disorders, Jan 5, 2017
The reconstruction of meta-/diaphyseal bone defects following bone tumour resection is challengin... more The reconstruction of meta-/diaphyseal bone defects following bone tumour resection is challenging, and biological treatment options should be applied whenever possible, especially in benign lesions and early stage sarcomas. We aimed to evaluate the results of segmental (SR) and hemicortical reconstructions (HR) at the extremities using non-vascularised fibula grafts. We retrospectively enrolled 36 patients who were treated with non-vascularised fibula reconstructions (15 SR, 21 HR) after bone tumour resection (15 malignant, 21 benign). All cases were evaluated regarding consolidation, hypertrophy at the graft-host junctions, and complications; moreover, the functional and oncological results were assessed. The mean follow-up was 8.3 years (2.1-26.6 years). Primary union was achieved in 94% (SR 87%, HR 100%) of patients, and 85% (SR 81%, HR 88%) showed hypertrophy at the graft-host junction. The overall complication rate was 36% with 4 patients (11%) developing local recurrence. The...