Publications by authors named "Susanna Lang"

40 Publications

Lethal encephalopathy in an infant with hypophosphatasia despite enzyme-replacement therapy.

Horm Res Paediatr 2021 Oct 21. Epub 2021 Oct 21.

Hypophosphatasia (HPP) is an inborn error of metabolism caused by loss‑of‑function mutations in the biomineralization associated alkaline phosphatase (ALPL) gene, encoding tissue-nonspecific alkaline phosphatase (TNSALP). Symptoms include skeletal hypomineralization and extra-skeletal manifestations such as pyridoxine (B6) responsive seizures due to impaired cerebral B6 passage. Since the introduction of enzyme-replacement therapy (ERT) skeletal manifestations and B6-responsive seizures were reported to improve significantly. Nevertheless, there is increasing evidence of B6-independent neurological manifestation of HPP including HPP-associated encephalopathy. Here, we present for the first time the brain alterations of an infant with neonatal HPP who died of neurological complications at the age of 5 months despite early initiation of ERT. CSF analysis showed normal concentrations of biogenic amines reflecting sufficient intracellular B6 availability. Post mortem histopathology revealed severe, localized affection of the cerebral cortex including cortical lesions in layers 2 and 3 in direct proximity to TNSALP-expressing neurons and hippocampal sclerosis. Our findings confirm that TNSALP deficiency may lead to a severe encephalopathy. We hypothesize that HPP-associated encephalopathy resistant to currently available ERT may develop in addition and probably independently of typical B6-responsive seizures in some patients. Prospective, controlled studies with close neurological follow-up including brain imaging are needed to identify patients at risk for severe neurological symptoms despite ERT.
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http://dx.doi.org/10.1159/000520341DOI Listing
October 2021

Avian extremity reconstruction via osseointegrated leg-prosthesis for intuitive embodiment.

Sci Rep 2021 06 11;11(1):12360. Epub 2021 Jun 11.

Clinical Laboratory for Bionic Extremity Reconstruction, Department of Surgery, Medical University of Vienna, Vienna, Austria.

For large avians such as vultures, limb loss leads to loss of ambulation and eventually death from malnutrition. Prosthetic devices may replace the limb, however, conventional prosthetic sockets are not feasible in feathered limbs and the extreme stress and strain of unreflected daily use in animals. Osseointegration is a novel technique, where external prosthetic parts are connected directly to a bone anchor to provide a solid skeletal-attachment. This concept provides a high degree of embodiment since osseoperception will provide direct intuitive feedback allowing natural use of the limb in gait and feeding. Here we demonstrate for the first time an osseointegrated bionic reconstruction of a limb in a vulture after a tarsometatarsal amputation with a longterm follow-up.
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http://dx.doi.org/10.1038/s41598-021-90048-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8195993PMC
June 2021

Extraosseous osteoblastoma: A rare cause of breast mass in a prepubertal girl.

Clin Case Rep 2021 May 31;9(5):e04094. Epub 2021 Mar 31.

Department of Obstetrics and Gynecology Medical University of Vienna Vienna Austria.

Extraosseous osteoblastoma of the breast, a rare disease, was diagnosed in a prepubertal girl. After tumor excision, the patient recovered well and an optimal cosmetic result was achieved. Interdisciplinary discussions about the case are essential.
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http://dx.doi.org/10.1002/ccr3.4094DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8142408PMC
May 2021

Survival and prognosis with osteosarcoma: outcomes in more than 2000 patients in the EURAMOS-1 (European and American Osteosarcoma Study) cohort.

Eur J Cancer 2019 03 25;109:36-50. Epub 2019 Jan 25.

CDC MRC Clinical Trials Unit at UCL, London, UK.

Background: High-grade osteosarcoma is a primary malignant bone tumour mainly affecting children and young adults. The European and American Osteosarcoma Study (EURAMOS)-1 is a collaboration of four study groups aiming to improve outcomes of this rare disease by facilitating randomised controlled trials.

Methods: Patients eligible for EURAMOS-1 were aged ≤40 years with M0 or M1 skeletal high-grade osteosarcoma in which case complete surgical resection at all sites was deemed to be possible. A three-drug combination with methotrexate, doxorubicin and cisplatin was defined as standard chemotherapy, and between April 2005 and June 2011, 2260 patients were registered. We report survival outcomes and prognostic factors in the full cohort of registered patients.

Results: For all registered patients at a median follow-up of 54 months (interquartile range: 38-73) from biopsy, 3-year and 5-year event-free survival were 59% (95% confidence interval [CI]: 57-61%) and 54% (95% CI: 52-56%), respectively. Multivariate analyses showed that the most adverse factors at diagnosis were pulmonary metastases (hazard ratio [HR] = 2.34, 95% CI: 1.95-2.81), non-pulmonary metastases (HR = 1.94, 95% CI: 1.38-2.73) or an axial skeleton tumour site (HR = 1.53, 95% CI: 1.10-2.13). The histological subtypes telangiectatic (HR = 0.52, 95% CI: 0.33-0.80) and unspecified conventional (HR = 0.67, 95% CI: 0.52-0.88) were associated with a favourable prognosis compared with chondroblastic subtype. The 3-year and 5-year overall survival from biopsy were 79% (95% CI: 77-81%) and 71% (95% CI: 68-73%), respectively. For patients with localised disease at presentation and in complete remission after surgery, having a poor histological response was associated with worse outcome after surgery (HR = 2.13, 95% CI: 1.76-2.58). In radically operated patients, there was no good evidence that axial tumour site was associated with worse outcome.

Conclusions: In conclusion, data from >2000 patients registered to EURAMOS-1 demonstrated survival rates in concordance with institution- or group-level osteosarcoma trials. Further efforts are required to drive improvements for patients who can be identified to be at higher risk of adverse outcome. This trial reaffirms known prognostic factors, and owing to the large numbers of patients registered, it sheds light on some additional factors to consider.
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http://dx.doi.org/10.1016/j.ejca.2018.11.027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6506906PMC
March 2019

A comparative study of intraoperative frozen section and alpha defensin lateral flow test in the diagnosis of periprosthetic joint infection.

Acta Orthop 2019 04 23;90(2):105-110. Epub 2019 Jan 23.

a Medical University of Vienna , Department of Orthopaedics and Trauma Surgery , Vienna.

Background and purpose - For decision-making (aseptic vs. septic), surgeons rely on intraoperatively available tests when a periprosthetic joint infection (PJI) cannot be confirmed or excluded preoperatively. We compared and evaluated the intraoperative performances of the frozen section and the alpha defensin lateral flow test in the diagnosis of PJI. Patients and methods - In this prospective study, consecutive patients with indicated revision surgery after arthroplasty were included. Patients were classified as having PJI using the MusculoSkeletal Infection criteria. The presence of alpha defensin was determined using the lateral flow test intraoperatively. During revision surgery, tissue samples were harvested for frozen and permanent section. Analysis of diagnostic accuracy was based on receiver-operating characteristics. Results - 101 patients (53 hips, 48 knees) were eligible for inclusion. Postoperatively, 29/101 patients were diagnosed with PJI, of which 8/29 cases were definitely classified as septic preoperatively. Of the remainder 21 septic cases, the intraoperative alpha defensin test and frozen section were positive in 13 and 17 patients, respectively. Sensitivities of the alpha defensin test and frozen section were 69% and 86%, respectively. The area under the curves of both tests showed a statistically significant difference (p = 0.006). Interpretation - The frozen section showed a significantly higher performance compared with the alpha defensin test and a near perfect concordance with the definitive histology, and therefore remains an appropriate intraoperative screening test in diagnosing PJI. Although the sensitivity of the alpha defensin test was lower compared with that of frozen section, this test is highly specific for confirming the diagnosis of PJI.
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http://dx.doi.org/10.1080/17453674.2019.1567153DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6461083PMC
April 2019

Ultrasound of horizontal instability of the acromioclavicular joint : A simple and reliable test based on a cadaveric study.

Wien Klin Wochenschr 2019 Feb 7;131(3-4):81-86. Epub 2019 Jan 7.

Department of Orthopaedic Surgery, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria.

Background: Horizontal instability influences the clinical outcome after acromioclavicular joint (ACJ) injuries and in joint degeneration. A standardized, dynamic examination of the horizontal instability has not been described before. This current study presents a sonographic method to analyze the dynamics between the clavicle and acromion in the horizontal plane.

Methods: The horizontal joint play, the ACJ space and the offset between clavicle and acromion were sonographically assessed by a 45° ventrally angulated longitudinal section. A total of four investigators with different experience in the field of musculoskeletal ultrasound examining 20 ACJs in 10 human cadavers. Measurements in the absence of any pressure and under standardized anterior and posterior pressure onto the clavicle were carried out in different ligament status intact acromioclavicular (AC) and coracoclavicular (CC) ligaments as well as in a Rockwood (RW) II and III model. A two-sided t‑test was used to examine the differences between joint positions and ligament status.

Results: The horizontal joint play was 1.3 ± 0.9 mm with intact ligaments, 1.4 ± 1.2 mm by transecting the AC ligaments and 1.9 ± 1.3 mm after additionally transecting the CC ligament. The joint space was 3.3 ± 1.1 mm with intact ligaments, 4.1 ± 1.8 mm in an iatrogenic RW 2 injury, and 5.3 ± 3.3 mm in an iatrogenic RW 3 injury. Manipulating the clavicle by applying anterior or posterior pressure did not change the difference within one injury pattern. Interobserver reliability was 83.9%.

Conclusion: Apart from evaluating the ligaments and the joint capsule, measurement of the dynamic horizontal instability is possible in a human cadaver model. The ultrasound-based measurement of horizontal instability dynamics avoids radiation exposure, is readily available and cost-efficient.
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http://dx.doi.org/10.1007/s00508-018-1433-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6394808PMC
February 2019

High-Grade Osteosarcoma of the Foot: Presentation, Treatment, Prognostic Factors, and Outcome of 23 Cooperative Osteosarcoma Study Group COSS Patients.

Sarcoma 2018 2;2018:1632978. Epub 2018 May 2.

Center for Pediatric, Adolescent and Women's Medicine, Olgahospital, Department of Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Stuttgart, Germany.

Osteosarcoma of the foot is a very rare presentation of a rare tumor entity. In a retrospective analysis, we investigated tumor- and treatment-related variables and outcome of patients registered in the Cooperative Osteosarcoma Study Group (COSS) database between January 1980 and April 2016 who suffered from primary high-grade osteosarcoma of the foot. Among the 23 eligible patients, median age was 32 years (range: 6-58 years), 10 were female, and 13 were male. The tarsus was the most commonly affected site (=16). Three patients had primary metastases. All patients were operated: 5 underwent primary surgery and 18 received surgery following preoperative chemotherapy. In 21 of the 23 patients, complete surgical remission was achieved. In 4 of 17 patients, a poor response to neoadjuvant chemotherapy was observed in the resected primary tumors. Median follow-up was 4.2 years (range: 0.4-18.5). At the last follow-up, 15 of the 23 patients were alive and 8 had died. Five-year overall and event-free survival estimates were 64% (standard error (SE) 12%) and 54% (SE 13%), which is similar to that observed for osteosarcoma in general. Event-free and overall survival correlated with primary metastatic status and completeness of surgery. Our findings show that high-grade osteosarcoma in the foot has a similar outcome as osteosarcoma of other sites.
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http://dx.doi.org/10.1155/2018/1632978DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5954899PMC
May 2018

C-reactive protein: An independent predictor for dedifferentiated chondrosarcoma.

J Orthop Res 2018 10 24;36(10):2797-2801. Epub 2018 May 24.

Department of Orthopaedics and Traumatology, Clinical Division of Orthopaedics, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria.

Dedifferentiated chondrosarcoma is a rare primary bone malignancy with a very poor prognosis. The aim of the study was to identify pretreatment serum markers as prognostic factors for the overall survival (OS) of patients with dedifferentiated chondrosarcoma. We retrospectively reviewed 33 patients with histologically confirmed dedifferentiated chondrosarcoma treated at our department from 1977 to 2015. Kaplan-Meier estimation, uni- and multivariable Cox proportional hazard model were performed to evaluate the association between serum markers such as the C-reactive protein and OS. In univariable analysis, CRP was strongly associated with OS (HR 1.35; 95%CI 1.13-1.61; p = 0.001). This association prevailed after adjustment for AJCC tumor stage (HR 1.31; 95%CI 1.02-1.57; p = 0.031) in multivariable analysis. In conclusion, our data gave evidence that baseline CRP is an independent predictor for OS in patients with dedifferentiated chondrosarcoma. CRP could be exploited for the clinical prediction of this disease in the future. © 2018 The Authors. Journal of Orthopaedic Research® Published by Wiley Periodicals, Inc. on behalf of Orthopaedic Research Society. J Orthop Res 36:2797-2801, 2018.
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http://dx.doi.org/10.1002/jor.24030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6220779PMC
October 2018

Matrix-associated chondrocyte transplantation for reconstruction of articulating surfaces in the temporomandibular joint: a pilot study covering medium- and long-term outcomes of 6 patients.

Oral Surg Oral Med Oral Pathol Oral Radiol 2018 08 9;126(2):117-128. Epub 2018 Mar 9.

Department of Oral and Maxillofacial Surgery, Medical University of Vienna, Waehringer Guertel 18-20, 1090, Vienna, Austria.

Objective: Matrix-associated chondrocyte transplantation is routinely used in joints of the extremities but not in the temporomandibular joint (TMJ).

Study Design: We report the first case series in 7 patients of a tissue engineering approach to regenerate severely degraded articulating surfaces in the TMJ by simultaneously completely resurfacing both the mandibular condyle and the articular eminence/glenoid fossa with a commercially available collagen sponge seeded with autologous cells stabilized within a fibrin matrix. To facilitate healing, we temporarily employed a silicone membrane to protect the engineered tissues. The indications for surgery were posttraumatic fibro-osseous ankylosis, ankylosing osteoarthritis, or late-stage osteoarthritis.

Results: Six of the patients were recalled for follow-up after 3 years 6 months to 12 years 1 month. The maximum incisal opening was 18.2 ± 9.2 mm (range, 9-33 mm) before and 31.2 ± 13.6 mm (range, 12-47 mm) at the latest follow-up. Histologic specimens taken at 4 months showed beginning differentiation of fibrocytes into chondrocytes, whereas at 3 and 11 years, mature hyaline cartilage-not typical for the TMJ-was present.

Conclusions: We conclude that the reconstruction of TMJ surfaces by matrix-associated chondrocyte transplantation may become a routine method for cartilage regeneration in the TMJ in the future.
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http://dx.doi.org/10.1016/j.oooo.2018.02.017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6057608PMC
August 2018

Increased zinc accumulation in mineralized osteosarcoma tissue measured by confocal synchrotron radiation micro X-ray fluorescence analysis.

Xray Spectrom 2017 Jan-Feb;46(1):56-62. Epub 2016 Dec 21.

Atominstitut TU Wien Vienna Austria.

Abnormal tissue levels of certain trace elements such as zinc (Zn) were reported in various types of cancer. Little is known about the role of Zn in osteosarcoma. Using confocal synchrotron radiation micro X-ray fluorescence analysis, we characterized the spatial distribution of Zn in high-grade sclerosing osteosarcoma of nine patients (four women/five men; seven knee/one humerus/one femur) following chemotherapy and wide surgical resection. Levels were compared with adjacent normal tissue. Quantitative backscattered electron imaging as well as histological examinations was also performed. On average, the ratio of medians of Zn count rates (normalized to calcium) in mineralized tumor tissue was about six times higher than in normal tissue. There was no difference in Zn levels between tumor fraction areas with a low fraction and a high fraction of mineralized tissue, which were clearly depicted using quantitative backscattered electron imaging. Moreover, we found no correlation between the Zn values and the type of tumor regression according to the Salzer-Kuntschik grading. The underlying mechanism of Zn accumulation remains unclear. Given the emerging data on the role of trace elements in other types of cancer, our novel results warrant further studies on the role of trace elements in bone cancer. Copyright © 2016 The Authors. published by John Wiley & Sons Ltd.
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http://dx.doi.org/10.1002/xrs.2727DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5299496PMC
December 2016

Bacillary angiomatosis presenting with facial tumor and multiple abscesses: A case report.

Medicine (Baltimore) 2016 Jul;95(28):e4155

Institute for Hygiene and Applied Immunology, Center for Pathophysiology, Infectiology and Immunology Division of Immunology, Allergy and Infectious Diseases, Department of Dermatology Clinical Institute for Pathology Department of Radiology and Nuclear Medicine, Medical University of Vienna, Vienna, Austria.

Background: The clinical manifestation of bacillary angiomatosis (BA) can be limited to one organ, most commonly the skin, but systemic courses can also occur. We report a human immunodeficiency virus (HIV)-positive patient with a systemic manifestation of BA caused by Bartonella quintana, diagnosed in Vienna, Austria. The pathogen was detected in multiple organs including a facial tumor which is an unusual finding for BA. Furthermore, infections with B quintana are rare in our area and no other autochthonous cases have been reported.

Methods And Results: The clinical manifestation included multiple papules and nodules on the entire body, several organic abscesses, and a facial tumor influencing the patient's view.The main laboratory finding indicated HIV infection combined with severe immunosuppression with 47 CD4 cells/μL. Contrast-enhanced computed tomography of the chest and the abdomen showed multiple and abscesses. Histological examination of the facial tumor confirmed inflammatory process. Bartonella quintana was detected by PCR in blood and in the facial tumor as well as by culture in the skin tissue. Antibiotic treatment with doxycycline and antiretroviral therapy resulted in clinical improvement.

Conclusion: Our case shows that rare opportunistic, vector-borne infections, usually associated with poverty, can lead to diagnosis of HIV even in well-developed countries. Furthermore, we provide details on clinical manifestation and diagnostic work-up which might expand the knowledge on disseminated infections with B quintana. As far, tumorous deformations have rarely been reported as consequence of BA. In our patient the pathogen was detected in the facial tumor using PCR techniques.
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http://dx.doi.org/10.1097/MD.0000000000004155DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4956801PMC
July 2016

Evaluation of six different airway devices regarding regurgitation and pulmonary aspiration during cardio-pulmonary resuscitation (CPR) - A human cadaver pilot study.

Resuscitation 2016 May 26;102:70-4. Epub 2016 Feb 26.

Institute of Anesthesiology, University Hospital Zurich, Zurich, Switzerland; Departments of Outcomes Research and General Anesthesiology, Anesthesiology Institute, The Cleveland Clinic, Cleveland, USA. Electronic address:

Background: Chest compressions and ventilation are lifesaving tasks during cardio-pulmonary resuscitation (CPR). Besides oxygenation, endotracheal intubation (ETI) during CPR is performed to avoid aspiration of gastric contents. If intubation is difficult or impossible, supraglottic airway devices are utilized. We tested six different airway devices regarding their potential to protect against regurgitation and aspiration during CPR in a randomized experimental human cadaver study.

Methods: Five-hundred ml of 0.01% methylene-blue-solution were instilled into the stomach of 30 adult human cadavers via an oro-gastric tube. The cadavers were then randomly assigned to one of six groups, resulting in 5 cadavers in each group. Airway management was performed with either bag-valve ventilation, Laryngeal Tube, EasyTube, Laryngeal Mask (Classic), I-Gel, or ETI. Thereafter 5min of CPR were performed according to the 2010 Guidelines of the European Resuscitation Council. Pulmonary aspiration was defined as the presence of methylene-blue-solution below the vocal cords or the ETI cuff as assessed by fiber-optic bronchoscopy.

Results: Thirty cadavers were included (14 females, 16 males). Aspiration was detected in three out of five cadavers receiving bag-valve ventilation and in two out of five intubated with LMA or I-Gel. In cadavers intubated with the LT, aspiration occurred in one out of five cases. No aspiration could be detected in cadavers intubated with ETI and EasyTube.

Conclusion: This study provides experimental evidence that, during CPR, ETI offers superior protection against regurgitation and pulmonary aspiration of gastric contents than supraglottic airway devices or bag-valve ventilation.
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http://dx.doi.org/10.1016/j.resuscitation.2016.02.017DOI Listing
May 2016

Multiple relapses in high-grade osteosarcoma: when to stop aggressive therapy?

Pediatr Blood Cancer 2015 Mar 8;62(3):529-30. Epub 2014 Dec 8.

Department of Pediatrics, St. Anna Children's Hospital, Medical University Vienna, Vienna, Austria.

The prognosis after relapse of high-grade osteosarcoma is poor and complete resection of all tumors is essential for survival. A 6-year old was diagnosed with high-grade osteosarcoma and treated according to the COSS-96 protocol. Within 5 years from initial diagnosis, five osteosarcoma relapses occurred and every time it was possible to achieve complete surgical remission. Additional treatments included chemotherapy and dendritic cell-based cancer immune therapy. Since the end of therapy of the 5th relapse, he is alive for 11½ years. Our experience further supports that aggressive surgery can help to achieve long-term survival even in patients with multiple osteosarcoma relapses.
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http://dx.doi.org/10.1002/pbc.25360DOI Listing
March 2015

Vacuum inversion and securing of distal colonic pseudodiverticula with novel spiked O-rings.

Gastrointest Endosc 2015 Mar 5;81(3):749-55. Epub 2014 Dec 5.

Department of Medicine III, Division of Gastroenterology and Hepatology, Medical University of Vienna, Vienna, Austria.

Background: Diverticular disease is increasingly prevalent in Western societies and is associated with significant morbidity.

Objective: Two-stage endoscopic device development for inversion and secured ligation of colonic diverticula; first, human cadaver studies were performed to measure forces required for diverticular inversion; second, a novel set of devices (elastic spiked O-ring with delivery system) was tested in animals.

Design: Prospective, observational study of human cadavers and prospective, interventional study of a porcine model.

Setting: University hospital pathology laboratory and animal facility.

Intervention: Full-thickness inversion of the colonic wall with a pipelike delivery instrument to produce an inverted pseudodiverticulum that was secured with a spiked O-ring.

Main Outcome Measurements: The forces required for diverticular inversion, the secured closure of inverted pseudodiverticula, and the time until necrotic tissue falls off.

Results: A total of 248 of 248 of cadaveric sigmoid diverticula could be inverted by means of vacuum or forceps. The forces required for inversion ranged from 0.28 to 0.47 N (median, 0.37 N). Twenty-four spiked O-rings were delivered in 6 living pigs to produce 24 inverted pseudodiverticula. One animal died the day after the procedure of a pulmonary thromboembolism. In the remaining 5 pigs, all delivered spiked O-rings remained in place for 7 to 22 days. At necropsy, none of the inverted sites showed signs of perforation but rather full-thickness reparative scarring with ingrowth of connective tissue.

Limitations: Animal model, stiff pipelike delivery instrument, variations in diverticular location, diameter, and size.

Conclusions: Endoluminal inversion and securing of colonic diverticula induces tissue necrosis, diverticular sloughing, and full-thickness scarring.
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http://dx.doi.org/10.1016/j.gie.2014.09.051DOI Listing
March 2015

Tumour response of osteosarcoma to neoadjuvant chemotherapy evaluated by magnetic resonance imaging as prognostic factor for outcome.

Int Orthop 2015 Jan 30;39(1):97-104. Epub 2014 Nov 30.

Department of Orthopaedic Surgery, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria,

Purpose: This study evaluated the feasibility of computed magnetic resonance imaging (MRI) volumetry in conventional osteosarcomas. Secondly, we investigated whether computed volumetry provides new prognostic indicators for histological response of osteosarcomas after neoadjuvant chemotherapy.

Methods: In a retrospective cohort study, data from the Vienna Bone Tumour Registry was used. MR images from 14 patients (male:female = 1.8, mean age 19 years) were analysed prior to and after neoadjuvant chemotherapy according to current therapy regimens. Histological response to chemotherapy was graded according to the Salzer-Kuntschik classification. Computed volumetry was performed for the intraosseous part, as well as the soft-tissue component and the tumour as a whole.

Results: In a setting of appropriate radiological equipment, the method has been considered to be well implementable into clinical routine. The mean tumour volume prior to chemotherapy was 321 (±351) ml. In good responders (n = 6), overall tumour volume decreased by 47% (p = 0.345), whereas poor responders (n = 8) showed a 19% decrease (p = 0.128). Neoadjuvant multidrug therapy remarkably changed the tumour composition. This is seen in a decrease of the mean ratio of soft-tissue to intraosseous tumour volume from 8.67 in poor responders and 1.15 in good responders to 1.26 and 0.45 (p = 0.065), respectively. Interestingly, the bony compartment of good responders showed a volume increase during neoadjuvant chemotherapy (p = 0.073). However, we did not find prognostic markers for histological tumour response to pre-operative chemotherapy.

Conclusions: Separated volumetry of tumour segments revealed interesting insights into therapy-induced growth patterns. If verified in a larger study population, these results should be taken into account when planning ablative surgery.
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http://dx.doi.org/10.1007/s00264-014-2606-5DOI Listing
January 2015

Non-occlusive mesenteric ischaemia: the prevalent cause of gastrointestinal infarction in patients with severe burn injuries.

Injury 2015 Jan 29;46(1):124-30. Epub 2014 Aug 29.

Head of Division of Plastic and Reconstructive Surgery, Department of Surgery, Medical University Vienna, Vienna, Austria.

Background: Gastrointestinal complications occur frequently in intensive care patients with severe burns. Intestinal infarction and its deleterious consequences result in high mortality despite rapid surgical intervention. Our objective was to evaluate the aetiology of gastrointestinal infarction in intensive care patients with severe burns.

Study Design: We retrospectively evaluated all of the severe-burn victims at the burn unit of the Medical University of Vienna from 01/2002 to 06/2012 for whom a gastrointestinal infarction was diagnosed during their inpatient stay on computed-tomography, in the context of acute laparotomy, or upon autopsy by aetiology.

Results: After a severe thermal injury, 17 patients suffered a gastrointestinal infarction during their stay. In 82% of those patients, non-occlusive mesenteric ischaemia (NOMI) was identified as the cause of the gastrointestinal infarction. Patients with an embolic infarction tended to be older (78.0years embolism vs. 53.4 NOMI, mean, p<0.01), with a lower abbreviated burn severity index (8.7 embolism vs. 10.4 NOMI, mean, p<0.02) and a smaller total body surface area burned (20% embolism vs. 48% NOMI, mean, p<0.01) than those with a non-occlusive mesenterial ischaemia. No patients with an embolic infarction or any of the females in the entire gastrointestinal infarction group survived this event, resulting in a mortality rate of 100% for the embolic infarction group and female group. The decisive factor for surviving a NOMI was age (median age: male survivors 28years vs. nonsurvivors 66years (of this median, males=72years and females=60years), p<0.02).

Conclusion: The results of our study clearly demonstrate that in severe-burn intensive care patients, non-occlusive mesenteric ischaemia is the most frequent cause of gastrointestinal infarction and that the decisive factor for survival is the patient's age.
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http://dx.doi.org/10.1016/j.injury.2014.08.035DOI Listing
January 2015

Surgical treatment of the haemophilic pseudotumour: a single centre experience.

Int Orthop 2012 Oct 4;36(10):2157-62. Epub 2012 Jul 4.

Department of Orthopaedics, Medical University of Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria.

Purpose: Haemophilic pseudotumour was defined by Fernandez de Valderrama and Matthews as a progressive cystic swelling involving muscle, produced by recurrent haemorrhage into muscles adjacent to the bone. The pseudotumour mainly occurs in the long bones and the pelvis. The treatment of the haemophilic pseudotumour poses a challenge, and extensive clinical experience is essential to appropriately address this serious complication in patients with haemophilia. Consequently, the aim of this study is to present our own clinical experience and treatment results of the haemophilic pseudotumour.

Methods: We retrospectively reviewed the records of 87 patients with bleeding disorders treated between 1967 and 2011 for musculoskeletal complications of congenital bleeding disorders. We identified six patients with a haemophilic pseudotumour who were treated at our department.

Results: The mean age at surgery was 45.9 (range, 40-61) years. The iliac bone was affected in three patients (one right, two left), the right tibia (distal diaphysis) in one, the right thigh in two and the right ulna (proximal part) in one patient. One patient had two pseudotumours. The perioperative course was easily controllable with adequate factor VIII substitution. At the latest follow-up after 8.4 (range, 4-24) years, normal healing with no recurrence was observed.

Conclusions: The haemophilic pseudotumour is a rare but severe complication of hereditary bleeding disorders. In the international literature the resection and postoperative course are described as challenging and difficult, requiring detailed preoperative planning. It is advisable to perform such operations in specialised centres with close co-operation between surgeons and haematologists.
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http://dx.doi.org/10.1007/s00264-012-1593-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3460086PMC
October 2012

Anatomy and variants of the triangular fibrocartilage complex and its MR appearance at 3 and 7T.

Semin Musculoskelet Radiol 2012 Apr 30;16(2):93-103. Epub 2012 May 30.

Department of Radiology, MR Centre-Highfield MR, Medical University of Vienna, Vienna, Austria.

Due to the small size and complexity of its constituents, the triangular fibrocartilage complex (TFCC) has been a challenging structure for magnetic resonance (MR) imaging. Higher-field MR units, at 3T and 7T, with increased spatial resolution and the development of novel MR sequences, are promising tools for an improved visualization of the ulnocarpal complex. Anatomically, the TFCC consists of the TFC proper, the ulnomeniscal homolog, the ulnar collateral ligament, the ulnotriquetral and ulnolunate ligament, and radioulnar ligaments at the volar (palmar) and the dorsal side, as well as the sheath of the extensor carpi ulnaris tendon and the capsule of the distal radioulnar joint. This article describes the normal anatomy of the TFCC and its appearance on high-field MRI. Anatomical variants, such as the positive ulnar variance, and changes during pronation and supination are addressed.
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http://dx.doi.org/10.1055/s-0032-1311761DOI Listing
April 2012

Growth potential of different zones of the growth plate-an experimental study in rabbits.

J Orthop Res 2012 Jan 27;30(1):162-8. Epub 2011 Jul 27.

University Clinic of Trauma Surgery, Medical University of Vienna, Waehringer Guertel 18-20, Wien, Austria.

Despite clinical efforts to treat growth disturbances only little is known about the growth potential of the different zones of the growth plate. The aim of this study was to investigate the growth potential of different zones of the growth plate. A total of 20 New Zealand White rabbits were used for this experiment. The right and left ulna of each animal were used resulting in a total of 40 ulnae. Animals were assigned into five groups. In groups I and II resection of the metaphyseal (n = 12) or the epiphyseal (n = 6) segment of the growth plate was performed. In group III resection of the growth plate and re-implantation was performed (n = 6). In group IV the growth plate was resected and re-implanted after a 180° rotation (n = 6). Animals in group V served as controls. Histologic and radiologic examinations were performed to evaluate the growth process at 1, 2, 4, and 12 weeks following surgery. In group I, III, and IV temporary growth disturbance which was compensated within a short time was observed. Resection of the epiphyseal part resulted in growth arrest of the distal ulna in combination with normal growth of the radius which led to and valgus deformity of the limb. The results of this study indicate the importance of the reserve zone for the functioning of the growth plate.
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http://dx.doi.org/10.1002/jor.21506DOI Listing
January 2012

The effect of drilling and screw fixation of the growth plate--an experimental study in rabbits.

J Orthop Res 2011 Dec 26;29(12):1834-9. Epub 2011 May 26.

Department of Trauma Surgery, Medical University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna, Austria.

Injury of the growth plate is a specific problem in traumatology and can cause limb deformity and length discrepancy as a result of growth arrest. The purpose of this study was to evaluate alterations of the growth plate after artificially created injuries. A total of 14 New Zealand White rabbits were used for this experiment. The right and left ulna of each animal was used resulting in a total of 28 ulnae. In six animals drill holes were driven into the growth plate either from the distal/epiphyseal side or from the proximal/metaphyseal side of the physis. In six animals a fracture of the distal ulna corresponding to a Salter-Harris fracture type IV was created. This fracture was fixed by screws from either the epiphyseal or the metaphyseal side. Two animals served as controls. Histologic and radiologic examinations were performed to evaluate the growth process at 1, 2, 4, and 12 weeks following surgery. Drilling or fixation of the growth plate from the metaphyseal side resulted in temporary growth disturbance which was compensated within a short time. In contrast fixation from the epiphyseal side caused severe growth disturbances. Based on our findings K-wires or screws should be inserted from the metaphyseal side and be placed in the center of the growth plate.
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http://dx.doi.org/10.1002/jor.21463DOI Listing
December 2011

Low-grade chondrosarcoma of bone: experiences from the Vienna Bone and Soft Tissue Tumour Registry.

Int Orthop 2011 Jul 28;35(7):1049-56. Epub 2010 Aug 28.

Medical University of Vienna Department of Orthopaedic Surgery, Vienna General Hospital, Waehringer Guertel 18-20, 1090, Vienna, Austria.

In a retrospective single-centre study, 70 low-grade chondrosarcoma (LCS) (31 female/39 male patients with a mean age of 40 years) were reviewed to evaluate surgical management. The mean overall follow-up was 81 months (median: 73 months, range: 6-317 months). Seventeen lesions (24.3%) in the trunk and 53 (75.7%) in the extremities were treated by curettage (48.6%) or resection (51.5%). Local recurrence occurred in eight patients (11.4%) 18 months postoperatively (median: 18 months, range: 0-41 months). Recurrence-free survival was significantly better for patients with extremity lesions compared to truncal lesions, but was not affected by resection margin. The anatomical site "trunk" and an "intralesional" resection margin had a significant independent prognostic influence in multivariate analysis. Curettage with local adjuvants is a viable treatment option for most extremity LCS. In truncal LCS wide resection is recommended despite a potentially higher complication rate.
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http://dx.doi.org/10.1007/s00264-010-1065-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3167418PMC
July 2011

Osteosarcoma in very young children: experience of the Cooperative Osteosarcoma Study Group.

Cancer 2010 Nov;116(22):5316-24

St. Anna Children's Hospital, Vienna, Austria.

Background: This study was conducted to investigate presentation, treatment, and outcome in very young children with osteosarcoma.

Methods: The authors retrospectively analyzed the data of 2706 consecutive COSS patients with newly diagnosed osteosarcoma and identified 28 (1.0%) patients aged younger than 5 years at diagnosis. Demographic, diagnostic, tumor, treatment-related variables, response, and survival data were analyzed.

Results: Of the 28 preschoolers, 27 presented with high-grade central osteosarcoma of an extremity, and 1 had a secondary osteosarcoma of the orbit. This analysis focused on the 27 patients with extremity tumors. The size of the primary was large (≥one-third of the involved bone) in 20 of 27 patients. Primary metastases were detected in 4 of 27 children. All patients received multiagent chemotherapy, and 11 of 18 analyzed tumors responded well (>90% necrosis) to neoadjuvant chemotherapy. Limb-sparing surgery was performed in 9 cases, ablative procedures were performed in 15, and, in 3 cases, no local surgery was performed. With a median follow-up of 4 years (6.2 years for survivors), 13 patients were alive. Four patients never achieved a complete remission, and 11 developed recurrences; 14 of these 15 patients died. Five-year overall and event-free survival probabilities were 51% (standard error of the mean [SE], 10%) and 48% (SE, 10%). Better survival was correlated with good response to chemotherapy and later time period of diagnosis.

Conclusions: Osteosarcoma is extremely rare in preschool children. These patients often have large tumors that may require mutilating resections. Prognosis is in the range of that reported for older patients.
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http://dx.doi.org/10.1002/cncr.25287DOI Listing
November 2010

Intimal sarcoma of the pulmonary valve.

Ann Thorac Surg 2010 Apr;89(4):e25-7

Division of Pulmonology, Department of Internal Medicine, Medical University Graz, Austria.

Pulmonary artery intimal sarcoma is a rare tumor of the cardiovascular system. Intimal sarcoma of the pulmonary valve itself has not been described. Embolization into pulmonary arteries originating from the pulmonary valve intimal sarcoma can mimic chronic thromboembolic pulmonary hypertension and mislead the diagnosis. We present and discuss a patient initially diagnosed as chronic thromboembolic pulmonary hypertension, treated by pulmonary endarterectomy. After 24 months, a tumor of the pulmonary valve was detected by echocardiography. The patient underwent removal and replacement of the pulmonary valve. Histology revealed pulmonary valve intimal sarcoma.
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http://dx.doi.org/10.1016/j.athoracsur.2010.01.053DOI Listing
April 2010

Histological evaluation of the healing potential of the anterior cruciate ligament by means of augmented and non-augmented repair: an in vivo animal study.

Knee Surg Sports Traumatol Arthrosc 2008 Dec 19;16(12):1087-93. Epub 2008 Aug 19.

Department of Trauma Surgery, Judenburg-Knittelfeld General Hospital, Oberweggasse 18, 8750, Judenburg, Austria.

The purpose of this animal study was to investigate the healing potential of the anterior cruciate ligament following transaction at the femoral end, by means of both the augmented and the nonaugmented primary repair, focusing on the histological changes during a 12-month postoperative observation period with respect to necrosis and the loss of normal structures during the healing period. Twenty sheep were randomly assigned to nonaugmented primary ACL repair, or to augmented ACL repair using a polyethylene terephthalate (PET) band. At 2, 6, 16, 26 and 52 weeks postoperatively four sheep out of each group were sacrificed. Histological healing was achieved after 16 weeks for the augmented repair group and after 26 weeks for the nonaugmented repair group. The main difference between the two techniques was that the PET augmentation device protected the ligament from necrosis and ligamentization. In this group a consistently normal ligament was seen histologically during the entire postoperative observation period. The nonaugmented repaired ACLs underwent the processes of necrosis and ligamentization.
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http://dx.doi.org/10.1007/s00167-008-0599-6DOI Listing
December 2008

Running has a negative effect on bone metabolism and proinflammatory status in male aged rats.

Exp Gerontol 2008 Jun 30;43(6):578-83. Epub 2008 Mar 30.

II. Medical Clinic, University of Veterinary Medicine Vienna, Veterinärplatz 1, A-1210 Vienna, Austria.

Animal models for male osteoporosis are scarce. This study aimed at identifying the impact of different living conditions on bone structure and metabolism as well as the inflammatory status in a rat model of age-related male osteoporosis. Bone mineral density, bone histomorphometric data, ex vivo osteoclast generation, and bone metabolism serum marker as well as intracellular cytokine expressions were evaluated in 23-month-old male Sprague-Dawley rats subjected to different housing conditions from the age of 5 months. Running rats were housed individually and were exercised voluntarily in running wheels attached to their cages. Dieting rats were housed individually, too, but were fed to pair weight with the running rats. Walking rats were exercised mildly by use of a treadmill (800m/day, 5 days a week) and social rats were kept as four in a cage and fed ad libitum. Whereas no marked differences could be found for bone mineral density, trabecular bone volume as well as trabecular bone surface were diminished in walking rats. The ex vivo osteoclast generation assay revealed no significant differences between groups. Osteoblasts of running rats were not only decreased in number, but displayed also a lower activity as indicated by decreased serum osteocalcin levels. Osteoclast activity was increased in the same group as indicated by elevated CTX (c-terminal telopeptide of type I collagen) levels. Additionally, production of tumor necrosis factor (TNF)-alpha and interferone (IFN)-gamma by CD8(+) T cells was elevated in running rats. In conclusion, running has a negative effect on bone metabolism and proinflammatory status in male aged rats.
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http://dx.doi.org/10.1016/j.exger.2008.03.008DOI Listing
June 2008

Creutzfeldt-Jakob disease in Austria: an autopsy-controlled study.

Neuroepidemiology 2008 18;30(4):215-21. Epub 2008 Apr 18.

Institute of Neurology and Austrian Reference Centre for Human Prion Diseases, Medical University of Vienna, Vienna, Austria.

Background: Definite diagnosis of prion diseases or transmissible spongiform encephalopathies (TSEs) requires neuropathology, usually at autopsy. Epidemiology of human TSEs has relied on definite as well as 'probable' cases in which neuropathological confirmation is lacking, usually because of low autopsy rates in most countries.

Methods: In Austria, an active surveillance program for human prion diseases was established in 1996. Since then, more than 900 referrals were analyzed. Postmortem investigation of brain tissue is mandatory in every suspect case of TSE. Thus, epidemiological data on TSEs from Austria may serve as autopsy-controlled reference for countries with lower autopsy rates.

Results: The total number of TSE cases in Austria since 1969 is 206. The average yearly mortality for the active surveillance period from 1996 to 30 June 2006 is 1.39 per million, with the highest rates for Vienna (2.37) compared with other provinces. Eighty-five percent of definite TSEs were classified as sporadic Creutzfeldt-Jakob disease (sCJD). We observed a significant linear increase in the mean age at death of 0.6 years per calendar year. Clinical diagnostic surveillance criteria had a sensitivity and specificity of 82.7 and 80.0% for probable CJD, respectively, and a positive predictive value of 80.5% for probable and 38.9% for 'possible' CJD. Alternative neuropathological diagnoses in suspect cases included Alzheimer's disease with or without Lewy body pathology, vascular encephalopathy, metabolic encephalopathies and viral or limbic encephalitis.

Conclusion: The steady increase in mortality rates, especially in old age groups, most likely reflects improved case ascertainment due to active surveillance causing higher awareness of the medical community. In comparison with other European countries, it is reassuring to note that the overall death rate of TSEs does not differ from the Austrian autopsy-controlled data, thus confirming the value of clinical surveillance criteria.
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http://dx.doi.org/10.1159/000126915DOI Listing
November 2008

Investigation of adenovirus occurrence in pediatric tumor entities.

J Virol 2007 Jul 9;81(14):7629-35. Epub 2007 May 9.

Division of Molecular Microbiology and Development of Genetic Diagnostics, St. Anna Kinderkrebsforschung, Children's Cancer Research Institute, Kinderspitalgasse 6, A-1090 Vienna, Austria.

Adenoviruses (AdVs) contain genes coding for proteins with transforming potential, and certain AdV serotypes have been shown to induce tumors in rodents. However, data on the possible oncogenicity of AdVs in humans are scarce. We have therefore employed a real-time quantitative PCR (RQ-PCR) assay permitting highly sensitive detection of all 51 currently known human AdV serotypes to screen more than 500 tumor specimens derived from 17 different childhood cancer entities including leukemias, lymphomas, and solid tumors. Most tumor entities analyzed showed no evidence for the presence of AdV sequences, but AdV DNA was detected by RQ-PCR in different brain tumors including 25/30 glioblastomas, 22/30 oligodendrogliomas, and 20/30 ependymomas. Nonmalignant counterparts of AdV-positive brain tumors, including specimens of ependymal cells, plexus choroideus, and periventricular white matter, were screened for control purposes and revealed the presence of AdV DNA in most specimens tested. Identification of the AdV types present in positive malignant and nonmalignant brain tissue specimens revealed predominantly representatives of species B and D and, less commonly, C. To exclude contamination as a possible cause of false-positive results, specimens with AdV sequences detectable by PCR were subsequently analyzed by in situ hybridization, which confirmed the PCR findings in all instances. The central nervous system appears to represent a common site of AdV infection with virus persistence, thus providing the first evidence for the possible contribution of AdVs to the multistep process of tumor pathogenesis in brain tissue.
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http://dx.doi.org/10.1128/JVI.00355-07DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1933336PMC
July 2007

Skip metastases in osteosarcoma: experience of the Cooperative Osteosarcoma Study Group.

J Clin Oncol 2006 Apr;24(10):1535-41

St Anna Children's Hospital, Vienna, Austria.

Purpose: The outlook for patients with osteosarcoma who present with synchronous regional bone metastases (skip metastases), either in the primary bone site or transarticular, is considered to be extremely poor. This study was conducted to further investigate the prognostic implication of skip metastases in osteosarcoma.

Patients And Methods: The authors retrospectively analyzed the collected data of 1,765 consecutive patients with newly diagnosed high-grade osteosarcoma of bone who were registered in the neoadjuvant Cooperative Osteosarcoma Study Group studies and identified 24 patients (1.4%) with unequivocally proven skip metastases. All 24 patients were treated by an aggressive surgical approach coupled with polychemotherapy. Demographic, diagnostic, tumor, and treatment-related variables and response and survival data were analyzed.

Results: Skip metastases were identified preoperatively in 11 of 24 patients by bone scan, eight of 22 patients by plain x-ray, 15 of 18 patients by magnetic resonance imaging, and five of 10 patients by computed tomography. A complete surgical remission (CSR) of all clinically detectable tumor sites was achieved in 22 of 24 patients during front-line therapy. With a median follow-up time of 4.4 years (8 years for survivors) from diagnosis, 12 patients were alive, all of whom were in continuous CSR. Survival did correlate with location of skip metastases and histologic response to neoadjuvant chemotherapy.

Conclusion: Synchronous regional bone metastases are rare in osteosarcoma, and preoperative detection relies on appropriate diagnostic imaging. Aggressive multimodal therapy holds the promise to achieve prolonged survival, especially in patients in whom these metastases occur within the same bone as the primary lesion and whose tumors respond well to chemotherapy.
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http://dx.doi.org/10.1200/JCO.2005.04.2978DOI Listing
April 2006

Chondrosarcoma of the hand: is a wide surgical resection necessary?

Clin Orthop Relat Res 2004 Jul(424):211-5

Department of Orthopaedics, University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna, Austria.

Chondrosarcomas of the hand are rare and generally treated with surgical resection. Thirteen patients with Grade 1 chondrosarcoma of the small bones of the hand were followed up for a mean of 99.8 months (range, 26-293 months). In eight patients (Group 1) curettage and reconstruction with cancellous bone was done and in five patients (Group 2) a wide resection was done. No patient experienced relapse in Group 2. In Group 1 one patient had a local relapse 18 months after intralesional resection. Using the Musculoskeletal Tumor Society score for evaluation, the clinical results showed an average of 98% and 95% of the normal function in Groups 1 and 2, respectively. None of the patients had evidence of systemic spread of the disease. With a relapse rate of 12.5% and no distant metastases after curettage, intralesional resection is the preferred method of treatment in Grade 1 chondrosarcoma of the hand, allowing the patient to avoid amputation and major loss of function.
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July 2004
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