ABSTRACT
BACKGROUND: Fractures of the distal radius are common and account for an estimated 17% of all fractures diagnosed. Two-thirds of these fractures are displaced and require reduction. Although distal radius fractures, especially extra-articular fractures, are considered to be relatively harmless, inadequate treatment may result in impaired function of the wrist. Initial treatment according to Dutch guidelines consists of closed reduction and plaster immobilisation. If fracture redisplacement occurs, surgical treatment is recommended. Recently, the use of volar locking plates has become more popular. The aim of this study is to compare the functional outcome following surgical reduction and fixation with a volar locking plate with the functional outcome following closed reduction and plaster immobilisation in patients with displaced extra-articular distal radius fractures. DESIGN: This single blinded randomised controlled trial will randomise between open reduction and internal fixation with a volar locking plate (intervention group) and closed reduction followed by plaster immobilisation (control group). The study population will consist of all consecutive adult patients who are diagnosed with a displaced extra-articular distal radius fracture, which has been adequately reduced at the Emergency Department. The primary outcome (functional outcome) will be assessed by means of the Disability Arm Shoulder Hand Score (DASH). Secondary outcomes comprise the Patient-Rated Wrist Evaluation score (PRWE), quality of life, pain, range of motion, radiological parameters, complications and cross-overs. Since the treatment allocated involves a surgical procedure, randomisation status will not be blinded. However, the researcher assessing the outcome at one year will be unaware of the treatment allocation. In total, 90 patients will be included and this trial will require an estimated time of two years to complete and will be conducted in the Academic Medical Centre Amsterdam and its partners of the regional trauma care network. DICUSSION: Ideally, patients would be randomised before any kind of treatment has been commenced. However, we deem it not patient-friendly to approach possible participants before adequate reduction has been obtained. TRIAL REGISTRATION: This study is registered at the Netherlands Trial Register (NTR3113) and was granted permission by the Medical Ethical Review Committee of the Academic Medical Centre on 01-10-2012.
Subject(s)
Fracture Fixation, Internal/methods , Immobilization/methods , Radius Fractures/therapy , Research Design , Biomechanical Phenomena , Bone Plates , Casts, Surgical , Clinical Protocols , Disability Evaluation , Fracture Fixation, Internal/instrumentation , Fracture Healing , Hand Strength , Humans , Immobilization/adverse effects , Netherlands , Pain Measurement , Quality of Life , Radius Fractures/diagnosis , Radius Fractures/physiopathology , Radius Fractures/surgery , Range of Motion, Articular , Recovery of Function , Single-Blind Method , Time Factors , Treatment OutcomeABSTRACT
BACKGROUND: Elbow dislocations can be classified as simple or complex. Simple dislocations are characterized by the absence of fractures, while complex dislocations are associated with fractures of the radial head, olecranon, or coronoid process. The majority of patients with these complex dislocations are treated with open reduction and internal fixation (ORIF), or arthroplasty in case of a non-reconstructable radial head fracture. If the elbow joint remains unstable after fracture fixation, a hinged elbow fixator can be applied. The fixator provides stability to the elbow joint, and allows for early mobilization. The latter may be important for preventing stiffness of the joint. The aim of this study is to determine the effect of early mobilization with a hinged external elbow fixator on clinical outcome in patients with complex elbow dislocations with residual instability following fracture fixation. METHODS/DESIGN: The design of the study will be a multicenter prospective cohort study of 30 patients who have sustained a complex elbow dislocation and are treated with a hinged elbow fixator following fracture fixation because of residual instability. Early active motion exercises within the limits of pain will be started immediately after surgery under supervision of a physical therapist. Outcome will be evaluated at regular intervals over the subsequent 12 months. The primary outcome is the Quick Disabilities of the Arm, Shoulder, and Hand score. The secondary outcome measures are the Mayo Elbow Performance Index, Oxford Elbow Score, pain level at both sides, range of motion of the elbow joint at both sides, radiographic healing of the fractures and formation of periarticular ossifications, rate of secondary interventions and complications, and health-related quality of life (Short-Form 36). DISCUSSION: The outcome of this study will yield quantitative data on the functional outcome in patients with a complex elbow dislocation and who are treated with ORIF and additional stabilization with a hinged elbow fixator. TRIAL REGISTRATION: The trial is registered at the Netherlands Trial Register (NTR1996).
Subject(s)
Arthroplasty , Elbow Joint/surgery , External Fixators , Fracture Fixation/instrumentation , Fractures, Bone/surgery , Joint Dislocations/surgery , Joint Instability/surgery , Research Design , Disability Evaluation , Elbow Joint/diagnostic imaging , Elbow Joint/physiopathology , Fractures, Bone/complications , Fractures, Bone/diagnosis , Fractures, Bone/physiopathology , Humans , Joint Dislocations/complications , Joint Dislocations/diagnosis , Joint Dislocations/physiopathology , Joint Instability/complications , Joint Instability/diagnosis , Joint Instability/physiopathology , Netherlands , Pain Measurement , Physical Therapy Modalities , Prospective Studies , Prosthesis Design , Quality of Life , Radiography , Range of Motion, Articular , Surveys and Questionnaires , Time Factors , Treatment OutcomeABSTRACT
BACKGROUND: The traditional view that the vast majority of midshaft clavicular fractures heal with good functional outcomes following non-operative treatment may be no longer valid for all midshaft clavicular fractures. Recent studies have presented a relatively high incidence of non-union and identified speciic limitations of the shoulder function in subgroups of patients with these injuries. AIM: A prospective, multicentre randomised controlled trial (RCT) will be conducted in 21 hospitals in the Netherlands, comparing fracture consolidation and shoulder function after either non-operative treatment with a sling or a plate fixation. METHODS/DESIGN: A total of 350 patients will be included, between 18 and 60 years of age, with a dislocated midshaft clavicular fracture. The primary outcome is the incidence of non-union, which will be determined with standardised X-rays (Antero-Posterior and 30 degrees caudocephalad view). Secondary outcome will be the functional outcome, measured using the Constant Score. Strength of the shoulder muscles will be measured with a handheld dynamometer (MicroFET2). Furthermore, the health-related Quality of Life score (ShortForm-36) and the Disabilities of Arm, Shoulder and Hand (DASH) Outcome Measure will be monitored as subjective parameters. Data on complications, bone union, cosmetic aspects and use of painkillers will be collected with follow-up questionnaires. The follow-up time will be two years. All patients will be monitored at regular intervals over the subsequent twelve months (two and six weeks, three months and one year). After two years an interview by telephone and a written survey will be performed to evaluate the two-year functional and mechanical outcomes. All data will be analysed on an intention-to-treat basis, using univariate and multivariate analyses. DISCUSSION: This trial will provide level-1 evidence for the comparison of consolidation and functional outcome between two standardised treatment options for dislocated midshaft clavicular fractures. The gathered data may support the development of a clinical guideline for treatment of clavicular fractures. TRIAL REGISTRATION: Netherlands National Trial Register NTR2399.
Subject(s)
Bone Malalignment/therapy , Bone Plates , Clavicle/injuries , Fracture Fixation, Internal/methods , Fractures, Bone/therapy , Orthotic Devices , Adolescent , Adult , Disability Evaluation , Female , Fracture Healing , Fractures, Ununited , Humans , Male , Middle Aged , Prospective Studies , Quality of Life , Recovery of Function , Treatment Outcome , Young AdultABSTRACT
BACKGROUND: Fractures of the proximal humerus are associated with a profound temporary and sometimes permanent, impairment of function and quality of life. The treatment of comminuted fractures of the proximal humerus like selected three-or four-part fractures and split fractures of the humeral head is a demanding and unresolved problem, especially in the elderly. Locking plates appear to offer improved fixation; however, screw cut-out rates ranges due to fracture collapse are high. As this may lead to higher rates of revision surgery, it may be preferable to treat comminuted fractures in the elderly primarily with a prosthesis or non-operatively. Results from case series and a small-sample randomized controlled trial (RCT) suggest improved function and less pain after primary hemiarthroplasty (HA); however these studies had some limitations and a RCT is needed. The primary aim of this study is to compare the Constant scores (reflecting functional outcome and pain) at one year after primary HA versus non-operative treatment in elderly patients who sustained a comminuted proximal humeral fracture. Secondary aims include effects on functional outcome, pain, complications, quality of life, and cost-effectiveness. METHODS/DESIGN: A prospective, multi-center RCT will be conducted in nine centers in the Netherlands and Belgium. Eighty patients over 65 years of age, who have sustained a three-or four part, or split head proximal humeral fracture will be randomized between primary hemiarthroplasty and conservative treatment. The primary outcome is the Constant score, which indicates pain and function. Secondary outcomes include the Disability of the Arm and Shoulder (DASH) score, Visual Analogue Scale (VAS) for pain, radiographic healing, health-related quality of life (Short-form-36, EuroQol-5D) and healthcare consumption. Cost-effectiveness ratios will be determined for both trial arms. Outcome will be monitored at regular intervals over the subsequent 24 months (1, 3 and 6 weeks, and 3, 6, 12, 18, and 24 months). Data will be analyzed on an intention to treat basis, using univariate and multivariable analyses. DISCUSSION: This trial will provide level-1 evidence on the effectiveness of the two mostly applied treatment options for three-or four part and split head proximal humeral fractures in the elderly. These data may support the development of a clinical guideline for treatment of these traumatic injuries. TRIAL REGISTRATION: Netherlands Trial Register (NTR2040).
Subject(s)
Arthroplasty/methods , Fractures, Comminuted/surgery , Humerus/injuries , Humerus/surgery , Shoulder Fractures/surgery , Shoulder Joint/surgery , Activities of Daily Living , Age Factors , Aged , Arthroplasty/trends , Belgium , Clinical Protocols , Female , Fractures, Comminuted/pathology , Fractures, Comminuted/physiopathology , Humans , Humerus/pathology , Male , Netherlands , Osteoporosis/complications , Outcome Assessment, Health Care/methods , Patient Selection , Postoperative Complications/etiology , Postoperative Complications/prevention & control , Prospective Studies , Prosthesis Design , Prosthesis Implantation/methods , Prosthesis Implantation/trends , Shoulder Fractures/pathology , Shoulder Fractures/physiopathology , Shoulder Joint/pathology , Shoulder Joint/physiopathologyABSTRACT
BACKGROUND: Elbow dislocations can be classified as simple or complex. Simple dislocations are characterized by the absence of fractures, while complex dislocations are associated with fractures. After reduction of a simple dislocation, treatment options include immobilization in a static plaster for different periods of time or so-called functional treatment. Functional treatment is characterized by early active motion within the limits of pain with or without the use of a sling or hinged brace. Theoretically, functional treatment should prevent stiffness without introducing increased joint instability. The primary aim of this randomized controlled trial is to compare early functional treatment versus plaster immobilization following simple dislocations of the elbow. METHODS/DESIGN: The design of the study will be a multicenter randomized controlled trial of 100 patients who have sustained a simple elbow dislocation. After reduction of the dislocation, patients are randomized between a pressure bandage for 5-7 days and early functional treatment or a plaster in 90 degrees flexion, neutral position for pro-supination for a period of three weeks. In the functional group, treatment is started with early active motion within the limits of pain. Function, pain, and radiographic recovery will be evaluated at regular intervals over the subsequent 12 months. The primary outcome measure is the Quick Disabilities of the Arm, Shoulder, and Hand score. The secondary outcome measures are the Mayo Elbow Performance Index, Oxford elbow score, pain level at both sides, range of motion of the elbow joint at both sides, rate of secondary interventions and complication rates in both groups (secondary dislocation, instability, relaxation), health-related quality of life (Short-Form 36 and EuroQol-5D), radiographic appearance of the elbow joint (degenerative changes and heterotopic ossifications), costs, and cost-effectiveness. DISCUSSION: The successful completion of this trial will provide evidence on the effectiveness of a functional treatment for the management of simple elbow dislocations. TRIAL REGISTRATION: The trial is registered at the Netherlands Trial Register (NTR2025).
Subject(s)
Casts, Surgical , Disability Evaluation , Elbow Injuries , Joint Dislocations/therapy , Physical Therapy Modalities , Adolescent , Adult , Aged , Aged, 80 and over , Braces , Cost-Benefit Analysis , Elbow Joint/physiopathology , Female , Humans , Male , Middle Aged , Netherlands , Outcome Assessment, Health Care , Quality of Life , Treatment Outcome , Young AdultABSTRACT
A 37-year-old recrute in the Royal Dutch Army developed blisters on the right cheek approximately 30 minutes after a boot camp exercise in the mangroves of the island of Curaçao. This bullous dermatitis was caused by direct contact with sap from the bark of the manzanilla tree (Hippomane mancinella). As a member of the family Euphorbiaceae, all parts of this tree are highly toxic. Even rain drops falling from the leaves can cause a bullous dermatitis. Usually, symptomatic treatment will allow healing of the dermatitis in a matter of weeks.
Subject(s)
Blister/etiology , Cheek , Dermatitis/etiology , Hippomane/adverse effects , Adult , Curacao , Humans , Male , Military PersonnelSubject(s)
Adenoma/diagnostic imaging , Gallbladder Neoplasms/diagnostic imaging , Gastric Mucosa/diagnostic imaging , Polyps/diagnostic imaging , Adenoma/surgery , Adult , Cholecystectomy, Laparoscopic , Gallbladder Neoplasms/surgery , Gastric Mucosa/surgery , Humans , Male , Polyps/surgery , Treatment Outcome , UltrasonographyABSTRACT
An 82-year-old woman without any previous medical history arrived in the emergency department with severe pain in the entire abdomen since 5 h. Blood tests showed, apart from a CRP of 28 mg/l, no abnormalities. We decided to perform an abdominal ultrasound, which showed an easily compressible gallbladder, containing a small, mobile gallstone and free fluid in the abdomen. During ultrasound-guided punction of this fluid, bile is aspirated. We performed laparoscopy and confirmed a large amount of intraperitoneal bile. Upon inspecting the gallbladder a perforation is seen in the anti-hepatic side of the gallbladder. After performing a cholecystectomy, we opened the gallbladder and detected a dissection-like lesion, which provided access to the peritoneal cavity. The confirmed diagnosis was acute onset free perforation of the gallbladder. The perforation was probably caused by the small obstructing gallstone seen on ultrasound or by another small stone, which could not be visualized.
ABSTRACT
OBJECTIVE: We describe 2 patients with surgical injury to the lateral femoral cutaneous nerve (LFCN) after bone harvesting from the iliac crest for autologous bone grafting. DESIGN: A case-series of 2 patients and literature study of all anatomical variants of the LFCN in relation to the anterior superior iliac spine and inguinal ligament. SETTING: A teaching hospital in The Hague, the Netherlands. PATIENTS: Two patients with surgical injury to the LFCN after bone harvesting from the iliac crest for autologous bone grafting. RESULTS: All 9 known anatomical variations of the LFCN in the literature are reviewed, and the importance of these anatomical variations for surgeons and anesthetists is stressed. CONCLUSIONS: For every trauma, orthopedic, plastic, and cranio-maxillofacial surgeon and anesthesiologist it is important to know the anatomy of the LFCN and its known variations. To prevent injury of the LFCN during bone harvesting, the bone should be harvested 4-5 cm posterior to the anterior superior iliac spine and the incision should be parallel to the iliac crest. LEVEL OF EVIDENCE: Therapeutic Level V. See Instructions for Authors for a complete description of levels of evidence.
Subject(s)
Bone Transplantation/adverse effects , Femoral Nerve/abnormalities , Femoral Nerve/injuries , Ilium/transplantation , Peripheral Nerve Injuries/etiology , Skin/innervation , Female , Humans , Middle Aged , Peripheral Nerve Injuries/diagnosis , Transplantation, Autologous/adverse effects , Treatment OutcomeABSTRACT
Infectious complications, sepsis, and multiple organ dysfunction syndrome (MODS) remain important causes for morbidity and mortality in patients who survive the initial trauma. Increasing evidence suggests that genetic variants, particularly single nucleotide polymorphisms (SNPs), are critical determinants for interindividual differences in both inflammatory responses and clinical outcome in sepsis patients. Although the effect of SNPs on sepsis and MODS has been studied in many populations and diseases, this review aimed to summarize the current knowledge on the effect of SNPs on infectious complication specifically in trauma patients. A review of available literature was performed in PubMed database. The following genes have been studied in populations of trauma patients: CD14, HMGB1, IFNG, IL1A, IL1B, IL1RN, IL4, IL6, IL8, IL10, IL17F, IL18, MBL2, MASP2, FCN2, TLR1, TLR2, TLR4, TLR9, TNF, LTA, GR, MYLK, NLRP3, PRDX6, RAGE, HSPA1B, HSPA1L, HSP90, SERPINE1, IRAK1, IRAK3, VEGFA, LY96, ANGPT2, LBP, MicroRNA, and mtDNA. In this review, we discuss the genes of the Pattern Recognition Receptors, Signal Transducing Adaptor Proteins, and Inflammatory Cytokines of the innate immune system. A number of genetic variations have so far been studied in cohorts of trauma patients. Studies are often unique and numbers sometimes small. No definitive conclusions can be reached at this time about the influence of specific sequence variations on outcome in trauma patients.
Subject(s)
Multiple Organ Failure/genetics , Sepsis/genetics , Wounds and Injuries/genetics , Adaptor Proteins, Signal Transducing/genetics , Cytokines/genetics , Genetic Predisposition to Disease , Humans , Immunity, Innate/genetics , Immunity, Innate/immunology , Multiple Organ Failure/etiology , Multiple Organ Failure/immunology , Polymorphism, Single Nucleotide , Receptors, Pattern Recognition/genetics , Sepsis/etiology , Sepsis/immunology , Wound Infection/genetics , Wound Infection/immunology , Wounds and Injuries/complications , Wounds and Injuries/immunologyABSTRACT
Trauma is a major public health problem worldwide. Infectious complications, sepsis, and multiple organ dysfunction syndrome (MODS) remain important causes for morbidity and mortality in patients who survive the initial trauma. There is increasing evidence for the role of genetic variation in the innate immune system on infectious complications in severe trauma patients. We describe a trauma patient with multiple infectious complications caused by multiple micro-organisms leading to prolonged hospital stay with numerous treatments. This patient had multiple single nucleotide polymorphisms (SNPs) in the MBL2, MASP2, FCN2 and TLR2 genes, most likely contributing to increased susceptibility and severity of infectious disease.
ABSTRACT
The ritual of Kambô or Sapo is a type of voluntary envenomation. During this purification ritual a shaman healer, from various South American countries, deliberately burns the right shoulder with a glowing stick from a fireplace. Excretions of Phyllomedusa bicolor (or Giant Leaf Frog, Kambô or Sapo) are then applied to these fresh wounds. This ritual is used as a means of purification of the body, supposedly brings luck to hunters, increases stamina and enhances physical and sexual strength. All the peripheral and most of the central effects of the secretion can be ascribed to the exceptionally high content of active peptides, easily absorbed through burned skin. This article describes the ritual and the bio-active peptides from the secretion.
ABSTRACT
BACKGROUND: Sepsis and subsequent multiple-organ failure are the predominant causes of late mortality in trauma patients. Susceptibility and response to infection is, in part, heritable. Single-nucleotide polymorphisms (SNPs) in Toll-like receptor (TLR) and cluster of differentiation 14 (CD14) genes of innate immunity may play a key role. The aim of this study was to assess if SNPs in TLR/CD14 predisposed trauma patients to infection. METHODS: A prospective cohort of trauma patients (age 18-80 years; injury severity score [ISS] ≥ 16) admitted to a Level I trauma center between January 2008 and April 2011 was genotyped for SNPs in TLR2 (T-16934A and R753Q), TLR4 (D299G and T399I), TLR9 (T-1486C and T-1237C), and CD14 (C-159T) using high-resolution melting analysis. Association of genotype with prevalence of positive culture findings (gram positive, gram negative, fungi), systemic inflammatory response syndrome (SIRS), sepsis, septic shock, and mortality was tested with χ(2) and logistic regression analysis. RESULTS: Genotyping was performed for 219 patients, of whom 51% developed positive culture findings in sputum, wounds, blood, or urine. SIRS developed in 64%, sepsis in 36%, and septic shock in 17%. The TLR2 T-16934A TA genotype increased the risk of a gram-positive infection (odds ratio, 2.816; 95% confidence interval, 1.249-6.348; p = 0.013) and SIRS (odds ratio, 2.386; 95% confidence interval, 1.011-5.632; p = 0.047). Trends were noted for TLR9 and CD14 SNPs but did not reach statistical significance. Sepsis and septic shock were unrelated to any of the SNPs studied. CONCLUSION: Aberrant functioning of the TLR/CD14 pathway of innate immunity changes the risk of infectious complications in severely injured trauma patients. Of the seven SNPs studied, the TLR2 T-16934A increased the risk, the TLR9 T-1486C SNPs may decrease the risk, and TLR4 variation seemed unrelated to outcome. Early genotyping may prove to be helpful in the future in identifying polytraumatized patients at risk for infectious outcome. LEVEL OF EVIDENCE: Prognostic and epidemiologic study, level II.
Subject(s)
DNA/genetics , Genetic Predisposition to Disease , Polymorphism, Single Nucleotide , Sepsis/genetics , Toll-Like Receptors/genetics , Wound Infection/genetics , Wounds, Penetrating/complications , Adolescent , Adult , Aged , Aged, 80 and over , Female , Genotype , Humans , Immunity, Innate , Injury Severity Score , Lipopolysaccharide Receptors , Male , Middle Aged , Polymerase Chain Reaction , Prospective Studies , Sepsis/blood , Sepsis/etiology , Toll-Like Receptors/blood , Wound Infection/blood , Wound Infection/etiology , Wounds, Penetrating/diagnosis , Wounds, Penetrating/metabolism , Young AdultABSTRACT
A 53-year-old patient presented with a rupture of the left patellar tendon. The tendon was reconstructed using Krackow whipstitch sutures through drill holes in the patella.
Subject(s)
Patellar Ligament/injuries , Patellar Ligament/surgery , Suture Techniques , Accidental Falls , Humans , Male , Middle Aged , Rupture/surgery , Suture Anchors , Treatment OutcomeABSTRACT
A 17-year-old woman was hit on the left ankle during hockey. There was pain over the dorsal fibula, though no swelling. During dorsiflexion the tendon of the M. peroneus could be luxated over the lateral malleolus towards the front. This was consistent with a peroneal tendon dislocation at the level of the lateral malleolus.
Subject(s)
Ankle Injuries/diagnosis , Hockey , Tendon Injuries/diagnosis , Adolescent , Ankle Injuries/surgery , Ankle Injuries/therapy , Athletic Injuries , Casts, Surgical , Female , Humans , Tendon Injuries/surgery , Tendon Injuries/therapyABSTRACT
The ritual of Kambô or Sapo is a type of voluntary envenomation. During this purification ritual a shaman healer, from various South American countries, deliberately burns the right shoulder with a glowing stick from a fireplace. Excretions of Phyllomedusa bicolor (or Giant Leaf Frog, Kambô or Sapo) are then applied to these fresh wounds. This ritual is used as a means of purification of the body, supposedly brings luck to hunters, increases stamina and enhances physical and sexual strength. All the peripheral and most of the central effects of the secretion can be ascribed to the exceptionally high content of active peptides, easily absorbed through burned skin. This article describes the ritual and the bio-active peptides from the secretion.
Subject(s)
Humans , Peptides , Skin/injuries , Wounds and Injuries , Bodily Secretions/chemistry , Anura , PoisoningABSTRACT
The ritual of Kambô or Sapo is a type of voluntary envenomation. During this purification ritual a shaman healer, from various South American countries, deliberately burns the right shoulder with a glowing stick from a fireplace. Excretions of Phyllomedusa bicolor (or Giant Leaf Frog, Kambô or Sapo) are then applied to these fresh wounds. This ritual is used as a means of purification of the body, supposedly brings luck to hunters, increases stamina and enhances physical and sexual strength. All the peripheral and most of the central effects of the secretion can be ascribed to the exceptionally high content of active peptides, easily absorbed through burned skin. This article describes the ritual and the bio-active peptides from the secretion.
Subject(s)
Animals , Ceremonial Behavior , Poisoning , Skin/anatomy & histology , Bodily Secretions , Bufo rana/classificationABSTRACT
INTRODUCTION: Leiomyosarcoma of the venous system is rare, even more so in the greater saphenous vein. In the 85 years since van Ree described the first case in 1919 only 25 cases have been reported in the world. METHODS: We describe a case of an 85-year-old woman who was successfully treated by excision of the tumour. We also reviewed pertinent literature with regard to age, gender spread, tumour size, survival, occurrence of metastases and therapy. RESULTS: The median age was 54 years (range 2-85 years), with a 3:2 female to male ratio. The median size of the tumours was 4.1 cm (range 2-12 cm) and metastases occurred in seven of the 25 cases. If any form of adjuvant therapy is used it is usually radiotherapy. Chemotherapy seems to be reserved for cases where metastasis occurs. Average survival was 4 years (range 1 month to 17 years). Currently the best treatment seems to be wide excision of the tumour, with selective vascular reconstruction combined with adjuvant radiotherapy.