Articulated distraction of the hip joint in the treatment of benign aggressive tumors located around the hip joint

Introduction The proximal femur and acetabulum are frequent sites for benign active and aggressive lesions. The risk of pathologic fracture is great when a bone-destroying pathology involves an anatomic location such as the hip joint that undergoes profound mechanical loading. If the destruction involves a large area around the joint, secure fixation cannot be achieved with internal fixation implants. The study investigates use of articulated hip distraction to protect reconstructions performed for the treatment of benign active or aggressive tumors presenting with pathologic fracture.Patients and methods Five patients with a pathologic fracture of the proximal, intracapsular femur or the acetabulum were operated on at the authors' institution between 1997 and 1999. Following histopathologic approval of a benign tumor, all lesions were curetted, chemocauterized, and grafted and osteosynthesis was performed. The reconstruction was protected with an articulated hip distraction external fixator. All patients were mobilized in the immediate postoperative period.Results The patients were kept in external fixators for an average of 19.8 weeks (range: 16–24). The fixator was removed when bony consolidation was observed in anteroposterior and lateral x-rays of the lesion. The patients were followed for an average of 47 months (range: 38–56) after frame removal. None of the lesions recurred. At the last follow-up examination, all patients displayed an excellent function according to the Musculoskeletal Tumor Society Rating Scale. Conclusion According to the authors' knowledge, this investigation is the first in the literature describing the use of articulated joint distraction in the treatment of benign active and aggressive lesions around the hip joint. The procedure adopts principles of joint distraction into bone tumor surgery.     

Malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1

We report 4 cases of malignant peripheral nerve sheath tumors (MPNST) with neurofibromatosis type 1 (NF1). Mean age was 29.5. Two of them had a family history. Three of them were male. All of them had enlarging mass and pain in the background of neurofibromas. Locations were popliteal, thigh and forearm. The masses were greater than 5 cm in diameter in each case. In two cases the mass was showing continuity with a nerve. One patient had a nonossifying fibroma as well as a MPNST. Wide excision and radiotherapy were applied to three of the patients. One of them did not take any therapy after surgical resection. Two of the patients died of lung metastases after a mean period of 12.5 months. In a majority of NF1 patients MPNST emerges from a preexisting neurofibroma. The patients with NF1 are at greatest risk for developing sarcomas, so they should be followed closely.     

Clinical outcome of rhabdomyosarcoma in adolescent and adult patients: single center experience from Turkey

Rhabdomyosarcoma (RMS) is rare disease in adults (age >or= 16 years). The data from randomized prospective trials are scarce; the clinical outcome of these patients seems poor with the currently available treatment strategies. In this study, we report a single institution's experience in the treatment of adult RMS. We reviewed the medical records of patients with RMS who were >or= 16 years and have been treated in our institution between 1988 and 2003 retrospectively. We analyzed the survival outcome of these patients and the prognostic impact of clinical/pathological factors on their survival. In total, 23 patients with RMS were identified. Median age was 26 years (range, 16-72 years). Majority of patients were male (n: 17, 73.9%), and had large tumors (>or= 5 cm, n: 13, 56.5%), localized disease (N0, M0, n: 12, 52.2%), and embryonal histology (n: 10, 43.5%). Median overall survival was 31.3 months, and the 3-year progression-free survival and overall survival rates were 19.9% and 34.94%, respectively. Patients with smaller tumors (< 5 cm) (p < 0.04), local disease (p < 0.01), and normal lactic dehydrogenase (LDH) level (p < 0.01) at the time of diagnosis were found to have better survival outcome. The tumor size, serum LDH level, and metastatic disease at the time of diagnosis are potential predictors of outcome in patients with adult RMS. Adult RMS is an aggressive disease with poor survival despite treatment. The data from prospective, randomized multicenter trials are necessary in order to improve the clinical outcome of adult RMS patients.     

Polygonal triple (Kotz) osteotomy (over 10 years experience)

We evaluated the results of polygonal triple (Kotz) osteotomy for the treatment of acetabular dysplasia over 10 years. This study included 31 hips of 27 patients who had the Kotz osteotomy for acetabular dysplasia. The mean age was 21.5 years. We performed the original Kotz osteotomy for the first 22 hips (group I), while the modified Kotz osteotomy through an intra-pelvic approach without damage to the abductor muscle was applied for the last 9 hips (group II). Patients were evaluated by clinically and radiologically. The average follow-up was 106 months in group I, and 18 months in group II. The Trendelenburg gait was unchanged for four patients in group I and for one patient in group II. The Harris Hip Score improved in all patients postoperatively. Radiographic assesment showed improvement in both groups in terms of the angle of CE, VCE, and Sharp postoperatively(P<0.05). The complication rate per hip was 0.29. The original Kotz osteotomy achieves adequate coverage for the treatment of acetabular dysplasia, and patients are generally satisfied by this procedure. Nonetheless, the modified Kotz osteotomy provides recovery of the abductor muscle strength in the early postoperative period and subsequently decreases the rate of the Trendelenburg gait compared to the original Kotz osteotomy.

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Résumé Nous avons évalué le résultat de l’ostéotomie de Kotz dans le traitement des dysplasies acétabulaires sur une période de 10 ans. Cette étude a inclus 31 hanches chez 27 patients. L’age moyen était de 21.5 ans. Nous avons réalisé la technique originale de Kotz pour 22 patients (groupe 1) et la technique de Kotz modifiée chez 9 patients (groupe 2). Les patients ont été évalués sur le plan clinique et radiologique. Le suivi moyen a été de 106 mois pour le groupe 1 et de 18 mois pour le groupe 2. Une boiterie de type Trendelenbourg a persisté chez 4 patients du groupe 1 et 1 patient du groupe 2. Le score de Harris post opératoire a été amélioré chez tous les patients. L’amélioration radiographique a été constatée dans les deux groupes sur l’angle CE et VCE et l’angle de Sharp (P<0.05). Le taux de complication a été de 0.29. La technique originale de Kotz donne de bons résultats quant à la couverture de la hanche, cependant la technique de Kotz modifiée permet de récupérer plus rapidement une force importante des muscles abducteurs en post opératoire et ce fait diminue le pourcentage de patients présentant une boiterie de type Trendelenbourg.

     

Enhancement effects of nicotine on neurogenic contractile responses in rabbit gastric fundus

Nicotine, a nicotinic acetylcholine receptor agonist, plays a role in the modulation of neurotransmitter release following nerve stimulation in both the central and the peripheral nervous system. Nitric oxide and prostaglandins modulate the release of various neurotransmitters in different tissues. We aimed to investigate the effects of nicotine on neurogenic contractile responses via nicotinic acetylcholine receptors and, if a change occurred, to investigate the effects of N(W)-nitro-L-arginine methyl ester (L-NAME) and indomethacin on this change in rabbit gastric fundus. Electrical field stimulation (EFS)-evoked contractile responses were recorded from gastric fundus strips obtained from rabbits with an isometric force displacement transducer. Nicotine was applied to preparations at varying concentrations. Then, the effects of hexamethonium, cadmium (Cd(2+)), indomethacin, and L-NAME were tested on the EFS-evoked contractions in the presence of nicotine. Nicotine-induced transient neurogenic contractions in a dose-dependent manner. Cd(2+) and hexamethonium inhibited nicotine-induced transient neurogenic contractions, but indomethacin and L-NAME produced no effect. In conclusion, nicotine increased EFS-evoked contractile responses, possibly by facilitating neurotransmitter release from nerve terminals by a mechanism dependent on the influx of Ca(2+) from voltage-gated Ca(2+) channels via activation of nicotinic acetylcholine receptors in isolated rabbit gastric fundus. Endogenous nitric oxide and prostaglandins do not play a physiological role in the regulation of this neurotransmitter release.     

Unusual localization of an aneurysmal bone cyst in ulnar coronoid process

Aneurysmal bone cysts are non-neoplastic, expansile, osteolytic tumor-like conditions. All bones may be involved, but the most commonly affected is the metaphysis of the long bones, especially the tibia, humerus and femur. We present a 13-year-old female patient with a cystic lesion with cortical continuity and a large bone cyst in the coronoid process of the ulna. The bone cyst was treated with curettage, phenol application and cement implantation following cyst debulking. Aneurysmal bone tumors are rare tumor-like conditions and localization at the coronoid process of the ulna with mechanical block of the elbow motion has yet to be reported. Debulking and curettage of the lesion and bone cement implantation are useful methods for local control of aneurysmal bone cysts.     

Familial tumoral calcinosis in three patients in the same family

Tumoral calcinosis is a rare condition characterized by large calcific soft tissue deposits occurring predominantly in a periarticular location. Familial tumoral calcinosis was detected in three members of a family, namely, the father and two offsprings. The father underwent many operations since age 10 for occurrences or recurrences of mass lesions in the right posterior elbow, both hips, left gluteal region, and perineal region. His 16-year-old son underwent his first operation at the age of 10 for a lesion in the posterior elbow, which recurred at the same site and required another operation two years later. He underwent subsequent surgeries for lesions that appeared in the right elbow and right dorsal foot. Finally, the 12-year-old daughter was treated with surgery for a lesion in the right dorsal foot. In all the patients, pathologic diagnoses of all surgical specimens were reported as tumoral calcinosis. All had normocalcemia, hyperphosphatemia, and D hypervitaminosis.     

Midfoot reconstruction with free vascularized fibular graft after wide resection: a case report

Malignant foot tumours are often treated with amputations due to anatomical difficulties. Limb salvage techniques are difficult to perform, as a stable, sensible and plantigrade foot should be obtained to prevent further problems.In this report, we present a midfoot reconstruction with a vascularized free fibula, osteomized in a V-shape after wide resection of a midfoot synovial sarcoma. We describe the reconstruction of both longitudinal arches in which using a vascularized autograft facilitated union, remodeling of the bone, and obtaining a functional foot.