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We report the results of a prospective Tunisian study using primary chemotherapy followed by conservative surgery in primitive limb osteosarcoma. From January 1988 to January 1998, 56 patients affected by limb osteosarcoma entered in a prospective study of neoadjuvant chemotherapy with the T10 protocol before surgery with a conservative intent. Initial work-up include: clinical exam with tumor measurements, chest and limb X-rays, limb CT-scan or MRI, chest CT-scan, bone scintigraphy and hematological and renal biological exams. Patients receive pre- and post-operative chemotherapy according to the T10 modified protocol. Fifty-six patients (33 M/23 F) with a mean age of 19 years (8 to 28) are included. Mean clinical and radiological tumor size is around 14 cm. Main histologic type is classic osteosarcoma (50% of cases) and 10 patients (9%) presented with initial metastasis; 42 patients on 56 receive the whole pre-operative protocol. Treatment is well tolerated excluding 18 episodes of mucositis, 29 of leucopenia (grade 3), 7 of thrombopenia (grade 3), 4 of cutaneous toxicity, 2 of pulmonary toxicity and 3 of nausea-vomiting. We observe 36% of good histological responders and 64% of bad responders to primary chemotherapy, 27 patients on 49 operated (53%) have a conservative surgery and 18 (47%) a radical surgery. With a median follow-up of 51 months (8 to 128), 29 patients remain alive free of disease (15/17 GR and 14/30 BR), 2 are alive with disease, 2 died by toxicity, 14 died by progressive disease and 9 are lost to follow-up with evolutive disease. Five year disease-free survival is 55% for the 46 non metastatic patients. In univariate analysis, seric alkaline phosphatase level (p = 0.0014) and histological response to chemotherapy (p = 0.0218) are significant factors for prognosis.

A variety of tissues in bone can be the origin of neoplastic primitive lesions. Consequently, it can be the site of various sarcomas of tissue type, incursing leiomyosarcoma. The leiomyosarcoma of the bone is considered as primitive after exclusion either a bony extension of a soft tissue tumor of vicinity or the presence of a leiomyosarcoma elsewhere. We report a case of a primary leiomyosarcoma arising on the left femur of a 40-year-old woman. The diagnosis was confirmed by immuno-histochemistry and electron microscopic study. A bloc resection followed by a prothesis was performed. The patient underwent a fractioned postoperative radiotherapy with a total dose of 75 Gy. The postoperative course one year later revealed local recurrence and pulmonary metastases. Despite the resection of pulmonary metastases and the amputation of left lower limb, the patient had developed local recurrence on the stump of amputated limb and was on the outside of all therapeutic resource. In this report, we present the clinicopathologic, immuno-histochemical and ultrastructural profiles of these rare primary bone tumors.

The authors report the results of a retrospective study of 36 cases of tibiotalar arthrodesis performed in 22 men and 14 women with an average age of 32 years. All patients were reviewed with an average of 8.5 years follow-up. The predominating etiologies were ankle osteoarthritis (15 cases) and neurologic deformities of the foot (13 cases). Arthrodesis was performed using the Meary technique in 60% of cases, using the Charnley technique in 20% and the Crawford-Adams technique or with clamps in the other cases. Fusion was obtained in 97% of cases. Long-term results were assessed using Duquennoy et al.'s scoring system. They were very good or good in 58% of cases, fair in 31% and poor in 11%. The study of distal repercussions of tibiotalar arthrodesis shows progressive deterioration of the subtalar joint in 70% of cases and appearance or increase of degenerative changes in 75% of cases. The final results of the procedure depend on this deterioration; the latter is related with the arthrodesis position. Midtalar joint is a compensation joint showing hypermobility in 40% of cases. Degenerative changes were limited and asymptomatic in 80% of cases. Based on the findings in this study and on the literature, we conclude that the foot should be fixed at 90 degrees or with less than 5 degrees of equinus, with 5 degrees of valgus and 10 to 15 degrees of external rotation.

The distal end of the radius is one of the sites commonly affected by giant-cell tumor of bone; it poses problems of reconstruction after resection of the distal radius. The authors present a retrospective study of 5 giant cells tumors treated by resection-arthrodesis of the wrist. These tumors were seen in 3 men and 2 women. From the radiological point of view, all tumors were extensive and lytic lesions centered in the epiphysis and involving the metaphysis; the wrist joint was respected. From the histological point of view, the tumors were grade I in 3 cases and grade II in 2 cases. With a mean follow up of 8 years, the results were considered good in 3 cases, fair in 1 case and poor in 1 case. A high incidence of bone complications was noted: delayed union (up to 11 months) and fracture of the graft, which occurred in 3 patients.

The authors report 10 segmental fractures of the femoral head associated with a dislocation of the hip. Using PIPKIN's classification, they noted 1 type I, 7 type II and 2 type III fractures. The initial treatment of the hip dislocation was conservative in all cases. Failure of reduction was noted in 6 cases and was complicated by fracture of the femoral neck in 2 cases. The attitude regarding the head fragment was: conservative in 5 cases, the reduction was considered satisfactory in 4 cases and poor in 1 case; surgical in the other 5 cases: screw fixation in 1 case, excision of the fragment in 1 case and prosthetic replacement of the femoral head in 3 cases. After a mean follow-up of 5 years, functional results were satisfactory in the majority of cases. The radiological assessment showed malunion without osteoarthrosis in one case and avascular necrosis of the femoral head in three cases. The authors propose a treatment policy based upon their experience and a review of the literature.

The purpose of the study was to clarify the clinico pathological features of periosteal ganglion. The authors present a case of an unusual diaphyseal bony tumor in a 27 years old man. The diagnosis of diaphyseal periosteal ganglion cyst was made on roentgenographic appearance, presence of mucoid material and histologic evidence of a synovial type cellular lining. The patient was successfully treated with excision. The follow-up examination showed no recurrence one year after surgery. The radiological differential diagnosis were considered and possible pathogenes for a periosteal ganglion was discussed. Periosteal ganglia must be considered as a cause of cortical erosions. This lesion may radiologically mimic other periosteal lesions. Treatment with excision is successful in most cases.

This paper presents a short study of 17 cases of tuberculosis of the greater trochanter seen during a period of 19 years at the National Orthopaedic Institute in Tunis. The onset of the infection is slow with patients presenting at a mean of 7 years after initial symptoms. The diagnosis is confirmed by biopsy and culture of the organism. Management is based on treatment with antituberculous drugs, although surgical excision of the lesion is sometimes required. Successful resolution of the symptoms is usually achieved unless the hip joint becomes involved. Our patients showed good results at a mean follow up of 5 1/2 years.

Spinal osteoid osteomas are rare. We report 21 cases, that have been studied and treated since 1985. The tumor affects young people, who are less than 25 years old. The pain is the most common sign. Diagnosis is difficult, but can be made by radiology. X ray sometimes enables to suspect the diagnosis. Scintigraphy shows a focus hyperfixation more suggestive. Computed tomography enables the diagnosis by showing a bony lacuna surrounded by osteosclerosis. Moreover, computed tomography localizes very well the nidus of the osteoid osteoma, and guides the surgical treatment. Since computed tomography, other techniques like angiography and tomography are less used for diagnosis.

An exceptional case of vertebral hydatidosis with involvement of the cervical, dorsal, lumbar and sacral segments of the spine is presented. Vertebral hydatidosis is a rare form of hydatid disease. It is found in less than 2% of all cases in echinococcosis. The initial parasite localization is usually univertebral. Extensive primary lesions were related to massive infestation by tapeworm ova at multivertebral levels. At this stage, the disease presents as a malignant tumor resistant to all surgical procedures. The prognosis is poor because of severe neurological complications and inexorable spinal destruction. The hope remains that in the future an effective medical parasiticidal treatment will be available.

A case of melanotic tumour was described localized close to the right cervical spine, destroying C6 and C7 vertebral bodies and pedicles, on a 27 years old woman. Histological and immunohistochemical findings, correlated with clinical and X-rays data, led up to the diagnosis of a melanotic schwannoma. Six years after the beginning of the illness, the outcome was preceded by a local vertebral involvement without metastasis and paradoxically with a good general state. The tumoral localization and the finding of nervous structures allowed to evoke a development from a cervical nerve root. On histological basis, we propose to classify this tumour along the new concept described by the Mayo Clinic pathologists team, under the name of psammomatous melanotic schwannoma. Some authors evoke a congenital outset in a context of endocrine disorders.

Summary Introduction As osteoclast, giant cell tumors express calcitonin receptors. The aim of this paper is to assess treatment using salmon calcitonin after curettage. Material and methods We retrospectively reviewed 25 patients with giant cell tumor of the appendicular skeleton treated with a single protocol of calcitonin administration following curettage in order to assess the effectiveness of calcitonin in reducing the rate of local recurrence. Results The mean duration follow-up was 68 months. Thirteen patients (52%) had local recurrence. Eight of them were treated successfully after repeated curettage and calcitonin. Four patients had bone resection and one patient had curettage and cement filling. All patients with cavity left empty had ossified and the functional score as assessed by the MSTS score was 28.02/30. Conclusion This study suggests that the use of calcitonin as adjuvant is not effective and that filling agents are not required after curettage of giant cell tumors. Level of evidence Level 4.

Two brothers and a sister with spondyloepiphyseal dysplasia tarda who subsequently developed severe generalised peripheral arthropathy are described. The condition appeared to be transmitted as an autosomal recessive trait.