[A giant sacral chordoma resection and reconstruction with a gluteal perforator flap, a case report and literature review]. / Chordome géant du sacrum et reconstruction par lambeau perforant glutéal supérieur, à propos d'un cas clinique et revue de la littérature.

BACKGROUND: Sacral chordomas are rare primary bone tumors and represent more than half of all primary malignant sacral tumors. Surgical resection is the only treatment with close to 50% of remission at 10 years, with or without radiotherapy. This tissue removal can be very extensive and morbid, particularly for evolved tumors. The reconstruction mostly uses myocutaneous flaps, notably the gluteus maximus flap and the latissimus dorsi flap, increasing morbidity of the surgical procedure. To avoid a muscular sacrifice and reduce the post-surgical morbidity, we describe the case of a patient who underwent a giant sacral chordoma resection and a reconstruction with a superior gluteal artery perforator flap. CASE REPORT: A 57-y.o. patient with a voluminous sacral chordoma had undergone a partial sacrectomy and abdomino-perineal resection. Firstly, a laparoscopy was realized to create a colostomy, to dissect an omental flap and to prepare the monobloc resection. In a prone position, the resection of the tumor was achieved and a de-epithelialized superior gluteal artery perforator flap was performed to fill the space and to support pelvic organs. CONCLUSION: For resections of sacral chordomas, coelioscopy has considerably reduced the surgical morbidity. However, the majority of reconstructions use myocutaneous flaps, specifically gluteus maximus and latissimus dorsi, which their postural function is considerable. Muscular sacrifice can lead to functional impotence with difficulty walking and standing up and run contrary to the diminution morbidity initiated by oncologic surgeons.

Late local recurrence, at 19 and 17 years, of sacral chordoma treated by en bloc resection.

UNLABELLED: Sacral chordoma (SC) is a malignant bone tumor with high risk of local recurrence (LR) even after en bloc resection, generally in the first 10 years after resection. We report two cases of late LR, at 17 and 19 years. Two male patients, aged 45 and 53 years, presented with large SC needing a combined approach for en bloc resection. Surgical margins were safe for the first patient and borderline for the second. The patients had yearly follow-up. The first patient developed LR on the posterior wall of the right acetabulum and the second developed LR in the right sciatic notch, at 17 and 19 years, respectively. These two cases of very late LR of SC advocate for yearly screening of patients even more than 20 years after resection. LEVEL OF EVIDENCE: IV (case report).

Intensive induction chemotherapy without methotrexate in adult patients with localized osteosarcoma: results of the Institut Gustave-Roussy phase II trial.

PURPOSE: To improve outcomes in localized osteosarcoma and to reduce the duration of preoperative chemotherapy, we conducted a phase ii trial assessing the efficacy of an intensive protracted regimen without methotrexate (api-ai regimen) in adolescent and adult patients with newly diagnosed disease. PATIENTS AND METHODS: Induction chemotherapy consisted of 2 cycles (4 courses) of doxorubicin 60 mg/m(2) (days 1 and 15), cisplatin 100 mg/m(2) (day 1), and ifosfamide 5 g/m(2) (days 2 and 15). The primary endpoint was good histologic response [ghr (≤5% identifiable tumour cells)]. RESULTS: From March 1993 to March 2000, 32 patients [median age: 21 years (range: 15-49 years)] were administered 126 induction courses. The median time between chemotherapy courses was 15 days (range: 12-32 days). All but 3 patients underwent conservative surgery. Toxicity was mainly hematologic, with febrile neutropenia occurring in 35% of patients and grades 3-4 thrombocytopenia in 35%. The ghr rate was 47%. The median follow-up was 64 months (range: 30-115 months). The 5-year event-free and overall survivals were 65% [95% confidence interval (ci): 48%-79%] and 69% (95% ci: 50%-83%) respectively. Two secondary hematologic malignancies occurred: 1 acute myelocytic leukemia (M5) in a poor responder with concomitant relapse, and 1 myelodysplastic syndrome in a patient achieving ghr. CONCLUSIONS: Despite hematologic toxicity, the results observed with the api-ai regimen compare favourably with those observed during previous induction chemotherapy containing methotrexate in adult patients and the pediatric population treated at our institution. These promising results have to be validated by an ongoing national multicentre trial coordinated by the French Sarcoma Group.

[Surgical treatment of stiff massive knee endoprosthesis for bone tumors in children and adolescents]. / Traitement chirurgical de la raideur après prothèse massive du genou pour tumeur osseuse chez l'enfant et l'adolescent.

PURPOSE OF THE STUDY: Improved oncologic outcome and technical advances in limb salvage surgery have made limb salvage therapy a feasible and valuable treatment option. Nevertheless, resection of a bone tumor followed by a reconstruction knee endoprosthesis can create gait abnormalities, of which one of the most frequent is knee stiffness. The aim of this retrospective study was to assess the outcomes of revision surgery for a stiff knee following reconstruction of a segmental long bone defect. PATIENTS AND METHODS: Between 1983 and 2005, 19 patients who had undergone wide resection of a tumor close to the knee followed by reconstruction with a massive endoprosthesis were revised for a diagnosis of stiffness. RESULTS: The mean age of the patients was 12 years (range: 7-19 years). Patients were followed for a mean five years (range: 1-21 years). Three patients were not assessed at the last follow up (two patients died, one patient was amputated for a local recurrence). The mean range of motion improved 80+/-24 degrees preoperatively to postoperatively. The Enneking score improved from 15+/-0.5 to 23+/-3 points at three months follow-up, and to 22+/-5 at last follow-up. Recurrent stiffness occurred three times and required a second operative release with a good final result. DISCUSSION: Outcome depends on the cause of the stiffness of the reconstruction knee arthroplasty. Stiffness can be caused by complications (trauma, implant failure, infection), and patient-related factors (lack of physiotherapy). Open arthrolysis is indicated for chronic stiffness in a motivated patient with an identified cause because failure to identify the cause of stiffness may result in recurrence of the problem.

Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients.

OBJECTIVE: To evaluate the impact of surgery as first-line treatment on event-free survival (EFS) of primary aggressive fibromatosis. PATIENTS AND METHODS: Treatments were categorized into: surgery with or without radiotherapy and nonsurgical strategies with systemic treatment alone or wait and see policy. Eighty-nine patients had initial resection of their primary tumour followed by postoperative radiotherapy in 13 cases. Twenty-three did not undergo surgery but received systemic treatment or watch and wait policy. RESULTS: Median follow-up was 76 months. Overall 3 years EFS was 49%. In the univariate analysis, patients with microscopically complete surgery had a similar outcome to patients in the no-surgery group (3 years EFS of 65% and 68%, respectively). Gender, age, tumour size, treatment period and strategy (surgery versus no-surgery) were not statistically significant. Quality of resection according to margins and the tumour site were the only prognostic factors. There was a significant correlation between tumour site and quality of surgery (p=0.0002). CONCLUSIONS: A subset of patients with extra-abdominal fibromatosis could be managed with a nonaggressive policy, as growth arrest concerned 2/3 of nonoperated patients. When surgery is finally necessary, it should be performed with the aim of achieving negative margins.

Imaging of sacral tumours.

All components of the sacrum (bone, cartilage, bone marrow, meninges, nerves, notochord remnants, etc.) can give rise to benign or malignant tumours. Bone metastases and intraosseous sites of haematological malignancies, lymphoma and multiple myeloma are the most frequent aetiologies, while primary bone tumours and meningeal or nerve tumours are less common. Some histological types have a predilection for the sacrum, especially chordoma and giant cell tumour. Clinical signs are usually minor, and sacral tumours are often discovered in the context of nerve root or pelvic organ compression. The roles of conventional radiology, CT and MRI are described and compared with the histological features of the main tumours. The impact of imaging on treatment decisions and follow-up is also reviewed.

[Function preserving surgery of limb sarcomas: history and improvements]. / Chirurgie des sarcomes des tissus mous des membres chez l'adulte : historique et avancées thérapeutiques.

In the seventies, limb-sparing surgery and radiation therapy appeared capable of successfully treating the great majority of adult patients with soft tissue sarcomas of the extremities. No survival benefit could be demonstrated in the patients selected for amputation. Microscopically positive surgical margins are related to a greater risk of local recurrence, whereas overall survival is compromised by high grade and large tumor size. Presurgery multidisciplinary discussion, management in reference center and management within cancer network independently predict conformity to the clinical practice guidelines. Reoperation should be a planned part of definitive management whenever the initial surgical procedure was done without a histologic diagnosis or was not planned to be a wide excision. Soft tissue flap reconstruction facilitates therapy for patients with locally advanced tumors so that patients with larger tumors can undergo resection, limiting complications and limb sacrifice. Vascular reconstruction is a feasible option in case of involvement of major vessels. Isolated limb perfusion (ILP) with TNF and chemotherapy is an efficient limb-sparing neoadjuvant therapy for locally advanced limb soft tissue sarcomas. Efficacy and safety of low-dose TNFalpha could greatly facilitate ILP procedures in the near future.

Surgical excision of bone sarcomas involving the sacroiliac joint.

Adequate (wide or marginal and uncontaminated) margins and reconstruction are difficult to achieve when performing an internal hemipelvectomy for bone sarcomas involving the sacroiliac joint. We evaluated whether adequate surgical margins could be achieved and if functional outcomes could be predicted based on the type of resection and reconstruction. Forty patients had resections of the sacroiliac joint. Vertical sacral osteotomies were through the sacral wing (n = 2), ipsilateral sacral foramina (n = 27), sacral midline (n = 9), or contralateral foramina (n = 2). Iliac resections were Type I, Type I-II with partial or total acetabular re-section, or Type I-II-III. Surgical margins were adequate in 28 of 38 patients (74%), two (7%) of whom experienced local recurrence, compared with seven of 10 (70%) patients with inadequate margins. Reconstruction consisted of restoring continuity between the spine and pelvis. Resection of the entire acetabulum and removal of the lumbosacral trunk were the two main determinants of function, as assessed using the Musculoskeletal Tumor Society score. There were no life-threatening or function-threatening complications. Internal hemipelvectomy with a limb salvage procedure can be achieved with adequate surgical margins in selected patients. Functional outcomes can be predicted based on the type of resection and reconstruction, which helps the surgeon plan the procedure and inform the patient.

Rotationplasty after failure of a knee prosthesis for a malignant tumour of the distal femur.

Failure of massive knee endoprostheses implanted for malignant tumours of the distal femur in children presents a difficult problem. We present the results of rotationplasty undertaken under these circumstances in four boys. They had been treated initially at a mean age of 9.5 years for a stage-IIB malignant tumour of the distal femur by resection and implantation of a massive knee endoprosthesis. After a mean period of eight years and a mean of four operative procedures, there was failure of the endoprosthesis because of aseptic loosening in two and infection in two. Function was poor with a mean Musculoskeletal Tumor Society score of 7.5/30, and considerable associated psychological problems. At a mean follow-up of 4.5 years after rotationplasty there was excellent function with a mean score of 27.5/30 and resolution of the psychological problems.

Recurrence of a primary malignant giant cell tumour of bone 14 years after initial surgery.

Giant cell tumour (GCT) is usually considered a benign entity. A small fraction of these tumours become malignant with time, and an extremely rare fraction may be malignant at onset. We report herein an unusual case of primary malignant GCT of the bone that relapsed locally with the same histology 14 years after a simple surgical curettage.

Posterior lumbar osteotomy for flat back in adults.

Several studies describe sagittal realignment for flat back and related kyphotic decompensation. Official guidelines for sagittal and the frontal realignment have not been developed. In this retrospective study, the authors examined 10 patients with flat back syndrome and treated a related kyphotic decompensation syndrome by posterior wedge osteotomy. The authors' goal was to determine the efficacy of one-level osteotomy on sagittal and frontal realignment. The effectiveness of osteotomy performed mainly in L3 or L4 was measured in terms of radiographic sagittal and frontal alignment. Sagittal unbalance was improved in all patients. The correction is related to the restitution of lumbar lordosis (5 degrees of lordosis allows 1 cm of sagittal correction). However, frontal balance is difficult to restore or even to maintain. In one patient, it was worsened and required repeated operation. One-level lumbar osteotomy is a safe procedure to correct sagittal unbalance. Peroperative lordosis correction allows reliable correction planning. The remaining problem is planning for frontal balance correction.

Knee reconstruction with prosthesis and muscle flap after total arthrectomy.

A massive prosthesis and medial gastrocnemius muscle transfer were used to reconstruct the knee after extracapsular en bloc excision for bone sarcoma. Magnetic resonance images showed intraarticular involvement. This technique was used in nine patients, six men and three women aged 18 to 51 years, with primary malignant bone tumors of the knee. Extraarticular resection of the knee, including the patella, was done in every case. A knee prosthesis was implanted, and the extensor mechanism was reconstructed by transfer of the medial gastrocnemius muscle and pes anserinus tendons. All resections had negative margins. There were no local recurrences, but metastases occurred in two patients. Infection was the only major complication and was seen in two patients. The mean postoperative Musculoskeletal Tumor Society score was 61% (range, 36%-100%). The mean postoperative range of flexion was 62 degrees (range, 30 degrees-90 degrees), and the mean extensor lag was 12 degrees (range, 0 degrees-40 degrees). Three patients required a crutch to walk. The functional outcome was poor in the two patients whose proximal tibia was removed with the joint, suggesting that arthrodesis may be best in this situation. In properly selected patients, prosthesis and muscle flap reconstruction provides acceptable function and a good cosmetic result.

[Iatrogenic tumor metastasis to the pelvis after treatment for hand osteosarcoma. A case report]. / Inoculation tumorale iatrogène du bassin après cure d'un ostéosarcome de la main. A propos d'un cas.

In this study, the case has been examined of a 49-year old male who initially presented with a pathological fracture of the second metacarpal. This was first treated by curettage, iliac bone graft and internal fixation, without any complementary investigation being carried out, i.e., no preliminary biopsy and histological diagnosis were made. The results of this inadequate approach were poor: neither satisfactory fixation of the fracture nor control of the primary lesion were obtained. Moreover, the definitive diagnosis was only made four months later, when biopsy findings confirmed the presence of an osteosarcoma. After neoadjuvant chemotherapy, an en-bloc resection of the second metatarsal and the trapezoid bone was carried out. At ten months post-surgery, one and a half years after the original fracture, the hand was found to be disease-free and functioning satisfactory. However, nearly a year later the discovery of a large tumor mass at the site where the iliac bone graft was originally taken necessitated resection of the hemi-pelvis, with chemotherapy prior to resection and radiotherapy following surgery. Unfortunately, this salvage procedure did not limit the spread of the disease, and subsequent pulmonary and vertebral metastases were found, leading to the death of the patient three years after the initial fracture. This particular case underlines the fact that the basic rules for the management of malignant tumors should be taken into consideration from the onset, so that a catastrophic prognosis such as that described can be avoided.

Postoperative radiotherapy in the management of adult soft tissue sarcoma of the extremities: results with two different total dose, fractionation, and overall treatment time schedules.

PURPOSE: This retrospective study was performed to evaluate two postoperative radiotherapy schedules in terms of dose, fractionation, and overall treatment time in soft tissue sarcoma (STS) of the extremities. METHODS AND MATERIALS: Between January 1984 and December 1993, 62 patients with newly diagnosed localized STS of the extremities were treated with maximal conservative surgery and postoperative radiotherapy (RT). Forty-five patients received 50 Gy with conventional fractionation plus a boost dose (5 to 20 Gy). Seventeen patients had hyperfractionated accelerated radiotherapy (HFART) up to a dose of 45 Gy in 3 weeks. RESULTS: With a median follow-up of 72 months, the 5-year local failure rate was 25%, the 5-year disease-free and overall survival rates were respectively 42% and 62%. The 3-year local relapse, disease-free, and overall survival rates were respectively 16%, 44%, and 70% in the conventional radiotherapy group, and 36%, 47%, and 82% in the HFART group (NS). No factor significantly influenced local control with a trend, however, in favor of conventional RT (p = 0.10). CONCLUSION: HFART at the dose of 45 Gy does not seem to be superior to the standard RT schedule, neither in terms of local control, survival, nor in terms of long-term side effects. However this dose could be considered too low as well as the power of comparison between the two groups to draw definitive conclusions.

Management of cancer in pregnancy: a case of Ewing's sarcoma of the pelvis in the third trimester.

Ewing's sarcoma of the pelvic bones was diagnosed in a 21-year childbearing woman, raising major medical and ethical problems. The diagnostic and therapeutic approaches during the sixth month of gestation were tailored in order to cure the patient and avoid unnecessary toxicity to the fetus. Ancillary tests included ultrasound and MRI studies of the pelvis. Ifosfamide and adriamycin, premedicated by granisetron, were administered during gestation, and were found to be safe. Cesarean section was the preferred way of delivery since the tumor involved the pelvic bones. The outcome was a disease-free patient and a small healthy baby who is now two years of age.

[Clinical imaging of osteo-condensed metastases]. / Imagerie clinique des métastases ostéocondensantes.

INTRODUCTION: Due to the occurrence of osteoblastic metastases in the course of various cancers, particularly in the course of prostate cancer, we are faced with diagnosis and follow-up issues different from those associated with lytic metastasis. We therefore analyzed the respective advantages of imaging techniques. CURRENT KNOWLEDGE AND KEY POINTS: Most of the time, osteoblastic metastases are evidenced by standard radiography. Due to its ability to demonstrate metastases localization, extent and signs, CT scan is not only of value when osteoblastic metastases are suspected but also for patient's follow-up. MRI provides further information in regard to both the lesion content and osteoblastic degree. Though MRI must be performed after all other imaging procedures, it is of value for multiplanar study of the whole spine. FUTURE PROSPECTS AND PROJECTS: Studies focusing on either the lesion content and volume or helical CT are in progress and aim at better monitoring follow-up, while the objective of dynamic MRI studies is to better analyze lesion content.

[Desmoid tumor of the thoracic wall. A case manifesting as painful stiffness of the shoulder]. / Tumeur desmoïde de la paroi thoracique. Un cas révélé par un enraidissement douloureux de l'épaule.

BACKGROUND: Desmoid tumors are uncommon fibromatous tumors arising from musculoaponeurotic tissue characterized by spindle cell fibroblast and myofibroblast proliferation. The cause is unknown (trauma, hormonal factors, genetic anomaly...). Locally invasive, they tend to recur increasing morbidity or even mortality. CASE REPORT: Stiff shoulder resulting from blockage of the scapulothoracic articulation was the inaugural sign. Three-phase bone scintigraphy demonstrated early uptake in the soft tissue in contact with the scapula. Magnetic resonance imaging and biopsy confirmed the diagnosis of desmoid tumor. Complete resection was performed. No adjuvant radiotherapy nor hormone treatment were given since no antiestrogen receptor antibodies were identified on the surgical specimen. DISCUSSION: The clinical manifestation (stiff shoulder) and the scapular localization observed in this case are unusual for desmoid tumors. Initially tendinopathy of the shoulder, acromio-clavicular arthropathy and capsular retraction of the shoulder joint had been entertained. Early diagnosis and wide surgical resection are indicated in desmoid tumors.

Red marrow recolonization induced by growth factors mimicking an increase in tumor volume during preoperative chemotherapy: MR study.

Growth factors associated with chemotherapy may induce red marrow reconversion. This may simulate a tumor increase on MRI. We report such a case.

[Reconstruction of the long bones of the legs in children via free vascularized transplant of the fibula after resection for sarcoma]. / Reconstruction des os longs des membres, chez l'enfant, par transplant libre vascularisé du péroné après résection pour sarcome.

Nineteen children were operated on between 1985 and 1994. All the patients presented a sarcoma of long bones: osteosarcoma: 12 and Ewing's sarcoma: 7. They were operated on: resection and reconstruction of the long bones with a free vascularised fibula. Pre and post-operative chemotherapy was used. The average follow-up was 3 years (between 10 years and 1 year). The mean bone defect was 20 cm (between 32 and 11 cm). Boys were more frequently encountered [12]. Average age was 9.5 years. The pathological bone was: femur: 14, tibia: 4, humerus 1. The approach needs usually two incisions for femoral bone: the internal incision allows us to prepare the recipient vessels: deep femoral vessels in 11 cases. In 18 cases, union occurred in less than 14 months. One case of pseudarthrosis occurred. Immediate follow-up was simple in 17 cases. Infection was observed in 2 cases. Secondly, the most frequent complications were: fracture of the fibula: 6 cases, vicious cal: 2 cases; delayed union: 6 cases; stiffness: 2 cases. One patient died later from pulmonary metastasis. Solid osteosynthesis of the member and of the free vascularised fibula permit to shorten these delays. Success depends on two criteria: the graft thickening of the fibula and union of the fibula at both ends. The micro vascular anastomoses must be excellent. Twelve children had bone union with only one operation. In the 6 cases of delayed union, secondary bone grafts give consolidation. The comparison of this technique with standard treatment showed an evident superiority of the free vascularised fibula transplant in extensive defects of bone. The fibula is a life bone which permits rapid union with a short hospitalization, a quick recovery and an early return to school. Free vascularised fibula permit to ameliorate the quality of survival.

Local control after surgical treatment of spinal metastatic disease.

Surgery was carried out on 118 patients with spinal metastatic diseases; 80 operations were palliative and 38 were curative. All patients who survived 1 year or more and all those who had local recurrence before dying (total n = 58) were included in this study. The aim of the study was to identify the factors that determine the success of local control in order to develop a new technique that could prevent local recurrence. From among the different factors that may influence the oncological result, a retrospective study concentrated particularly on the following items: sex of the patient, location and extension of the tumor on the spine, tumor involvement on the vertebra itself, and quality of tumor excision. These factors seem to have no or little influence on local control. However, sensitivity of the primary cancer to adjuvant treatments (e.g., chemotherapy, radiation therapy, hormonotherapy) and correct timing of the radiation therapy, which must be performed after, rather than before, surgery, seem to improve local control significantly. The authors therefore suggest two options for treatment. When the primary cancer is sensitive to adjuvant treatments, 'palliative' surgery with posterior fixation and nerve decompression seems sufficient to attain good function and adequate oncological results. On the other hand, when the primary cancer is resistant to adjuvant treatment or when the lesion recurs after radiation therapy, more aggressive surgery must be carried out. Complete excision of the tumor after embolization, with may be even associated local chemotherapy, is required. However, even when this is carried out, local control is difficult to achieve.