Goals: Sarcomatous degeneration of giant cell tumours (GCT) occurs rarely. It occurs in less than 1% of the cases, and most of them are GCT previously treated with radiotherapy. The goal of this presentation is to review the CGT cases treated at our unit that have evolved towards malignization.

Methods: Retrospective study of 96 GCT treated at our Hospital between 1983 and 2005. 5 presented sarcomatous degeneration in their evolution. These were the cases of 3 men and 2 women with a mean age of malignization of 42 years (32 years – 54 years). The median follow-up period was 155 months (5 months – 209 months). 3 cases affected the distal femur, one case affected distal radius and one case affected proximal humerus, with a slight tendency to the right hemibody. The primary treatment for GCT in these patients was curettage and bone graft. Only one case had received previous radiotherapy. In the same period of time we had two cases of lung dissemination of CGT with typical histology, without previous malignization of tumour.

Results: Malignization takes place, on average, at the 1.8th recurrence (1.3). Histologically, we find 3 osteosarcomas and 2 indifferentiated tumours. Three patients developed distant dissemination; 2 patients died due to lung metastases, with a mean time between the first surgery and the sarcomatous degeneration of 90 months (40 monts – 183 months) and a mean time between malignization and mestastases of 22.3 months (9 months – 34 months) The treatment, once the malignization was diagnosed, consisted in wide resection and substitution with mega-arthroplasty in cases of distal femur and osteoarticular graft at the shoulder. 2 cases required amputation of the affected limb due to irresecable recurrence in soft tissues.

Conclusions: There is no predictive criteria of which type of primary typical CGT will evolve into sarcoma. The malignization always has as a result high grade sarcomas, with a high tendency to hematogenous dissemination. When lung metastases appear the survival prognosis is a number of months. We must suspect malignization of a benign CGT when one of the relapses shows a very rapid growth with radiologic aggressive characteristics; in these cases we prefer wide resection of the tumour instead of curettage and thus we prevent the possible sarcomatous degeneration.

Introduction: Aggressive fibromatosis is a benign but locally aggressive process. It arises from musculo-aponeurotic tissues, and invades locally without respect for tissue planes, surrounding vessels and nerves, which makes treatment of local recurrences difficult.

Aims: Our aim is to review our experience in the management of aggressive fibromatosis, focussing on the cases of multiple recurrences, as well as to evaluate the need for disabling surgery.

Material and methods: We present the series of 33 patients (15 male and 18 female) diagnosed of aggressive fibromatosis treated between 1993 and 2003; the follow-up period was no less than two years. The locations were shoulder girdle (8), lower extremity (8), upper extremity (6), gluteus (5), paravertebral (4) and thorax (2). There were 6 cases with 3 or more episodes of local recurrences; in these cases, depending on location and size, and considering high surgical morbidity, associations of radiotherapy, chemotherapy and hormone therapy were given, avoiding disabling surgery.

Results: With an average follow-up of 32 months (25 to 50), there were no deaths and, in the 6 cases of multiple recurrences, there were no amputations. The control MRI demonstrated stability of the process in 5 out of 6 cases, and minimum growth without clinical correlation in the other one. In the other 27 cases, there were 14.8% surgical treated local recurrences, with no need for amputation.

Conclusion: We consider that cases of multiple recurrences of aggressive fibromatosis benefit from adjuvant treatment (radiotherapy, chemotherapy and hormone therapy), avoiding disabling surgery, which is unnecessary following our criteria. This requires strict clinical and radiological control.

Aims: The knee is the commonest articular location in osteosarcoma (OS). We study the complications in limb salvage due to OS in knee reconstructions.

Methods: In our series of 107 OS for the period 1983–1998, limb salvage procedure was possible in 78 cases and the amputation was necessary in 29 patients. The Knee reconstruction includes 62 cases (39 due to femur OS and 23 due to tibia OS). All cases were treated with preoperative and postoperative chemotherapy. The average follow-up was 87.4 months with a range of 55 to 183 months.

Results: Complications in re-constructions due to femur OS:

– Local recurrence 2/39 (5.13%)

– Infection 2/39 (5.13%)

– Fatigue fracture (7.6%) Complications in tibia re-constructions:

– Local recurrences 3/23 (13%)

– Infection 5/23 (21.7%)

Patellar tendon tear off 2/23 (8.7%)

Fatigue fracture 1/23 (4.35%).

Conclusions: The rate of complications in tibia is higher than in femur.

The infections in tibia limb salvage are related to skin coverage.

The local recurrence in tibia is related to anatomical problems to achieve wide resections

Aims: Extraskeletal bone tumours are rare and high-grade tumours including osteosarcoma (OS), chondrosarcoma (CHO) and Ewingñs sarcoma (EW) of the soft tissues and its variants. A retrospective study of 22 cases were made in our Hospital in the period from 1983–2000. Methods: The study of 22 cases revealed that this tumours affect adults (median age 50.86; range 17 to 70 years). The thigh (36.36%) and the arm-elbow (18.18%) were the most common tumour locations. For diagnostic type of tumours 11 were CHO, 8 OS and 3 EW. The median follow-up was 48.9 months with a range 6 to a97 months. All of the cases of OS and EW were treated with preoperative chemotherapy and postoperative radiotherapy. Results: The preoperative duration of symptoms was ranged from 6 weeks to 6 years (median 6 months). Local recurrences after wide and radical surgery developed in 8 cases Ð35.45%- (3 CHO, 4 OS and 1 EW) and distant metastases developed in 3 cases (13.18%). 14 cases were alive with no evidence of recurrence (63.6%), 5 were alive with one or more recurrences (22.72%), 1 were alive with metastases (4.59%) and 2 ulcerated tumours were exitus form metastases (10.05%). Five amputations were performed. Conclusions: 1. All cases were high-grade tumours. 2. The prevailing sites of metastases were the lung, the regional lymph nodes and the skeleton. 3. Cure may be achieved by wide or radical local excision of the tumour at and early stage of the disease (combined with radiation and chemotherapy).

Aims: The purpose of this report is to asses the prognostic factors that could influence management and clinical outcome of malignant fibrous histiocytoma (MFH) of soft tissues. Methods: Between 1990 and 2000, 79 patients diagnosed with localized disease by MFH, seen at Santa Creu i Sant Pau Hospital, have been reviewed. 45 were women and 34 were men. The median age at presentation was 62 years (range: 20–85). The median follow-up was 60 months (range: 1–119). Low grade tumors constituted 8% and the remaining 92% were high grade. Several treatment modalities with or without radiotherapy and/or chemotherapy were associated and assessed. We used Kaplan-Meier method for calculating survival rates and Log-Rank test to assess presumed prognostic factors like sex, age, anatomic side, Enneking stage and associated quimio/radiotherapy. Results: Thigh were the most common location (50%). Isolated local recurrence occurred in 19 patients (24%), isolated meta-static disease without local recurrence in 4 patients (5%), and combined local and metastatic disease occurred in 4 patients (5%). The 2- and 5-year relapse-free survival (RFS) rates were 71% and 67%, respectively.

The 2- and 5-year overall survival (OS) rates were 92% and 86% respectively. We found that only Enneking stage was significant prognostic factor (p< 0.05). The best results in SLE were found in surgery + radiotherapy group but it was not statistically significant (p> 0.05). Conclusions: In our opinion, wide complete surgical resection at the time of primary tumor in combination with local radiotherapy is likely to afford the best chance for RFS and OS. Enneking stage seems to be the most important prognostic factor.

The role of adyuvant chemotherapy remains investigational.

Introduction and Objectives: Extraskeletal osseous sarcomas are very rare high-grade tumours. They include osteosarcoma, chondrosarcoma, and Ewing’s soft tissue sarcoma and their respective variants. We present a retrospective study of 25 cases covering the period from December 2002 to January 2003.

Materials and Methods: This study of 25 cases shows that this heterogeneous group of soft tissue sarcomas primarily affects adults (mean age 50.68; range 17 to 70 years). The thigh (36.60%) and the elbow-forearm (18.18%) are the most common locations for these tumours. In these cases, 14 chondrosarcomas, 8 osteosarcomas, and 3 Ewing’s sarcomas were diagnosed. Mean follow-up time was 48.9 months with a range of 16 to 197 months. There were 16 surgeries performed with wide or compartmental margins, while in the 5 remaining cases, amputations were performed, and one case of Ewing’s sarcoma of the pelvis was treated by means of chemotherapy and radiation therapy, as it was considered non-resectable. All cases of osteosarcoma and Ewing’s sarcoma were treated by means of pre- and postoperative chemotherapy and postoperative radiation therapy.

Results: The postoperative duration of symptoms ranged from 2 weeks to 6 years with a mean of 6 months. Local recurrence following surgery with wide or compartmental margens occurred in 8 cases, representing 35.45% (3 chondrosarcomas, 4 osteosarcomas, and 1 Ewing’s sarcoma). The total number of cases surviving without recurrence is 14 (63.6%), surviving cases with one or more recurrences is 5 (22.72%), one case is alive with metastasis (4.59%), and 3 sarcomas (2 ulcerated upon admission) had disseminated and the patients died (10.05%).

Discussion and Conclusions: All these tumours were high-grade sarcomas. The most common metastatic localizations were lungs, regional ganglia, and skeleton. The recommended treatment is surgery with wide or compartmental margins, if possible in the early stages, in conjunction with chemotherapy and radiation therapy. The worst prognosis corresponds to the osteosarcoma.

Introduction and Objectives: Giant cell tumour (GCT) of the bone is an “aggressive tumor characterized by highly-vascularized tissue consisting of ovoid or fusiform cells and the presence of numerous gigantic osteoclast-like cells distributed uniformly throughout the tumor tissue” (WHO). The aim of this report is to present our experience over the past 19 years (1983–2002) with GCT of the bone treated in our unit.

Materials and Methods: From January 1983 to January 2002, we have treated 67 cases of GCT of the bone, excluding all cases with less than 12 months of follow up. Age at presentation ranged 10 to 17 years. There was a higher incidence from 20 to 40 years of age. There was a moderately higher rate in women compared to men (1.5:1). The most common locations were the distal epiphysis of the femur, proximal epiphysis of the tibia, and the distal end of the radius. This type of tumour generally localises to the epiphysis and subsequently invades the metaphysis. Localization to the axial skeleton is rare. Radiologic diagnosis was achieved by simple local radiology, CT scan, and MRI. Histopathologic diagnosis was done by means of biopsy using a trocar guided by an image intensifier. In cases of central localization, we obtained the sample by CT-guided biopsy. The treatment of choice is aggressive curettage (high-velocity burr) and filling with frozen cancellous chips. Radiation therapy is useful in cases of localization that are not accessible by surgery.

Results: GCT of the bone possesses several unique characteristics, which make it different from other intermediate tumors: a high rate of recurrence (up to 50%), the possibility of sarcomatous degeneration, and the possibility of pulmonary metastasis (even in non-malignant cases). We believe the ideal treatment is resection of the bone where the tumour is located (useful on the head of the fibula, distal end of the ulna, ribs, some bones of the hand and feet, and the patella). In view of its usual localization near the knee (50%), our usual treatment is aggressive curettage (high-speed burr) and filling with frozen cancellous chips. We have treated 26 recurrent cases out of 67 patients treated in our unit (38.8%). Of these, approximately 50% were referred from other centres. Treatment of recurrence has generally been aggressive curettage and addition of allografts. Sarcomatous degeneration occurred in 3 cases (4.5%), all of which were high grade sarcomas of the malignant fibrous histiocytoma type. One case survived following amputation of the extremity, and the other 2 cases died as a result of pulmonary metastasis.

Discussion and Conclusions: Alternative local treatment methods exist for GCT of the bone such as cryosurgery, phenolization, and cementing. However, we cannot comment on these methods due to a lack of experience with them. The aim of all these methods is to cauterize the tumour bed. We prefer aggressive curettage and filling with cancellous bone in an attempt to use a more biological treatment.

Introduction and Objectives: From January 1983 to December 2000 we treated a total of 542 intermediate to high-grade primary bone tumours. We present our experience in reconstruction following tumour resection from the hip, excluding cases of pelvic reconstruction and cases of localised benign bone tumours of the hip.

Materials and Methods: This study included 49 cases of primary bone tumours localised to the proximal humerus (9% of the total). Of all cases treated by means of massive structural allograft for femoral reconstruction (44), only 11 cases were in the hip region. We performed 28 hip tumour resections for high-grade bone tumors with the following diagnoses: osteosarcoma (7), chondrosarcoma (9), Ewing’s sarcoma (6), malignant fibrous histiocytoma (2), and selected cases of metastasis-myeloma (4). Limb salvage procedures (22) with reconstruction after the first surgery included: modular megaprosthesis (11), megaprosthesis associated with massive structural allograft of the proximal third of the femur (8), and osteoarticular allografts (3). The remaining cases (6) required immediate agressive surgery including coxofemoral disarticulation (4) and hemipelvectomy (2).

Results: Limb salvage cases treated by means of mega-prosthesis associated with a composite allograft presented the following complications: 1 case of deep infection with local recurrence and immediate postoperative dislocation (12.5%), 3 isolated coxofemoral dislocations (37.5%), and one case of homograft osteolysis (12.5%). Mean follow-up time was greater than 5 years, and two patients died due to dissemination of the primary tumour (1 chondrosarcoma and 1 osteosarcoma). Cases of limb salvage treatment using modular mega-prosthesis had the following complications: 2 isolated coxofemoral dislocations (18%) and 2 cases of recurrence or local tumour progression (18%). Mean follow-up time was greater than 3 years, and two patients died due to progression of the primary tumour. Cases of salvage treatment using osteoarticular allografts had a mean follow-up time of 2 years and showed no significant complications.

Discussion and Conclusions: 1) In cases of disease up to 12 years of age (primarily Ewing’s sarcomas) we prefer osteoarticular reconstruction. 2) In cases of young adults (from 12 to 60 years of age) we prefer reconstruction using a megaprosthesis (Wagner type) with massive structural allograft. 3) In cases of older adults (greater than 60 years of age) we perform reconstruction using a modular megaprosthesis for tumour treatment.

Introduction and Objectives: Half of primary tumors tend to disseminate to bones, and metastasis to bone is the third most common localisation for disseminated disease, after the lungs and liver. It is also the most common form of neoplasia in the skeleton. Treatment of bone metastasis is essentially palliative, and in select cases improves patient survival. We present results from the last 15 years in our centre.

Materials and Methods: Between the years 1988–2003, our surgical oncology unit has treated 451 patients with bone metastasis. Of these, 49% were male, and 51% were female. Average age was 64 years (19–98). The most common causes were metastatic breast cancer (34%), unknown tumours (17%), multiple myeloma (9%), prostate cancer (9%), lung cancer (7%), bladder cancer (6%), and others (18%). Tumours localised to the following locations: femur (31%), spine (27%), multiple locations (13%), pelvis (11.5%), humerus (9%), and other locations (8.5%). In 69% of cases the first symptom was pain, in 28% pathologic fracture, and in the remaining 3% medullary compression. Of the 125 pathologic fractures, 71% were on the femur, 18% on the humerus, and the remaining 11% in other locations.

Results: In 60% of cases (271 patients) conservative treatment was used, and in the remaining 40% (180 patients) surgical treatment was used. Of the 180 surgeries, 50.5% were for pathologic fractures, and 49.5% were prophylactic surgeries. Of the 125 pathologic fractures, 91 (73%) received surgical treatment, and the other 34 (27%) were treated conservatively. Intramedullary nailing was the most commonly used form of osteosynthesis (47%). Total resolution of pain was achieved in 86.5% of cases, and partial resolution in 13.5%. Mean time in bed from prophylactic surgery was 3 days. Mean time for recovery of function was 7 days for the arms and 11 days for the legs.

Discussion and Conclusions: The fundamental goal is to offer short-term individualized treatment to control pain and avoid bedrest and hospitalization of these patients. Prophylactic surgery does not increase life expectancy of these patients. However, it does alleviate pain, avoids bedrest, and improves functionality. It should be kept in mind that the least aggressive surgical technique possible should be used.

Introduction and Objectives: This study analyses the results of our experience in reconstruction of high-grade sarcomas of the proximal humerus.

Materials and Methods: A total of 37 patients were treated from March 1983 to December 2001. Average age was 24 years (19–38), with 21 males and 16 females, all presenting with high-grade sarcomas of the proximal humerus. The primary tumour was osteosarcoma in 49% (n=18) of cases, chondrosarcoma in 22% (n=8), Ewing’s sarcoma in 13% (n=5), parosteal sarcoma in 8% (n=3), malignant fibrous histiocytoma in 5% (n=2), and adamantinoma in 3% (n=1). All cases were classified as Type 1 Malawer resections (intraarticular resections of the proximal humerus).

Results: During this period, scapulohumeral or scapulothoracic disarticulation was performed in 32% of cases (12 patients). In the remaining 68% (25 cases), limb salvage surgery was performed (relative limb salvage rate: 67.5%). In terms of reconstruction type, 6 cases received megaprotheses, and the remaining 19 cases received osteoarticular allografts of the humerus. Four of the 25 patients had reconstructions with sufficient follow-up time to be considered survivors without recurrence. Results were “acceptable” based on the Enneking-MTS functional scale (overall movement arcs in all directions between 60–120° with tolerable subluxations.) From a subjective point of view, all the patients obtained good results in terms of physical health and mobility of distal joints. No analysis was done of survival due to the varied nature of the neoplasias and adjuvant therapies. The main complication was fracture of the osteoarticular allograft, which occurred in 4 of 19 cases (21%). Subluxation was practically constant throughout our series but was of relatively minor clinical significance.

Discussion and Conclusions: The results obtained in this study in terms of local and functional control are similar to those reported in the literature. In spite of the poor functional results for the shoulder joint, the functional results for distal joints and the cosmetic appearance of the extremity are satisfactory. However, the high rate of allograft fractures forces us to reconsider our reconstruction technique, placing priority on a mixed reconstruction using both allograft and mega-prosthesis.