Purpose. Extraskeletal chondrosarcoma is a rare tumor with an indolent course and high propensity for local recurrence and metastasis. This tumor most commonly presents in the proximal extremities of middle-aged males, and is commonly asymptomatic. Although slow growing, these tumors have a significant risk of eventual relapse and metastases, especially to the lung. There are no clinical trials that investigated the best treatment options for this tumor given its very low incidence. The aim of this study is to present the surgical and clinical results of extraskeletal chondrosarcoma, which is a rare tumor. Methods. In our clinic, the information of 13 patients who were diagnosed with extra-skeletal chondrosarcoma between 2006 and 2018 were retrospectively reviewed. Demographic information, tumor size, surgical treatments, chemotherapy and radiotherapy status, follow-up times, recurrence and metastases of the patients were recorded. Results. This study included 13 patients with an average age of 53.6 ± 15 (range, 28 to 73) years diagnosed with extraskelatal chondrosarcoma. In 8 of the patients, the tumor was located in the lower limbs and it was observed that the thigh was located mostly (46.2%). The mean follow-up period of the patients was 52.8 ± 19.9 (range, 24 to 96) months. All patients underwent extensive resection and only one patient had a positive surgical margin. In the follow-up, 5 (38.5%) of the patients developed recurrence, while 6 patients had
Lymph node metastasis are a rare occurrence in soft tissue sarcomas of the extremity, arising in less than 5% of patients. Few studies have evaluated the prognosis and survival of patients with a lymph node metastasis. Early reports compared lymph node involvement to
Impaired bone healing biology secondary to soft tissue deficits and chemotherapy contribute to non-union, fracture and infection following limb salvage surgery in Osteosarcoma patients. Approved bone healing augments such as recombinant human bone morphogenetic protein-2 (rhBMP-2) have great potential to mitigate these complications. rhBMP-2 use in sarcoma surgery is limited, however, due to concerns of pro-oncogenic signalling within the tumour resection bed. To the contrary, recent pre-clinical studies demonstrate that BMP-2 may induce Osteosarcoma differentiation and limit tumour growth. Further pre-clinical studies evaluating the oncologic influences of BMP-2 in Osteosarcoma are needed. The purpose of this study is to evaluate how BMP-2 signalling affects Osteosarcoma cell proliferation and metastasis in an active tumour bed. Two Osteosarcoma cell lines (143b and SaOS-2) were assessed for proliferative capacity and invasion. 143b and SaOS-2 cells were engineered to upregulate BMP-2. In vitro proliferation was assessed using a cell viability assay, motility was assessed with a scratch wound healing assay, and degree of osteoblastic differentiation was assessed using qRT-PCR of Osteoblastic markers (CTGF, ALP, Runx-2 and Osx). For in vivo evaluation, Osteosarcoma cells were injected into the intramedullary proximal tibia of immunocompromised (NOD-SCID) mice and local tumour growth and metastases were assessed using weekly bioluminescence imaging (BLI) and tumour volume measurements for 4–6 weeks. At the experimental end point we assessed radiographic tumour burden using ex-vivo micro-CT, as well as tibial and pulmonary gross and histologic pathology. SaOS-2 was more differentiated than 143b, with increased expression of Runx-2 (p = 0.009), Osx (p = 0.004) and ALP (p = 0.035). BMP-2 upregulation did not stimulate an osteoblast differentiation response in 143b, but stimulated an increase in Osx expression in SaOS-2 (p = 0.002). BMP-2 upregulation in 143b cells resulted in increased proliferation in vitro (p = 0.014), faster in vitro wound healing (p = 0.03), significantly increased tumour volume (p = 0.001) with enhanced osteolysis detected on micro-CT, but did not affect rates of
To set up an osteosarcoma mouse model with spontaneous
Impaired bone healing biology secondary to soft tissue deficits and chemotherapy contribute to non-union, fracture and infection following limb salvage surgery in Osteosarcoma patients. Approved bone healing augments such as recombinant human bone morphogenetic protein-2 (rhBMP-2) have great potential to mitigate these complications. rhBMP-2 use in sarcoma surgery is limited, however, due to concerns of pro-oncogenic signalling within the tumour resection bed. To the contrary, recent pre-clinical studies demonstrate that BMP-2 may induce Osteosarcoma differentiation and limit tumour growth. Further pre-clinical studies evaluating the oncologic influences of BMP-2 in Osteosarcoma are needed. The purpose of this study is to evaluate how BMP-2 signalling affects Osteosarcoma cell proliferation and metastasis in an active tumour bed. Two Osteosarcoma cell lines (143b and SaOS-2) were assessed for proliferative capacity and invasion. 143b and SaOS-2 cells were engineered to upregulate BMP-2. In vitro proliferation was assessed using a cell viability assay, motility was assessed with a scratch wound healing assay, and degree of osteoblastic differentiation was assessed using qRT-PCR of Osteoblastic markers (CTGF, ALP, Runx-2 and Osx). For in vivo evaluation, Osteosarcoma cells were injected into the intramedullary proximal tibia of immunocompromised (NOD-SCID) mice and local tumour growth and metastases were assessed using weekly bioluminescence imaging and tumour volume measurements for 4–6 weeks. At the experimental end point we assessed radiographic tumour burden using ex-vivo micro-CT, as well as tibial and pulmonary gross and histologic pathology. SaOS-2 was more differentiated than 143b, with significantly increased expression of the Osteoblast markers Osx (p = 0.004) and ALP (p = 0.035). BMP-2 upregulation did not stimulate an osteoblast differentiation response in 143b, but stimulated an increase in Osx expression in SaOS-2 (p = 0.002). BMP-2 upregulation in 143b cells resulted in increased proliferation in vitro (p = 0.014), faster in vitro wound healing (p = 0.03), significantly increased tumour volume (p = 0.001) with enhanced osteolysis detected on micro-CT, but did not affect rates of
Purpose: To evaluate the clinico-pathological features and outcome of osteosarcoma in patients over the age of 40 in Scotland. Methods: A retrospective review was performed using data collected by the Scottish Bone Tumour Registry on patients diagnosed with osteosarcoma over the age of 40 between 1960 and 2004. Information about tumour location, age of diagnosis, gender,
Navigation-assisted surgery has been reported to enhance resection accuracy in bone sarcoma surgery. Patient-specific instruments (PSIs) have been proposed as a simpler alternative with fewer setup facilities. We investigated the use of 3D surgical planning and PSI in realising computer planning of complex resections in bone sarcoma patients with regards to surgical accuracy, problems, and early clinical results. We retrospectively studied twelve patients with bone sarcoma treated surgically by PSIs with 3D planning. The procedure was planned using engineering software. The resection accuracy was accessed by comparing CT images of tumour specimens with the planned in seven patients. Mean age was 30.9 (9 – 64). Mean follow-up was 3.1 year (0.5 – 5.3). 31 planes of bone resections were successfully performed using the technique and were considered accurate. The mean time required for placing PSIs was 5.7 minutes (1 – 10) and performing bone osteotomies with the assistance of PSIs was 4.7 minutes (2 – 7). The mean maximum deviation error was 1.7mm (0.5 – 4.4). One PSI was broken during bone resection, and one patient needed re-resection using the same PSI. One pelvic patient died of local recurrence and lung metastases six months postoperatively. One patient developed a soft tissue local recurrence and
Introduction: Soft tissue sarcomas (STS) are rare tumors. A multidisciplinary approach including surgery, chemotherapy and radiation therapy is recommended. Materials and Methods: In the last 12 years, 249 patients with STS were teated in our Institution. All of them were treted with a multidisciplinary approach using all or some of the previous refered treatments. Results: The overall local recurrence rate in the group of patients submited to surgery was 25% and this factor was related mostly with contaminated margins. Surgical resections were associted with soft tissue reconstructions when needed. Radiation therapy was used in both regimens pre and post operativly, chemotherapy was also used in 85% of the patients, and was not dependent of tumor histotype. 23 patients were submited to surgery of
To present the oncological outcome of eleven patients with stage-3 GCT of bone. Thirty-nine cases of GCT who were treated the past nine years at our department were reviewed. Five tumors were classified as stage I, twenty-three tumors as stage II and eleven as stage III tumors. In stage I or II tumors we proceeded to an intraoperative biopsy (frozen biosy).In cases where the intraoperative pathological findings confirmed our diagnosis of GCT we proceeded to operative management. In cases where the intraoperative pathologist’s findings were not clear as well as in cases of stage III tumors we performed only a traditional open biopsy proceeding surgery in a second stage. In stage III tumors we aimed wide margins. Ten of these patients underwent wide surgical excision and limb salvage, while in one patient curettage with cementation was the treatment of choice in order to obtain a fair functional outcome. With a minimum follow up of 3 years, we had no case of local recurrence in cases treated with wide excision and limb salvage. One stage III GCT treated with curettage recurred. Two stage III tumors metastized to the lung. The average interval from initial operation to
Introduction and Objectives: Synovial sarcoma is an infrequent mesenchymal neoplasia (between 8–10% of soft tissue sarcomas) that can originate in the joint capsule, bursa and tendon sheaths. Materials and Methods: We studied 52 cases of synovial sarcomas reviewed between 1983 and 2006, with a mean follow-up of 91.4 months (24–204 months). The mean age of the patients was 38.4 years of age (range 13–86). The most frequent location was the knee and the popliteal area. In 25 cases a wide resection was performed and in 17 cases amputation was carried out. The most frequently used treatment protocol was preoperative chemotherapy plus postoperative chemo and radiotherapy. In one of the recurrence cases isolated perfusion of the limb was performed due to a recurrence of synovial sarcoma in a hand. Results: One or more recurrences during follow-up were seen in 24 patients. There were 2 cases (3.8%) of skin and cervical spine metastasis and 20 cases of
Chondrosarcoma is the second most frequent primary malignant tumour of bone, representing approximately 25% of all primary osseous neoplasms. Chondrosarcomas are a group of tumours with highly diverse features and behavior patterns, ranging from slow-growing non-metastasizing lesions to highly aggressive metastasizing sarcomas. As radio and quimio-resistant tumours, the surgery constitutes the unique chance of cure. Nowadays, besides the curative intention, the reconstructive surgery is also a priority in order to save the limb and optimize the function. This case report is about a young woman, of 24 years old, with hip-related pain and a large mass in the left pelvis. The imagiologic study showed a large mass of about 8 cm of large diameter, starting at the anterior wall of the acetabulum, involving the pubic arcs and with matrix calcification. The core needle biopsy confirmed the presence of a chondrosarcoma, staged as a IIB of Enneking. Because of its size and localization the limb salvage surgery has been a challenge. The surgery included a broad approach of the left hemipelvis, with wide excision of the tumour, reconstruction of the abdominal wall with a propylene prothesis and reconstruction of the hemipelvis with a “custom-made” prothesis with preservation of the femoral neurovascular bundle. The patient started to walk with total bearing after three months and had a normal gait and a nearly normal life during eleven months. Fifteen months after the surgery
Background. Bone lesions in Ewing's sarcoma (ES/PNET) have been traditionally diagnosed with bone Scan. PET-scan is emerging as a promising investigative modality for detection of metastatic lesions. In this prospective study, we compare the utility of both to detect the metastatic sites. Methods. One hundred and seventy five histologically proven cases of ESPNET from 2004-2009 were prospectively staged with bone scan and PET-scan with Breath- hold CT scan- thorax. The diagnostic value of PET-scan to pick up metastatic lesions was compared with bone scan. Results. The site of primary disease was axial in 62(35.4 %) patients, appendicular in 94(53.7 %) patients, and extraskeletal in 19 (10.8 %) patients. 24(13.7 %) patients were metastatic at presentation, while 151(86.2 %) patients had localized disease. In all patients with localized disease, bone scan did not detect any lesion other than that detected on PET-scan. In metastatic patients, PET-scan detected 12 patients with lymph node involvement which were not detected by bone scan, 10 patients were found to have
Summary Statement. Combination of sorafenib with irradiation achieved synergistic effect with dose reduction in both 143B and HOS cell lines. This demonstrated the potential application of sorafenib in the treatment of osteosarcoma metastasis and radiation resistance. Introduction. More than 20% of patients with osteosarcoma die of the disease within 5 years due to tumour relapse and metastasis. Identifying new treatment that works singly or in combination with conventional therapies is urgently required. We previously found that the Ras/Raf/MAPK pathway was associated with
Osteosarcoma is the most frequent bone tumor in adolescents and young adults. Already, the bisphophonates were introduced as the first line therapy for metastatic disease as well as the maintenance therapy, but new drugs are still in researchers interest. Between 2005 and 2008, we have been treated 17 osteosarcoma patients, from 4 till 18 years of age. All patients have been followed up for 11 months average (range 4–18). At the time of diagnosis 15 of them had local disease, and 2 had metastatic disease. They were treated according to EURAMOS protocol. There were two groups of patients; the high risk patients who has received the pamidronat disodium (pamidronat) after the standard postoperative chemotherapy, and the other group who hadn’t received pamidronat. One patient, who had bone and pulmonary metastasis at the diagnosis, received the pamidronat as the first line therapy. We have introduced the 2 mg/kg mothly of pamidronat to 7 patients, median age of 13. Patients have received 8 cycles average of pamidronat (range 4–12). Two patients had to be excluded from therapy due to nephrotoxycity and pregnancy. The patient with metastatic disease, bone and
In the last ten years, 172 bone sarcomas and 126 soft tissue sarcomas, were treated by our Orthopaedic Oncology Unit. From those patients 49 (16.5%) meet criteria for pulmonary metastasis resection. Patient group were 27 males (55%) and 22 females (45%); median age 28.8 years (range 12–71); histology of the primary tumours were in 33 cases bone lesions (67%): 17 osteosarcomas, 8 Ewing sarcomas, 3 Malignant Fibrous Histiocytoma (MFH) and 1 Giant Cell Tumor and 16 cases (23%) for soft tissue tumours: 5 synovial sarcoma, 3 schwannoma and MFH, 2 leiomiosarcoma and liposarcoma and 1 rhabdomyosarcoma. From those sarcomas 8 (17.8%) were metastatic at presentation. Treatment included surgical resections of the secondary lesions and chemotherapy. The
Aims: To evaluate the outcome of surgical treatment of benign and aggressive chondroblastoma in the Prague bone tumours register. Methods: Between 1969–2001 57 patients (38 men and 19 women) with chondroblastoma have been registered. The age ranged from 7 to 52 years – in average 19 years. The most frequent localizations were epiphyses of long bones (13 proximal humerus, 10 proximal femur, distal femur and proximal tibia each 11). We also observed atypical localizations (3 patella, 2 pelvis and 1 each in, fibula, talus, 5th metatarsal). All patients had available x-rays for evaluation and some arteriography or CT. All had histological verification. The treatment of choice was intralesional curetting and filling with auto or allografts. The femoral head lesions were treated through an original femoral neck approach to prevent hip luxation. We registered 5 aggressive variants with a different clinical course. They recur after intralesional surgery, are purely osteolytic and richly vascularized. One patient even developed
Our purpose was to assess the role of preoperative radio-therapy +/− neoadjuvant chemotherapy in nonmetastatic soft tissue sarcoma of extremities for limb-sparing surgery and identify the role of neoadjuvant therapies on local control and survival rate. Forty-seven patients with soft tissue sarcoma of extremities who were treated at Cerrahpasa Medical Faculty within a limb salvage protocol, including preoperative radiotherapy +/− chemotherapy were retrospectively analized. Median age was 45 years (17–72 years). The tumor size was between 5–33 cm. Seventeen patients were in stage I, 11 in stage II, 19 in stage III. The most common histology was synovial sarcoma. Nine patients were treated for locally recurrent tumour. The tumour and surrounding tissues with probable microscopic tumour involvement observed clinically and radiologically, were irradiated. Thirty-two patients, with a high grade tumour and/or tumours larger than 8 cm, also received neoadjuvant chemotherapy. Neoadjuvant chemotherapy regimen was consisted of doxorubicine and ifosphamide with mesna. Preoperative radiotherapy was applied, usually between the second and third cycles of chemotherapy. Definitive surgery was administered 2–6 weeks after radiotherapy or after the third cycle of chemotherapy. Chemotherapy was completed to 6 courses after the surgery. Postoperative external beam radio-therapy boost of 16 Gy was given who had close or positive surgical margins. Median follow-up time was 67 months (12–217 months). All of the patients had limb-sparing surgery. Patients had; 30 marginal excision, 13 wide local excision, 4 radical resection. Nine patients locally recurred. Limb-sparing surgery was performed for 8 patients. 25 patients had distant metastases. Metastasectomy were applied for 10 patients with
Haemangioendothelioma of bone is a rare intermediate grade malignancy. Because of its rareness there is a lack of information in the literature about the well established treatment strategies depending on series with large numbers. The outcome of wide resection with postoperative external irradiation would be presented. 4 patients (2 females, 2 males) with a mean age of 40.5 (26–52) with solitary haemangioendothelioma of bone admitted with local pain on the affected bone and limited restriction of function. Anatomical sites were scapula, calcaneum, midshaft of radius and metaphysodiaphyseal region of femur. Plain X-ray, CT, MRI, Tc 99 tecnetium wholebody bone scan investigations were applied. All lesions were hot on bone scan and lytic irregular permeative lesions T1 hypo, T2 hyper with gadolinium enhancement were present. Open biopsy resulted with the diagnosis of intermediate haemangioendothelioma of bone. Wide resection of tubular bones and intercalary lyophilised allograft recostruction with IM rod and cerclage wire and total calcaneum resection and allograft replacement with talar arthrodesis, total scapulectomy subsequent autoclaved bone reimplantation were the surgical procedures applied. Mean follow-up was 96 months (40–132). Three patients except scapula case received 50 Gy external irradiation. No patient developed local recurrence in the follow up. Regarding complications calcaneum patient developed skin necrosis after the irradiation which led to removal of the allograft but eventually healed. Scapula patient had late infection treated by antibiotics. All patients had satisfactory function. Intercalary allografts united in 6 months time. Calcaneum patient developed multiple small
Giant cell tumour of bone (GCTB) is a primary tumour of bone characterised by a proliferation of mononuclear stromal cells and infiltrating macrophages and osteoclast-like giant cells. GCTB has a variable and unpredictable course and can produce metastatic lesions, mostly in the lungs, in up to 3% of cases. Whether these represent tumour implants rather than true neoplastic secondaries is uncertain. In this study, we analysed morphological and immunophenotypic features of primary GCTBs which metastasised to the lung as well as the metastatic lesions themselves in order to determine if these would provide a clue as to the mechanism of
Forty-six hemangioendotheliomas (HE) of bone treated at Rizzoli from 1985 to 2004 were studied with minimum follow up of 4 years: 19 females and 27 males, mean age 37 years, mean follow-up 9 years, 35 cases unifocal at diagnosis (10 spine – 1 with
