The Efficacy of Radiofrequency Ablation for Bone Tumors Unsuitable for Radical Excision.

BACKGROUND: Bone tumors can cause severe pain and poor quality of life due to recurrence and non-achievement of complete remission after surgery, chemotherapy, or radiotherapy. Radiofrequency ablation (RFA) can be considered for minimally invasive treatment of bone tumors that are difficult to radically excise. In this study, RFA was performed for bone tumors that were difficult to radically excise and did not respond to surgery, chemotherapy, or radiotherapy due to their large sizes and/or locations. The purpose of this study was to retrospectively analyze the clinical characteristics and survival rates of bone tumors after RFA and provide one more treatment option for the future. METHODS: There were 43 patients with bone tumors who underwent percutaneous RFA at our hospital from April 2007 to October 2017. The median age of the patients was 59 years (range, 31-75 years), and the median follow-up duration was 67.2 months (range, 10.2-130.5 months). Of the 43 patients, 26 were male and 17 were female. Thirty-four cases were metastatic bone tumors, 5 were chordomas, 3 were osteosarcomas, and 1 was a giant cell tumor. Pain and functional ability of the patients were evaluated using a visual analog scale (VAS) and the Musculoskeletal Tumor Society (MSTS) functional scoring system, respectively. Scores were recorded preoperatively, 1 week postoperatively, and 4 weeks postoperatively. The 1-year, 2-year, and 5-year survival rates were evaluated using the Kaplan-Meier method. RESULTS: The mean VAS score was 8.21 preoperatively. The mean VAS score at 1 week, 4 weeks, 12 weeks, and 24 weeks postoperatively were 3.91, 3.67, 3.31, and 3.12, respectively. The mean preoperative MSTS score was 64.0% (range, 32%-87%). The mean postoperative MSTS score was 71.0% (range, 40%-90%). The 1-year, 2-year, and 5-year survival rates were 95.3%, 69.8%, and 30.2%, respectively. CONCLUSIONS: As per our study findings, RFA was effective in reducing pain and improving functional ability of patients with bone tumors that were difficult to radically excise.

Denosumab for Effective Tumor Size Reduction in Patients With Giant Cell Tumors of the Bone: A Systematic Review and Meta-Analysis.

BACKGROUND: Denosumab is a human monoclonal antibody that has been used successfully in the treatment of giant cell tumors of bone. These tumors are rare and, in principle, benign, but they are highly aggressive, locally advanced, osteolytic bone tumors that can metastasize to the lungs. Denosumab is an effective treatment when these tumors cannot be surgically removed or when surgical resection is likely to lead to severe morbidity (eg, loss of limbs or joints). The aim of this systematic review and meta-analysis was to investigate patients with giant cell tumors of bone who experienced tumor progression during treatment with denosumab and to compare them with patients who experienced reduction of their giant cell tumors of bone during treatment with denosumab. METHODS: Embase, Cochrane Library, and MEDLINE/PubMed databases were searched for trials submitted by January 7, 2020, that reported the efficacy and safety of denosumab in patients with giant cell tumors of bone. RESULTS: Sixty studies were reviewed, involving a total of 1074 patients who had giant cell tumors of bone and were treated with denosumab. Of the 60 studies, 58% of the patients were from case series studies, 39% from open-label phase II studies, and 3% from case reports. The response rate for denosumab as a treatment for giant cell tumors of bone was 97.5%, with statistical significance (P < .0001). Pain in the limbs was statistically the most common adverse event for denosumab treatment in case series studies (P < .0001). No treatment-related deaths occurred in the reviewed studies. CONCLUSION: Cumulative evidence supports the addition of surgery to optimal medical therapy with denosumab to reduce tumor size, clinical symptoms, and mortality among patients with giant cell tumors of bone.

Clinical Characteristics and Prognosis of Osteoclast-like Giant Cell Tumors of the Pancreas Compared with Pancreatic Adenocarcinomas: A Population-Based Study.

BACKGROUND The incidence of osteoclast-like giant cell tumor of the pancreas (OGTP) is very low, and relatively little OGTP clinical data is available. The present study, therefore, sought to conduct a more comprehensive analysis of the clinical characteristics and prognosis of OGTP. MATERIAL AND METHODS A large population-based cohort analysis was conducted using the Surveillance, Epidemiology and End Results (SEER) registry. We conducted a systematic assessment of the demographic and clinical characteristics of these patients, in addition to assessing available prognostic and therapeutic data corresponding to their disease. We further compared overall survival (OS) in these OGTP and pancreatic adenocarcinoma (PA) patient cohorts, adjusting for sex, grade, stage, and surgical treatment by propensity score matching (PSM). RESULTS We included a total of 47 OGTP patients and 73 150 PA patients in the present analysis. The mean ages of PA and OGTP diagnosis were 68.0 and 62.8 years, respectively. Compared with PA patients, OGTP patients were more likely to be female (70.2% versus 48.7%, P<0.01), to have early-stage disease, to have lower rates of lymph node metastasis (17.0% versus 28.8%, P<0.01) and distant metastasis (17.0% versus 45.1%, P<0.01), and to have higher rates of tumor resection (70.2% versus 15.4%, P<0.01). OGTP patients also had a significantly longer median OS than did PA patients (13 months versus 6 months; hazard ratio [HR] 0.55, 95% confidence interval [CI] 0.37-0.57, P<0.0001). No significant differences in tumor site preferences were detected. Our findings also suggested that being female, having early-stage disease, and undergoing surgical resection may be associated with a more favorable prognosis in patients with OGTP. CONCLUSIONS OGTP patients had distinctive clinical characteristics and a better prognosis compared with PA patients. Understanding these differences will help clinicians accurately recognize these diseases. Radical resection was beneficial to the survival of OGTP patients.

Malignant giant cell tumor of bone or soft tissue treated by surgery with or without radiotherapy.

Malignant giant cell tumor of bone or soft tissue (MGCTBS) is one of the malignant tumors associated with poor prognosis. However, it remains controversial whether the combined treatment of both surgery and radiotherapy (surgery + RT) leads to better survival outcomes than surgical treatment alone (surgery alone) does for patients diagnosed with MGCTBS. We compared the two treatment strategies using the data provided by Surveillance, Epidemiology, and End Results (SEER) program. About 357 patients with MGCTBS who received either surgery + RT or surgery alone between 1975 and 2016 in the USA were identified and then matched based on their propensity scores estimated using the patients' baseline characteristics. We also performed a subgroup analysis for patients with high-grade and regional/distant tumor extension. Unadjusted Kaplan-Meier curves suggested that the surgery alone group had a better 10-year survival profile than the surgery + RT group. After propensity score matching, there was no statistical difference between the two treatment groups with respect to the 10-year cancer-specific survival and overall survival distributions. A subsequent subgroup analysis demonstrated that the surgery alone group has a similar 10-year survival comparing with the surgery + RT group for patients with high-grade and regional/distant tumor extension. The results of our study suggest that RT should not be recommended as a regular therapeutic method for MGCTBS, even for patients with high-grade histology and distant metastasis Clinical Significance: This study may provide better decision making for surgeons dealing with malignant giant cell tumor of bone or soft tissue. Type of study: Observation study. Level of evidence: Level III.

Functional and oncological outcomes of limb-salvage surgery for foot and ankle tumors.

BACKGROUND: Limb-salvage surgery has become the backbone treatment for musculoskeletal tumors in the last decades. However, limb-salvage with safe margins for tumors of the foot and ankle is challenging, due to the complex anatomy of this region. This study aimed to evaluate functional and oncological outcomes, complications, and local recurrence of limb-salvage procedures for tumors of the foot and ankle. METHODS: This study was a retrospective review of 18 patients with primary tumors of the foot and ankle who underwent limb-salvage surgery between 1996 and 2015, with a minimum follow-up of 2 years. Clinical presentations, radiological and histopathological investigations, surgical procedures, functional and oncological outcomes, complications and local recurrences were analyzed. The Musculoskeletal Tumor Society (MSTS) scoring system was utilized for evaluation of the functional outcome. RESULTS: With a mean follow-up period of 44.2 months, 7 males and 11 females with a mean age of 29.2 years were included. Nine patients were presented as recurrent cases. The most common location was forefoot in 7 patients. Synovial sarcoma was the histopathologic diagnosis in 7 patients. The final surgical margins were wide in 15 patients, wide-contaminated in one patient, and marginal in two patients. After resection, soft tissue reconstruction and wound coverage were done by soft tissue flaps in 4 patients. The mean MSTS score was 27.5. Four patients developed local recurrence, all of them were recurrent cases. CONCLUSION: Limb-salvage surgery for foot and ankle tumors is an effective treatment option with good functional outcome. LEVEL OF EVIDENCE: Level IV, retrospective case series.

Surgical management and long-term outcomes of intracranial giant cell tumors: a single-institution experience with a systematic review.

OBJECTIVE: Intracranial giant cell tumors (GCTs) are extremely rare neoplasms with dismal survival and recurrence rates. The authors aimed to confirm independent adverse factors for progression-free survival (PFS) and to propose an optimal treatment algorithm. METHODS: The authors reviewed the clinical data of 43 cases of intracranial GCTs in their series. They also reviewed 90 cases of previously reported GCTs in the English language between 1982 and 2017 using Ovid MEDLINE, Embase, PubMed, and Cochrane databases with keywords of "giant cell tumor" or "osteoclastoma" and "skull," "skull base," "temporal," "frontal," "sphenoid," or "occipital." These prior publication data were processed and used according to PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Aforementioned risk factors for the authors' series and the pooled cases were evaluated in patients not lost to follow-up (m = 38 and n = 128, respectively). RESULTS: The authors' cohort included 28 males and 15 females with a mean age of 30.5 years. Gross-total resection (GTR) was achieved in 15 (34.9%) patients. Fifteen patients (39.5%) who did not undergo GTR received postoperative radiotherapy with a mean total dose of 54.7 ± 4.1 Gy. After a mean follow-up of 71.3 months, 12 (31.6%) patients experienced recurrence, and 4 (10.5%) died of disease. The actuarial 5-year PFS and overall survival (OS) were 68.6% and 90.0% in the authors' cohort, respectively. A multivariate Cox regression analysis verified that partial resection (HR 7.909, 95% CI 2.296-27.247, p = 0.001), no radiotherapy (HR 0.114, 95% CI 0.023-0.568, p = 0.008), and Ki-67 ≥ 10% (HR 7.816, 95% CI 1.584-38.575, p = 0.012) were independent adverse factors for PFS. Among the 90 cases in the literature, GTR was achieved in 49 (54.4%) cases. Radiotherapy was administered to 33 (36.7%) patients with a mean total dose of 47.1 ± 5.6 Gy. After a mean follow-up of 31.5 months, recurrence and death occurred in 17 (18.9%) and 5 (5.6%) cases, respectively. Among the pooled cases, the 5-year PFS and OS were 69.6% and 89.2%, respectively. A multivariate model demonstrated that partial resection (HR 4.792, 95% CI 2.909-7.893, p < 0.001) and no radiotherapy (HR 0.165, 95% CI 0.065-0.423, p < 0.001) were independent adverse factors for poor PFS. CONCLUSIONS: GTR and radiotherapy were independent favorable factors for PFS of intracranial GCTs. Based on these findings, GTR alone or GTR plus radiotherapy was advocated as an optimal treatment; otherwise, partial resection plus radiotherapy with a dose ≥ 45 Gy, if tolerable, was a secondary alternative. Lack of randomized data of the study was stressed, and future studies with larger cohorts are necessary to verify these findings.Systematic review no.: CRD42018090878 (crd.york.ac.uk/PROSPERO/).

Cytologic features and clinical implications of undifferentiated carcinoma with osteoclastic giant cells of the pancreas: An analysis of 15 cases.

BACKGROUND: The cytologic features of undifferentiated pancreatic carcinoma with osteoclastic giant cells (UOC) are rarely described. METHODS: Cytologic and clinicopathologic characteristics in 15 UOC fine-needle aspiration (FNA) specimens were analyzed. RESULTS: FNA specimens were obtained from 6 men and 8 women with a mean age of 65 years who had UOCs (head, n = 7; body, n = 3; and tail, n = 4) with a mean radiologic size 7.3 cm, and some had a cystic component (n = 9). Three cell types (osteoclastic giant cells, pleomorphic tumor giant cells, and spindled/histiocytoid cells) were observed in 12 of 15 specimens (80%); and pancreatic ductal adenocarcinoma (PDAC) was present in 11 specimens. FNA diagnoses were UOC (n = 6), PDAC (n = 5), poorly differentiated carcinoma (n = 2), "suspicious for neoplasm" (n = 1), and "negative" (n = 1). Five of 5 specimens with osteoclastic giant cells were positive for cluster of differentiation 68 (CD68) (a glycoprotein that binds to low-density lipoprotein). Pleomorphic tumor giant cells and spindled/histiocytoid cells were positive for pancytokeratin (6 of 7 specimens), CAM5.2 (2 of 3 specimens), and epithelial membrane antigen (2 of 2 specimens). INI-1 protein expression was retained in 3 of 3 specimens. The Ki-67 labeling index was assessed in 3 specimens and was 12%, 18%, and 40%; 4 of 12 resected UOCs were pure, and 8 were mixed with PDAC. One resection specimen had intraductal papillary mucinous neoplasm, and 2 had mucinous cystic neoplasms. The median overall survival (OS) of patients who had UOCs identified on FNA was 8 months (6 died [OS, 8 months; range, 2-22 months], and 8 remained alive [OS, 3 months; range, 1-27 months]), which was similar to the survival of 74 patients who had PDACs identified on FNA (OS, 15 months; P = .279) but worse than that of the 27 patients with UOCs who did not undergo FNA (OS, 92 months; P = .0135). CONCLUSIONS: The 3 classical UOC cell types are identifiable on FNA, making cytologic diagnosis possible if considered in the differential. A PDAC component is often also observed. The survival advantage of UOC over pure PDAC appears to be negated by FNA and requires further investigation. Cancer Cytopathol 2017;125:563-75. © 2017 American Cancer Society.

En Bloc Resection Versus Intralesional Surgery in the Treatment of Giant Cell Tumor of the Spine.

STUDY DESIGN: Multicenter, ambispective observational study. OBJECTIVE: The aim of this study was to quantify local recurrence (LR) and mortality rates after surgical treatment of spinal giant cell tumor and to determine whether en bloc resection with wide/marginal margins is associated with improved prognosis compared to an intralesional procedure. SUMMARY OF BACKGROUND DATA: Giant cell tumor (GCT) of the spine is a rare primary bone tumor known for its local aggressiveness. Optimal surgical treatment remains to be determined. METHODS: The AOSpine Knowledge Forum Tumor developed a comprehensive multicenter database including demographics, presentation, diagnosis, treatment, mortality, and recurrence rate data for GCT of the spine. Patients were analyzed based on surgical margins, including Enneking appropriateness. RESULTS: Between 1991 and 2011, 82 patients underwent surgery for spinal GCT. According to the Enneking classification, 59 (74%) tumors were classified as S3-aggressive and 21 (26%) as S2-active. The surgical margins were wide/marginal in 27 (36%) patients and intralesional in 48 (64%) patients. Thirty-nine of 77 (51%) underwent Enneking appropriate (EA) treatment and 38 (49%) underwent Enneking inappropriate (EI) treatment. Eighteen (22%) patients experienced LR. LR occurred in 11 (29%) EI-treated patients and six (15%) EA-treated patients (P = 0.151). There was a significant difference between wide/marginal margins and intralesional margins for LR (P = 0.029). Seven (9%) patients died. LR is strongly associated with death (Relative Risk 8.9, P < 0.001). Six (16%) EI-treated patients and one (3%) EA-treated patients died (P = 0.056). With regards to surgical margins, all patients who died underwent intralesional resection (P = 0.096). CONCLUSION: En bloc resection with wide/marginal margins should be performed when technically feasible because it is associated with decreased LR. Intralesional resection is associated with increased LR, and mortality correlates with LR. LEVEL OF EVIDENCE: 3.

[Efficacy of reconstruction with modular endoprosthesis after resection of periacetabular malignant tumors].

OBJECTIVE: To explore the efficacy of the resection of periacetabular malignant tumors and the reconstruction with modular endoprosthesis.
 METHODS: From August 2006 to December 2012, 22 patients with periacetabular malignant tumors, who received the resection and reconstruction with modular prosthesis, were retrospectively reviewed. There were 11 males and 11 females, and the average age was 44 (16-65) years old. Pathological results showed there were 13 cases of chondrosarcoma, 5 cases of osteosarcoma, 2 cases of Ewing's sarcoma, 1 case of maligant fibrous histiocytoma, and 1 case of giant cell tumor. According to the classification system by Enneking, there were 11 cases of Type II+III resection, 5 cases of Type I+II+III resection, 3 cases of Type I+II resection, and 3 cases of Type II resection.
 RESULTS: All patients were followed up. The average time for follow-up was 49 (11-103) months. At the last time of follow-up, 13 patients (59%) were still alive, 9 patients (41%) died due to their primary disease. Metastasis occurred in 8 patients (36%), and local recurrence occurred in 5 patients (23%). The mean Musculoskeletal Tumor Society (MSTS) score for 13 cases of alive patients at the latest time of follow-up was (18.5±5.7) months. The mean score for 11 patients, whose limb salvage were successful, was 20.7±2.0.
 CONCLUSION: Reconstruction with modular prosthesis after wide resection for periacetabular malignant tumor can achieve satisfied outcome.

Treatment options and prognosis for repeatedly recurrent giant cell tumor of the spine.

BACKGROUND: Giant cell tumor of the spine has high rate of postoperative recurrence. There are not much published studies on repeatedly recurrent GCTS (RRGCTS). Also, there are controversies as to the prognostic factors and treatment options for RRGCTS. METHODS AND MATERIALS: A retrospective survival analysis between 2000 and September 2014 was performed on the 74 times of in-hospital treatment of the 21 patients. Recurrence-free survival was defined as the time between the date of surgery and the date of recurrence. Factors with P values <0.05 in univariate analysis were subjected to multivariate analysis by means of proportional hazard analysis. RESULT: A total of 21 patients comprising ten males and 11 females with a mean age of 29.7 years (range 15-49) were included, with a total of 74 times of surgery performed. Eighteen patients had no evidence of disease. Univariate and multivariate analysis suggested that total spondylectomy and bisphosphonate therapy were independent prognostic factors for better RFS in RRGCTS patients. CONCLUSION: Patients of RRGCTS are suitable for further surgical treatment with relatively good healing process and restoration of nervous function. Early diagnosis of recurrence may be associated with better prognosis. Total spondylectomy in combination with bisphosphonate therapy could reduce postoperative recurrence rate.

Giant Cell Tumors of the Bone With Pulmonary Metastasis.

Giant cell tumors of the bone are benign but locally aggressive, and they rarely metastasize to the lungs. The purpose of this study was to retrospectively review the clinical presentation, long-term outcomes, and treatment of pulmonary metastasis of these tumors. Between 1991 and 2004, a total of 168 patients with giant cell tumors of the bone were treated at the authors' institution, 7 of whom developed lung metastasis. Four of the 7 patients were men, and mean age of these patients at initial surgery was 40 years (range, 19-56 years). All patients underwent wide excision and reconstruction or curettage and bone grafting for the bony lesions. Lung metastases were detected at a mean of 44 months after the treatment of bone lesions. Five patients had multiple metastases, and 2 had solitary pulmonary metastases. Six of these patients underwent delayed treatment, locally aggressive, or multiple recurrent and surgical procedures. All of the aforementioned procedures had similar risk factors to those previously reported in the literature. One patient had multiple giant cell tumors of the bone. At last follow-up, 2 patients had died due to complications from the pulmonary metastases or chemotherapy. One patient underwent a metastasectomy 4 years after treatment due to the progression of pulmonary metastasis. The remaining 4 patients were alive and healthy after chemotherapy or conservative treatment. Therefore, early detection, adequate treatment of the primary bone lesion, conservative treatment of lung metastases, and regular long-term follow-up are recommended.

More than 10-year follow-up after total en bloc spondylectomy for spinal tumors.

BACKGROUND: There are many reports of en bloc resection for spinal tumors. However, no studies have evaluated the clinical outcomes with follow-up exceeding 10 years after surgery. METHODS: We reviewed 82 patients who had undergone total en bloc spondylectomy (TES) before January 2002 and identified 29 (19 with primary tumors and 10 with metastatic tumors) who had survived for more than 10 years after surgery. We evaluated the clinical outcomes including patient-based outcomes using questionnaire. The questionnaire included subjective assessment of the results of TES and SF-36. RESULTS: Overall, 33 % of patients with metastases from kidney cancer and 25 % of those with metastases from thyroid cancer survived for more than 10 years after TES for solitary spinal metastases. Four patients with metastatic tumors had no evidence of disease at the time of survey. There were no tumor recurrences in any of the 23 patients who underwent TES as the primary surgery. No revision surgeries have been required as a result of instrumentation failure in any of the 29 patients. About 90 % of the patients were satisfied or very satisfied with the results of TES. The SF-36 results demonstrated that the both physical and mental health of patients with primary tumors was equivalent to those of healthy individuals, and the mental health of patients with metastatic tumors was almost similar to them. CONCLUSIONS: This study showed the long-term clinical outcomes after TES to be favorable. TES played an important role in the treatment strategy for spinal tumors including metastases.

Giant cell tumors of the skull: a series of 18 cases and review of the literature.

Giant cell tumors (GCTs) are generally benign, locally aggressive lesions mostly located in the metaphysis of long bones. GCTs of the skull are rare and the majority of the cases have been presented as case reports. The authors retrospectively reported 18 patients with GCTs of the skull at a single institution from April 1994 to February 2012 and summarized the clinical, radiological, pathological characteristics and management of the disease. Meanwhile, a systematic review of 94 case reports of GCTs of the skull was performed. Headache and symptoms related to the involvement of intracranial nerves were the most common symptoms. Over 90 % of the tumors originated from sphenoid and temporal bones. On MRI, very low signal on T2-weighted images were found highly indicative of GCTs of the temporal bone. Univariate analysis revealed that extent of tumor resection and post-operative radiation therapy (RT) were prognostic factors significantly influencing the survival of the patients. We concluded that complete tumor resection is the optimal goal in treating this disease and adjuvant RT should be given once tumor residual is inevitable.

Factors affecting prognosis of patients with giant cell tumors of the mobile spine: retrospective analysis of 102 patients in a single center.

BACKGROUND: Giant cell tumor (GCT) of the mobile spine is a benign tumor, but it can be potentially aggressive. There is not much published information on GCT of the mobile spine as a result of rarity of the disease, and there are controversies over prognostic factors of the condition. METHODS: A retrospective analysis of GCT of the mobile spine was performed by survival analysis. Recurrence-free survival (RFS) was defined as the interval between the date of surgery and the date of recurrence. The postoperative RFS rate was estimated by the Kaplan-Meier method. Factors with P values of ≤0.1 were subjected to multivariate analysis for RFS by proportional hazard analysis. P values of ≤0.5 were considered statistically significant. RESULTS: A total of 102 patients with GCT of the mobile spine were included in the study. The mean follow-up period was 39.9 (median 26.0, range 2-153) months. Thirty-eight patients developed recurrence. The univariate and multivariate analysis suggested that age less than 40 years, total spondylectomy either by en bloc or piecemeal method, and administration of bisphosphonate were independent favorable prognostic factors. Subgroup analysis by excluding patients before the year 2000 further confirmed our findings. CONCLUSIONS: The removal of the entire osseous compartment either by en bloc or piecemeal method in combination with the long-term use of bisphosphonate could significantly reduce the recurrence rate of GCT of the mobile spine. Age less than 40 years is a favorable prognostic factor for GCT in the mobile spine.

Efficacy of first-line chemotherapy in patients with advanced lung sarcomatoid carcinoma.

BACKGROUND: Sarcomatoid carcinomas (SCs) are rare tumors that may arise in the lung, accounting for 0.4% of non-small-cell lung cancers; the prognosis is poor. Only few retrospective small-size series have studied the efficacy of chemotherapy (CT) for metastatic SC. METHODS: Multicenter study of patients with advanced or metastatic SC who received first-line CT. Clinical characteristics at baseline, response to first-line CT (Response Evaluation Criteria in Solid Tumors version 1.1), progression-free survival (PFS), and overall survival (OS) were retrospectively collected. RESULTS: Ninety-seven patients were included. Median age was 62 (54-72) years. The majority of patients were men (70%), white (84%), and smokers (84%). Overall, 73% of patients received first-line platinum-based CT. At first tumor evaluation, 69% of patients experienced progression, 31% had disease control, and 16.5% had partial response. Partial response was observed in 20% of patients receiving platinum-based CT, and in none of those receiving non-platinum-based CT (p = 0.018). Median PFS was 2.0 months (confidence interval [CI] 95%: 1.8-2.3). PFS was not statistically different between patients receiving or not receiving a platinum-based CT. Median OS was 6.3 months (CI 95%: 4.7-7.8). There was a trend toward better OS for patients treated with platinum-based CT (7.0 months [CI 95%: 4.9-9.0] versus 5.3 months [CI 95%: 2.8-7.6]; p = 0.096). In multivariate analysis, disease control at first evaluation (hazard ratio = 0.38 [CI 95%: 0.21-0.59]) and at platinum-based CT (hazard ratio = 0.92 [CI 95%: 0.85-0.99]) was associated with better OS. CONCLUSION: SC is associated with poor prognosis and high rate of resistance to conventional first-line CT. New therapeutic strategies are needed, based on better knowledge of the carcinogenesis of SC.

Prosthetic limb salvage surgery for bone and soft tissue tumors around the knee.

In this study, we analyzed long-term survival, limb function and associated complications after prosthetic limb salvage treatment in patients with bone and soft tissue tumors around the knee joint. A total of 63 patients treated with prosthetic limb salvage surgery around the knee were reviewed. The bone tumors involved the distal femur in 45 patients, the proximal tibia in 14 patients and the soft tissue tumors of the proximal lower leg in 4 patients. The median follow-up period after the first operation was 8.0 years. The medical records of the patients, surgical reports, radiographs and histological specimens were retrospectively reviewed. The 5-year overall survival rate was 63.2% in the patients with distal femur tumors and 86.2% in those with tumors of the proximal lower leg. The 5­year prosthetic survival rate was 72.8% in the distal femur and 74.6% in the proximal lower leg. The mean functional score according to the scoring system of the Musculoskeletal Tumor Society (MSTS) was 81% in the patients with distal femur tumors and 82% in the patients with proximal lower leg tumors. Post-operative complications occurred in 27 patients. Limb salvage surgery is considered to be an effective treatment option. However, the high complication rate is a major concern for prosthetic replacement. Future improvements of prostheses are very important.

Clinical and immunohistochemical characteristics of benign giant cell tumour of bone with pulmonary metastases: case series.

PURPOSE: Giant cell tumour of bone with pulmonary metastases is rare. However, some patients die of pulmonary metastases, and histological examination cannot distinguish between benign tumour and malignant metastases. In this study, we present clinical and immunohistochemical findings associated with giant cell tumour of bone with pulmonary metastases. METHODS: Five patients with benign giant cell tumour of bone with pulmonary metastases (one man and 4 women) were studied. Patients' ages ranged between 20 and 23 years (mean age, 21.8 years). Tumours were in the distal femur in 2 cases, and in the proximal tibia, distal tibia, and lumbar spine in one case each. The tissue specimens from primary tumours, recurrent tumours, and pulmonary metastases were studied using immunohistochemical techniques. RESULTS: Three of the 5 primary tumours were of the spontaneous regression or growth cessation type, or the continuously slow-growing type, showing 4.2% to 6.2% of positive cells for Ki-67 after immunohistochemical staining. However, 2 patients with the rapid-growing type of disease died of pulmonary metastases; their primary, recurrent, and metastatic tumour specimens contained 9.0% to 11.5% of positive cells for Ki-67. CONCLUSION: Three of the 5 primary tumours had a benign clinical pattern and immunohistochemistry. Two of the 5 patients died of pulmonary metastases, which had an aggressive clinical pattern and a high prevalence of positive cells in Ki-67. Examination of Ki-67 should be carried out for aggressive type of giant cell tumour.

Proximal femoral reconstruction with custom mega prosthesis.

Seventy-seven patients with proximal femoral tumours underwent limb salvage surgery with custom mega prostheses between July 1989 and January 2002. We describe 44 cases with a mean follow-up of 57.8 months. Forty-one patients presented with malignant neoplasms of which 11 were malignant giant cell tumours, eight were chondrosarcoma and five were metastases. Wide margins were achieved for all malignant tumours, and marginal resection was done for all benign and metastatic lesions. Six patients died of disease and two were lost to follow-up. Mechanical complications were observed in six cases, fracture being the most common. Oncological complications occurred in five patients. Functional activity was satisfactory in 79% of patients.

Oncologic and functional results after treatment of giant cell tumors of bone.

Surgical treatment of giant cell tumor of bone has always been a difficult problem because of its local aggressive behavior. Oncologic results and functional outcome are reported here in a retrospective study of 36 patients, treated by various surgical procedures. The average age at the time of diagnosis was 34 years, and the median follow-up period was 7 years. Twenty-three patients were treated by intralesional excision with local adjuvant therapy, and 11 patients by extralesional excision. Two patients received radiotherapy only. Seven local tumor recurrences (30%) were encountered after intralesional procedures, while local tumor control was the rule after extralesional excision. Intralesional excision with local adjuvant therapy resulted in significantly better functional results compared with extralesional excision. Wide excision was associated with a poor functional outcome and marginal excision with a good functional outcome. For the treatment of giant cell tumor of bone, intralesional excision with local adjuvant therapy is recommended because of a good functional outcome. When applying cryosurgery as the local adjuvant, more vigorous freezing may be necessary to improve local tumor control.