Chondrosarcomas of the small bones: analysis of 44 patients.

BACKGROUND: Chondrosarcomas of the small bones of the hands and feet are uncommon and account for less than 2% of all chondrosarcomas in the skeleton; a 4.2% rate of malignant degeneration of enchondromas to secondary chondrosarcomas has been reported. We performed this study to assess the outcome of the patients with chondrosarcomas of the small bones. We hypothesized that the presumed better prognosis of chondrosarcomas in these locations could be biased as the majority of these tumors tend to be of lower grades and are removed when still small sized, and that less aggressive surgery has an adverse effect on local control MATERIALS AND METHODS: We retrospectively studied the files of 44 patients with chondrosarcomas of the small bones of the hands and feet. There were 23 female and 21 male patients with a mean age of 50.9 years (range, 6-86 years). The mean follow-up was 13 years (range, 5-40 years). We recorded the patients' details including gender and age at diagnosis, type and duration of symptoms, tumor location and histology, type of surgery and complications, and outcome (local recurrences and metastases). RESULTS: The most common anatomical location for chondrosarcomas of the hands was the metacarpals and proximal phalanges. The most common presenting symptom was a slowly enlarging palpable mass. Overall, 36 chondrosarcomas were secondary to a pre-existing cartilaginous tumor. Patients with syndromes were affected in younger age compared to the others. The mean age at diagnosis was higher for higher grade chondrosarcomas. Overall, 13 patients (29.5%) experienced a local recurrence; the rate of local recurrence was higher after curettage regardless the histological grade of the tumors. After wide resection of the first local recurrence, five patients experienced local re-recurrence. Five patients (11.4%) experienced lung metastases, two patients at presentation. All these patients had a high grade chondrosarcomas. At the last follow-up, one patient with lung metastases died from disease, and another patient died from unrelated cause. CONCLUSIONS: The patients with chondrosarcomas of the small bones of the hands and feet may have a dismal outcome if treated improperly. A careful treatment planning is required to avoid unnecessary amputations. Curettage is associated with a high rate for local recurrence that should be treated with a more aggressive surgical resection to avoid re-recurrences. Although the risk is low, the patients may develop lung metastases, especially those with higher grade chondrosarcomas, therefore, they should be staged and followed closely.

Chondroblastoma-like primary malignant giant cell tumor of the humerus - a case report.

35-year-old woman suffered prolonged pain in the left shoulder, where an aggressively growing tumor of the proximal humerus was revealed thereafter. The lesion caused massive osteolysis of the metaepiphysis with cortical disruption, but no soft tissue extension was evident. Given the unsatisfactory effect, the ongoing neoadjuvant chemotherapy was prematurely ceased and the resection 13 cm long segment of bone with modular prosthesis replacement followed. Histologically, clear-cut malignant tumor with both the presence of numerous reactive osteoclast-like giant cells and geographic structural deposition of chondroid matrix bore a close resemblance to chondroblastoma. Dominant cellular composition formed solid mosaic clusters of large, atypical, frequently binucleated cells with voluminous eosinophilic cytoplasm. Impressive nuclear pleomorphism was accentuated by both the grooving and atypical mitotic figures. Thorough sampling disclosed limited, but sharply contrasting parts, where biphasic arrangement of small uniform stromal elements together with regularly distributed, reactive osteoclasts suggested putative precursor giant cell lesion. Except the osteoclasts, all matrical and stromal cells were strongly SOX9 and D2-40 positive; in contrary desmin, SATB2, S100 and p63 yielded completely negative results. Detected H3F3A c.103G>T mutation in exon 2 finally established true nature of that peculiar neoplastic proliferation and lead to descriptive term of primary chondroblastoma-like malignant giant cell tumor. In the setting of all the microscopic variability, histogenesis and complex differential diagnosis of skeletal (malignant) giant cell lesions, there are discussed e.g. aggressive/malignant chondroblastoma, chondroblastoma-like osteosarcoma or giant cell-rich osteosarcoma and practical impact of specific mutational analysis results as well.

Complex surgery for locally advanced bone and soft tissue sarcomas of the shoulder girdle.

Surgical management of primary musculoskeletal tumors of the shoulder girdle is cognitively and technically demanding. Over the last decades, advances in the medical treatments, imaging and surgical techniques have fostered limb salvage surgery and reduced the need for amputation. Despite well-accepted general principles, an individualized approach is often necessary to accommodate tumor extension, anatomical challenges and patient characteristics. A combination of techniques is often required to achieve optimal oncologic and durable functional outcome. Goal of this article is to review approach and management of patients with locally advanced sarcomas of the shoulder girdle requiring major tumor surgery, to illustrate principles of surgical strategy, outcome and complications, and to provide useful guidelines for the treating physicians.

[Periosteal osteosarcoma - personal experience with five cases]. / Periostální osteosarkom - osobní zkusenost s peti prípady.

The authors present five cases of periosteal osteosarcoma located in the femur (4) and tibia (1) in children and young adults (1 female and 4 males) with an age range of 9 - 23 years (mean age 15 years). Radiographs in all cases showed a broad-based soft tissue mass attached to the cortex with periosteal reaction and in two of them cortical disruption with extensive medullary involvement. Follow-ups were available in four cases (range 11 - 73 months) and revealed pelvic metastasis after 15 months with ultimately rapid dissemination and death in a 9-year-old girl and metastasis to the humerus after 13 months in a 15-year-old boy. The former tumor widely extended into the medullary cavity and an amputation was carried out, the latter had a pure juxtacortical position and an en block resection was performed; both of them were treated with chemotherapy. All the lesions displayed distinctive structural patterns combining a large island of tumorous cartilage and hypocellular, bland-looking myxoid mesenchymal stroma with abrupt transition between both components. Contrary to conventional osteosarcoma, the delicate flocculent osteoid deposits were produced by innocuous stromal cells lacking apparent atypia. They were strictly situated outside the prevailing chondroid areas and disclosed sometimes only after a meticulous search. Immunohistochemical detection of SATB2, S100protein and D2-40 assisted effectively not only in recognition of the real stromal histogenetic derivation, but also in distinction of true differentiation of a heavily mineralized extracellular matrix. Molecular analysis revealed no IDH1/2 mutation in four examined cases. Regardless of unique low-grade morphology in rare periosteal osteosarcoma, an aggressive therapeutical approach similar to conventional osteosarcoma is justified, particularly in the case of a medullary extension.

A quantitative CT analysis of fibula inlayed in a massive allograft for femoral diaphysis reconstruction.

Introduction: In diaphyseal reconstructions for bone tumor resection, massive bone allografts (MBA) are historically regarded as the gold standard. However, these are not without complications, and they present an elevated risk of infection, nonunion and structural failure that increases over time as the graft remains largely avascular. To counteract this disadvantage, a combination of allograft with a vascularized fibula has been proposed. The aim of our study was to objectively review the results of combined vascularized fibula-allograft constructs compared to plain allograft reconstruction for bone defects in tumor patients and to assess fibular vitality predictive factors from imaging studies. Materials and methods: Our data was retrospectively reviewed for patients with femoral diaphysis reconstructions in the past ten years. Ten patients (six males and four females) with a mean average follow-up time of 43.80 months (range 20-83, SD 18.17) with combined graft (Group A) were included in the study. As a control group 11 patients (six males and five females) with a mean average follow-up of 56.91 months (range 7-118, SD 41.33) with a simple allograft reconstruction were analyzed (Group B). Demographic and surgical data, adjuvant therapy as well as complications were analyzed in both groups. Both groups were assessed with plain radiographs for bony fusion at the osteotomy sites. Patients in "Group A" had consecutive CT scans at 6 months and then annually to check for potential bone stock and bone density changes. We analyzed total bone density as well as incremental changes in three different areas of the reconstruction. This was done at two defined levels for each patient. Only patients with at least two consecutive CT scans were included in the study. Results: There were no statistical differences between the groups in terms of demographics, diagnosis or adjuvant therapy (p = 1.0). The mean average surgical time (599.44 vs 229.09) and mean average blood loss (1855.56 ml vs. 804.55 ml) were significantly higher in the combined graft group A (p < 0.001 and p = 0.01, respectively). The mean average length of resection (19.95 cm vs. 15.50 cm) was higher in the combined graft group (p = 0.04). The risk for non-union and infectious complication was higher in the allograft group, however, the difference was not significant (p = 0.09 and p = 0.66, respectively). The mean average time to union at junction sites was 4.71 months (range 2.5-6.0, SD 1.19) for cases of successful fibula transfer, 19.50 months (range 5.5-29.5, SD 12.49) for the three cases where we presumed the fibula was not viable and 18.85 months (range 9-60, SD 11.99) for the allograft group. The difference in healing time was statistically significant (p = 0.009). There were four cases of non-union in the allograft group.Seven out of ten patients in Group A exhibited incremental changes in all CT scan measured values. This difference was statistically significant already at 18 months from the index surgery (p = 0.008). The patients with a non-viable fibula had a smaller increase in the percentage of total bone density area measured in the CT scan compared to those patients with a successful fibula transfer (4.33, SD 2.52 vs. 52.29, SD 22.74, p = 0.008). The average bone density incremental increase in-between the fibula and allograft was different among patients with an unsuccessful fibula transfer (32.22, SD 10.41) and the ones with a viable fibula (288.00, SD123.74, p = 0.009). Bony bridges were observed in six cases of viable fibula and in none of the tree presumably dead fibulas (p = 0.03). The mean average MSTS score was higher for the subgroup of successful fibular transfer (26.7/30, SD 2.87) when compared to the group of non-viable fibular graft (17.00/30, SD 6.08) and this was also statistically significant (p = 0-007). Conclusion: A viable fibula enhances incorporation of the allograft and decreases the risk for both structural failure as well as infectious complications. Viable fibula also contributes to better functional status of the recipient. Consecutive CT scans proved to be a reliable method for assessing fibular vitality. When no measurable changes are present at 18-month follow-up, we can declare the transfer unsuccessful with a good amount of certainty. These reconstructions behave as simple allograft reconstructions with analogue risk factors. The presence of either axial bridges between the fibula and allograft or newly formed bone on the inner surface of the allograft is indicative of a successful fibular transfer. The success rate of fibular transfer in our study was only 70% and skeletally mature and taller patients seem to be at increased risk for failure. The longer surgical times and donor site morbidity therefore warrant stricter indications for this procedure.

Dedifferentiated chondrosarcoma: prognostic factors and outcome from a European group.

BACKGROUND: Dedifferentiated chondrosarcoma has a very poor prognosis. Because of its rarity, there are few large studies of outcome which might identify potential prognostic factors. In particular there remains uncertainty about the value of chemotherapy for this condition. METHOD: A retrospective study was done using data supplied by members of the European Musculo Skeletal Oncology Society (EMSOS). We obtained data on 337 patients from nine European centres with this rare condition, with details on patients, treatment and outcome which were then analysed in an attempt to identify prognostic features. RESULTS: The median age was 59 years and there was a slight predominance of males (53%). The most common sites were the femur and pelvis. Twenty-nine percent of patients with a long bone tumour had a pathological fracture. 71 patients (21%) had metastases at the time of diagnosis and these patients had a median survival of 5 months with a 10% chance of survival at 2 years. For the 266 patients without metastases at diagnosis, 254 underwent surgery with 79% having limb salvage. Thirty-one percent of these 266 patients had chemotherapy with 47% of those under 60 receiving it. In this group of 266 patients, overall survival was 28% at 10 years and poor prognostic factors were the presence of a pathological fracture at diagnosis, a pelvic location and increasing age. Local recurrence and overall survival were related to inadequate margins of excision. We did not find that the histological subtype, size of the tumour or the use of chemotherapy significantly affected outcome. For all patients the overall survival was 24% at 5 years. CONCLUSIONS: The prognosis for patients with dedifferentiated chondrosarcoma remains dismal. Surgery with clear margins remains the principal treatment for this condition. Further use of chemotherapy should be within a trial or treatment protocol.

The association of enchondromatosis with malignant transformed chondrosarcoma and ovarian juvenile granulosa cell tumor (Ollier disease).

OBJECTIVE: Ovarian juvenile granulosa cell tumor has an interesting association with multiple enchondromatosis (Ollier disease and Maffucci syndrome) and should be considered a leading diagnosis when an ovarian mass is found in young patients with these conditions. Besides the association with nonskeletal malignancies, there is a high risk of malignant transformation of enchondroma in chondrosarcoma as was also the case in this instance. CASE REPORT: The report uses multiple images to document the representative and characteristic markers of multiple enchondromas in a 22-year-old patient with Ollier disease complicated by malignant transformation of chondrosarcoma and in whom the disease is associated with ovarian juvenile granulosa cell tumor of the right ovary. CONCLUSION: It is important to recognize that when the female patient presents with enchondromatosis and a large unilateral multilocular-solid ovarian mass, the specific diagnosis of granulosa cell tumor can be made with high accuracy.

Local recurrence of giant cell tumor of bone after intralesional treatment with and without adjuvant therapy.

BACKGROUND: The use of adjuvants after curettage has been well established for the treatment of giant cell tumor of bone. The purpose of this study was to analyze the rates of recurrence following different types of treatment as well as the influence of various factors of tumor presentation on those rates. METHODS: The data regarding benign giant cell tumors of the appendicular skeleton from ten bone tumor centers were evaluated. Axial and malignant tumors were excluded. The recurrence rates associated with the different treatment modalities were analyzed, and hazard ratios for a recurrence were calculated for multiple factors of tumor presentation. RESULTS: The study included 384 surgical procedures, involving 256 primary and 128 recurrent tumors. The mean duration of follow-up was 64.2 months. Wide excision was performed in seventy-eight cases (20.3%), and an intralesional procedure was done in 306 (79.7%). Of the intralesional procedures, 103 (33.7%) were performed without the use of adjuvants, 102 (33.3%) included filling with polymethylmethacrylate, seventy-four (24.2%) included polymethylmethacrylate filling after phenolization, and twenty-seven (8.8%) included use of local toxins. The overall recurrence rate after the intralesional procedures was 49% when no adjuvants had been used, 22% when polymethylmethacrylate only had been used as an adjuvant, 27% when polymethylmethacrylate had been used after phenolization, and 15% when phenol or other local toxins had been used (without polymethylmethacrylate). The highest rate of recurrence (36%) after curettage with adjuvants was associated with extracompartmental tumors. Recurrent tumors were not at increased risk for another recurrence, even when they were extracompartmental. The recurrence rate following curettage of a primary tumor without the use of adjuvants (55%) was higher than that following the same treatment of a recurrent tumor (39%) (p = 0.033). CONCLUSIONS: Use of polymethylmethacrylate as an adjuvant significantly reduces the recurrence rate following intralesional treatment of benign giant cell tumors, and it appears to be the therapy of choice for primary as well as recurrent giant cell tumors of bone. The significantly better results following treatment of recurrent tumors without adjuvants compared with the results of the same treatment of primary tumors were probably related to increased surgical thoroughness brought about by the surgeon's awareness of dealing with a riskier tumor.