SELNET clinical practice guidelines for bone sarcoma.

Bone sarcoma are infrequent diseases, representing < 0.2% of all adult neoplasms. A multidisciplinary management within reference centers for sarcoma, with discussion of the diagnostic and therapeutic strategies within an expert multidisciplinary tumour board, is essential for these patients, given its heterogeneity and low frequency. This approach leads to an improvement in patient's outcome, as demonstrated in several studies. The Sarcoma European Latin-American Network (SELNET), aims to improve clinical outcome in sarcoma care, with a special focus in Latin-American countries. These Clinical Practice Guidelines (CPG) have been developed and agreed by a multidisciplinary expert group (including medical and radiation oncologist, surgical oncologist, orthopaedic surgeons, radiologist, pathologist, molecular biologist and representatives of patients advocacy groups) of the SELNET consortium, and are conceived to provide the standard approach to diagnosis, treatment and follow-up of bone sarcoma patients in the Latin-American context.

Dedifferentiated chondrosarcoma with "adamantinoma-like" features: A case report and review of literature.

Dedifferentiated chondrosarcoma is defined by the presence of a low grade malignant cartilaginous component juxtaposed to a high grade malignant non-cartilaginous sarcomatous components. Only 4 cases in which the high grade component showed epithelial differentiation have been reported in the literature; three featured a squamous and the one a glandular epithelial component. Here we describe a case of dedifferentiated chondrosarcoma exhibiting epithelial "adamantinoma-like" basaloid features. The patient underwent wide resection of the proximal tibia and post-operative chemotherapy and died 8 months after the diagnosis due to lung and bone metastases.

Primary extraskeletal myxoid chondrosarcoma of bone: Report of three cases and review of the literature.

Extraskeletal myxoid chondrosarcoma is a rare neoplasm of soft tissue. The usual location is in deep parts of the proximal extremities and limb girdles in middle-aged adults. The bone location as primary location is extremely rare and few cases are reported. We present three cases arising in bone with molecular confirmation using both RT-PCR and FISH analysis. Patients include two men and one woman with an age of 62, 69 and 73 years old. The mean size of the lesion was 13cm (range 8-18cm). Tumors arose in the iliac bone in two cases and in the proximal humerus in the other case. At time of diagnosis the three cases show bone cortex and soft tissue involvement. On imaging, lesions have a lobular pattern, are purely lytic, but take up contrast medium after injection. Two patients are alive with disease (local recurrence and lung metastasis) after five years and five years and six months, respectively and one patient died of disease two years after the diagnosis. The primary extraskeletal myxoid chondrosarcoma of bone seems to have a more aggressive behavior than the soft tissue counterpart. The molecular confirmation of diagnosis using RT-PCR is necessary to do the differential diagnosis with other entities, in particular with myoepithelioma that shows similar morphological features and EWSR1 and FUS genes rearrangement.

Metastasizing tenosynovial giant cell tumour, diffuse type/pigmented villonodular synovitis.

Tenosynovial giant cell tumour, diffuse type, also known under a variety of other terms including diffuse pigmented villonodular synovitis, tends to be locally aggressive and not infrequently can show multiple recurrences. The differential diagnosis with the extremely rare and somewhat controversial malignant variant of tenosynovial giant cell tumour, diffuse type, is challenging due to overlapping radiologic features of these two entities. Malignant tenosynovial giant cell tumour is defined by the presence of overtly malignant sarcomatous areas. We describe a very unusual case of a 63-year-old man affected by tenosynovial giant cell tumour, diffuse type of the knee that, despite absence of morphologic evidence of sarcomatous transformation, developed inguinal lymph node metastases following multiple surgical procedures.

Clear cell sarcoma of soft tissue: a retrospective review and analysis of 31 cases treated at Istituto Ortopedico Rizzoli.

INTRODUCTION: Clear cell sarcoma (CCS) of soft tissue is a rare melanocytic soft tissue sarcoma with different cytogenetic and natural history than that of melanoma. Objective of this study was to determine outcome predictors in patients treated in our Institute. This objective included the effectiveness of surgical intervention and disease progression after surgery. MATERIALS AND METHODS: Thirty-one patients were diagnosed at our institute with clear cell sarcoma through tissue pathology and immunohistochemistry. Patients received multimodality treatment (surgery, radiotherapy and chemotherapy). Five-year survival rates and prognostic predictors were determined. RESULTS: Sixteen patients were males and 15 females with a median age of 37 years (8-72-years). Twenty-eight tumors were located in extremities and 3 in the trunk area. Eight patients had metastases at their first presentation (6 local lymph nodes and 2 pulmonary metastases). Five and ten-year disease-specific survival rates were 56% and 41%. Two-year disease-specific survival rates for lymph node and pulmonary metastasis groups were 40% and 0%. All metastatic patients died within 5 years follow-up. Five and ten-year disease-specific survival rates for localized tumor cases were 72% and 53%. Male gender, less than 30-years of age, trunk tumor location and size greater than 5 cm were poor prognostic factors according to univariate analysis. Tumor location in the trunk was the only negative prognostic determinant in multivariate model. CONCLUSIONS: Although surgical treatment may be beneficial for tumors without systemic involvement, new chemotherapeutic agents and molecular targeted therapy should be implemented to improve the oncologic outcome in both early and late stage disease.

Fresh osteochondral allograft is a suitable alternative for wide cartilage defect in the knee.

INTRODUCTION: There are several surgical options to restore a wide osteochondral defect in the knee. Fresh osteochondral allografts are usually considered a poor alternative due to their difficulties in surgical application. The aim of this work is first to present our experience including the surgical technique and the functional results of patients receiving fresh osteochondral allograft to restore major knee lesions, then, to compare our results with other results presented in literature. METHODS: Between 2006 and 2011, we treated 11 patients with osteochondral lesion of the knee (Outerbridge IV°). The average lesion size was 10.3 cm(2) (range 3-20 cm(2)). The average age was 34 years (range 18-66). Patients were followed from 12 to 55 months (average of 26.5) through clinical examination, X-ray film and MRI every 3 months for the first year, then every 6 months. RESULTS: The treatment was successful in 10 patients showing pain regression and mean IKDC subjective score improvements from 27.3 to 58.7. The IKDC objective score also improved of at least one class for each patient except the who failed. The radiographs show good osteointegration in all cases but one. CONCLUSIONS: Fresh allograft is an effective therapy for osteochondral defects repair because it allows functional recovery in a considerable number of patients. This technique obtains better results in lesion smaller than 8 cm(2). However larger lesion show good results. LEVEL OF EVIDENCE: Therapeutic study, Level IV.

Factors affecting outcome of massive intercalary bone allografts in the treatment of tumours of the femur.

We retrospectively reviewed 101 consecutive patients with 114 femoral tumours treated by massive bone allograft at our institution between 1986 and 2005. There were 49 females and 52 males with a mean age of 20 years (4 to 74). At a median follow-up of 9.3 years (2 to 19.8), 36 reconstructions (31.5%) had failed. The allograft itself failed in 27 reconstructions (24%). Mechanical complications such as delayed union, fracture and failure of fixation were studied. The most adverse factor on the outcome was the use of intramedullary nails, followed by post-operative chemotherapy, resection length > 17 cm and age > 18 years at the time of intervention. The simultaneous use of a vascularised fibular graft to protect the allograft from mechanical complications improved the outcome, but the use of intramedullary cementing was not as successful. In order to improve the strength of the reconstruction and to advance the biology of host-graft integration, we suggest avoiding the use of intramedullary nails and titanium plates, but instead using stainless steel plates, as these gave better results. The use of a supplementary vascularised fibular graft should be strongly considered in adult patients with resection > 17 cm and in those who require post-operative chemotherapy.

OP-1 application in bone allograft integration: preliminary results in sheep experimental surgery.

UNLABELLED: Massive bone allografts are frequently used in orthopaedic reconstructive surgery. However the failure rate at long term follow-up is around 25%. AIM: Stimulation of allograft incorporation. MATERIALS AND METHODS: In order to stimulate bone remodeling of an allograft we applied recombinant human osteogenic protein-1 (rh-OP-1, also know as bone morphogenetic protein-7, BMP-7) to a long bone critical size defect sheep model. In nine sheep we created a 3 cm osteoperiosteal metatarsal defect replaced with a structural allograft alone (control group, 4 animals), or an allograft added with rh-BMP-7 (BMP group, 5 animals). Radiographic, mechanical, histological and histomorphometric analysis were performed. RESULTS: X-rays in the BMP group showed a better and faster callus formation, compared to the control group within the first 8 weeks after surgery. After 16 weeks there was a higher evidence of bone remodeling in the BMP group. Radiographic healing at junction sites was more evident in the BMP group at 4, 8 and 16 weeks. Mechanical testing on screw extraction showed no statistical differences between the two groups and histomorphometry showed no difference in terms of newly formed bone inside the allograft as well. The resorption rate of the graft was higher in the BMP group in comparison to the control group. The penetration of newly formed vessels was significantly higher in the BMP group. CONCLUSIONS: These findings indicate that BMP-7 added to a structural bone allograft inducing early remodeling of the graft through stimulation of neo-angiogenesis and osteoclastic activity, without negative effects in mechanical strength and clinical outcome.