Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study.

BACKGROUND: Extra-abdominal desmoid tumor fibromatosis (DTF) is a rare, locally aggressive soft tissue tumour. The best treatment modality for this patient cohort is still object of debate. QUESTIONS/PURPOSE: This paper aimed to (1) to compare the outcomes of DTF after different treatment modalities, (2) to assess prognostic factors for recurrence following surgical excision, and (3) to assess prognostic factors for progression during observation. METHODS: This was a retrospective multicenter study under the patronage of the European Musculoskeletal Oncology Society (EMSOS). All seven centres involved were tertiary referral centres for soft tissue tumours. Baseline demographic data was collected for all patients as well as data on the diagnosis, tumour characteristics, clinical features, treatment modalities and whether they had any predisposing factors for DTF. RESULTS: Three hundred eighty-eight patients (240 female, 140 male) with a mean age of 37.6 (±18.8 SD, range: 3-85) were included in the study. Two hundred fifty-seven patients (66%) underwent surgical excision of ADF, 70 patients (18%) were observed without therapy, the residual patients had different conservative treatments. There were no significant differences in terms of tumour recurrence or progression between the different treatment groups. After surgical excision, younger age, recurrent disease and larger tumour size were risk factors for recurrence, while tumours around the shoulder girdle and painful lesions were at risk of progression in the observational group. CONCLUSION: Local recurrence rate after surgery was similar to progression rates under observation. Hence, observation in DTF seems to be justified, considering surgery in case of dimensional progression in 2 consecutive controls (3 and 6 months) and in painful lesions, with particular attention to lesions around the shoulder girdle.

Clinical features, prognostic factors and outcome in a series of 29 extra-skeletal Ewing Sarcoma. Adequate margins and surgery-radiotherapy association improve overall survival.

OBJECTIVE: Authors review a series of 29 extra-skeletal Ewing Sarcoma (EES). METHODS: They analyzed characteristics, prognostic factors and outcome of EES. RESULTS: Authors report 60% Overall Survival (OS) and 56% of Event Free Survival (EFS) at 5 years. Better 5 years EFS was found in patients with localized disease (68.8%) compared to metastatic EES (33.3%) (p = 0.042). Radiotherapy + surgery offered the best local treatment (p=0.017). Volume (p = 0.032), Surgical margins (p = 0.01), metastatic disease (p = 0.0013) were a significant prognostic factor for OS at 5-yrs. CONCLUSION: Adequate margins and surgery+radiotherapy improve Overall Survival.

Dedifferentiated soft tissue leiomyosarcoma with heterologous osteosarcoma component: case report and review of the literature.

BACKGROUND: Soft tissue dedifferentiated leiomyosarcoma with heterologous osteosarcomatous component is an extremely rare entity described in only few cases in the literature. CASE PRESENTATION: We report the case of a 65-year-old male patient who, after initial inadequate surgery of a tumor of the left forearm, developed local recurrence that was treated with neoadjuvant chemotherapy, surgery and postoperative radiation therapy. Histologically the tumor showed an abrupt separation of two different patterns. One component consisted of interlacing fascicles of spindle cells with cigar-shaped nuclei strongly positive for smooth muscle actin, desmin and H-caldesmon. The other component consisted of a high-grade pleomorphic sarcoma with osteoid and chondroid matrix production, which positive for SATB2. Thus, a final diagnosis of dedifferentiated leiomyosarcoma was rendered. Fifteen months after treatment, the patient presented further local and distant relapse with pulmonary metastases and died 23 months after the first presentation. DISCUSSION AND CONCLUSIONS: Dedifferentiated leiomyosarcoma is a highly malignant neoplasm with a poor outcome. Extensive sampling of soft tissue leiomyosarcomas is recommended to detect possible dedifferentiated areas as they represent a crucial prognostic parameter.

Multimodal Treatment in Pelvic Ewing Sarcoma: A Prognostic Factor Analysis.

BACKGROUND: Although multidisciplinary therapies have improved local control and overall survival in Ewing sarcoma (ES), the prognosis of pelvic lesions remains markedly worse than that of limb ES. METHODS: We retrospectively evaluated the influence of the type of local treatment, margins, necrosis and sacrum involvement on overall survival (OS) and disease-free survival (DFS) in a series of 21 non-metastatic pelvic ES. RESULTS: The average follow-up was 46.3 months (range 3-156). Only one patient had recurrence, at 11 months after surgery. Eight patients showed pulmonary metastasis and five showed bone metastases. Necrosis was the only significant prognostic factor for overall survival at 5 years (p=0.0132) and disease-free survival (p=0.0086). Overall survival at 5 years was 40.1%. CONCLUSION: Local control in pelvic Ewing sarcoma is comparable for patients treated with surgery (S), surgery plus radiotherapy (S/RT), or definitive radiotherapy (RT). The combination of surgery plus radiotherapy could be indicated in cases of large tumor, a poor necrosis response (< 90%), or an inadequate margin with involvement of the sacrum. A poor response to neoadjuvant therapy is a significant risk factor for both local control and overall survival.

Liposarcoma: Clinico-pathological analysis, prognostic factors and survival in a series of 307 patients treated at a single institution.

BACKGROUND AND OBJECTIVES: Liposarcoma (LPS) is a malignant mesenchymal tumor and the most common soft tissue sarcoma. Four different subtypes are described: well differentiated (WD) LPS or atypical lipomatous tumor (ALT), dedifferentiated (DD) LPS, myxoid LPS, and pleomorphic LPS (PLS). The objective of the study was to investigate prognostic factors and clinical outcome of liposarcoma. METHODS: We retrospectively examined the clinico-pathological features of a series of 307 patients affected by Liposarcoma at a mean follow-up of 69 months (range 6-257). ALT/WD LPS were analyzed separately. The influence of site, size, type of presentation, grading, histotype and local recurrence on local and systemic control and survival was assessed. RESULTS: The statistical analysis indicated that only surgical margins represented a significant prognostic factor for local recurrence in ALT/WD LPS (P = 0.0007) and other subtypes of LPS (P = 0.0055). In myxoid, PLS and DD LPS, significant prognostic factors for metastasis free survival (MFS) were surgical margins (P = 0.0009), size of the tumor (P = 0.0358), histology (P = 0.0117) and local recurrence (P = 0.0015). In multivariate analysis, surgical margins (0.0180), size (0.0432) and local recurrence (0.0288) correlated independently with MFS. Margins (P = 0.0315), local recurrence (P = 0.0482) and metastases (P < 0.0001) were prognostic factors for overall survival (OS). CONCLUSION: Marginal surgery can be an accepted treatment for ALT/WD LPS. In other liposarcoma subtypes (Myxoid, DD, PLS) wide or radical surgery is recommended as the margins significantly influence local recurrence-free survival (LRFS), metastasis-free survival (MFS) and overall survival (OS). Local recurrence and metastases were significant prognostic factors for OS.

Is an Intramedullary Nail a Valid Treatment for Limb-Length Discrepancy After Bone Tumor Resection? Case Descriptions.

One of the most frequent outcomes after resection of bone tumors in children is a limb-length discrepancy. An intramedullary nail is a valid method for lengthening the limb. We report our experience with four cases of limb-length discrepancy in the lower limbs several years after the primary treatment of bone tumor resection and subsequent reconstruction. Two femoral PRECICE® nails (NuVasive, Inc., San Diego, CA) were introduced retrograde and two were introduced in an anterograde manner. All four cases healed and showed a reduction of the limb-length discrepancy, early loading, and complete bone osteogenesis. In one case, a reduction of the joint ROM recovered after release of the iliotibial band and a quadriceps release according to Judet's arthrolysis.

Pre-operative diclofenac HPßCD for pain control of needle biopsy in musculoskeletal neoplasm: preliminary results.

Needle biopsy is the main standard method used for diagnosis of musculoskeletal tumors of the limbs and superficial trunk. Pain control during this procedure is through the use of Local Anaestetic (L.A.). In order to achieve a complete pain control in our cases, recently we started using diclofenac HPßCD 50 mg via s.c. preoperativly. We present the clinical results of a non-randomized study of two eterogeneous groups of patients: "Experimental" Group (1): diclofenac HPßCD 50 mg via s.c. one hour before surgical procedure, local anesthesia and ev. diclofenac HPßCD 50 mg via s.c. 12 hours postoperative; "Conventional" Group (2): local anesthesia and ev. postoperative tramadol 100 mg via oral for pain control. In October 2014, at the Department of Orthopedic Oncology and Reconstructive Surgery of Florence, 37 musculoskeletal biopsies for a bone or a soft tissue lesion were performed. Exclusion criteria for this study were: known allergies to lidocaine, diclofenac, tramadol; known gastric or duodenal ulcers; known gastrointestinal bleed or perforation; refusal of the patients to collaborate. For one or more of these reasons, 6 patients were excluded from this study. In the Group 1, 10 patients (59%) referred no pain during the surgical procedure (8/14 biopsies on soft tissue and 2/3 on bone). In 5 cases (29%) no exacerbation of previous chronic pain, and in 2 cases (12%) a progression of local pain after biopsy (average 1 points higher in the VAS). In Group 2, only 6 patients (42%) did not have any pain during the procedure, 4 (29%) no exacerbation of previous chronic pain and 4 (29%) a progression of local pain (average 2 points higher in the VAS). Despite similar results in both Groups, Group 1 seemed to have a mild better control of perioperative pain. The use of diclofenac HPßCD 50 mg preoperative seems to be a rational approach for minimizing perioperative pain and the preliminary data of our study seem encouraging. Obviously many bias are present in this study (small numbers of cases, heterogeneity of diseases, association with local anesthetic, non-randomized study, comparison between preoperative versus postoperative treatment) and this cannot absolutely be considerate as definitive conclusions.

Synovial chondrosarcoma of the hip: report of two cases and literature review.

Synovial chondrosarcoma (SCH) is a rare malignant tumour. The lesion may arise de novo in intra-articular space or, more frequently, it may occur as a malignant degeneration of synovial chondromatosis (SC). The authors report two cases of SCH of the hip joint occurring on a SC diagnosed 23 years earlier in one case and 10 years earlier in another case. In both cases the SCH showed an intermediate grade of malignancy at histology (grade 2). In the first case, the patient underwent an external hemipelvectomy and he was disease-free 10 years after the operation. In the second case, the patient refused the proposed amputation and, after palliative radiation therapy, she was alive with disease 58 months after diagnosis. A review of literature reports on SCH was carried out. The knee was the most affected joint, followed by the hip. An amputation was performed in two thirds of cases. Amputation was done as primary surgery in one fifth of cases and for tumour recurrence after synovectomy in four fifth of cases. Metastatic dissemination was seen in 29% of cases. An early detection of malignant transformation of SC might improve the rate of limb salvage surgery in these patients, although no reliable clinical and imaging signs of malignant degeneration have been identified so far.