Novel fusion sarcomas including targetable NTRK and ALK.

BACKGROUND: Challenging emerging entities with distinctive molecular signatures may benefit from algorithms for diagnostic work-up. METHODS: Fusion sarcomas (2020-2021, during pandemic) were diagnosed by clinicoradiology, morphology, phenotype, and next-generation sequencing (NGS). RESULTS: Six fusion sarcomas in two males and four females involved the chest-wall, neck, or extremities; ages ranged 2-73, median 18 years. Sizes ranged 5.3-25.0, median 9.1 cm. These include high grade 1) TPR-NTRK1 of proximal femur with a larger rounded soft tissue mass, previously considered osteosarcoma yet without convincing tumor matrix. A pathologic fracture necessitated emergency hemipelvectomy (NED) and 2) novel KANK1-NTRK2 sarcoma of bone and soft tissue with spindled pleomorphic to epithelioid features (AWD metastases). 3) Novel ERC1-ALK unaligned fusion, a low grade infiltrative deep soft tissue hand sarcoma with prominent-vascularity, myopericytoid/lipofibromatosis-like ovoid cells, and collagenized stroma, was successfully treated with ALK-inhibitor (Crizotinib), avoiding amputation. These NTRK and ALK tumors variably express S100 and CD34 and were negative for SOX10. 4) and 5) CIC-DUX4 round cell tumors (rapid metastases/demise), one with COVID superinfection, were previously treated as Ewing sarcoma. These demonstrated mild pleomorphism and necrosis, variable myxoid change and CD99 reactivity, and a distinctive dot-like-Golgi WT1 immunostaining pattern. 6) A chest wall/thoracic round cell sarcoma, focal CD34/ keratins/CK7, revealed nuclear-STAT6, STAT6-NAB2 by NGS, confirming malignant solitary fibrous tumor, intermediate-risk-stratification (AWD metastases). CONCLUSIONS: Recent fusion sarcomas include new KANK1-NTRK2 and ERC1-ALK, the latter successfully treated by targeted-therapy. ALK/NTRK fusion partners TPR and KANK1 suggest unusual high-grade morphology/behavior. Clinicoradiologic, morphologic, and phenotypic algorithms can prompt molecular-targeted immunostains or NGS for final classification and promising inhibitor therapy.

Reconstruction using a frozen autograft for a skull and humeral lesion of synchronous multicentric osteosarcoma after undergoing successful neoadjuvant chemotherapy: a case report and review of the literature.

BACKGROUND: Synchronous multicentric osteosarcoma (SMOS) is a rare disease characterized by simultaneous multicentricity of intraosseous osteosarcoma without visceral involvement. SMOS, including a skull lesion, which occurs relatively rarely, and reconstruction using a frozen autograft after the excision of a lesion of SMOS has been infrequently reported previously. CASE PRESENTATION: We report an 18-year-old girl with SMOS, with lesions located in the left distal femur, right proximal humerus, and left occipital bone. Her major complaint was pain and swelling around the left knee joint. Asymptomatic lesions of the humerus and skull bone were detected on a systemic bone scan. No visceral organ metastasis was observed. A biopsy of the distal femoral lesion revealed osteosarcoma. Based on the histological findings, multiple bone lesions, and absence of visceral lesion, the clinical diagnosis of SMOS was made. After five courses of neoadjuvant chemotherapy with a regimen of doxorubicin and cisplatin, reconstruction using a tumor prosthesis following wide excision of the left distal femur was performed, and total necrosis was histologically observed in the retracted specimen. Following three cycles of adjuvant chemotherapy, tumor excision and reconstruction with a frozen autograft treated with liquid nitrogen was conducted for both lesions of the humerus and skull, rather than tumor prosthesis or synthetics, in order to retain a normal shoulder function, and to obtain a good cosmetic and functional outcome after treatment of the skull lesion. Further adjuvant chemotherapy could not be administered after the completion of the surgical treatment for all lesions because the adverse events due to chemotherapy were observed. At over 5 years after the diagnosis, she remains clinically disease-free. CONCLUSIONS: An early correct diagnosis, the proper management of chemotherapy, and surgical treatment for all lesions are essential for achieving a good clinical outcome, even in SMOS including a skull lesion. By performing reconstruction using a frozen autograft for a proximal humeral lesion and a skull lesion after confirming the good histological efficacy of neoadjuvant chemotherapy for the primary lesion, the excellent function of the shoulder joint and a good cosmetic outcome at the site of the skull lesion was acquired without complications or recurrence.

Use of tranexamic acid is not associated with complications following bipolar hemiarthroplasty for metastatic disease.

INTRODUCTION: Advances in the care of cancer patients have resulted in increased survival. The proximal femur is a common site for metastatic disease, often requiring surgical intervention. Tranexamic acid (TXA) has proven to be safe in elective and traumatic femoral hemiarthroplasty; however, its use has not been investigated in oncologic patients. METHOD: We reviewed 66 patients (37 males) with a mean age of 64 ± 3 years undergoing a hemiarthroplasty for metastatic disease in the femoral neck. A total of 22 (33%) patients received intraoperative TXA. Primary outcomes included postoperative blood loss, intraoperative and postoperative transfusion requirement, and postoperative complications. RESULTS: There was no difference in the baseline characteristics between the TXA and non-TXA groups. When comparing the TXA and non-TXA groups, there were no differences in 72 h postoperative blood loss between groups (1.21 L vs. 1.33 L, p = 0.61), percentage of patients requiring transfusion (36.4% vs. 36.4%, p = 1.0), or the incidence of postoperative complications including venous thromboembolism (14% vs. 11%, p = 0.70) and pulmonary embolism (0% vs. 5%, p = 1.0). CONCLUSION: Oncology patients are a high-risk population for thromboembolic events. This initial study supports the safe use of TXA intraoperatively in femoral hemiarthroplasty performed for metastatic disease.

Therapy-Induced Neural Differentiation in Ewing's Sarcoma: A Case Report and Review of the Literature.

Ewing's sarcoma (ES) is a small round cell tumor of adolescents or young adults that usually arises in the deep soft tissues of the extremities. The tumor cells have uniform round nuclei, fine powdery chromatin and indistinct nucleoli. CD99 (O13) is a product of the MIC 2 gene that is highly sensitive to ES but not specific. A panel of markers should be used for the differential diagnosis of small round cell tumors because nearly all others, on occasion, show membranous staining for CD99. One of the defining feature of ES is the presence of 22q12 gene rearrangement. The presented case is a 6 year-old boy complaining of swelling on his right leg. The biopsy was compatible with classic ES in terms of histopathological, immunohistochemical and cytogenetic criteria. Wide surgical resection was performed after chemotherapy. The posttreatment specimen was composed of uniformly small round cells mixed with areas of ganglion cells embedded in neurophil-like fibrillary background. Immunohistochemically, neoplastic cells revealed strong CD99 (O13) and NSE staining and the tumor had EWSR1 gene rearrangement. Morphologic alterations due to treatment are commonly seen in pediatric tumors. Single case reports have defined neural differentiation in ES but to the best of our knowledge this is the first report of ES in the literature with all histopathological, immunohistochemical, and cytogenetic criteria evaluated in both pretreatment and posttreatment specimens.

Compliance and satisfaction with intensive physiotherapy treatment during chemotherapy in patients with bone tumours and evaluation of related prognostic factors: An observational study.

The purpose of this study was to evaluate compliance and satisfaction of adult patients to intensive rehabilitation treatment during chemotherapy cycles after surgery for bone-musculoskeletal tumours, as well as to identify possible predictive factors. An observational, prognostic, prospective study was conducted. The study enrolled 27 patients who previously had undergone modular knee prosthesis surgery in the period between October 2014 and October 2015. The outcome was compliance to intensive rehabilitation treatment during hospitalisations in the chemotherapy unit and patient satisfaction 6 months' post-surgery. The variables taken into account were linked to the patient's characteristics, to the oncological pathology and to the chemotherapy treatment administered. Patients' compliance was 100% (range, 61-100). The presence of surgery complications (29.6%) produced 5% loss in compliance to treatment; likewise, chemotherapy treatment with prevalent use of ifosfamide reduced compliance to rehabilitation by 6%. The mean patient satisfaction score was 7.9 in the Likert scale from 0 to 10. Intensive physiotherapy starting during chemotherapy administration is a feasible treatment for bone tumour patients that have shown to be able to positively adhere to it. Rehabilitation treatments, within chemotherapy wards, should therefore be promoted according to satisfaction level as reported by patient.

[Clinical, radiologic and pathologic features of giant cell tumor of bone treated with denosumab].

Objective: To investigate the radiological and histopathological features of giant cell tumor of bone treated with RANKL inhibitor denosumab. Methods: Eleven cases were retrieved from the surgical pathology records between March 2015 and June 2017 in Beijing Jishuitan Hospital. Formalin fixed, paraffin embedded specimens were collected and the histological features were evaluated. The imaging features including X ray, magnetic resonance imaging, and computed tomography were also reviewed. Results: These 11 cases of giant cell tumor of bone were derived from five female and six male patients, with age ranged from 20 to 62 years (mean age, 35 years). The tumors were located in the sacrum (6 cases), femur (2 cases), radius (1 case), tibia (1 case) and patella (1 case), respectively. Histologically, all cases showed depletion of giant cells, proliferation of mononuclear cells and different degrees of ossification 3 to 6 months after denosumab therapy. Radiography showed marked osteosclerosis and sclerotic rim formation. Three cases of the sacrum recurred after 5, 6 and 11 months of surgery, and the remaining cases showed no recurrence within follow-up of 1 to 14 months. Conclusions: Denosumab treated giant cell tumors morphologically differ from untreated tumors. Careful attention to a history of denosumab administration is crucial to avoid misdiagnosis and to allow proper differentiation from other tumors and tumor-like lesions.

Molecular Tumor Boards: Realizing Precision Oncology Therapy.

Technological advances in high-throughput next-generation sequencing (NGS) along with advances in computational processes have brought about the dawn of the genomic medicine era. NGS has enabled molecular characterization of malignancies, and facilitated the development and approval of gene- and immune-targeted therapies, both of which impact the mutanome. Clinical implementation of this technology, approval of novel targeted agents, and establishment of molecular tumor boards has enabled precision oncology to become a reality.

Therapeutic effect of neoadjuvant chemotherapy combined with curettage to treat distal femoral osteosarcoma: A case report.

RATIONALE: Osteosarcoma is the most common malignant bone tumor in children and adolescents. Metastasis occurs early, the mortality rate is high, and the tumor results in a tremendous physical, mental, and economic burden on patients. Therefore, the treatment of osteosarcoma has been important for orthopedic surgeons. However, treatment has always been a difficult problem globally. PATIENT CONCERN: We present the case of a 22-year-old girl with increasing local pain in the distal left thigh. DIAGNOSES: The patient was initially diagnosed as bone cancer according to computed tomography (CT) and X-ray imaging. And the patient was further diagnosed as osteosarcoma via to puncture biopsy of the left distal femur. INTERVENTIONS: Local arterial infusion chemotherapy, systemic intravenous chemotherapy, and curettage was conducted because of limb salvage program. OUTCOMES: The patient was doing well with no evidence of local or distant recurrence 7 years after the surgery. LESSONS: Our case indicated that limb osteosarcoma patients can undergo a limb salvage program of local arterial infusion chemotherapy, systemic intravenous chemotherapy, and curettage.

Intercalary allograft augmented with intramedullary cement and plate fixation is a reliable solution after resection of a diaphyseal tumour.

AIMS: Intercalary allografts following resection of a primary diaphyseal tumour have high rates of complications and failures. At our institution intercalary allografts are augmented with intramedullary cement and fixed using compression plating. Our aim was to evaluate their long-term outcomes. PATIENTS AND METHODS: A total of 46 patients underwent reconstruction with an intercalary allograft between 1989 and 2014. The patients had a mean age of 32.8 years (14 to 77). The most common diagnoses were osteosarcoma (n = 16) and chondrosarcoma (n = 9). The location of the tumours was in the femur in 21, the tibia in 16 and the humerus in nine. Function was assessed using the Musculoskeletal Tumor Society (MSTS) scoring system and the Toronto Extremity Salvage Score (TESS). The survival of the graft and the overall survival were assessed using the Kaplan-Meier method. RESULTS: The median follow-up was 92 months (4 to 288). The mean MSTS 87 score was 29.1 (19 to 35), the mean MSTS 93 score was 82.2 (50 to 100) and the mean TESS score was 81.2 (43 to 100). Overall survival of the allograft was 84.8%. A total of 15 patients (33%) had a complication. Five allografts were revised for complications and one for local recurrence. CONCLUSION: Intercalary allografts augmented with intramedullary cement and compression plate fixation provide a reliable and durable method of reconstruction after the excision of a primary diaphyseal bone tumour, with high levels of function and satisfaction. Cite this article: Bone Joint J 2017;99-B:973-8.

A case report of apatinib in treating osteosarcoma with pulmonary metastases.

RATIONALE: Osteosarcoma is the most common malignant bone tumor in children and adolescents. Pulmonary metastases lead to a significantly increased risk of death. Apatinib, a new potent oral small-molecule tyrosine kinase inhibitor targeting the intracellular domain of vascular endothelial growth factor receptor 2 (VEGFR-2), shows survival benefits in treating advanced or metastatic gastric adenocarcinoma, non-squamous non-small cell lung cancer and metastatic breast cancer. However, its efficacy in metastatic osteosarcoma has not been reported yet. PATIENT CONCERNS: Herein, we presented a 50-year-old man patient who visited hospital due to local bone pain in the left leg. DIAGNOSES: He was initially diagnosed with osteoblastic osteosarcoma. INTERVENTIONS: The patient suffered repeated resection surgeries but developed multiple lung metastases. Positive staining for CD31, CD34, and VEGFR-2 were detected in the tumor section. As he refused to receive chemotherapy due to concerns regarding the chemotherapy toxicities and sorafenib due to high cost, apatinib was given at a dose of 500 mg daily. OUTCOMES: Eleven months following apatinib administration, the patient achieved a partial response according to the RECIST 1.1 standard. No severe toxicity or drug-related side effect was observed during the treatment. LESSONS: Therefore, apatinib could be a new option for the treatment of metastatic osteosarcoma. Clinical trials are required to further confirm the efficacy and safety of apatinib in treating pulmonary metastases from osteosarcoma.

Metal wear-induced pseudotumour following an endoprosthetic knee replacement for Ewing sarcoma.

Pseudotumours are well recognised as a complication of metal-on-metal hip arthroplasties and are thought to develop on the basis of an innate and adaptive immune response to cobalt-chrome (Co-Cr) wear particles. We report a case of a large pseudotumour that developed following a knee endoprosthetic replacement (EPR) undertaken for Ewing sarcoma. The lesion contained necrotic and degenerate connective tissue in which there were numerous scattered metal wear-containing macrophages, eosinophil polymorphs, lymphocytes, plasma cells and aseptic lymphocyte-dominated vascular-associated lesion-like lymphoid aggregates. Metal ion levels were elevated. No evidence of infection or tumour was noted and it was concluded that the lesion was most likely an inflammatory pseudotumour developing on the basis of an innate and adaptive immune response to components of Co-Cr metal wear derived from the knee EPR.

Denosumab in advanced/unresectable giant-cell tumour of bone (GCTB): For how long?

BACKGROUND: Giant-cell tumours of bone (GCTB) are RANK/RANK-ligand (RANKL) positive, aggressive and progressive osteolytic tumours. Denosumab, a RANKL inhibitor, was FDA-approved for adults and skeletally mature adolescents with unresectable GCTB or when surgical resection is likely to result in severe morbidity. Data on long-term toxicity and activity of denosumab monthly 'GCTB-schedule' (120 mg per 12/year, 1440 mg total dose/year) are lacking. METHODS: Patients with GCTB receiving denosumab, 120 mg on days 1, 8, 15, 29 and every 4 weeks thereafter, from 2006 to 2015 treated in two centres were included. Long-term toxicity was evaluated. RESULTS: Ninety-seven patients were identified. 43 patients underwent resection of the tumour with a median time on denosumab treatment of 12 months (range 6-45 months). Fifty-four patients had unresectable GCTB's (male/female 23/31, median age 35 years [range: 13-76 years], 26% presented with lung metastases, 31% had primary tumor located to the spine, 63% were relapsed after previous surgery) with a median time on denosumab of 54 months (9-115 months). In the unresectable GCTB group, tumour control and clinical benefits were observed in all patients undergoing denosumab, whereas 40% of patients discontinuing denosumab had tumour progression after a median of 8 months (range 7-15 months). ADVERSE EVENTS: Overall, six (6%) patients developed osteonecrosis of jaw (ONJ): 1/43 (2%) in the resectable group, 5/54 (9%) in the unresectable group, with a 5-year ONJ-free survival of 92% (95% CI 84-100). Only patients with prolonged treatment experienced mild peripheral neuropathy (6/54, 11%), skin rash (5/54, 9%), hypophosphataemia (2/54, 4%) and atypical femoral fracture (2/54, 4%). CONCLUSIONS: Prolonged treatment with denosumab has sustained activity in GCTB, with a mild toxicity profile. The dose-dependent toxicity observed recommends a careful and strict monitoring of patients who need prolonged treatment. Decreased dose-intensity schedules should be further explored in unresectable GCTB.

Non-Hodgkin's lymphoma involving a femur bone and bilateral adrenal glands alone with adrenal insufficiency.

Primary bone lymphoma and primary adrenal lymphoma are rare clinicopathological entities of non-Hodgkin's lymphoma (NHL). We present the first case of diffuse large B-cell lymphoma with the involvement of a single bone and both adrenal glands alone with adrenal insufficiency. As primary extranodal NHL may have other unusual extranodal lesions, which may present unexplained clinical findings, patients with primary extranodal NHL require careful systemic examination, even when lymphadenopathy is absent.

Successful Therapy with Nivolumab in Metastatic Renal Cell Carcinoma After Multiple Prior Treatments.

Renal cell carcinoma (RCC) is responsible for 80 to 85 percent of all primary renal malignancies. In the United State%, there are about 63,000 new cases and almost :14,000 deaths per year from RCC. Surgical resection of localized RCC can be curative but many patients eventually recur. Immunotherapy appears to be a promising new modality for many malignancies, including RCC. Nivolumab, a specific immunotherapy agent indicated for advanced RCC, may restore antitumor immunity and allow for greater progression-free survival by targeting proteins that negatively regulate T cell immunity. This case study aims to demonstrate the integration of nivolumab into the management of a patient with advanced RCC and provide a stimulus for further investigation and research into this treatment modality.

Ewing Sarcoma of the Bone With EWS/FLI1 Translocation After Successful Treatment of Primary Osteosarcoma.

Although prognosis in patients with localized osteosarcoma has been dramatically improved by the introduction of multiple chemotherapy agents known as combination chemotherapy, there is growing concern about the development of secondary malignant neoplasms. We report the case of a 13-year-old girl in whom the diagnosis of Ewing sarcoma of bone localized on the shaft of left femur was made 2 years after successful treatment without radiotherapy for osteosarcoma of right proximal femur. EWS-FLI1 fusion gene was detected by reverse transcriptase-polymerase chain reaction. To our knowledge, this is the first case with Ewing sarcoma of the bone as a secondary malignant neoplasm developed in osteosarcoma survivor. We collected 15 cases, included this case, with secondary Ewing sarcoma family of tumor by utilizing the PubMed search and might consider the causes of this secondary cancer.

Maturation toward neuronal tissue in a Ewing sarcoma of bone after chemotherapy.

BACKGROUND: Ewing sarcoma is the second most common bone tumor, occurring mainly in children and young adults. It shows a typical primitive, small round cell morphology and a characteristic fusion oncogene involving EWSR1 and members of the ETS family in most of the cases. Neuronal maturation after chemotherapy is a rare phenomenon and we herein describe such an exceptional case. CASE PRESENTATION: An 8-year old boy was diagnosed with a Ewing sarcoma in the left femur. On biopsy the morphology was typical and there was an EWSR1-FLI1 gene fusion. He underwent neo-adjuvant chemotherapy and resection of the tumor. On microscopic evaluation, part of the tumor showed ganglioneuroblastoma-like differentiation with expression of neuronal markers. The continued presence of EWSR1 rearrangement in both the blue round cell component and the ganglioneuroblastoma-like component was shown by FISH analysis. CONCLUSIONS: In conclusion, this case describes the possibility of a Ewing sarcoma to differentiate into a ganglioneuroblastoma-like lesion after neo-adjuvant chemotherapy treatment; the prognostic value of this phenomenon remains questionable.

Local Treatment with Adjuvant Therapy for Central Atypical Cartilaginous Tumors in the Long Bones: Analysis of Outcome and Complications in One Hundred and Eight Patients with a Minimum Follow-up of Two Years.

BACKGROUND: A central atypical cartilaginous tumor (ACT)--formerly known as chondrosarcoma grade 1 (CS1)--is a tumor of intermediate-type malignancy, often treated with surgery. The extent of surgery remains controversial, as some advocate resection and others favor local treatment by curettage. Because of the low prevalence of ACT/CS1, the available data are limited and generally not uniform. The purpose of this study was to present the outcome for a large cohort of patients with ACT/CS1 in the long bones who were treated with curettage and adjuvant phenolization and followed for a minimum of two years according to national guidelines. METHODS: A retrospective study was designed to analyze data from 108 patients treated for central ACT/CS1 in the long bones between 2006 and 2012. All patients were treated with curettage and adjuvant phenolization, and defects were filled with polymethylmethacrylate, bone graft, or bone substitutes. The primary end point was local recurrence or residual tumor. Secondary end points included the type and rate of complications and reoperations. RESULTS: All patients were free from local recurrence at a mean follow-up of 48.7 months (range, 24.3 to 97.5 months). Residual tumor was suspected in five patients, leading to a 95.4% disease-free survival rate. A fracture occurred in eleven patients (10.2%). Other complications were osseous penetration during the surgery (two patients), wound infection (one patient), arthrofibrosis (one patient), and skin necrosis (one patient). Tumor volume was related neither to the risk of fracture nor to the occurrence of residual tumor. CONCLUSIONS: In our experience, curettage of ACT/CS1 in the long bones with adjuvant phenolization is safe, even with large tumors of up to 100 cm(3). Most worrisome is the risk of fracture, which occurred in 10.2% of our patients. Considering the relatively mild behavior of ACT/CS1, less aggressive treatment, by observation or by minimally invasive surgery, could be the next step that should be evaluated prospectively.

Risk-Based Therapy for Localized Osteosarcoma.

BACKGROUND: The outcome of localized osteosarcoma has remained constant over the past 30 years. Histological response to preoperative chemotherapy is the best predictor of outcome. Strategies to alter treatment based on histological response have not resulted in increased survival. PROCEDURE: Patients with localized osteosarcoma received preoperative chemotherapy with cisplatin, doxorubicin, and methotrexate. Patients whose tumors had a good histological response (≥90% necrosis) continued with the same treatment postoperatively. Patients with poor histological response (<90% necrosis) received three courses of melphalan 100 mg/m(2) on day -4, cyclophosphamide 2,000 mg/m(2) on days -3, and -2 followed by stem cell infusion. RESULTS: Fifty-two patients were enrolled. Median age was 14 years, and 56% of patients were male. The femur was the most common site. Forty patients underwent limb salvage surgery and amputation was performed in six patients. Forty-eight percent of tumors showed good histological response. Forty patients were evaluable for outcome; 18 patients with poor histologic response received high-dose chemotherapy. The 5-year event-free survival (EFS) and overall survival (OS) for patients treated on the high-dose chemotherapy arm were 28% (95% confidence interval [CI], 10-49) and 48% (95% CI, 23-69), respectively. The 5-year EFS and OS for patients treated on the standard chemotherapy arm were 62% (95% CI, 36-80) and 74% (95% CI, 44-90), respectively. All patients who received high-dose chemotherapy developed grade 3 or higher hematological toxicity. There were no treatment-related deaths. CONCLUSIONS: Postoperative alkylator intensification with high-dose cyclophosphamide and melphalan in patients with localized osteosarcoma with poor histological response failed to improve survival.

Trabectedin clinical cases: use according to indication in diverse clinical scenarios.

BACKGROUND: Key distinguishing characteristics of trabectedin in the treatment of advanced soft tissue sarcoma are its prolonged tumor control activity in multiple histological subtypes, positive outcomes in translocation-related sarcomas, maintenance of response, option to rechallenge after treatment interruption and lack of cumulative toxicity. Trabectedin is indicated for use in advanced soft tissue sarcoma after failure of anthracyclines and ifosfamide, or as front-line treatment in patients unsuited to receive these agents. METHODS: In this review, cases studies are presented in which trabectedin was used according to its indication but in diverse clinical settings. RESULTS: As second-line treatment of uterine leiomyosarcoma, trabectedin produced prolonged tumor control with good quality of life. In treatment of recurrent synovial sarcoma, the best objective response (partial response) and longest disease control (37 months) was achieved under treatment with trabectedin. As neoadjuvant treatment of undifferentiated pleomorphic sarcoma in a patient unsuited to receive doxorubicin-based chemotherapy, trabectedin induced a pathological response with 85% of necrosis. CONCLUSION: These cases illustrate the broad range of indications for trabectedin in advanced soft tissue sarcoma and highlight how its unique characteristics can be optimized to achieve maximum clinical benefit.

Outcomes of curettage and anhydrous alcohol adjuvant for low-grade chondrosarcoma of long bone.

BACKGROUND: Low-grade chondrosarcoma of long bones can be treated successfully with extended intralesional curettage using adjuvants. However, there is no study reporting the use of anhydrous alcohol as an adjuvant in the treatment of low-grade chondrosarcoma. We asked (1) whether intralesional curettage and anhydrous alcohol adjuvant for low-grade chondrosarcoma is associated with good oncologic outcomes; and we report (2) the complications of the procedure. METHODS: Thirty-six patients (13 men, 23 women) with a mean age of 46 years (range, 18-67 years) were treated for low-grade chondrosarcoma and followed up for a median of 62 months (range, 24-169 months). After intralesional curettage, and additional burring, anhydrous alcohol was used as an adjuvant therapy. RESULTS: At the time of last follow-up, there were no local recurrences or distant metastases. Six patients developed complications: 4 postoperative fractures (11%), 1 intra-articular loose body (3%) and 1 postoperative joint stiffness (3%). CONCLUSION: Anhydrous alcohol is a reasonable adjuvant for the curettage of low-grade chondrosarcoma of long bones. A long-term follow-up study is necessary, considering the slow biological progression of low-grade chondrosarcoma.