Image-guided core needle biopsy for musculoskeletal lesions.

BACKGROUND: Image-guided percutaneous core needle biopsy (CNB) has been an important diagnostic procedure for musculoskeletal lesions. Here we surveyed the variety of diagnostic strategies available and assessed the clinical usefulness and limitations of image-guided CNB carried out by a multidisciplinary team comprising specialists in various fields. METHODS: We conducted a retrospective study of 284 image-guided CNBs among 1899 consecutive biopsy procedures carried out at our institution for musculoskeletal tumorous conditions, focusing on their effectiveness including diagnostic accuracy and utility for classification of specimens according to malignant potential and histological subtype as well as their correlation with biopsy routes. RESULTS: Among the 284 studied biopsies, 252 (88.7%) were considered clinically "effective". The sensitivity for detection of malignancy was 94.0% (110/117) and the specificity was 95.3% (41/43). The diagnostic accuracy for detection of malignancy was 94.4% (151/160) and that for histological subtype was 92.3% (48/52). The clinical effectiveness of the procedure was correlated with the complexity of the biopsy route (P = 0.015); the trans-pedicular, trans-retroperitoneal and trans-sciatic foramen approaches tended to yield ineffective results. Repeat biopsy did not have a significant impact on the effectiveness of image-guided CNB (P = 0.536). CONCLUSIONS: The diagnostic accuracy rates of image-guided CNB performed at multidisciplinary sarcoma units were usable even for patients who have variety of diagnostic biopsy procedures. It is important to establish and implement diagnostic strategies based on an understanding that complicated routes, especially for spine and pelvic lesions, may be associated with ineffectiveness and/or complications.

Survivin: A novel marker and potential therapeutic target for human angiosarcoma.

Human angiosarcoma is a rare malignant vascular tumor associated with extremely poor clinical outcome and generally arising in skin of the head and neck region. However, little is known about the molecular pathogeneses and useful immunohistochemical markers of angiosarcoma. To investigate the mechanisms of angiosarcoma progression, we collected 85 cases of human angiosarcoma specimens with clinical records and analyzed ISO-HAS-B patient-derived angiosarcoma cells. As control subjects, 54 cases of hemangioma and 34 of pyogenic granuloma were collected. Remarkably, consistent with our recent observations regarding the involvement of survivin expression following Hippo pathway inactivation in the neoplastic proliferation of murine hemangioendothelioma cells and human infantile hemangioma, nuclear survivin expression was observed in all cases of angiosarcoma but not in hemangiomas and pyogenic granulomas, and the Hippo pathway was inactivated in 90.3% of yes-associated protein (YAP) -positive angiosarcoma cases. However, survivin expression modes and YAP localization (Hippo pathway activation modes) were not correlated with survival. In addition, we confirmed that survivin small interference RNA (siRNA) transfection and YM155, an anti-survivin drug, elicited decreased nuclear survivin expression and cell proliferation in ISO-HAS-B cells which expressed survivin consistently. Conclusively, these findings support the importance of survivin as a good marker and critical regulator of cellular proliferation for human angiosarcoma and YM155 as a potential therapeutic agent.

Discoidin, CUB and LCCL domain-containing protein 2 (DCBLD2) is a novel biomarker of myxofibrosarcoma invasion identified by global protein expression profiling.

Myxofibrosarcoma (MFS) is a mesenchymal malignancy characterized by frequent recurrence even after radical wide resection. To optimize therapy for MFS patients, we aimed to identify candidate tissue biomarkers of MFS invasion potential. Invasion characteristics of MFS were evaluated by magnetic resonance imaging and protein expression profiling of primary tumor tissues performed using two-dimensional difference gel electrophoresis (2D-DIGE). Protein expression profiles were compared between invasive and non-invasive tumors surgically resected from 11 patients. Among the 3453 protein spots observed, 59 demonstrated statistically significant difference in intensity (≥2-fold) between invasive and non-invasive tumors (p<0.01 by Wilkoxon test), and were identified by mass spectrometry as 47 individual proteins. Among them, we further focused on discoidin, CUB and LCCL domain-containing protein 2 (DCBLD2), a receptor tyrosine kinase with aberrant expression in malignant tumors. Immunohistochemistry analysis of 21 additional MFS cases revealed that higher DCBLD2 expression was significantly associated with invasive properties of tumor cells. DCBLD2 sensitivity and specificity, and positive and negative predictive values for MFS invasion were 69.2%, 87.5%, 90%, and 63.6%, respectively. The expression level of DCBLD2 was consistent in different portions of tumor tissues. Thus, DCBLD2 expression can be a useful biomarker to evaluate invasive properties of MFS. Further validation studies based on multi-institutional collaboration and comprehensive analysis of DCBLD2 biological functions in MFS are required to confirm its prognostic utility for clinical application.

Impact of geriatric factors on surgical and prognostic outcomes in elderly patients with soft-tissue sarcoma.

OBJECTIVE: Patients aged ≥65 years requiring surgery for soft-tissue sarcoma are a concern in an aging society. We aimed to reveal the association of clinical/geriatric factors with survival period or postoperative events in such patients who underwent surgery. METHODS: We enrolled patients aged ≥65 years who underwent surgery for localized soft-tissue sarcoma at five institutions. We retrospectively collected clinical/geriatric factors and laboratory data, and analyzed their association with outcomes using univariate and multivariate analyses. RESULTS: Among the 202 patients included, mean age at presentation was 73 years. Surgical margin was R0 in 139 patients (69%). The Eastern Cooperative Oncology Group performance status was ≥2 in 15 (7%). Thirty patients (15%) showed thinness (body mass index <18.49 kg/cm2). High-sensitivity-modified Glasgow prognostic score ≥1 was seen in 52 patients (26%). Multivariate analysis showed that R1 surgical margin was significantly correlated with poor sarcoma-specific survival (hazard ratio for R1 vs. R0, 3.17; P = 0.001) and event-free survival (hazard ratio for R1 vs. R0, 2.56; P < 0.001). Higher Eastern Cooperative Oncology Group performance status was significantly associated with poor sarcoma-specific survival (hazard ratio for ≥2 vs. 0 or 1, 2.15; P = 0.038), and higher sensitivity-modified Glasgow prognostic score was significantly associated with poor event-free survival (hazard ratio for ≥1 vs. 0, 1.74; P = 0.046). Severe thinness (body mass index <16.00) was a risk factor for postoperative events (odds ratio for body mass index <16.00 vs. ≥16.00, 8.15, P = 0.010). CONCLUSIONS: Negative surgical margin was associated with better survival. Coexisting conditions had an impact on outcomes in elderly soft-tissue sarcoma patients.

A histological positive margin after surgery is correlated with high local re-recurrence rate in patients with recurrent myxofibrosarcoma.

OBJECTIVE: Myxofibrosarcoma has high frequency of local recurrence after surgery. To determine an optimal treatment for recurrent tumors, clinical features of recurrent cases should be characterized. METHODS: We performed a retrospective analysis of 30 patients with recurrent myxofibrosarcoma who underwent surgery between 1999 and 2008. RESULTS: A negative margin after surgery was achieved in only 12 patients (40.0%). The 5-year re-recurrence free-survival rate was 31.7%. The 5-year re-recurrence free survival for those with positive histological margin and those with negative margin were 9.8% and 62.3%, respectively, which indicated that a positive margin was the significant predictor of poor prognosis (P = 0.006). In 21 patients with recurrent myxofibrosarcoma in the extremities, 10 patients (47.6%) ultimately underwent amputation in the follow-up period and the 5-year amputation-free survival rate was 62.5%. The 5-year metastasis-free survival rates and the 5-year overall survival rates were 84.8% and 83.6%, respectively. CONCLUSIONS: In this study, the majority of recurrent cases could not achieve negative margins; notably, a positive margin is a significant poor prognostic indicator of local re-recurrence in patients with recurrent myxofibrosarcoma. To control local recurrence of myxofibrosarcoma was extremely difficult and amputation is often needed in the extremity cases.

Angiomatoid fibrous histiocytoma: a series of seven cases including genetically confirmed aggressive cases and a literature review.

BACKGROUND: Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor of intermediate biologic potential. Because of its rarity and nonspecific radiological and diverse pathological findings, AFH is often clinically misdiagnosed. However, few clinical reports have described this tumor. As reported herein, we analyzed the clinical and radiological features and clinical outcomes of AFH. METHODS: We retrospectively reviewed the medical records of seven cases histopathologically diagnosed as AFH. We examined clinical features, MRI findings, histopathological diagnoses, treatments, and outcomes. RESULTS: These seven cases comprised five male and two female patients with ages ranging from 8 to 50 years old. The primary locations included upper extremities in 2, lower extremities in 4, and the inguinal region in one patient. Of the tumors, 4 occurred in subcutaneous tissues and 3 occurred in deep tissues. No cases were diagnosed as AFH from MRI and needle biopsy results. All cases were diagnosed histopathologically after excision. After treatment, 2 patients (29%) had tumor recurrence and metastasis, one of whom died from disease progression. These 2 aggressive cases involved both EWSR1 and CREB1 gene rearrangements as determined by FISH. The other patients were alive and well without recurrence or metastasis. CONCLUSION: AFH is a rare tumor that is difficult to diagnose. Therefore, it tends to be misdiagnosed and to be treated inadequately by referring physicians. Surgeons must therefore be mindful of the presence of AFH, learn about appropriate treatment necessary for this tumor, and conduct careful follow-up because AFH can engender poor outcomes.

Factors associated with the decision of operative procedure for proximal femoral bone metastasis: Questionnaire survey to institutions participating the Bone and Soft Tissue Tumor Study Group of the Japan Clinical Oncology Group.

BACKGROUND: Pathological fracture of the proximal femur is a main cause of cancer patients losing their ability to walk. Although both osteosynthetic devices (predominantly intramedullary nails) and prosthetic replacement have been widely performed for treatment, controversies exist regarding which procedure should be used for the various conditions. In order to decide the eligibility criteria of a planned randomized prospective study about the treatment of pathological fractures of the proximal femur, we assessed the factors affecting the selection of operative procedures using questionnaires sent to the members of the Bone and Soft Tissue Tumor Study Group (BSTTSG) of the Japan Clinical Oncology Group (JCOG). METHODS: Questionnaire surveys to evaluate (1) the priority levels of the factors, (2) the equipoise range of each factor in situations where either procedure could be applied, (3) risk and benefit of each procedure, and (4) the degree of bone destruction affecting the selection of operative procedures, were sent to 26 institutions. RESULTS: Over 80% of the institutions answered. Orthopaedic surgeons of BSTTSG decided on the procedure according to the following factors in descending order: life expectancy, performance status before fracture, the degree of bone destruction, walking ability before fracture, general complications, the number of bone metastases in other sites, and the visceral metastasis status. With regard to bone destruction, (1) the involvement of the head, neck, calcar, and intertrochanteric region, (2) transverse destruction >1/2, and (3) soft-tissue tumor extension, were the factors that led to the choice of prosthesis treatment. CONCLUSIONS: Using these identified factors, the inclusion criteria for the prospective randomized study of the surgical treatment of metastatic bone tumors of the proximal femur were optimized. The evaluation system about the bone destruction of metastases needs to be refined through the following prospective randomized study.

Feasibility and efficacy of gemcitabine and docetaxel combination chemotherapy for bone and soft tissue sarcomas: multi-institutional retrospective analysis of 134 patients.

BACKGROUND: Bone and soft tissue sarcomas (BSTS) are rare malignant tumors. Recently, the combination of gemcitabine and docetaxel (GD) was shown to have activity as second-line setting in BSTS. However, the efficacy as first-line and adjuvant settings and precise profiles of adverse events in Japanese patients are not known yet. In the present study, the feasibility and efficacy of GD in patients with BSTS were investigated. METHODS: Patients with BSTS treated with GD in our institutions were retrospectively analyzed. Information regarding clinical features, adverse events, and outcome was collected and statistically studied. Factors related to survival were analyzed using log-rank test and Cox proportional hazard regression method. RESULTS: A total of 134 patients were analyzed. GD was carried out as adjuvant setting in 9, first-line in 23, second-line in 56, and third-or-greater line in 46 patients. The response rate (RR) for all patients was 9.7%. RR for the patients treated as adjuvant or first-line setting was 18.8%, whereas that as second-or-greater line was 6.9%. The median progression-free survival (PFS) and overall survival (OS) of all patients were 4.8 (95% CI 3.5-6.1) and 16.4 (95% CI 9.8-22.9) months, respectively. Survival tended to be better in the patients treated as first-line than in those treated as second-or-greater line. Multivariate analysis demonstrated that history of prior chemotherapy (p = 0.046) and response to GD (p = 0.009) was significantly associated with PFS and OS, respectively. The leucopenia and neutropenia were the most frequent adverse events, and grade 3 or 4 leucopenia and neutropenia were observed in 69.4 and 72.4% of the patients. Grade 2 or 3 pneumonitis was observed in one (0.7%) and four (3.0%) patients, respectively. All the patients with pneumonitis had experienced prior chemotherapy and/or radiotherapy. CONCLUSIONS: GD used as both first- and second/later line is effective chemotherapy for a proportion of patients with advanced BSTS. Higher response rate and better outcome was achieved in chemotherapy-naïve patients. This regimen is associated with high incidence of severe hematological toxicity, as well as the risk of severe pneumonitis, especially in pre-treated patients. GD is promising for further analysis by phase III study for the patients with BSTS.

Anterolateral thigh flap for axillary reconstruction after sarcoma resection.

BACKGROUND: Reconstruction of extensive axillary defects after sarcoma resection presents a challenging problem in reconstructive microsurgery. The purpose of this report was to investigate the feasibility of the free anterolateral thigh (ALT) flap for oncologic axillary reconstruction. METHODS: The extensive axillary defects in six patients with sarcoma was reconstructed using a free ALT flap. The defect size ranged from 15 × 11 to 28 × 25 cm2 . Five patients had recurrent cases and the ipsilateral latissimus dorsi flap had been already used in three patients. Two patients with a full-thickness defect underwent chest wall reconstruction with the iliotibial tract. RESULTS: All flaps survived completely and the wounds healed without complications in all patients. CONCLUSIONS: The free ALT flap is an ideal flap for axillary reconstruction after extensive sarcoma resection. It can be tailored to the requirements of the individual's defect and provides durable coverage for the axillary neurovascular bundle and intrathoracic structures. Flap harvesting in the lateral decubitus position enables a two-team approach. © 2015 Wiley Periodicals, Inc. Microsurgery 36:378-383, 2016.

Early detection of local recurrence after soft tissue sarcoma resection and flap reconstruction.

PURPOSE: Flap reconstruction has become an essential component in soft tissue sarcoma treatment. However, the clinical features of local recurrence after soft tissue sarcoma resection and flap reconstruction remain unclear. The present study aimed to establish effective follow-up strategies after soft tissue sarcoma resection and flap reconstruction. METHODS: Data from patients who underwent soft tissue sarcoma resection and immediate flap reconstruction were retrospectively reviewed. Follow-up after surgery included history taking and physical examination during every visit to the hospital. Magnetic resonance imaging to evaluate the primary site was performed six months after the end of treatment then annually for ten years. The methods of detection of local recurrence were assessed. RESULTS: A total of 229 consecutive patients were included in the present study. During a median follow-up period of 40 months, 33 patients (14.4%) developed local recurrence. Twenty-three recurrences that occurred on the margin of the transferred flap were detected as palpable mass prior to radiological assessment; among the remaining ten recurrences that occurred in the deep layer of the transferred flap, six were detected by abnormal clinical findings and four were clinically occult and detected by surveillance radiological assessment. CONCLUSIONS: Surveillance radiological assessment has an important role in early detection of local recurrence that develops in the deep layer of the transferred flap. Therefore, meticulous clinical assessment combined with routine radiological study should be performed during follow-up evaluation for local recurrence after soft tissue sarcoma resection and flap reconstruction.

[A Case of Severe Hematological Toxicity in Response to Pazopanib].

The patient(woman, approximately 46 years old)began pazopanib (PAZ) treatment (800 mg/day)f ollowing the recurrence of retroperitoneal leiomyosarcoma. Prior to treatment, the patient's platelet count was 18.6×10(4)/µl and her neutrophil count was 1.61×10(3)/µl . The platelet count decreased to 9.2×10(4)/µl on day 7 and to 5.4×10(4)/µl on day 21 after commencement of treatment. The neutrophil count was 0.97×10(3)/µl on day 28 and 0.68×10(3)/µl on day 35 after commencement of treatment. Thus, PAZ treatment was stopped on day 35. The blood sampling results on day 42 after commencement of treatment showed that the platelet count was 13.0×10(4)/µl and that the neutrophil count had recovered to 1.28×10(3)/µl . At that time, PAZ treatment was resumed at a reduced dose of 600 mg/day. By day 84 after commencement of treatment, the platelet count had increased from 12.7 to 13.8×10(4)/µl and the neutrophil count had increased from 1.02 to 1.34×10(3)/µl ; treatment was subsequently continued. The main adverse effects that have been reported for PAZ are hypertension and frequent liver dysfunction; these reports also indicate that the incidence of severe cytopenia(thrombocytopenia, neutropenia)is quite low. However, our patient exhibited cytopenia after commencement of PAZ treatment and her blood cell counts recovered once treatment was ceased, independent of other possible medications. Our findings suggest that cytopenia should be considered as an adverse effect of PAZ.

Prognostic Value of Relevant Clinicopathologic Variables in Epithelioid Sarcoma: A Multi-Institutional Retrospective Study of 44 Patients.

BACKGROUND: Epithelioid sarcoma (ES) is an extremely rare soft tissue sarcoma. Recently, the proximal variant has been reported to be a more aggressive subtype; however, as most reports of ES have involved small case series, the actual prognostic implications remain unclear. We investigated the clinicopathological features of patients with ES to identify the prognostic factors that influence survival. METHODS: We retrospectively analyzed the clinicopathological features of 44 patients with ES who had been treated at our institutions between 1991 and 2011. Among these patients, 26 were diagnosed histologically as having classic-type ES, whereas the remaining 18 had proximal-type ES. Thirty-three of the patients, all without distant metastases, underwent curative surgery, and the remaining 11 with distant metastases (M1) received palliative treatment. RESULTS: The proximal subtype was significantly correlated with a proximal tumor location, distant metastases at presentation, presence of rhabdoid cells, a higher tumor grade, and vascular invasion. The overall survival (OS) rate at 5 years for the 44 patients was 45 %. A superficial tumor location and lymph node metastases (N1) at presentation were independently predictive of local recurrence-free survival (LRFS), and N1 and M1 tumors were independently predictive of distant metastasis-free survival and OS, respectively. The proximal subtype was associated with unfavorable LRFS and OS, although not to a statistically significant degree. CONCLUSIONS: Proximal-type ES has significantly more aggressive clinicopathological features than classic-type ES, and lymph node or distant metastasis has the most critical impact on prognosis.

Perioperative chemotherapy with ifosfamide and doxorubicin for high-grade soft tissue sarcomas in the extremities (JCOG0304).

OBJECTIVE: The efficacy of perioperative chemotherapy for soft tissue sarcomas is controversial and only a few prospective studies of pre-operative chemotherapy for soft tissue sarcomas in the extremities have been reported. We therefore carried out Phase II study of perioperative chemotherapy for patients with soft tissue sarcomas in the extremities. METHODS: Patients with Stage III non-round cell soft tissue sarcomas in the extremities were eligible. The patients were treated with pre-operative chemotherapy consisting doxorubicin 60 mg/m(2) and ifosfamide 10 g/m(2) for three courses. After the tumor resection, two additional courses of the same regimen were carried out. RESULTS: A total of 72 patients were enrolled and 70 patients were eligible. The median age of the patients was 49 years. The major pathological subtypes were synovial sarcoma in 20 and undifferentiated pleomorphic sarcoma in 17 patients. The protocol treatments were completed in 74% of the eligible cases. The 2 and 5-year progression-free survival rates were 75.7% (95% CI, 63.9-84.1%) and 63.8% (95% CI, 51.3-73.9%), respectively. The 5-year overall survival was 82.6% (95% CI, 71.3-89.7%). There was no treatment-related death. Grade 3 or 4 hematological toxicities (leukopenia and neutropenia) were observed in most of the patients. CONCLUSIONS: Although the toxicities of the regimen were significant, pre-operative chemotherapy followed by post-operative chemotherapy using doxorubicin and high-dose ifosfamide was feasible. The outcome of the trial for the patients with high-grade soft tissue sarcomas in the extremities was favorable, and this regimen is promising for further investigation. This trial was registered at the UMIN Clinical Trials Registry (www.umin.ac.jp/ctr/) as C000000096.

Pediatric myositis ossificans mimicking osteosarcoma.

Myositis ossificans (MO) is a rare benign cause of heterotopic bone formation in soft tissue that most commonly affects young adults, typically following trauma. We report the case of an 11-year-old girl who developed MO mimicking osteosarcoma in her right shoulder. Plain radiography and computed tomography showed poorly defined flocculated densities in the soft tissue and a periosteal reaction along the proximal humerus. On magnetic resonance imaging, the mass displayed an ill-defined margin and inhomogeneous signal change. Histologically, the mass had a pseudosarcomatous appearance. Based on these findings, the patient was initially misdiagnosed with osteosarcoma at another hospital. The diagnosis was difficult because the patient was 11 years old and had no trauma history, with atypical radiographic changes and a predilection for the site of origin for osteosarcomas. We finally made the correct diagnosis of MO by carefully reviewing and reflecting on the pathological differences between stages.

Cross-cultural adaptation and validation of the Japanese version of the Toronto Extremity Salvage Score (TESS) for patients with malignant musculoskeletal tumors in the lower extremities.

BACKGROUND: Before this work a Japanese version of the Toronto Extremity Salvage Score (TESS), a disease-specific patient-completed questionnaire widely used to assess the physical function of patients with musculoskeletal tumors, had not been developed. The purpose of this study was cross-cultural adaptation and validation of the English-language version of the TESS to facilitate international comparisons of treatment results. METHODS: The TESS was translated into Japanese, back-translated into English, and reviewed by a committee to develop a consensus Japanese version of the TESS. One hundred and two patients were assessed by use of this Japanese version to examine its reliability and validity. RESULTS: Test-retest reliability and internal consistency determined by using the intraclass correlation coefficient (0.941) and Cronbach's alpha test (0.978), respectively, were excellent. Factor analysis showed that the structure consisted of a three-item cluster; the Akaike information criterion (AIC) network also demonstrated that the items could be divided into three domains in accordance with their content. The Japanese version of the TESS correlated with the Musculoskeletal Tumor Society rating scale (r = 0.811; P < 0.001) and the Short Form-36 physical component summary (r = 0.785; P < 0.001). CONCLUSIONS: Our study suggested that the Japanese version of the TESS is a reliable and valid instrument for measuring patient-reported functional outcome for patients with lower extremity sarcoma, and that it enables international comparisons of treatment results. The spatial association of each item demonstrated by using the AIC network also suggested that the underlying structure of the TESS reflected its coverage of a wide range of physical functions.

Pelvic ring reconstruction with a double-barreled free vascularized fibula graft after resection of malignant pelvic bone tumor.

INTRODUCTION: In patients undergoing limb-salvage internal hemipelvectomy, pelvic ring reconstruction is mandatory to maintain the stability of the pelvis and the spinal column, which finally expected to achieve a good functional outcome. However, no optimal reconstruction method has been established. In addition, no previous reports have highlighted the long-term complications of pelvic ring reconstruction after internal hemipelvectomy. We aimed to analyze the outcome of pelvic ring reconstruction using a double-barreled free vascularized fibula graft (VFG) after internal hemipelvectomy with special reference to long-term complications. MATERIALS AND METHODS: We conducted a retrospective review of 9 consecutive patients (5 male, 4 female; mean age 31 years) who underwent pelvic ring reconstruction using a double-barreled free VFG after internal hemipelvectomy (P1, n = 4; P1 + 4, n = 3; P1 + 2, n = 2) at our institution between 1998 and 2013. The mean follow-up period was 55 months (range 3-131 months). RESULTS: The mean length of the bone defect was 9 cm. The methods of fixation included a Cotrel-Dubosset rod (n = 4), screw (n = 3), and screw and plate (n = 2). Bone union was achieved in 5 of 8 patients (63 %) over a 1-year follow-up. The mean period required for bone union was 5.4 months (range 3-7 months). There were 3 early postoperative complications: 2 deep infections resulting in graft removal and 1 implant failure resulting in non-union. Among 3 patients, 2 developed scoliosis within 5 years. One patient developed lumbar disc hernia as a result of scoliosis, for which surgical intervention was required. The mean Musculoskeletal Tumor Society score was 57 % at the last follow-up. CONCLUSIONS: In conclusion, this reconstruction method can achieve an early and high rate of bone union and provide good functional outcome. However, follow-up with careful attention to postoperative complications, including deep infection in the early postoperative period and spinal deformity in the long term, is necessary.

Ewing sarcoma arising after treatment of diffuse large B-cell lymphoma.

We report the case of a patient in whom the diagnosis of Ewing sarcoma arising from a soft tissue was made after successful treatment of diffuse large B-cell lymphoma. A 65-year-old woman presented with a rapidly growing mass in her left scapular region 8 years after successful chemotherapy with the cyclophosphamide, hydroxydaunomycin hydrochloride, vincristine, prednisolone regimen for diffuse large B-cell lymphoma. Computed tomographic examination and magnetic resonance imaging of the thorax revealed an intramuscular tumour measuring 40 mm in size in the left scapular region. Histopathological examination of an open biopsy specimen revealed a small round cell tumour that showed positive staining for CD99. Fluorescence in situ hybridization showed a split signal by a break-apart probe for the EWS gene in chromosome 22q12. Reverse transcriptase-polymerase chain reaction confirmed the expression of EWS-FLI1 fusion transcripts. Based on these findings, the patient was diagnosed as having secondary Ewing sarcoma. Despite adjuvant chemotherapy, however, she died of pulmonary metastases 2 years after the diagnosis of Ewing sarcoma. Therapy-related haematological malignancies with balanced translocations have been reported previously. A mechanism similar to that underlying the development of secondary malignancy might explain the occurrence of this solid cancer.

Prediction of treatment outcomes in patients with chest wall sarcoma: evaluation with PET/CT.

OBJECTIVE: The aim of this study was to investigate the prognostic implications of (18)F-2-fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography in patients with chest wall sarcoma. METHODS: Positron emission tomography/computed tomography scans of 42 patients (mean age: 46 years) with chest wall sarcomas were analyzed. Pathologic confirmation was obtained by surgical specimens in all patients. Tumor grade assessed by Ki-67 (MIB-1) immunohistochemical analysis and expression of glucose transporter protein 1 were compared with a maximum standardized uptake value. Univariate and multivariate analyses were conducted for estimates of overall and event-free survivals. RESULTS: The median maximum standardized uptake value of the tumor was 10.2 and the median MIB-1 index of the tumor was 32.5%. Glucose transporter protein 1 expression was found in 29 patients (69%). Univariate analyses revealed that surgery, chemotherapy, MIB-1 labeling index (cut-off 32.5%), MIB-1 grade, glucose transporter protein 1 expression and maximum standardized uptake value were possible predictors for overall and event-free survival. Multivariate analysis revealed that surgery (hazard ratio, 4.852; P = 0.017), maximum standardized uptake value (hazard ratio, 3.077; P = 0.037) and MIB-1 labeling index (hazard ratio, 6.549; P = 0.003) were independent predictors of event-free survival. In addition, surgery (hazard ratio, 4.092; P = 0.021) and maximum standardized uptake value (hazard ratio, 2.968; P = 0.027) were independent predictors of overall survival. CONCLUSIONS: (18)F-2-fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography allows the prediction of prognosis after treatment in patients with chest wall sarcoma and may be useful in selecting high-risk patients for more risk-adapted treatments.

Extraskeletal myxoid chondrosarcoma: a review of 23 patients treated at a single referral center with long-term follow-up.

BACKGROUND: Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma. Although it has been regarded as a low-grade sarcoma unassociated with tumor-related death, a recent study has suggested an insidious nature with a high propensity for relapse during a long disease course. The aim of this study was to clarify the long-term clinical features of EMC treated at a single referral center using state-of-the-art techniques. METHODS: A retrospective review of 23 consecutive patients (10 males, 13 females; mean age 58 years) treated between 1979 and 2008 (mean follow-up; 109 months) was performed. RESULTS: Surgery for the primary tumor was performed in 22 patients, and 7 cases recurred locally due to inadequate resection. Eleven patients had metastatic disease, either at diagnosis (3) or developing later (8). The 5/10-year overall survival rates were 91/84 %, and the 5/10-year local recurrence-free and metastasis-free survival rates for patients with localized disease were 89/62 and 89/61 %, respectively. Larger tumor size (>10 cm) and metastases at diagnosis were significant negative prognostic factors. Four patients received ifosfamide-based chemotherapy with no objective response. There was no local recurrence in three patients who underwent R1 resection followed by adjuvant radiotherapy. Clinical palliation and retarded progression of the metastatic disease were achieved in three patients who underwent radiotherapy. CONCLUSIONS: EMC is indolent but has a high propensity for relapse over 5 years of follow-up. Definitive initial surgery and careful monitoring for a prolonged period are important. Radiotherapy seems beneficial in an adjuvant setting and as palliative therapy for metastatic disease.