Post-Systemic Chemotherapy Prognoses of Recurrent/Metastatic Soft Tissue Sarcoma Patients with Retroperitoneal/Intra-Abdominal Origin versus Those with Extremities/Trunk Origin.

BACKGROUND: The clinical benefit of systemic chemotherapy for recurrent/metastatic retroperitoneal/intra-abdominal soft tissue sarcoma (STS) compared to its benefits for other primary lesions has not been known or sufficiently evaluated. METHODS AND PATIENTS: We retrospectively reviewed the cases of the STS patients who consulted a department of medical oncology in Tokyo between June 2011 and March 2018, and we extracted the cases of patients with primary sites at the retroperitoneum/intra-abdomen (cohort R) or extremities/trunk (cohort E) who received systemic chemotherapy in a recurrent/metastatic setting, comparing the cohorts' characteristics, chemotherapy details, and prognoses. RESULTS: Of all 337 STS patients, we enrolled 49 patients in cohort R and 75 patients in cohort E. Liposarcoma was more frequently observed in cohort R (51.0%) than cohort E (22.7%). The median chemotherapy treatment line was two lines (range: 1-6) in cohort R and three lines (range: 1-9) in cohort E. The doxorubicin usage rates differed in recurrent/metastatic settings (90.0% in cohort R and 55.0% in cohort E), due mainly to the higher rate of a perioperative chemotherapy treatment history in cohort E (52.0% vs. 6.1% in cohort R). The median overall survival from the start of salvage chemotherapy was 31.9 months (cohort R; 95% CI: 20.9-42.8) and 27.1 months (cohort E; 95% CI: 21.6-32.5) (p = 0.549). CONCLUSION: There were differences in the distributions of pathology and antitumor drugs used in a salvage setting between retroperitoneal/intra-abdominal and extremities/trunk STS patients in recurrent/metastatic settings, but the prognoses with salvage chemotherapy were similar in the two cohorts.

Primary intramucosal synovial sarcoma of the sigmoid colon in a patient with a germline mutation in the MSH2 gene: A case report.

Synovial sarcoma is a high-grade soft tissue sarcoma that occurs primarily in the deep soft tissue of extremities, and primary colorectal synovial sarcoma is extremely rare. In this report, we present a synovial sarcoma mostly located within the mucosa of the sigmoid colon. The patient was a man in his forties with a germline deletion in the MSH2 gene. He had experienced undifferentiated pleomorphic sarcoma of the left forearm 7 years before and adenocarcinoma of the transverse colon 6 years before, both of which were successfully treated and exhibited no recurrence to date. A surveillance colonoscopy for Lynch syndrome revealed the tumor which had a submucosal tumor-like appearance with central erosion and endoscopic resection was performed. Histologically, it was composed of monotonous proliferation of spindle cells arranged in cellular fascicles; these findings were compatible with monophasic fibrous synovial sarcoma. In the tumor cells, the presence of the SS18-SSX1 fusion gene was confirmed. Protein expression of mismatch repair genes was intact in the tumor cells, indicating the association between microsatellite instability and synovial sarcoma was weak. The present case highlights a rare primary site of synovial sarcoma in a patient with Lynch syndrome.

Definitive surgery of primary lesion should be prioritized over preoperative chemotherapy to treat high-grade osteosarcoma in patients aged 41-65 years.

BACKGROUND: Recently, the number of osteosarcomas in middle-aged and older patients has demonstrated an increasing trend; moreover, their results are comparatively worse than those of young patients. In Europe and the USA, the prognosis for osteosarcoma in middle-aged and older patients has improved with adjuvant chemotherapy. In Japan, however, the prognosis has remained poor. MATERIALS AND METHODS: We retrospectively analyzed the outcomes of osteosarcoma, especially in regards to preoperative chemotherapy, from January 1980 to July 2014. A total of 29 patients with high-grade osteosarcoma between the age of 40 and 65 years were included. We included patients without distant metastasis and with primary lesions that were deemed resectable. The mean age was 52.8 years (range 41-65 years), and the mean follow-up period was 103.2 months (range 5-314 months). RESULTS: Adjuvant chemotherapy was administered to 27 of 29 patients (93%), and 8 of 15 cases (53%) were able to undergo preoperative chemotherapy as planned, including CDDP. A major complication of chemotherapy was acute kidney injury due to CDDP (26%). The 5-year OS and 5-year EFS were 64.9% and 57.1%, respectively. After 2006, a policy to prioritize the resection of the primary lesion was implemented, and if the primary lesion was deemed resectable, preoperative chemotherapy was either not administered or administered for only a short duration. The 5-year OS after 2006 improved to 78.8%. CONCLUSIONS: This study shows that administration of high-dose intensity preoperative chemotherapy was difficult in middle-aged and older patients due to their high rate of acute kidney injury by CDDP. For cases of osteosarcoma in middle-aged and older patients, if the primary lesion is resectable, preoperative chemotherapy should be minimized to prioritize the resection of the primary lesion. It was considered that, with appropriate measures to prevent complications, adjuvant chemotherapy may lead to improved prognosis. LEVEL OF EVIDENCE: V.

Giant cell tumor of the distal femur: Outcome beyond 20 years of follow-up after curettage with polymethylmethacrylate.

BACKGROUND: Polymethymethacrylate (PMMA) is often used to reconstruct defects after curettage of Giant Cell Tumors (GCT). While GCTs usually originate in the epiphysis, the use of PMMA in distal femoral lesions may induce the risk of degenerative osteoarthritis (OA). We investigated the limb function of patients after curettage with PMMA beyond 20 years of follow-up. METHODS: Patients with more than 20 years of follow-up who underwent curettage with PMMA for distal femoral GCTs were observed. We retrospectively investigated the radiographic assessment of OA and functional assessment of the limb. The Kellgren and Lawrence (KL) grading system was used for radiographic evaluation. RESULTS: Five patients were included in this study. The mean age was 33 years, and the mean period from application of PMMA to final follow-up observation was 28.1 years. Four lesions were primary, and one lesion was recurrent. There were no patients with postoperative recurrence. There were no OA changes in preoperative radiographs. The shortest mean distance from PMMA to the articular cartilage was 4.6 mm on radiographs immediately after surgery. On radiographs at final follow-up observation, the KL grading were as follows: grade 1, 2 patients; grade 2, 1 patient; grade 3, 2 patients. All patients were able to independently ambulate without a crutch, and there was not enough pain to require nonsteroidal anti-inflammatory drugs. The mean flexion of the knee joint was 116°. CONCLUSIONS: Although PMMA used for distal femoral GCTs exhibited OA changes beyond a 20 year follow-up period, there were no cases requiring artificial joints, and the affected limbs demonstrated good function.

Pulmonary metastasis from giant cell tumor of bone: clinical outcome prior to the introduction of molecular target therapy.

OBJECTIVE: We analyzed the risk factors for pulmonary metastasis from giant cell tumor of bone and aimed to discuss their therapeutic strategy and appropriate follow-up period. METHODS: We analyzed 141 patients of giant cell tumor of bone. The variables analyzed included age, gender, primary site, Campanacci grading, surgical treatment on the primary lesion, radiotherapy and local recurrence. RESULTS: Pulmonary metastasis occurred in 12 patients. The risk factors were young age, Campanacci Grade III and local recurrence. Median time from initial surgery to metastasis was 1.3 years (0-3.1 years). Among them, eight patients experienced local recurrence of the primary tumor, and the median time from initial surgery to local recurrence was 0.8 years (0.3-2.9 years). Among seven patients who underwent wide resection, three patients showed local recurrence of the soft tissue. Nine patients underwent metastasectomy for pulmonary metastases. Of three patients who did not undergo metastasectomy, one patient died of uncontrollable metastases, and two patients showed no changes in their metastatic lesions. CONCLUSIONS: Although we found a correlation between local recurrence and pulmonary metastasis, we were still unable to prevent local or metastatic recurrence by wide resection. Local recurrence and metastasis have been found within ~3 years after initial surgery, and routine image examinations of the primary site and chest after initial surgical treatment should be considered for at least 3 years postoperatively.

Multicentric giant cell tumor of bone: Case series of 4 patients.

BACKGROUND: We aimed to retrospectively investigate patients with multicentric giant cell tumor (MCGCT) who were treated at our hospital and to clarify their clinical features, treatment policy, and follow-up method. METHODS: Four patients with two or more giant cell tumor (GCT) that occurred in the same patient were treated at our institution between 1978 and 2015. These patients were evaluated for the following: frequency, age of onset, number and site of occurrence, time to occurrence of the next lesion, treatment, recurrence, malignant transformation, metastasis, and oncological outcome. RESULTS: The rate of occurrence was 1.7%. The average age was 25.2 (17-44). The total number of lesions was three in two cases and two in two cases. All four cases had only one lesion during the initial visit. The most frequent site of occurrence was the proximal femur, followed by two lesions that occurred in the metaphysis. The interval between confirmation of the initial lesion and occurrence of the second lesion was in average 12.1 years (0.8-27.0). Initial presentations of lesions were treated by en bloc resection in one case and curettage in three cases. Local recurrences occurred in two cases that underwent curettage. The six lesions that occurred after the initial lesion were treated as follows: en bloc resection in four lesions, curettage and radiation therapy in one, and embolization and radiation therapy in one. Pathologically, no lesions presented malignancy. Pulmonary metastasis occurred in one case. The oncological outcome was NED in three cases and AWD in one case. CONCLUSIONS: No lesions were malignant, and by providing the same treatment as solitary GCT, the oncological outcome was good. It is unnecessary to be concerned of its risks and postoperatively conduct long-term searches for focal lesions across the body.

Risk factors for pneumothorax in advanced and/or metastatic soft tissue sarcoma patients during pazopanib treatment: a single-institute analysis.

BACKGROUND: After the approval of pazopanib for the treatment of soft tissue sarcoma (STS), pneumothorax was reported as an unexpected adverse event during pazopanib treatment. The incidence and risk factors of pneumothorax during pazopanib treatment for STSs have not been established yet. METHODS: We retrospectively reviewed the cases of all of the STS patients treated with pazopanib between November 2012 and December 2014 at our institute and evaluated the prevalence, incidence, treatment details and risk factors for pneumothorax in the STS patients during pazopanib treatment. RESULTS: A total of 58 patients were enrolled; 45 of them had lung and/or pleural lesions at the start of pazopanib treatment. During the median follow-up time of 219 days (range 23-659), 13 pneumothorax events occurred in six patients; the prevalence and incidence of pneumothorax were 10.3 % and 0.56 per treatment-year, respectively. The median onset of pneumothorax was day 115 (range 6-311). No patients died of pneumothorax, but pazopanib was interrupted in 10 events and chest drainage was performed in eight events. Pazopanib continuation or restart after the recovery from pneumothorax was conducted after 9 of the 13 events. The median progression-free survival of patients with and without pneumothorax events were 144 and 128 days (p = 0.89) and the median overall survival periods were 293 and 285 days (p = 0.69), respectively. By logistic regression analyses, the maximum diameter of the lung metastases ≥ 30 mm (OR 13.3, 95 % CI 1.1-155.4, p = 0.039) and a history of pneumothorax before the pazopanib induction (OR 16.6, 95 % CI 1.1-256.1, p = 0.045) were significantly predictive of pneumothorax. CONCLUSIONS: In our retrospective analysis, pneumothorax was observed in 10.3 % of 58 STS patients during pazopanib treatment. The diameter of the lung metastases and a history of pneumothorax could be useful for evaluating the risk of pneumothorax in pazopanib treatment.

Risk factors for distant metastasis of dermatofibrosarcoma protuberans.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) may recur locally but rarely metastasizes. Fibrosarcomatous transformation in dermatofibrosarcoma protuberans (FS-DFSP) is said to have worse prognosis compared with ordinary DFSP (O-DFSP). Since DFSP rarely metastasizes, there have been few reports summarizing data on distant metastasis cases at single institution. The aim of this retrospective study is to review DFSP cases in order to analyze risk factors for metastasis. PATIENTS AND METHODS: This retrospective study involved 67 patients. We analyzed O-DFSP and FS-DFSP metastasis rates, metastasis sites, time to metastasis, the relationship between frequency of local recurrence and metastasis, and the relationship between primary tumor size and metastasis. RESULTS: Distant metastasis was found in 5 (7.4 %) of 67 cases with DFSP. Of the five cases, the histopathological diagnosis was FS-DFSP in four cases and O-DFSP in one case. Out of five cases with metastasis, three had not recurred and two had recurred twice. No clear correlation was identified (Fisher's exact test: p = 0.216). The primary tumor diameters in the metastatic cases were 15.0, 12.6, 20.5, 13.0, and 5.0 cm, respectively. The tumor diameters in metastatic cases were significantly larger (Fisher's exact test: p < 0.0001). CONCLUSIONS: In this study, we identified a stronger correlation between DFSP metastasis and tumor size. There was a high possibility that the cases with large tumors might be FS-DFSP, having high rate of metastasis and poor prognosis. In treatment of DFSP, early diagnosis before primary tumor growth and wide resection is considered important.

Differences in the responses to pazopanib and the prognoses of soft tissue sarcomas by their histological eligibility for the PALETTE study.

OBJECTIVE: In Japan, pazopanib has been made available to soft tissue sarcoma patients, also to patients histologically diagnosed as ineligible for the international Phase 3 study (PALETTE). However, clinical evidence for the use of pazopanib in PALETTE-ineligible patients is currently insufficient. METHODS: We retrospectively reviewed medical records of soft tissue sarcoma patients treated with pazopanib at our institute. By pathological review, the patients' eligibility for the PALETTE study was evaluated and the differences in their responses to pazopanib and incidences of adverse events were investigated. RESULTS: From November 2012 to August 2014, a total of 47 patients received pazopanib, 38 (81%) of whom were histologically eligible for the PALETTE study, and 9 of whom (19%) were not. The median follow-up time was 7.5 months (range 1.4-20.3 months). An objective response was observed in both groups, but the patients' survival tended to be longer in the PALETTE-eligible patients; median progression-free survival was 4.5 months vs. 2.9 months (P = 0.15) and overall survival was 10.7 months vs. 7.8 months (P = 0.55), though these differences were not statistically significant. There were no significant differences in the incidence of adverse events by PALETTE eligibility, but dose skipping or dose reduction was more likely to be observed in PALETTE-ineligible patients. CONCLUSION: Pazopanib is tolerable to soft tissue sarcoma patients ineligible for the PALETTE study and some of them respond to pazopanib, but the prognoses of patients ineligible for the PALETTE study might be worse than those of PALETTE-eligible patients. The indication of pazopanib for soft tissue sarcoma patients with PALETTE-ineligible histologies should be decided carefully.

New endoprosthesis suspension method with polypropylene monofilament knitted mesh after resection of bone tumors in proximal humerus.

BACKGROUND: Endoprosthetic reconstruction of the proximal humerus is one of the standard procedures after resection of tumors of the proximal humerus and has been considered a reliable method to reconstruct the proximal humerus in recent reports. However, instability of the shoulder joint caused by loss of the rotator cuff and deltoid muscle function is often observed after such an endoprosthetic reconstruction. METHODS: We performed the endoprosthesis suspension method with polypropylene monofilament knitted mesh. This suspension method, by which the endoprosthesis is suspended from the bone structure, was used after resection of tumors in 9 patients. We assessed postoperative stability of the shoulder joint by comparing these patients with 12 patients who underwent the conventional surgical technique, by which the mesh-wrapped endoprosthesis is attached only to soft tissue. RESULTS: In radiographic and physical evaluation, 4 of the 12 patients in the soft tissue reconstruction group showed shoulder joint instability. No patient in the suspension method group showed subluxation of the humeral prosthesis. The mean shoulder flexion was 35° and 65° and the mean shoulder abduction was 40° and 40° for the soft tissue reconstruction group and the suspension method group, respectively. DISCUSSION: Shoulder joint subluxation sometimes occurs because of elongation of the attached soft tissue in the conventional reconstruction with mesh, whereas no shoulder joint subluxation occurs after endoprosthetic reconstruction in the suspension method because the bone structure has no leeway for elongation. Excellent stability of our new method enables exercise of the surgical shoulder at an early stage, leading to improved range of shoulder joint motion.

Bipolar Head Perforation With Rhabdomyosarcoma of the Thigh: A Case Report With Literature Review.

Background/Aim: High complication rates during the perioperative management of sarcomas around the pelvis have been reported; however, few include the detailed clinical course or complications in the late postoperative period. Radiotherapy is a multidisciplinary strategy for treating sarcomas. However, irradiated bone and soft tissues show a permanent loss of repair and immunocompetence. We present a case of pleomorphic rhabdomyosarcoma of the thigh that resulted in acetabular collapse induced by radiation and intestinal perforation during long-term follow-up. Additionally, we discuss the risk factors for late complications and pelvic reconstruction methods. Case Report: A 75-year-old man presented with a 1-month history of a recurring fever. Ten years prior, he was diagnosed with pleomorphic rhabdomyosarcoma of the right thigh and underwent a wide resection and bipolar hip arthroplasty, followed by chemotherapy and radiotherapy. Radiographs showed central dislocation of the bipolar head. Computed tomography revealed free air in the hip joint and thickening of the colon wall. Colonoscopy revealed displacement of the bipolar head into the colon wall. Colon resection and hip disarticulation were performed, as the bipolar head was contaminated with intestinal contents. Currently, he is able to walk stably with a walker, and there is no evidence of recurrence or metastasis of the tumor. Conclusion: Irradiation of the periacetabular bone may induce resorptive destruction, resulting in future structural failure. Hardware should not be used for periacetabular bone reconstruction; the risk of pelvic organ damage should be considered when the acetabular collapse becomes deteriorated. Therefore, other reliable and permanent reconstruction options are required.

Bone metastases with "false negative" findings on 18F-FDG PET/CT in patients with angiosarcoma: A case series with literature review.

RATIONALE: Fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) is considered a reliable and indispensable imaging method when evaluating distant metastases and clinical staging of angiosarcomas. Here, we report 2 cases of angiosarcoma with bone metastases with "false negative" findings on 18F-FDG PET/CT. PATIENT CONCERNS: Case 1, a 39-year-old woman, who had undergone mastectomy for primary angiosarcoma 2 years prior, presented with a 5-month history of right coxalgia. Case 2 was a 37-year-old woman, who had undergone mastectomy for primary angiosarcoma 4 months prior. During postoperative follow-up, multiple bone lesions were detected on magnetic resonance imaging (MRI). DIAGNOSES: Based on the histopathological findings, both cases were diagnosed with bone metastases of angiosarcoma. Although MRI showed multiple bone metastatic lesions, 18F-FDG PET/CT showed no uptake or osteolytic destruction in both cases. INTERVENTIONS: Weekly paclitaxel was initiated as a salvage chemotherapy in both cases. OUTCOMES: No uptake or osteolytic lesions were observed on 18F-FDG PET/CT, despite multiple bone metastases detected on MRI. LESSONS: False-negative findings on 18F-FDG PET/CT should be considered when evaluating bone metastases of angiosarcoma. Even with negative findings on 18F-FDG PET/CT, open biopsy should be performed if MRI indicates bone metastases.

Clinical characteristics of sarcoma cases in which long-term disease control was achieved with trabectedin treatment: A retrospective study.

Trabectedin is a therapeutic option for patients with advanced sarcoma. While a randomized trial demonstrated its prolonged progression-free survival (PFS), the reported PFS was <6 months. Some patients can achieve long-term disease control with this treatment. However, the reference information is insufficient. Herein, we retrospectively reviewed 51 sarcoma patients who received trabectedin. We analyzed the clinicopathological features, trabectedin dose, administration schedule, and clinical outcomes, including the overall response rate (ORR) and PFS. Among them, we assessed the detailed data of patients who achieved long-term disease control (PFS >1 year). The ORR in the 49 evaluable patients was 8%, and the median PFS in 51 patients was 7.5 months. Six patients (12%) achieved PFS of >1 year. Five of the six patients had metastatic lesions at trabectedin initiation. The pathological subtypes were myxoid liposarcoma (n = 2), leiomyosarcoma (n = 2), synovial sarcoma (n = 1), and Ewing sarcoma (n = 1). The final administration dose was the minimum dose (0.8 mg/m2) in two patients who continued the treatment over 20 cycles. The best radiological response was partial response (PR) in two myxoid liposarcoma patients and stable disease in four. The durations from trabectedin initiation to the first response in the two PR cases were 163 and 176 days, respectively. Our results support the validity of continuing trabectedin at a sustainable dose and interval in patients who can tolerate it. These results may be useful when considering the clinical application of trabectedin.

SYT-SSX breakpoint peptide vaccines in patients with synovial sarcoma: a study from the Japanese Musculoskeletal Oncology Group.

In the present study, we evaluated the safety and effectiveness of SYT-SSX-derived peptide vaccines in patients with advanced synovial sarcoma. A 9-mer peptide spanning the SYT-SSX fusion region (B peptide) and its HLA-A*2402 anchor substitute (K9I) were synthesized. In Protocols A1 and A2, vaccines with peptide alone were administered subcutaneously six times at 14-day intervals. The B peptide was used in Protocol A1, whereas the K9I peptide was used in Protocol A2. In Protocols B1 and B2, the peptide was mixed with incomplete Freund's adjuvant and then administered subcutaneously six times at 14-day intervals. In addition, interferon-α was injected subcutaneously on the same day and again 3 days after the vaccination. The B peptide and K9I peptide were used in Protocols B1 and B2, respectively. In total, 21 patients (12 men, nine women; mean age 43.6 years) were enrolled in the present study. Each patient had multiple metastatic lesions of the lung. Thirteen patients completed the six-injection vaccination schedule. One patient developed intracerebral hemorrhage after the second vaccination. Delayed-type hypersensitivity skin tests were negative in all patients. Nine patients showed a greater than twofold increase in the frequency of CTLs in tetramer analysis. Recognized disease progression occurred in all but one of the nine patients in Protocols A1 and A2. In contrast, half the 12 patients had stable disease during the vaccination period in Protocols B1 and B2. Of note, one patient showed transient shrinkage of a metastatic lesion. The response of the patients to the B protocols is encouraging and warrants further investigation.

Intraneural metastasis of gastric carcinoma leads to sciatic nerve palsy.

BACKGROUND: Soft tissue metastases, in particular intraneural metastasis, from any carcinomas seldom occur. To our knowledge, no case of sciatic nerve palsy due to intraneural metastasis of gastric carcinoma is reported in the literature. CASE PRESENTATION: A case is reported of a 82-year old woman with sciatic nerve palsy with intraneural metastasis of gastric carcinoma. Although she had undergone partial gastrectomy with T2b, N0, M0 two years ago and primary site was cured, she developed sciatic nerve palsy from the carcinoma metastasis directly to the nerve. Operative resection and Histological examination revealed poorly differentiated adenocarcinoma, the same as her primary site adenocarcinoma. CONCLUSIONS: Sciatica is usually caused by a herniated disc or spinal canal stenosis. Sciatic nerve palsy may be caused by nondiscogenic etiologies that may be either intrapelvic or extrapelvic. It is important to image the entire course of the nerve to distinguish these etiologies quickly. The longer the nerve compression the less likely a palsy will recover. Surgery is a good intervention that simultaneously obtains a tissue diagnosis and decompresses the nerve.

What are risk factors for local recurrence of deep high-grade soft-tissue sarcomas?

BACKGROUND: Patients with local recurrence of soft-tissue sarcomas have a poor overall survival. High-grade, soft-tissue sarcomas in deep locations may have a poorer prognosis regarding local recurrence than low-grade sarcomas or those located superficially. Although previous reports evaluated tumors at various depths, it is unclear what factors influence recurrence of deep, high-grade sarcomas. QUESTIONS/PURPOSES: We therefore determined whether possible risk factors (tumor size, location, histologic subtype, unplanned excision, local recurrence at presentation, metastasis at diagnosis, surgical procedure, surgical margin, and adjuvant treatments) influenced local recurrence of deep, high-grade, soft-tissue sarcomas. PATIENTS AND METHODS: We retrospectively reviewed 433 patients with deep, high-grade, soft-tissue sarcomas surgically treated between 1985 and 2005. For each patient, we reviewed tumor size, location, histologic subtype, unplanned excision, local recurrence at presentation, metastasis at diagnosis, surgical procedure, surgical margin, and adjuvant treatments and determined the effect of each prognostic variable on local recurrence. The minimum followup was 1 month (median, 51 months; range, 1-305 months). RESULTS: Forty-seven patients had local recurrence at a median of 10.7 months. Local recurrence at presentation, metastasis at diagnosis, and positive margins independently predicted local recurrence. No other factors independently predicted local recurrence. CONCLUSIONS: Unplanned excisions did not increase the rate of local recurrence of deep, high-grade, soft-tissue sarcomas if treated appropriately. Aggressiveness of tumor represented by metastasis or local recurrence at presentation may be a risk for local recurrence. LEVEL OF EVIDENCE: Level II, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.

Indications for and surgical complications of rotationplasty.

BACKGROUND: Rotationplasty is one treatment option for femoral bone sarcomas in skeletally immature patients. This procedure can also be used to save failed limb salvage procedures such as infected prostheses and failed bone grafts in adults. Rotationplasty is only rarely indicated, and the surgical complications and risk factors for failure of the procedure that might influence the treatment or patient choices have not been well described. METHODS: We retrospectively reviewed 19 patients who underwent rotationplasty focusing on surgical indications, surgical procedures such as the type of rotationplasty, and whether vessels were sacrificed and reconstructed with vascular anastomosis or preserved. Risk factors for failure of rotationplasty were categorized into either early or late postoperative complications. RESULTS: Thirteen of 19 rotationplasties were done for patients diagnosed with primary sarcoma, 3 patients for unplanned excisions of sarcomas, and 3 patients for infection. Two of 19 patients did not have sufficient circulation resulting in amputation. Six of 19 patients underwent vascular anastomoses, and all of these anastomoses were successful. Three patients had multiple operations for infection before rotationplasty, and one patient's treatment failed so an amputation was required. As late complications, one patient had delayed bone union and one had postoperative infection that healed after removal of plate fixation. Two patients had malrotation of the tibia that resolved with revision surgery. One patient had a skin ulcer that healed with conservative treatment. One patient who had an unplanned excision had a hemipelvectomy for non-union 11 months after rotationplasty. CONCLUSIONS: Rotationplasty was successfully accomplished in 16 of the 19 patients (84 %) with sarcomas, unplanned excision of sarcomas or infection. Vascular anastomosis did not increase the risk of rotationplasty failure in our series. Patients appeared to have a higher risk of failure of rotationplasty after multiple operations, possibly because of inflammation around the vessels. The surgeon should be aware of the higher potential risk and consider vascular anastomosis for these patients.

Long-term results from use of pasteurized bone.

BACKGROUND: Pasteurized bone (PB) is recycled bone. The pasteurization has a tumor cell-killing effect wit retention of initial strength. However, few reports have been published on its long-term course; thus, in this study, we evaluated the long-term course of use of PB and examined appropriate reconstruction methods. PATIENTS AND METHODS: We reviewed 27 cases in which reconstructive surgery using PB was performed between 1990 and 2002. Of these, we excluded 12 fatal cases and 1 case in which follow-up was discontinued. Therefore, our final analysis consisted of 14 cases with an average follow-up period of 165 months. The reconstruction methods used were: osteoarticular graft in 6 cases, composite graft with prosthesis in 3 cases, intercalary graft in 1 case, and reconstruction using PB from the pelvis in 4 cases. RESULTS: The PB survived in 7 of the 14 cases. Five and 10-year survival of the PB was 78.6 and 47.6%, respectively. Three of the 6 osteoarticular cases failed because of late-onset absorption or infection of the PB. For patients with composite graft or intercalary graft, long-term survival was achieved when small amounts of PB were used. For patients with pelvic grafts, long-term survival was achieved in a case of P1 pelvis, but large PB grafts on small bone-junction surfaces were not successful in the long term. On the basis of these results, we were able to achieve successful long-term results with small PB grafts on large bone-junction surfaces. CONCLUSIONS: To prevent bone absorption and achieve long-term survival of PB, it is important to use a small PB graft and create a large surface area of contact with normal bone. These factors are advantageous to PB survival and to gaining limb function.

The Clinical Features of Multicentric Extra-abdominal Desmoid Tumors.

Background: Extra-abdominal desmoid tumors often occur in the necks, shoulder, chest wall, back, arm, buttock, thigh and leg. Multicentric extra-abdominal desmoids are rather rare and seem to have other clinical features. The aim of our study was to investigate clinical features, especially multicentric occurrence of extra-abdominal desmoid tumors. Patients and Methods: A total of 135 patients diagnosed with extra-abdominal desmoid were enrolled in this study from January 2005 to December 2019 at the Cancer Institute Hospital of The Japanese Foundation for Cancer Research. The operative procedure was principally wide excision. The clinicopathological factors [e.g., age, gender, pain, restriction of range of motion (ROM), tumor site, tumor size, surgical margin, multicentric occurrence, local recurrence, tumoral regression] were collected and assessed by univariate analysis. We assessed how multicentric occurrence influenced clinicopathological factors of desmoid tumors. Results: The median follow-up was 39.9 months (range=0.29-259 months). Among 135 patients, 20 had multicentric occurrence. Multicentric extra-abdominal desmoids occurred in the neck in six cases, shoulder in four, chest wall in three, back in three, thigh in two and leg in two. In the case of multicentric occurrence on thighs and legs, tumors arose not in the anterior compartment but in the posterior compartment. Univariate analysis showed association of multicentric extra-abdominal desmoids with high local recurrence (p=0.0003), restriction of ROM (p=0.0012) and tumor size larger than 5 cm (p=0.04) but surgical margins were not correlated with local recurrence (p=0.37). Conclusion: Surgery should be performed in those who have severe pain or restriction of ROM. A 'Wait and see' policy is a first-line management, especially for those with multicentric extra-abdominal desmoids.