The MR features described in this study are useful for distinguishing malignant peripheral nerve sheath tumors from neurofibromas. If a tumor has two or more of the four statistically significant features, it can be considered to be highly suspicious of malignancy and should be subjected to a biopsy for early diagnosis.

Background Myxoid liposarcoma is generally considered a low grade tumor but the presence of areas of round cells exceeding 5% is reportedly associated with a worse prognosis. Whether ''pure'' tumors without round cells are low grade has not been confirmed. While radiotherapy has been used for patients' myxoid liposarcoma it is unclear whether it reduces local recurrences. Questions/purposes We therefore determined the survival, roles of radiotherapy for local control, and prognostic factors of pure myxoid liposarcoma of the extremities and trunk wall. Methods We retrospectively reviewed 53 patients histologically diagnosed with pure myxoid liposarcoma arising in extremities and the trunk wall. Nine patients of the 53 received radiotherapy for primary tumors. Clinical features and prognosis was determined, and various factors were analyzed as to their usefulness as prognostic factors (age, gender, location, size, depth, surgical margin, and adjuvant radiotherapy). The minimum followup was 12 months (mean, 60 months; range, 12-226 months). Results Seven (13%) and 6 (11%) patients developed a local recurrence and distant metastasis, respectively. The 5-and 10-year disease-specific and disease-free survival rates were 90% and 83% and 77% and 77%, respectively.

The data suggest an adequate additional wide excision may improve the local control and survival in patients with an unplanned excision as well as the patients with a planned excision. While patients with unplanned excisions had superficial and smaller tumors, survival and postoperative function were similar to those with planned excisions.

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BackgroundGiant cell tumor of bone (GCTB) is an intermediate tumor known to be locally aggressive, but rarely metastasizing. To plan a prospective study of GCTB, we performed a questionnaire survey for institutions participating in the Bone and Soft Tissue Tumor Study Group (BSTTSG) in the Japan Clinical Oncology Group (JCOG) in 2015.MethodsWe reviewed 158 consecutive patients with primary GCTB treated with curettage without perioperative denosumab from 2008 to 2010 in Japan. We investigated local and distant recurrence rates after definitive curettage. We also investigated the recurrence rate after treatment with preoperative and/or postoperative denosumab with curettage in recent years. There were 40 patients treated with perioperative denosumab, and the factors affecting recurrence in them were investigated.ResultsAnswers were available from 24 of 30 institutions (80.0%) participating in JCOG BSTTSG. Thirty (19.0%) and 4 (2.5%) of 158 patients developed local and distant recurrence after curettage without perioperative denosumab from 2008 to 2010, respectively. Campanacci grade and embolization before surgery were significantly associated with increasing incidence of local recurrence after curettage (p = 0.034 and p = 0.022, respectively). In patients treated with perioperative desnosumab, 120 mg denosumab was administered subcutaneously for a median 6 (2–41) and 6 (1–14) times in preoperative and postoperative settings, respectively. The recurrence rates were 6 of 21 (28.6%), 2 of 9 (22.2%), and 0 of 10 (0.0%) in the preoperative, postoperative, and both pre- and postoperative denosumab treatment groups, respectively. With all of the preoperative treatments, administration exceeding five times was significantly associated with a decreased incidence of local recurrence after curettage (p < 0.001).ConclusionThe recurrence rate of GCTB was still high after curettage, especially in Campanacci grade III, and improvements in the therapeutic strategy are needed in this cohort. There is a possibility that a sufficient dose of preoperative denosumab can reduce recurrence after curettage. Recently, we have started a clinical trial, JCOG1610, to investigate the efficacy of preoperative denosumab in patients who can be treated with curettage in GCTB.