Objective The mainstay of treatment modality for extra-abdominal desmoid-type fibromatosis (DF) has shifted from surgery, which often impairs ADL/QOL, to conservative treatment including active surveillance. In the present study, we conducted a longitudinal survey on the diagnosis and treatment of DF at facilities belonging to the Japanese Musculoskeletal Oncology Group, which is a research group of facilities specializing in the treatment of bone and soft tissue tumors in Japan to clarify the transition of medical care for extra-abdominal DF. Methods The same questionnaire was administered in 2015 and 2018, and responses were obtained from 46 (69%) of 67 facilities and 42 (53%) of 80 facilities in 2015 and 2018, respectively. Results Although immunostaining for β-catenin was often used for the pathological diagnosis in both 2015 and 2018, CTNNB1 mutation analysis was not performed either in 2015 or in 2018. As for the treatment strategy for resectable cases, surgical treatment including wide resection was selected at 11 facilities (24% of respondents) in 2015, and further decreased to 5 facilities (12%) in 2018. Conservative treatment with active surveillance or medical treatment was the most common treatment for both resectable and difficult-to-resect cases. COX-2 inhibitors and tranilast were often used in the drug treatment of both resectable and difficult-to-resect cases. Few facilities provided radiotherapy, methotrexate and vinblastine, or DOX-based chemotherapy for refractory cases in both 2015 and 2018. Conclusions A good trend was found in the questionnaire survey. It will be further necessary to disseminate clinical practice guidelines to physicians more widely, and to have them understand and implement the most up-to-date medical practice strategies for this rare disease.
Objective: Primary undifferentiated pleomorphic sarcoma (UPS) of the bone is rare. However, the common sites are the knee and proximal femur and humerus, while spinal involvement is rare. We report a case of primary UPS of the 11th thoracic vertebra, where corpectomy would have been difficult and extensive, treated with carbon ion radiotherapy. Case report: A 76-year-old man presented with an osteolytic tumor of the 11th thoracic vertebra on plain computed tomography (CT). The spinal cord was compressed and displaced posteriorly by the tumor on magnetic resonance imaging (MRI), and extraosseous extension was observed. An incisional biopsy was performed, and primary UPS of the 11th thoracic vertebra was diagnosed pathologically. Total en bloc spondylectomy was considered to be challenging because of the extraosseous extension and the patient's age; thus, carbon ion radiotherapy (70.4 GyE / 32 fraction) was performed. Denosumab (120 mg) was administered subcutaneously every four weeks. No adjuvant chemotherapy was administered. Four years post-treatment, imaging revealed a compression fracture of the 11th thoracic vertebra, but there was no recurrence. Conclusion: Despite a poor prognosis and an aggressive course of UPS of the spine, the tumor continues to be controlled without local recurrence four years after carbon ion radiotherapy.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
customersupport@researchsolutions.com
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.