Diagnostic and therapeutic analysis of primary angiosarcoma of long bone / 中华骨科杂志

ABSTRACT

Objective:To explore the clinical, imaging manifestations, treatment methods and prognosis of primary angiosarcoma of longbone, with the aim of improving the diagnosis and treatment.Methods:During April 2014 to April 2018, 5 patients with histopathologically-proven primary angiosarcoma of longbone were enrolled in this retrospective study, clinicaland imagingcharacteristics wereanalysiaed. Thetreatment and follow-up results werediscussed.There were 3 males and 2 females; the age ranged from 27 to 79 years and an average age of 45.2±3.5 years. Theproximal humeruswas1case. Thedistal humeruswas1case.The proximalfemur was 2 cases.Thediatalfemur was 1case. Pathological fracturewas2case.According to the tumor site and the presence or absence of pathological fractures, lesion curettage and large-scale resection were taken separately. Adjuvant treatment was given according to the patient's condition after surgery. The length of the lesion wasmeasured and recorded. The X-ray, CT, MRI and nuclear medicine imaging features of the tumor and its characteristics were recorded and analyzed. The pathological characteristics of the tumor were recorded and analyzed. The treatment of the tumor and treatment effect and prognosis were analyzed.Results:The clinical manifestations of angiosarcoma of long bone are non-specific,local pain and swelling. The imaging manifestationsin X-ray, CT and MRI imaging have certain characteristics, such as osteolytic bone destruction in bone marrow cavity and/orcorti-calmostly,without scleroticrim, soft tissue mass, centripetal non-progressive filling and unevenenhancement or nodules gradual enhancement. The length of the tumor lesion is usually larger, the size is about 6.6±1.2 cm. The expression of CD31 and CD34 inosteoangio sarcoma lesions was higher, and the positiverate of ERG expression was about 100%. The survival time of 2 patients who underwent adjuvant chemotherapy after surgery was15 months, 37 months with median survival time was28 months. One patient who survived radiotherapy survived for 40 months. The survival time of 2 patients who underwent surgery alone was 8 months, 30 months, and the median survival time was19 months.Conclusion:Imaging finding scan beused as adjunctive diagnois of the primary angio sarcoma of bone. ERG is the most specific and sensitive marker in the pathological diagnosis of osteoangiosarcoma. Surgical resectionis still the first choice of treatment for long bone angiosarcoma. Surgical combined with radiotherapy can appropriately extend the survival time, and this program can be used as a treatment method chosen by doctors and patients.