Alveolar soft part sarcoma in children and young adults: A report of 69 cases

2018 ◽  
Vol 65 (5) ◽  
pp. e26953 ◽  
Author(s):  
Ricardo J. Flores ◽  
Douglas J. Harrison ◽  
Noah C. Federman ◽  
Wayne L. Furman ◽  
Winston W. Huh ◽  
...  
Keyword(s):  
Young Adults ◽  
Soft Part ◽  
Alveolar Soft Part Sarcoma ◽  
Children And Young Adults

Alveolar soft part sarcoma of the head and neck. A disease of children and young adults

1989 ◽  
Vol 17 (2) ◽  
pp. 139-153 ◽  
Author(s):  
W.Bradley Simmons ◽  
Holly Susan Haggerty ◽  
B. Ngan ◽  
Cynthia K. Anonsen
Keyword(s):  
Young Adults ◽  
Head And Neck ◽  
Soft Part ◽  
Alveolar Soft Part Sarcoma ◽  
Children And Young Adults

Vincristine, irinotecan, and temozolomide as a salvage regimen for relapsed or refractory sarcoma in children and young adults.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 10040-10040
Author(s):  
Byung-Kiu Park ◽  
Hee Young Ju ◽  
Meerim Park ◽  
Hyeon Jin Park
Keyword(s):  
Young Adults ◽  
Survival Rate ◽  
Synovial Sarcoma ◽  
Ewing Sarcoma ◽  
Soft Part ◽  
Alveolar Soft Part Sarcoma ◽  
Salvage Regimen ◽  
Children And Young Adults ◽  
Grade 3 ◽  
One Year

10040 Background: No standard salvage regimen is available for recurrent or refractory sarcoma. Only a few studies using vincristine (V), irinotecan (I), and temozolomide (T) for rhabdomyosarcoma or Ewing sarcoma have been conducted mostly in pediatric age. We investigated the efficacy and toxicity of VIT regimen for several relapsed or refractory sarcomas in children and young adults. Methods: We retrospectively reviewed the relapsed or refractory sarcoma patients who were treated at the Center for Pediatric Cancer of the National Cancer Center, Korea between 2012 and 2018. VIT regimen given every 3 week schedule was as follows; V, 1.5mg/m2 i.v. on day 1, I, 50mg/m2 i.v. on days 1-5, and T, 100mg/m2 p.o. on days 1-5. Cefixime prophylaxis at 4mg/kg/day p.o. was administered to reduce irinotecan-induced diarrhea. Results: Total 26 patients (12 males) were treated with VIT during the study period. Patients were diagnosed with rhabdomyosarcoma ( n= 8), osteosarcoma ( n= 7), Ewing sarcoma ( n= 3), synovial sarcoma ( n= 3), alveolar soft part sarcoma ( n= 2), and mixed rhabdomyosarcoma and liposarcoma, hemangiopericytoma and desmoplastic small round cell tumor, one for each. Median age at the start of VIT was 18.5 years (range 2.0-39.9). VIT was delivered as the 2nd to 7th line of treatment, with the 4th line most common (9/26, 34.6%). Number of administered courses were 1-18, with two courses most common (6/26, 23.1%). Of the 25 evaluable patients, we had 2 partial response (PR), 13 stable disease (SD), and 11 progressive disease (PD) with an overall control rate (CR + PR + SD) of 60.0% as assessed by RECIST 1.1. According to diagnoses, we observed 4SD and 4PD for rhabdomyosarcoma; 1PR, 2SD and 4PD for osteosarcoma; 1PR, 1SD and 1 non-evaluable for Ewing sarcoma; 2SD and 1PD for synovial sarcoma; 2SD for alveolar soft part sarcoma. With a median follow-up of 24 months, 5 patients were alive without disease; 9 were alive with disease; 12 died of PD. Progression-free survival rate at one year was 33.8%, and overall survival rate was 79.3% at one year and 45.5% at 2 years. Of the 26 patients, two patients had grade 4 neutropenia; two had grade 3 colitis and another two had grade 3 neutropenic fever; 8 had grade 2 diarrhea. Grade 4 non-hematologic toxicity or treatment-related mortality was not observed. Conclusions: VIT was effective especially for disease control and relatively safe in our cohort of sarcoma patients.

scholarly journals Imaging and Pathological Features of Alveolar Soft Part Sarcoma: Analysis of 16 Patients

2021 ◽  
Author(s):  
Malvika Gulati ◽  
Abhenil Mittal ◽  
Adarsh Barwad ◽  
Rambha Pandey ◽  
Sameer Rastogi ◽  
...  
Keyword(s):  
Young Adults ◽  
Soft Tissue ◽  
Soft Part ◽  
Alveolar Soft Part Sarcoma ◽  
Preoperative Imaging ◽  
Histopathological Diagnosis ◽  
Imaging Features ◽  
Common Site ◽  
Hyperintense Signal ◽  
Characteristic Imaging

Abstract Context Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor most commonly occurring in deep intramuscular plane of lower extremities of adolescents and young adults. It is a highly vascular, slow growing tumor with malignant potential having lung as the most common site of metastases at the time of presentation. Aims The aim is to review the imaging findings of ASPS and determine characteristic imaging features of this rare tumor. Materials and Methods Sixteen patients having histopathological diagnosis and preoperative imaging of ASPS attending the dedicated sarcoma clinic at our institute were included in the study. The demographic, clinical, and imaging data were retrieved from the case records and then evaluated for characteristic imaging features which may raise suspicion of ASPS. Results The patients ranged from 3 to 72 years of age and with a slight male preponderance. Of the eight CECTs evaluated, 62.5% tumors showed well-defined lobulated margins, 87.5% cases showed intense enhancement with presence of feeder vessels. On CEMRI of 10 patients, 70% had well circumscribed lobulated margins with intense enhancement and tortuous flow voids in most of them. All cases showed T2 hyperintense signal. Fourteen of 16 (87.5%) patients had metastatic disease with lung as the most common site (92.8%). Conclusion ASPS is a rare soft tissue sarcoma seen in children and young adults. Imaging may mimic a vascular malformation due to the presence of tortuous feeders. Misdiagnosis at an early stage may lead to later metastatic presentation of the disease, thus emphasizing the need to suspect it on imaging.

Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults

2006 ◽  
Vol 41 (1) ◽  
pp. 187-193 ◽  
Author(s):  
Mark L. Kayton ◽  
Paul Meyers ◽  
Leonard H. Wexler ◽  
William L. Gerald ◽  
Michael P. LaQuaglia
Keyword(s):  
Young Adults ◽  
Clinical Presentation ◽  
Soft Part ◽  
Adolescents And Young Adults ◽  
Alveolar Soft Part Sarcoma
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