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

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 th...

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Autores principales: Malvika Gulati, Abhenil Mittal, Adarsh Barwad, Rambha Pandey, Sameer Rastogi, Ekta Dhamija
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Publicado: Thieme Medical and Scientific Publishers Pvt. Ltd. 2021
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spelling oai:doaj.org-article:cf83dea8b60644cdb03be5536c3637c72021-11-13T23:32:30ZImaging and Pathological Features of Alveolar Soft Part Sarcoma: Analysis of 16 Patients0971-30261998-380810.1055/s-0041-1735501https://doaj.org/article/cf83dea8b60644cdb03be5536c3637c72021-07-01T00:00:00Zhttp://www.thieme-connect.de/DOI/DOI?10.1055/s-0041-1735501https://doaj.org/toc/0971-3026https://doaj.org/toc/1998-3808Context 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.Malvika GulatiAbhenil MittalAdarsh BarwadRambha PandeySameer RastogiEkta DhamijaThieme Medical and Scientific Publishers Pvt. Ltd.articlealveolar soft part sarcomasoft tissue sarcomaimagingfeeding vesselsmetastatic diseaseMedical physics. Medical radiology. Nuclear medicineR895-920ENIndian Journal of Radiology and Imaging, Vol 31, Iss 03, Pp 573-581 (2021)
institution DOAJ
collection DOAJ
language EN
topic alveolar soft part sarcoma
soft tissue sarcoma
imaging
feeding vessels
metastatic disease
Medical physics. Medical radiology. Nuclear medicine
R895-920
spellingShingle alveolar soft part sarcoma
soft tissue sarcoma
imaging
feeding vessels
metastatic disease
Medical physics. Medical radiology. Nuclear medicine
R895-920
Malvika Gulati
Abhenil Mittal
Adarsh Barwad
Rambha Pandey
Sameer Rastogi
Ekta Dhamija
Imaging and Pathological Features of Alveolar Soft Part Sarcoma: Analysis of 16 Patients
description 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.
format article
author Malvika Gulati
Abhenil Mittal
Adarsh Barwad
Rambha Pandey
Sameer Rastogi
Ekta Dhamija
author_facet Malvika Gulati
Abhenil Mittal
Adarsh Barwad
Rambha Pandey
Sameer Rastogi
Ekta Dhamija
author_sort Malvika Gulati
title Imaging and Pathological Features of Alveolar Soft Part Sarcoma: Analysis of 16 Patients
title_short Imaging and Pathological Features of Alveolar Soft Part Sarcoma: Analysis of 16 Patients
title_full Imaging and Pathological Features of Alveolar Soft Part Sarcoma: Analysis of 16 Patients
title_fullStr Imaging and Pathological Features of Alveolar Soft Part Sarcoma: Analysis of 16 Patients
title_full_unstemmed Imaging and Pathological Features of Alveolar Soft Part Sarcoma: Analysis of 16 Patients
title_sort imaging and pathological features of alveolar soft part sarcoma: analysis of 16 patients
publisher Thieme Medical and Scientific Publishers Pvt. Ltd.
publishDate 2021
url https://doaj.org/article/cf83dea8b60644cdb03be5536c3637c7
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AT adarshbarwad imagingandpathologicalfeaturesofalveolarsoftpartsarcomaanalysisof16patients
AT rambhapandey imagingandpathologicalfeaturesofalveolarsoftpartsarcomaanalysisof16patients
AT sameerrastogi imagingandpathologicalfeaturesofalveolarsoftpartsarcomaanalysisof16patients
AT ektadhamija imagingandpathologicalfeaturesofalveolarsoftpartsarcomaanalysisof16patients
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