CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2021; 31(03): 573-581
DOI: 10.1055/s-0041-1735501
Original Article

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

Malvika Gulati
1   Department of Radiodiganosis, Dr. B. R. A. IRCH, All India Institute of Medical Sciences, New Delhi, India
,
Abhenil Mittal
2   Department of Medical Oncology, Dr. B. R. A. IRCH, All India Institute of Medical Sciences, New Delhi, India
,
Adarsh Barwad
3   Department of Pathology, Dr. B. R. A. IRCH, All India Institute of Medical Sciences, New Delhi, India
,
Rambha Pandey
4   Department of Radiation Oncology, Dr. B. R. A. IRCH, All India Institute of Medical Sciences, New Delhi, India
,
Sameer Rastogi
2   Department of Medical Oncology, Dr. B. R. A. IRCH, All India Institute of Medical Sciences, New Delhi, India
,
1   Department of Radiodiganosis, Dr. B. R. A. IRCH, All India Institute of Medical Sciences, New Delhi, India
› Author Affiliations

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.

Institute Ethics Obtained Vide Letter Number

IEC 527/05.06.2020


Source(s) of Support

None.


Presentation at a Meeting

None.




Publication History

Article published online:
07 September 2021

© 2021. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 American Cancer Society. Cancer Facts & Figures 2020. Atlanta: American Cancer Society; 2020
  • 2 Sood S, Baheti AD, Shinagare AB. et al. Imaging features of primary and metastatic alveolar soft part sarcoma: single institute experience in 25 patients. Br J Radiol 2014; 87 (1036): 20130719
  • 3 Kallen ME, Hornick JL. The 2020 WHO Classification: what's new in soft tissue tumor pathology?. Am J Surg Pathol 2021; 45 (01) e1-e23
  • 4 Viry F, Orbach D, Klijanienko J. et al. Alveolar soft part sarcoma-radiologic patterns in children and adolescents. Pediatr Radiol 2013; 43 (09) 1174-1181
  • 5 Prasad A, Chauhan B, Pal H, Bhardwaj M. Alveolar soft part sarcoma with cerebral and skeletal metastases. Indian J Radiol Imaging 2001; 11: 44-45
  • 6 Pennacchioli E, Fiore M, Collini P. et al. Alveolar soft part sarcoma: clinical presentation, treatment, and outcome in a series of 33 patients at a single institution. Ann Surg Oncol 2010; 17 (12) 3229-3233
  • 7 Christopherson WM, Foote Jr FW, Stewart FW. Alveolar soft-part sarcomas; structurally characteristic tumors of uncertain histogenesis. Cancer 1952; 5 (01) 100-111
  • 8 Ladanyi M, Lui MY, Antonescu CR. et al. The der(17)t(X;17)(p11;q25) of human alveolar soft part sarcoma fuses the TFE3 transcription factor gene to ASPL, a novel gene at 17q25. Oncogene 2001; 20 (01) 48-57
  • 9 Crombé A, Brisse HJ, Ledoux P. et al. Alveolar soft-part sarcoma: can MRI help discriminating from other soft-tissue tumors? A study of the French sarcoma group. Eur Radiol 2019; 29 (06) 3170-3182
  • 10 McCarville MB, Muzzafar S, Kao SC. et al. Imaging features of alveolar soft-part sarcoma: a report from Children's Oncology Group Study ARST0332. AJR Am J Roentgenol 2014; 203 (06) 1345-1352
  • 11 Li X, Ye Z. Magnetic resonance imaging features of alveolar soft part sarcoma: report of 14 cases. World J Surg Oncol 2014; 12: 36
  • 12 Cui J-F, Chen H-S, Hao D-P, Liu J-H, Hou F, Xu W-J. Magnetic resonance features and characteristic vascular pattern of alveolar soft-part sarcoma. Oncol Res Treat 2017; 40 (10) 580-585
  • 13 Kim HS, Lee HK, Weon YC, Kim H-J. Alveolar soft-part sarcoma of the head and neck: clinical and imaging features in five cases. AJNR Am J Neuroradiol 2005; 26 (06) 1331-1335
  • 14 Itani M, Shabb NS, Haidar R, Khoury NJ. AIRP best cases in radiologic-pathologic correlation: alveolar soft-part sarcoma. Radiographics 2013; 33 (02) 585-593
  • 15 Daly BD, Cheung H, Gaines PA, Bradley MJ, Metreweli C. Imaging of alveolar soft part sarcoma. Clin Radiol 1992; 46 (04) 253-256
  • 16 Tian L, Cui C-Y, Lu S-Y, Cai P-Q, Xi S-Y, Fan W. Clinical presentation and CT/MRI findings of alveolar soft part sarcoma: a retrospective single-center analysis of 14 cases. Acta Radiol 2016; 57 (04) 475-480
  • 17 Fong Y, Coit DG, Woodruff JM, Brennan MF. Lymph node metastasis from soft tissue sarcoma in adults. Analysis of data from a prospective database of 1772 sarcoma patients. Ann Surg 1993; 217 (01) 72-77
  • 18 Portera Jr CA, Ho V, Patel SR. et al. Alveolar soft part sarcoma: clinical course and patterns of metastasis in 70 patients treated at a single institution. Cancer 2001; 91 (03) 585-591
  • 19 Paoluzzi L, Maki RG. Diagnosis, prognosis, and treatment of alveolar soft-part sarcoma: a review. JAMA Oncol 2019; 5 (02) 254-260
  • 20 Read WL, Williams F. Metastatic alveolar soft part sarcoma responsive to pazopanib after progression through sunitinib and bevacizumab: two cases. Case Rep Oncol 2016; 9 (03) 639-643
  • 21 Kodama K, Doi O, Higashiyama M. et al. Surgery for multiple lung metastases from alveolar soft-part sarcoma. Surg Today 1997; 27 (09) 806-811
  • 22 Murphey MD, Fairbairn KJ, Parman LM, Baxter KG, Parsa MB, Smith WS. From the archives of the AFIP. Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. Radiographics 1995; 15 (04) 893-917