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Alveolar soft-part sarcoma: can MRI help discriminating from other soft-tissue tumors? A study of the French sarcoma group

  • Oncology
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European Radiology Aims and scope Submit manuscript

Abstract

Objectives

To investigate the imaging features of alveolar soft-part sarcomas (ASPS) on pre-treatment MRI in order to identify relevant criteria to distinguish ASPS from other soft-tissue tumors.

Methods

A series of 25 patients (mean age, 18.5 years old) with histologically proven ASPS from five French comprehensive cancer centers was compared to a control cohort of 292 patients with various histologically proven benign and malignant soft-tissue tumors representative of the 10-year long activity of one center. All had a baseline MRI with contrast-agent administration. Two radiologists independently reviewed the MRIs. Features assessing location, size, signal, architecture, periphery, and vascularization were reported. Their association with the histological diagnosis of ASPS was evaluated with chi-square or Fisher’s test. Their prevalence, sensitivity, specificity, odds ratio, and reproducibility were calculated.

Results

Eight MRI features were significantly associated with ASPS: deep location (p < 0.001), high signal intensities on T1-weighted imaging (p < 0.001), central area of necrosis (p = 0.001), absence of fibrotic component (p = 0.003), infiltrative growth pattern (p = 0.003), absence of tail sign (p = 0.001), presence of intra- and peritumoral flow-voids (p < 0.001), and number of flow-voids ≥ 5 (p < 0.001). Twenty out of the 25 (80%) ASPS showed at least 7 of these 8 features compared to only four out of 292 (1.4%) tumors of the control cohort (1 benign vascular tumor, 1 solitary fibrous tumor, 2 high-grade soft-tissue sarcomas). The five ASPS with less than 7 out of 8 features measured less than 40 mm.

Conclusion

The striking histological uniformity of ASPS translates into imaging. However, ASPS may be misdiagnosed as benign tumors or pseudo-tumors, notably intramuscular benign vascular tumors or vascular malformations.

Key Points

• ASPS are rare aggressive mesenchymal tumors displaying recurrent MRI features highly reminiscent of the diagnosis.

• Deep-seated tumors presenting with mainly high signal intensity on T1-weighted imaging, an absence of fibrotic component, ill-defined margins without aponeurotic extension, and more than five central and peripheral flow-voids are very likely to be ASPS.

• ASPS may be misdiagnosed as intramuscular benign vascular tumor or vascular malformation, which occur in the same age group.

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Abbreviations

ASPS:

Alveolar soft-part sarcoma

CI95%:

95% confidence interval

FNCLCC:

French Federation of Cancer Centers Sarcoma Group

ICC:

Interclass correlation coefficient

ISSVA:

International Society for the Study of Vascular Anomalies

OR:

Odds ratio

PACS:

Picture archiving and communication system

SFT:

Solitary fibrous tumor

STS:

Soft-tissue sarcoma

WI:

Weighted imaging

κ:

Cohen’s kappa

κw:

Weighted kappa

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Acknowledgements

The authors would like to thank Ms. Camille Martinerie for medical writing services.

Funding

The authors state that this work has not received any funding.

Author information

Authors and Affiliations

  1. Department of Radiology, Institut Bergonié, Regional Comprehensive Cancer Center, 229 cours de l’Argonne, F-33076, Bordeaux, France

    Amandine Crombé, Pauline Ledoux & Michèle Kind

  2. University of Bordeaux, F-33000, Bordeaux, France

    Amandine Crombé & François Le Loarer

  3. Imaging Department, Institut Curie, 75005, Paris, France

    Hervé J. Brisse

  4. Paris Sciences et Lettres Research University, Paris, France

    Hervé J. Brisse

  5. Department of Radiology, Institut Gustave Roussy, Comprehensive Cancer Center, 94800, Villejuif, France

    Leila Haddag-Miliani

  6. Department of Radiology, Centre Léon Bérard, Comprehensive Cancer Center, 69008, Lyon, France

    Amine Bouhamama

  7. Department of Radiology, Centre Oscar Lambret, Comprehensive Cancer Center, 59000, Lille, France

    Sophie Taieb

  8. Department of Pathology, Institut Bergonié, Comprehensive Cancer Center, 33076, Bordeaux, France

    François Le Loarer

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  1. Amandine Crombé
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  8. Michèle Kind
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Corresponding author

Correspondence to Amandine Crombé.

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Guarantor

The scientific guarantor of this publication is Dr. Michèle Kind, MD, MSc, Department of Radiology, Institut Bergonié, Bordeaux, France.

Conflict of interest

The authors of this manuscript declare no relationships with any companies, whose products or services may be related to the subject matter of the article.

Statistics and biometry

No complex statistical methods were necessary for this paper.

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Written informed consent was waived by the Institutional Review Board.

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Institutional Review Board approval was obtained.

Study subjects or cohorts overlap

Six patients were previously reported in the study by Viry et al, which was a series of cases without extensive radiological analysis and without comparison to other potential differential diagnoses (Pediatr Radiol. 2013;43(9):1174–81).

Methodology

• Retrospective

• Case/control study

• Multicenter study

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Crombé, A., Brisse, H.J., 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 29, 3170–3182 (2019). https://doi.org/10.1007/s00330-018-5903-3

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