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Lingual Alveolar Soft Part Sarcoma in a 78-Year-Old Woman: A Case Report and Comprehensive Review of the Literature from 1952 to 2022

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Abstract

Background

Alveolar soft part sarcoma (ASPS) is a rare translocation-related soft tissue sarcoma, occurring mainly in the limbs and trunk in young adults and adolescents. ASPS is rarely seen in the head and neck and one fourth of those cases described are tongue primary. Given its nonspecific symptoms, clinical findings, and rarity in this location, lingual ASPS (L-ASPS) has been reported to be commonly misdiagnosed as various benign tumors, leading to adverse outcomes.

Methods

We report a case of L-ASPS occurring in the oldest (78 years) female patient published to date and comprehensively review the literature from 1952 to 2022.

Results

She presented with a slow-growing (2-year duration) tongue mass, measuring 3.5 cm on palpation. Intraoperative frozen section could not render the definitive diagnosis. The pathological findings of the tumor were characteristic of ASPS with eosinophilic polygonal cells in an organoid/nested pattern, rich sinusoidal capillaries, and TFE3 immunoreactivity, except for the strong diffuse aberrant cytoplasmic CD68 immunoexpression and absence of intracytoplasmic crystalline inclusions on PAS with diastase. After TFE3 gene rearrangement had been identified with fluorescent in-situ hybridization, reflex testing confirmed a rearrangement of TFE3 gene with the known fusion partner ASPSCR1.

Conclusions

ASPS should be included in the differential diagnoses in cases of any slow-growing lingual masses (especially vascular ones) with non-specific clinical pictures, regardless of the patient’s age.

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Data Availability

No datasets were generated or analyzed during the current study.

Code Availability

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Acknowledgements

We would like to thank Mr. Brandon Hartman, BS (an electron microscopy supervising technician) and his team for their help in performing ultrastructural analysis and obtaining EM images. We would like to thank Dr. Rhett P. Ketterling, MD and Mayo Clinic Laboratories (Rochester, MN) for the molecular testing and Fluorescence in situ hybridization testing images.

Funding

The authors received no financial support for the research, authorship, and/or publication of this article.

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Authors and Affiliations

  1. Department of Pathology and Translational Pathobiology, LSU Health Shreveport, 1501 Kings Hwy, Shreveport, LA, 71103, USA

    Aliaksandr Aksionau & Hidehiro Takei

  2. Department of Pathology, Baylor Scott & White Health, Irving, TX, USA

    Nestor Enriquez Dela Cruz

  3. Department of Oral & Maxillofacial Surgery, LSU Health Shreveport, Shreveport, LA, USA

    Andrew T. Meram

  4. Department of Radiology, LSU Health Shreveport, Shreveport, LA, USA

    Hugo Cuellar-Saenz

  5. Calpath Medical Associates, Campbell, CA, USA

    Jillian R. Aveni

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Contributions

Concept: AA. Design: AA, HT. Definition of intellectual content: AA, HT. Literature search: AA. Data acquisition: AA, NDC, ATM, HCS, JRA. Data analysis: AA, HT. Statistical analysis: AA. Manuscript preparation: AA, NDC, ATM, HCS, JRA, HT. Manuscript editing: AA, NDC, ATM, HCS, HT. Manuscript review: AA, NDC, ATM, HCS, JRA, HT. Guarantor: AA.

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Correspondence to Aliaksandr Aksionau.

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Aksionau, A., Dela Cruz, N.E., Meram, A.T. et al. Lingual Alveolar Soft Part Sarcoma in a 78-Year-Old Woman: A Case Report and Comprehensive Review of the Literature from 1952 to 2022. Head and Neck Pathol 17, 265–274 (2023). https://doi.org/10.1007/s12105-022-01505-x

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