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A successful trimodality therapy for difficult-to-diagnose primary mediastinal dedifferentiated liposarcoma, which originated from the perihilar fat and invaded the right lungs

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Abstract

A 40-year-old man with high fever, hemoptysis, and fatigue showed a 10-cm mass in the middle and lower lobes of the right lung on computed tomography. Histological examination of transbronchial biopsy specimens showed sheets of small round tumor cells and mild staining for CD99. Primary Ewing sarcoma was suspected, and a trimodality therapy consisting of chemotherapy, intensity-modulated radiation therapy, and right pneumonectomy with surrounding tissue resection was performed. In surgical specimens, negative outcome of NKX2.2 in immunostaining and EWSR1 rearrangement in fluorescence in situ hybridization did not support the diagnosis of Ewing sarcoma. Positive immunostaining for MDM2 and CDK4 led to a diagnosis of dedifferentiated liposarcoma, which probably originated from an adipose tissue of the right perihilar mediastinum, and then invaded the lungs. The postoperative course was uneventful, without recurrence for more than 16 months.

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References

  1. WHO Classification of Tumours Editorial Board. WHO classification of tumours of soft tissue and bone. 5th edition. International Agency for Research on Cancer, Lyon; 2020.

  2. Chen M, Yang J, Zhu L, Zhou C, Zhao H. Primary intrathoracic liposarcoma: a clinicopathologic study and prognostic analysis of 23 cases. J Cardiothorac Surg. 2014;9:119.

    Article  CAS  Google Scholar 

  3. Agaimy A, Michal M, Hadravsky L, Michal M. Dedifferentiated liposarcoma composed predominantly of rhabdoid/epithelioid cells: a frequently misdiagnosed highly aggressive variant. Hum Pathol. 2018;77:20–7.

    Article  CAS  Google Scholar 

  4. Thway K, Jones RL, Noujaim J, Zaidi S, Miah AB, Fisher C. Dedifferentiated liposarcoma: updates on morphology, genetics, and therapeutic strategies. Adv Anat Pathol. 2016;23:30–40.

    Article  CAS  Google Scholar 

  5. Doyle LA, Tao D, Mariño-Enríquez A. STAT6 is amplified in a subset of dedifferentiated liposarcoma. Mod Pathol. 2014;27:1231–7.

    Article  CAS  Google Scholar 

  6. Beird HC, Wu CC, Ingram DR, Wang WL, Alimohamed A, Gumbs C, et al. Genomic profiling of dedifferentiated liposarcoma compared to matched well-differentiated liposarcoma reveals higher genomic complexity and a common origin. Cold Spring Harb Mol Case Stud. 2018;4:a002386.

    Article  Google Scholar 

  7. Yang JC, Chang AE, Baker AR, Sindelar WF, Danforth DN, Topalian SL, et al. Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol. 1998;16:197–203.

    Article  CAS  Google Scholar 

  8. Pervaiz N, Colterjohn N, Farrokhyar F, Tozer R, Figueredo A, Ghert M. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer. 2008;113:573–81.

    Article  Google Scholar 

  9. Gronchi A, Ferrari S, Quagliuolo V, Broto JM, Pousa AL, Grignani G, et al. Histotype-tailored neoadjuvant chemotherapy versus standard chemotherapy in patients with high-risk soft-tissue sarcomas (ISG-STS 1001): an international, open-label, randomised, controlled, phase 3, multicentre trial. Lancet Oncol. 2017;18:812–22.

    Article  CAS  Google Scholar 

  10. Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med. 2003;348:694–701.

    Article  CAS  Google Scholar 

  11. Keung EZ, Hornick JL, Bertagnolli MM, Baldini EH, Raut CP. Predictors of outcomes in patients with primary retroperitoneal dedifferentiated liposarcoma undergoing surgery. J Am Coll Surg. 2014;218:206–17.

    Article  Google Scholar 

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Acknowledgements

We thank Yasuo Ishida from Chibaken Saiseikai Narashino Hospital for support in pathologic diagnosis.

Funding

The authors have not declared funding for this study.

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Authors and Affiliations

  1. Department of Thoracic Surgery, Chibaken Saiseikai Narashino Hospital, 1-1-1, Izumi-cho, Narashino, 275-8580, Japan

    Takahiro Ochi, Teruaki Mizobuchi & Kaoru Nagato

  2. Department of Pathology, Tokyo Women’s Medical University Yachiyo Medical Center, Yachiyo, Japan

    Kenzo Hiroshima

  3. Department of Respiratory Medicine, Chibaken Saiseikai Narashino Hospital, Narashino, Japan

    Taku Itoh & Fuminobu Kuroda

  4. Department of Pathology, Teikyo University Chiba Medical Center, Ichihara, Japan

    Kazuto Yamazaki

  5. Department of Molecular Pathology, Yokohama City University, Yokohama, Japan

    Ikuma Kato

  6. Department of Pathology and Oncology, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan

    Masanori Hisaoka

  7. Department of Pathology, Yokosuka Kyosai Hospital, Yokosuka, Japan

    Yukio Nakatani

Authors
  1. Takahiro Ochi
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  2. Teruaki Mizobuchi
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  3. Kenzo Hiroshima
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  4. Kaoru Nagato
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  5. Taku Itoh
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  6. Fuminobu Kuroda
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  7. Kazuto Yamazaki
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  8. Ikuma Kato
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  9. Masanori Hisaoka
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  10. Yukio Nakatani
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Correspondence to Teruaki Mizobuchi.

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Ochi, T., Mizobuchi, T., Hiroshima, K. et al. A successful trimodality therapy for difficult-to-diagnose primary mediastinal dedifferentiated liposarcoma, which originated from the perihilar fat and invaded the right lungs. Gen Thorac Cardiovasc Surg 70, 298–302 (2022). https://doi.org/10.1007/s11748-021-01735-w

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  • DOI: https://doi.org/10.1007/s11748-021-01735-w

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