Journal of Chest Surgery

The Korean Society for Thoracic and Cardiovascular Surgery (대한심장혈관흉부외과학회)
권호 표지
DOI QR코드

DOI QR Code

Diaphragmatic Fibromatosis: A Diagnostic and Therapeutic Challenge: A Case Report and Review of the Literature

  • Omar Hamdy (Surgical Oncology Department, Oncology Center, Mansoura University) ;
  • Reem Ayman (Faculty of Medicine, Mansoura University) ;
  • Randa Abdelaal (Faculty of Medicine, Mansoura University) ;
  • Ransy Elhadidy (Faculty of Medicine, Mansoura University) ;
  • Mariam Tarek (Faculty of Medicine, Mansoura University) ;
  • Gehad Ahmad Saleh (Department of Radiology, Faculty of Medicine, Mansoura University) ;
  • Sara Rafat (Department of Pathology, Faculty of Medicine, Mansoura University)
  • Received : 2024.05.14
  • Accepted : 2024.08.30
  • Published : 2024.11.05
Download PDF
⟨ Previous Next ⟩

Abstract

Diaphragmatic fibromatosis is an exceptionally rare condition, with only 2 previously reported cases in the literature. We present the case of a 23-year-old woman with a left hypochondrial swelling measuring 19×18×13 cm on computed tomography. An endoscopic ultrasound-guided biopsy indicated low-grade papillary proliferation. Surgical exploration revealed a large mass originating from the left hemidiaphragm. Complete excision of the mass and reconstruction of the diaphragm with double-layer mesh were performed. Microscopic examination of the mass revealed a bland-looking spindle cell proliferation. Immunohistochemical staining showed a positive nuclear reaction in tumor cells for β-catenin, a focal positive reaction for SMA, and negative reactions for S100, CD34, and desmin. Diaphragmatic fibromatosis is an extremely rare tumor for which complete excision and reconstruction of the diaphragm is the best suggested modality of treatment.

Keywords

Acknowledgement

All the clinical, radiological & pathological data used in this manuscript are available on the Mansoura University medical system (Ibn Sina Hospital management system; http://srv137.mans.edu.eg/mus/newSystem/).

References

  1. Guo C, Liu L, Zhang J, et al. Analysis of clinicopathological features of primary diaphragm tumors: a single-center study. Thorac Cancer 2021;12:1810-3. https://doi.org/10.1111/1759-7714.13988
  2. Timbergen MJ, Schut AW, Grunhagen DJ, Sleijfer S, Verhoef C. Active surveillance in desmoid-type fibromatosis: a systematic literature review. Eur J Cancer 2020;137:18-29. https://doi.org/10.1016/j.ejca.2020.06.022
  3. Endara SA, Davalos GA, Molina GA, Armijos CA, Narvaez DS, Montalvo N. Case report: desmoid fibromatosis in the mediastinum of a 6-month-old toddler, what to do? Front Surg 2023;9:1007760.https://doi.org/10.3389/fsurg.2022.1007760
  4. Garcia-Ortega DY, Martin-Tellez KS, Cuellar-Hubbe M, et al. Desmoid-type fibromatosis. Cancers (Basel) 2020;12:1851. https://doi.org/10.3390/cancers12071851
  5. Zhang Z, Shi J, Yang T, Liu T, Zhang K. Management of aggressive fibromatosis. Oncol Lett 2021;21:43. https://doi.org/10.3892/ol.2020.12304
  6. Suzuki H, Miyazaki T, Kasahara A, et al. Fibromatosis of the diaphragm-a case report. J Jpn Pract Surg Soc 1992;53:599-604. https://doi.org/10.3919/ringe1963.53.599
  7. Huang P, Chen N, Guo L. Desmoid-type fibromatosis of diaphragm. Asian J Surg 2022;45:2921-2. https://doi.org/10.1016/j.asjsur.2022.06.104
  8. Puls R, Kreissig R, Hosten N, Gaffke G, Stroszczynski C, Felix R. Tumour of the diaphragm mimicking liver lesion. Eur J Radiol 2002;41:168-9. https://doi.org/10.1016/s0720-048x(01)00357-6
  9. Guo C, Wang G, Ma D, Li J, Liu H, Li S. Primary desmoplastic fibroblastoma of diaphragm. Thorac Cancer 2021;12:2961-3. https://doi.org/10.1111/1759-7714.14168