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Exploring Head and Neck Fibromatosis: A Case Series and Literature Review

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Indian Journal of Surgical Oncology Aims and scope Submit manuscript

Abstract

This study aimed to retrospectively review the clinical data, management protocols, clinical outcomes, and literature review of patients diagnosed with head and neck fibromatoses. This case series aims to present and discuss seven cases of rare head and neck fibromatosis. The study design involves retrospective analysis of medical records, radiological imaging, and histopathological reports of the included cases. Ethical approval was obtained from the relevant Institutional Review Board. The results of the study reveal that individuals diagnosed with fibromatosis had a wide age range, starting from 6 to 58 years, with the median age being 44.3 years. Out of the 8 patients, 5 (62.5%) were females and 3 (37.5%) were males. The maxilla was found to be the most common subsite of origin in the head and neck area, followed by the masseter and temporal fossa. At the time of diagnosis, 2 (25%) patients were found to have unresectable disease. Multidisciplinary management therapies, including surgery, chemotherapy, hormonal, targeted therapy, and radiotherapy, were used. Out of the 6 patients who underwent surgery, 5 (83.3%) experienced tumor recurrence. In conclusion, the retrospective analysis underscores the complexity of managing fibromatoses of the head and neck region; complete tumor resection is not achievable always due to the inherent challenges, complex anatomy, and proximity of vital structures in the head and neck. Adjuvant strategies including radiotherapy, chemotherapy, and hormonal therapy have demonstrated efficacy in disease management. The rarity of these tumors necessitates the development of standardized treatment protocols to guide clinical practice effectively. Moving forward, collaborative efforts are crucial to enhance our understanding of fibromatoses and refine therapeutic strategies for improved patient outcomes.

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Abbreviations

CT :

Computed tomography

MRI :

Magnetic resonance imaging

IHC :

Immunohistochemistry

TNM :

Tumor, node, metastasis (staging system)

WHO :

World Health Organization

NSAIDs :

Nonsteroidal anti-inflammatory drugs

SERMs :

Selective estrogen receptor modulators

SMA :

Smooth muscle actin

H-caldesmon :

Heavy caldesmon

SCC :

Squamous cell carcinoma

References

  1. De Bree E, Zoras O, Hunt JL, Takes RP, Suárez C, Mendenhall WM, Hinni ML, Rodrigo JP, Shaha AR, Rinaldo A, Ferlito A (2014) Desmoid tumors of the head and neck: a therapeutic challenge. Head Neck 36(10):1517–1526

    Article  PubMed  Google Scholar 

  2. Otero S, Moskovic EC, Strauss DC, Benson C, Miah AB, Thway K, Messiou C (2015) Desmoid-type fibromatosis. Clin Radiol 70(9):1038–1045

    Article  CAS  PubMed  Google Scholar 

  3. Wang W, Koirala U, Ma S, Liu G, Ding M, Hu X, Lei D (2014) Age-based treatment of aggressive fibromatosis in the head and neck region. J Oral Maxillofac Surg 72(2):311–321

    Article  PubMed  Google Scholar 

  4. Kahraman D, Karakoyunlu B, Karagece U, Ertas U, Gunhan O (2021) Desmoplastic fibroma of the jaw bones: a series of twenty-two cases. J Bone Oncol 1(26):100333

    Article  Google Scholar 

  5. Wanebo HJ, Koness RJ, Macfarlane JK, Eilber FR, Byers RM, Elias EG, Spiro RH (1992) Head and neck sarcoma: report of the head and neck sarcoma registry. Head Neck 14(1):1–7

    Article  CAS  PubMed  Google Scholar 

  6. Dei Tos AP, Bonvalot S, Haas R (2018) The key role of pathology, surgery and radiotherapy in the initial management of soft tissue sarcoma. Future Oncol 14(10s):15–23

    Article  CAS  PubMed  Google Scholar 

  7. Jo VY, Demicco EG (2022) Update from the 5th edition of the World Health Organization classification of head and neck tumours: soft tissue tumours. Head Neck Pathol 16(1):87–100

    Article  PubMed  PubMed Central  Google Scholar 

  8. Skubitz KM (2017) Biology and treatment of aggressive fibromatosis or desmoid tumour. Mayo Clin Proc 92(6):947–964 (Elsevier)

    Article  PubMed  Google Scholar 

  9. Castellote A, Vázquez E, Vera J, Piqueras J, Lucaya J, Garcia-Peña P, Jiménez JA (1999) Cervicothoracic lesions in infants and children. Radiographics 19(3):583–600

    Article  CAS  PubMed  Google Scholar 

  10. Thway K, Gibson S, Ramsay A, Sebire NJ (2009) Beta-catenin expression in pediatric fibroblastic and myofibroblastic lesions: a study of 100 cases. Pediatr Dev Pathol 12(4):292–296

    Article  CAS  PubMed  Google Scholar 

  11. Howard JH, Pollock RE (2016) Intra-abdominal and abdominal wall desmoid fibromatosis. Oncol Ther 4(1):57–72

    Article  PubMed  Google Scholar 

  12. Azzarelli A, Gronchi A, Bertulli R, Tesoro Tess JD, Baratti D, Pennacchioli E, Dileo P, Rasponi A, Ferrari A, Pilotti S, Casali PG (2001) Low-dose chemotherapy with methotrexate and vinblastine for patients with advanced aggressive fibromatosis. Cancer 92(5):1259–64

    Article  CAS  PubMed  Google Scholar 

  13. Bhati M, Balakrishna G, Joshi K, Bhattacharya K, Bal M, Laskar SG, Sinha S, Joshi A, Joshi P, Nair S, Chaturvedi P (2021) Fibromatoses of head and neck: case series and literature review. Rambam Maimonides Medical Journal 12(3)

  14. Emori M, Matsumoto Y, Murahashi Y, Yoshida M, Nishida Y (2021) Efficacy and safety of cyclooxygenase 2 inhibitors for desmoid tumor management: a systematic review. Nagoya J Med Sci 83(4):673

    CAS  PubMed  PubMed Central  Google Scholar 

  15. Cho JY, Gupta S, Cho HS, Park MS, Mok SJ, Han I, Kim HS (2018) Role of nonsteroidal anti-inflammatory drug in treatment of extra-abdominal desmoid tumors. Clin Orthop Surg 10(2):225–233

    Article  PubMed  PubMed Central  Google Scholar 

  16. Hansmann A, Adolph C, Vogel T, Unger A, Moeslein G (2004) High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors. Cancer 100(3):612–620

    Article  CAS  PubMed  Google Scholar 

  17. Gounder MM, Mahoney MR, Van Tine BA, Ravi V, Attia S, Deshpande HA, Gupta AA, Milhem MM, Conry RM, Movva S, Pishvaian MJ (2018) Sorafenib for advanced and refractory desmoid tumors. N Engl J Med 379(25):2417–2428

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Batalni JP, Belloir C, Mazabraud A, Pilleron JP, Cartigny A, Jaulerry C, Ghossein NA (1988) Desmoid tumors in adults: the role of radiotherapy in their management. Am J Surg 155(6):754–760

    Article  Google Scholar 

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Funding

This research did not receive any specific grant from funding agencies in the public, commercial or not-for-profit sectors.

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Authors and Affiliations

  1. Department of Surgical Oncology, Sawai Man Singh Medical College and Attached Hospitals, Jaipur, Rajasthan, 302004, India

    Agil Babu, Suresh Singh, Pinakin Patel, Kamal Kishor Lakhera, Deeksha Mehta & Pranav Mohan Singhal

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  1. Agil Babu
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  2. Suresh Singh
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  3. Pinakin Patel
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  4. Kamal Kishor Lakhera
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  5. Deeksha Mehta
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  6. Pranav Mohan Singhal
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Contributions

AB and KKL conceived the idea. PMS, PP, and AB designed the study and laid the framework for data collection. AB, DM, and KKL did the data collection and data entry. KKL and SS supervised data entry and did the data analyses. AB and SS laid down the framework for the paper and supervised data analysis. AB and DM wrote the manuscript. DM and AB helped review the literature. KKL and PP helped in the editing and formation of the final draft.

Corresponding author

Correspondence to Deeksha Mehta.

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Ethics Approval

Approval from the Institutional Ethics Committee of SMS Medical College, Jaipur, was taken for the conduct of this study.

Consent to Participate

Well-informed consent from patients was taken.

Conflict of Interest

The authors declare no competing interests.

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Babu, A., Singh, S., Patel, P. et al. Exploring Head and Neck Fibromatosis: A Case Series and Literature Review. Indian J Surg Oncol (2024). https://doi.org/10.1007/s13193-024-02010-z

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