Journal of Spine Practice
ISSN: 2789-9462
Leading research in all spine subspecialties focusing on orthopaedic spine, neurosurgery, radiology, and pain management.
A Unique Collaboration Between Otolaryngology, Cardiothoracic Surgery, and Spine Surgery for Paraspinal Desmoid-Type Fibromatosis: A Case Report and Systematic Review
Published date: Nov 15 2025
Journal Title: Journal of Spine Practice
Issue title: Journal of Spine Practice (JSP): Volume 5, Issue 1
Pages: 46 - 58
Authors:
Abstract:
Purpose
Desmoid-type fibromatosis is a rare, aggressive tumor of mesenchymal origin that mimics malignancy. Though non-metastasizing, desmoid tumors exhibit local invasion and tissue destruction with high rates of recurrence, leading to severe morbidity. However, there is no standard approach for management. Hence, we present the case of a 69-year-old male with cervicothoracic desmoid-type fibromatosis and conduct a systematic review to analyze the risk factors, treatment modalities, and recurrence.
Methods
An extensive chart review of the patient was conducted, with special attention to contributing risk factors and follow-up examinations. A systematic search was conducted in PubMed, SCOPUS, and Embase with the terms ((vertebral) OR (spine)) AND (desmoid fibromatosis). Publications with desmoid-type fibromatosis at the cervical or cervicothoracic spine were included. A manual reference search was conducted to include all relevant publications.
Results
Our patient’s tumor extended from the right anterolateral C3 to the T1 level, with C5-C7 vertebral infiltration. The patient underwent a radical neck dissection, hemithyroidectomy, and hemisternotomy for tumor resection. No spinal intervention or adjuvant medical therapy was utilized. His postoperative course was complicated by right vertebral artery occlusion and transient right-sided Horner syndrome. He was discharged on postoperative day 5 with no subsequent tumor recurrence or complications. Our systematic review resulted in 21 studies with 24 patients (8 males, 16 females) at an average age of 39.98±17.23 years. The most common treatment modality was resection (n=17, 70.83%). Five patients (20.83%) had concomitant spinal decompression with or without fusion. Recurrence occurred in three patients (12.5%), at an average of 7±5.57 months after initial intervention.
Conclusion
In our case report and systematic review, we found that resection is the most common treatment modality for paraspinal desmoid-type fibromatosis confined to the cervical or cervicothoracic spine, with minimal rates of recurrence. Depending on tumor localization and invasion, concomitant spinal intervention may not be necessary.
Keywords: desmoid-type fibromatosis, desmoid tumor, aggressive fibromatosis, paraspinal, cervical, cervicothoracic
References:
[1] Master SR, Mangla A, Shah C. Desmoid Tumor[Updated 2024 Mar 1] StatPearls [Internet]Treasure Island (FL): StatPearls Publishing; 2024 Jan., Available from https: //www.ncbi.nlm.nih.gov/books/NBK459231/
[2] Otero S, Moskovic EC, Strauss DC, Benson C, Miah AB, Thway K, et al. Desmoid-type fibromatosis. Clin Radiol. 2015 Sep;70(9):1038– 45.
[3] Bansal A, Goyal S, Goyal A, Jana M. WHO classification of soft tissue tumours 2020: an update and simplified approach for radiologists. Eur J Radiol. 2021 Oct;143:109937.
[4] Okuda M, Yoshida K, Kobayashi S, Gabata T. Desmoid-type fibromatosis: imaging features and course. Skeletal Radiol. 2023 Jul;52(7):1293–303.
[5] Alman B, Attia S, Baumgarten C, Benson C, Blay JY, Bonvalot S, et al.; Desmoid Tumor Working Group. The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients. Eur J Cancer. 2020 Mar;127:96–107.
[6] Colombo C, Gronchi A. Desmoid-type fibromatosis: what works best? European journal of cancer. 2009;45(45 Suppl 1):466–7. https://doi.org/10.1016/S0959-8049(09)70092-9.
[7] Peng PD, Hyder O, Mavros MN, Turley R, Groeschl R, Firoozmand A, et al. Management and recurrence patterns of desmoids tumors: a multiinstitutional analysis of 211 patients. Ann Surg Oncol. 2012 Dec;19(13):4036–42.
[8] Hansmann A, Adolph C, Vogel T, Unger A, Moeslein G. High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors. Cancer. 2004 Feb;100(3):612–20.
[9] Riedel RF, Agulnik M. Evolving strategies for management of desmoid tumor. Cancer. 2022 Aug;128(16):3027–40.
[10] de Bruyns A, Li H, MacNeil A, Simmons C, Clarkson P, Goddard K, et al. Evolving Practice Patterns Over Two Decades (1993-2013) in the Management of Desmoid-type Fibromatosis in British Columbia. Clin Oncol (R Coll Radiol). 2020 Apr;32(4):e102–10.
[11] Page MJ, McKenzie JE, Bossuyt PM, et al. The PRISMA 2020 statement: an updated guideline for reporting systematic reviews. BMJ. 2021;372:(71). https://doi.org/10.1136/bmj.n71.
[12] Gonatas NK. Extra-abdominal desmoid tumors. Report of six cases. Arch Pathol. 1961 Feb;71:214– 21.
[13] Wyler AR, Harris AB. Recurrent desmoid tumor following cervical laminectomy. Case report. J Neurosurg. 1973 Jul;39(1):114–6.
[14] Kriss TC, Warf BC. Cervical paraspinous desmoid tumor in a child: case report. Neurosurgery. 1994 Nov;35(5):956–9.
[15] Lewin JS, Lavertu P. Aggressive fibromatosis of the prevertebral and retropharyngeal spaces: MR and CT characteristics. AJNR Am J Neuroradiol. 1995 Apr;16(4 Suppl):897–900.
[16] Rist CE, Mitznegg P. A Desmoid Tumor Mimicking Common Neck Pain. Oncol Res Treat. 2000;23(3):267–9.
[17] Ogino-Nishimura E, Okamura HO, Kishimoto S. Successful treatment of an extra-abdominal fibromatosis (desmoid tumor) arising from the prevertebral fascia of the neck. Eur Arch Oto- Rhino-Laryngol. 2003 Sep;260(8):446–9.
[18] Cohen S, Ad-El D, Benjaminov O, Gutman H. Post-traumatic soft tissue tumors: case report and review of the literature a propos a post-traumatic paraspinal desmoid tumor. World J Surg Oncol. 2008;6. https://doi.org/10.1186/1477-7819-6-28.
[19] Kenning TJ, Kanwar VS, Qian J, Deshaies EM. A de novo desmoid tumor of the surgical site following foramen magnum meningioma resection in a patient with Gardner’s Syndrome: a case report and review of the literature. J Neurooncol. 2009 Jan;91(1):107–11.
[20] Chung KH, Charlton A, Arbuckle S, Chaseling R, Owler BK. Metachronous multifocal desmoidtype fibromatoses along the neuraxis with adenomatous polyposis syndrome. J Neurosurg Pediatr. 2010 Oct;6(4):372–6.
[21] Lee YC, Lee JW. Innovative treatment for huge nuchal desmoid tumour: a case report with a 2- year follow-up. J Plast Reconstr Aesthet Surg. 2010 Aug;63(8):e622–6.
[22] Sevak S, Blount AL, Cottingham S, DeLano M, Vander Woude DL, Stevenson J, et al. Fibromatosis of the cervical region following laminectomy: a case report and literature review. Spine. 2012 Apr;37(7):E456–9.
[23] Hood B, Benglis DM, Levi AD, Vanni S. Occiput to thoracic fusion after surgical resection of desmoid tumor. World Neurosurg. 2013;79(1). https://doi.org/10.1016/j.wneu.2011.01.041.
[24] Shakur SF, Takagi I, Jacobsohn JA, Golden BM, Karahalios DG. Spinal fibromatosis: a report of two cases and review of the literature. Spine J. 2013 Aug;13(8):e1–6.
[25] Lacayo EA, Glastonbury CM, Hoang JK, Magliocca KR, Hill KL, Hudgins PA. Deep Neck Fibromatosis After Diskectomy and Cervical Fusion: Case Series and Review of the Literature. AJR Am J Roentgenol. 2016 May;206(5):1068–72.
[26] Goldstein A, Hoang S, Miller DC, Mesfin FB. Extra-abdominal Desmoid Tumor Mimicking Cervical Spine Schwannoma. Cureus. 2018;10(8). https://doi.org/10.7759/cureus.3145.
[27] Bohl MA, Leveque JC, Bayles S, Sethi R. Postoperative Development of Desmoid Tumor After Surgical Correction of Adult Spinal Deformity: Case Report and Review of Literature. World Neurosurg. 2019 Aug;128:4–10.
[28] Luo N, He X, Li G, Liao Y, Tang Q, Ye R, et al. Desmoid Tumor Presenting as a Typical Cervical Dumbbell Tumor: Case Report and Review of the Literature. World Neurosurg. 2019 Apr;124:151–6.
[29] Mujtaba B, Call C, Rowland F, et al. Desmoid fibromatosis following surgical resection of spinal meningioma. Radiol Case Rep. 2020;15(6):697- 701. https://doi.org/10.1016/j.radcr.2020.02.004.
[30] Gupta A, Batta NS, Batra V. Postoperative Recurrent Paraspinal Fibromatosis after Resection of Cervical Meningioma and Review of Literature. Indian J Radiol Imaging. 2021 Apr;31(2):514–8.
[31] Schlag H, Neuhoff J, Castein J, Hoffmann C, Kandziora F. Sporadic desmoid fibromatosis of the neck after dorsal spondylodesis of the cervical spine. Surg Neurol Int. 2022;13:64. https://doi. org/10.25259/SNI_1240_2021.
[32] Rachida Bouatay, Nawres Bouaziz, Khaled Harrathi, Koubaa J. Aggressive and recurrent desmoid tumor of the head and neck: A therapeutic challenge. Otolaryngology Case Reports. 2024;20. https://doi.org/10.1016/j.xocr. 2024.100578.
[33] Shindle MK, Khanna AJ, McCarthy EF, O’Neill PJ, Sponseller PD. Desmoid tumor of the spinal canal causing scoliosis and paralysis. Spine. 2002 Jun;27(12):E304–7.
[34] Kawashima A, Fishman EK, Hruban RH, Kuhlman JE. Intrathoracic paraspinal desmoid with intracanalicular extension: computed tomography and magnetic resonance imaging findings. Orthopedics. 2000 Apr;23(4):381–2.
[35] Muller E, Castagnaro M, Yandel DW, Wolfe HJ, Alman BA. Molecular genetic and immunohistochemical analysis of the tumor suppressor genes Rb and p53 in palmar and aggressive fibromatosis. Diagn Mol Pathol. 1996 Sep;5(3):194–200.
[36] Kotiligam D, Lazar AJ, Pollock RE, Lev D. Desmoid tumor: a disease opportune for molecular insights. Histol Histopathol. 2008 Jan;23(1):117–26.
[37] Shields CJ, Winter DC, Kirwan WO, Redmond HP. Desmoid tumours. Eur J Surg Oncol. 2001 Dec;27(8):701–6.
[38] Griffiths HJ, Robinson K, Bonfiglio TA. Aggressive fibromatosis. Skeletal Radiol. 1983;9(3):179–84.
[39] Taylor LJ. Musculoaponeurotic fibromatosis. A report of 28 cases and review of the literature. Clin Orthop Relat Res. 1987 Nov;224:294–302.
[40] Francis IR, Dorovini-Zis K, Glazer GM, Lloyd RV, Amendola MA, Martel W. The fibromatoses: CT-pathologic correlation. AJR Am J Roentgenol. 1986 Nov;147(5):1063–6.
[41] Kim SJ, Ha DH, Lee SM, Kang H. Desmoid type fibromatosis in the facet joint of lumbar spine: case report and review of literature. Korean J Radiol. 2013;14(5):818–22.
[42] Hartman TE, Berquist TH, Fetsch JF. MR imaging of extraabdominal desmoids: differentiation from other neoplasms. AJR Am J Roentgenol. 1992 Mar;158(3):581–5.
[43] de Camargo VP, Keohan ML, D’Adamo DR, Antonescu CR, Brennan MF, Singer S, et al. Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor). Cancer. 2010 May;116(9):2258–65.
[44] Fiore M, MacNeill A, Gronchi A, Colombo C. Desmoid-Type Fibromatosis: Evolving Treatment Standards. Surg Oncol Clin N Am. 2016 Oct;25(4):803–26.