Pathology
Age-Based Treatment of Aggressive Fibromatosis in the Head and Neck Region

https://doi.org/10.1016/j.joms.2013.07.021Get rights and content

Purpose

To review our experience regarding the difference in management and treatment outcomes of aggressive fibromatosis of the head and neck region in children and adults, emphasizing, in particular, the role of conservative surgery in comprehensive treatment strategies.

Patients and Methods

A retrospective analysis of patients with aggressive fibromatosis was performed during a 5-year period (2008 to 2012). Nine patients were enrolled in the present study, including 5 children (age, <18 years) and 4 adults (age, >18 years). All patients underwent surgical intervention and were treated by surgical resection with different surgical margins. Adjuvant low-dose chemotherapy and radiotherapy were given to pediatric and adult patients, respectively, with macroscopically or microscopically positive surgical margins.

Results

All 5 pediatric patients (3 females and 2 males) received low-dose chemotherapy after conservative surgical resection (in 4 patients, microscopically incomplete resection; and in 1 patient, macroscopic residual tumor). Of the 4 adults (3 females and 1 male), 2 underwent complete surgical resection and 2 underwent surgery and postoperative radiotherapy (1 patient had microscopically suspected residual tumor and 1 had macroscopic residual tumor). The patients were followed up for a period of 7 to 51 months. Two pediatric patients and one adult patient had disease progression after resection and became stable after continued adjuvant therapy. None of the patients had functional or cosmetic defects. All patients had good long-term outcomes, with no disease progression.

Conclusions

For the treatment of aggressive fibromatosis, conservative resection with preservation of form and function should be given greater priority in all age groups. Also, postoperative adjuvant therapy is vital for patients with gross or microscopic residual tumor to obtain progression-free survival.

Section snippets

Patients and Methods

A retrospective chart review was performed to identify all patients with a diagnosis of AF of the head and neck region treated at the Department of Oral and Maxillofacial Surgery, Fourth Military Medical University School of Stomatology from January 1, 2008 to December 31, 2012. The patients were identified from the institutional pathology database. A senior pathologist reviewed the original histopathologic reports and tumor specimens to confirm the diagnosis of AF. The clinical data of all the

Results

Of the 5 children, 4 had a microscopically incomplete resection and 1 (patient 7), with a deeply infiltrative tumor in the suprahyoid groups of muscles, had macroscopic residual tumor. All 5 patients received low-dose chemotherapy after conservative tumor resection. Three patients (patients 1, 3, and 7) experienced mild nausea and vomiting. Patient 1 had moderate neutropenia (grade III), 750 cells/μL after 10 doses. The level had increased to normal after missing 2 doses and was not associated

Discussion

Despite their nonmetastatic nature, desmoid tumors have been classified by the World Health Organization as intermediate grade tumors because of their propensity for locally invasive growth and a tendency toward local recurrence, leading to considerable morbidity and, in rare circumstances, death.29 AF is a rare benign tumor accounting for approximately 0.03% of all neoplasms and 3% of all soft tissue tumors. Of the extra-abdominal fibromatosis, only 12% develop in the head and neck region.30

References (42)

  • L.G. Morris et al.

    Tamoxifen therapy for aggressive fibromatosis of the posterior triangle of the neck

    Otolaryngol Head Neck Surg

    (2007)
  • S. Buitendijk et al.

    Pediatric aggressive fibromatosis: A retrospective analysis of 13 patients and review of literature

    Cancer

    (2005)
  • Y. Zhou et al.

    Clinical management of pediatric aggressive fibromatosis involving the mandible

    Pediatr Blood Cancer

    (2012)
  • S. Tejpar et al.

    Predominance of beta-catenin mutations and beta-catenin dysregulation in sporadic aggressive fibromatosis (desmoid tumor)

    Oncogene

    (1999)
  • L.B. Faulkner et al.

    Pediatric desmoid tumor: retrospective analysis of 63 cases

    J Clin Oncol

    (1995)
  • J.K. Wanjeri et al.

    A massive abdominal wall desmoid tumor occurring in a laparotomy scar: A case report

    World J Surg Oncol

    (2011)
  • L. Bertario et al.

    Multiple approach to the exploration of genotype-phenotype correlations in familial adenomatous polyposis

    J Clin Oncol

    (2003)
  • A.M. Carothers et al.

    Mesenchymal stromal cell mutations and wound healing contribute to the etiology of desmoid tumors

    Cancer Res

    (2012)
  • C. Wu et al.

    Aggressive fibromatosis (desmoid tumor) is derived from mesenchymal progenitor cells

    Cancer Res

    (2010)
  • A. Gronchi et al.

    Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: A series of patients surgically treated at a single institution

    J Clin Oncol

    (2003)
  • J.P. Dormans et al.

    Fibromatoses in childhood: The desmoid/fibromatosis complex

    Med Pediatr Oncol

    (2001)
  • Cited by (14)

    • Desmoid tumors of the head and neck in the pediatric population: Has anything changed?

      2021, International Journal of Pediatric Otorhinolaryngology
      Citation Excerpt :

      Historically, surgical resection with negative margins was considered the standard of care, but complete excision in the head and neck may result in significant morbidity with long-term deleterious cosmetic and functional effects in children [9]. Therefore, conservative surgical management with acceptance of positive surgical margins along with use of chemotherapy, radiotherapy, or other more selective medical management has been increasingly utilized in pediatric patients to preserve form and function [1,9]. Due to the rarity of these lesions, few institutions have extensive experience with desmoid tumors of the head and neck in the pediatric population.

    • Imaging in Otolaryngology

      2018, Imaging in Otolaryngology
    • Diagnostic Imaging: Head and Neck

      2017, Diagnostic Imaging: Head and Neck
    • Pediatric desmoid fibromatosis of the parapharyngeal space: A case report and review of literature

      2016, American Journal of Otolaryngology - Head and Neck Medicine and Surgery
      Citation Excerpt :

      Preservation of function and avoiding unnecessary morbidity should be given a high priority. Adjuvant radiotherapy or chemotherapy may be valuable in controlling microscopic or macroscopic residual disease [1–3,5]; however, interpretation of data is limited by heterogeneity and rarity of the disease. A 14-month-old male presented with a 1-month history of a rapidly enlarging painless right neck mass, preceded by blunt cervical trauma from a 2-feet fall.

    • Desmoid tumors in neurosurgery: A review of the literature

      2015, Clinical Neurology and Neurosurgery
      Citation Excerpt :

      Friede et al. [15] describe an adult case with a tumor centered on the coronal suture operated upon by professor Yaşargil, but due to fact that it was classified as a fibroma and had a recurrence 11 months postoperatively with a histological transformation considered to be malignant, we do not include this case. Interestingly, common carotid and vertebral angiography in the case of Dolman [7] showed an avascular tumor while in the case of Wang et al. [63] the superficial temporal artery supplied the tumor and underwent preoperative embolization. While the head and neck region is a predilection site for extra-abdominal DTs, reported to constitute 6% to 15% of all DTs [1,34], we found only 16 cases with intracranial extension through the skull base described in the literature.

    View all citing articles on Scopus

    Drs Wang, Koirala, and Ma contributed equally to the present study.

    Conflict of Interest Disclosures: None of the authors reported any disclosures.

    View full text