Six cases of fibromatosis of the head and neck in children

doi:10.1016/S0165-5876(00)82013-3

International Journal of Pediatric Otorhinolaryngology

Volume 53, Issue 3, 14 July 2000, Pages 235-244

https://doi.org/10.1016/S0165-5876(00)82013-3 Get rights and content

Abstract

Six cases of aggressive fibromatosis occurring in the head and neck in children are described. Fibromatosis is a rare, benign but locally infiltrative condition. Primary excision is not always possible in the head and neck areas. It is not always appropriate to treat children using the same modalities as adults due to associated growth problems. The case histories illustrate the management difficulties that can be encountered when treating the paediatric population.

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Cited by (23)

Aggressive fibromatosis involving the cheek: A case report with review of literature
2021, Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology
Aggressive or Desmoid fibromatosis (AF) is a benign fibrous soft tissue tumor that consists of dense masses of fibroblasts and myofibroblasts with marked collagen production. The lesions are considered to be aggressive and non-metastasizing but invade locally and recur frequently after surgical excision. Although the head and neck are not an unusual site of involvement, very few cases of fibromatosis of the cheek are reported till date. We report a case of fibromatosis of cheek in an adult female that was managed successfully by surgical intervention and to discuss the review of literature on this subject.
Treatment of extensive post tonsillectomy oropharyngeal stenosis secondary to fibromatosis
2018, International Journal of Pediatric Otorhinolaryngology
Aggressive fibromatosis is an uncommon, benign tumor of fibroblastic origin with high potential for local invasion. Less than a quarter of these lesions are located in the head and neck, and although extremely rare, associations have been demonstrated with physical trauma. We describe a unique case of oropharyngeal fibromatosis with traumaticetiology, managed successfully with surgical excision of the lesion with negative surgical margins. A 5-year old patient was found to have an aggressive fibromatosis causing oropharyngeal stenosis following tonsillectomy. We demonstrate that surgical resection with a clear margin allowed for alleviation of stenosis without recurrences reported since the procedure.
Aggressive fibromatosis of the head and neck in the pediatric population
2014, International Journal of Pediatric Otorhinolaryngology
Citation Excerpt :
Fibromatosis do not metastasize, however without adequate resection, they are locally aggressive and tend to recur [4,11]. Overall recurrence rates in the literature are reported around 50% [13]. In our review of those within the head and neck, and those that received primary surgery, the recurrence rate is 19% (Table 3).
Aggressive fibromatosis, previously referred to as desmoid tumor, is a rare tumor. It is classified as benign, however, has a high potential for local invasion and recurrence. It is most commonly found in the abdomen, with an origin in the head and neck accounting for less than a quarter of the total cases.
Literature review using PubMed and OVID and a combination of the words, “desmoid”, “fibromatosis”, “pediatric”, “children”, and “head and neck”.
Annual occurrence is 0.2 – 0.4 per 100,000, with origins in the head and neck accounting for 10–25%. Since 1954, 97 cases of pediatric head and neck fibromatosis were reported. The age range was from birth to 16 years, with the average being 4 years and 5 months old. The overwhelming majority of tumors were of the mandible (38%). 74% patients underwent a large resection as the primary treatment modality. The most common treatment complication was tumor recurrence (16%).
Fibromatosis is a rare tumor of muscoloaponeurotic origin. Surgery is the mainstay of treatment; however recurrence rates are high, even despite negative surgical margins.
Facial infantile aggressive fibromatosis: A frequent localization of an exceptional tumor!
2012, Annales de Chirurgie Plastique Esthetique
Les fibromatoses agressives sont des tumeurs bénignes ; mais elles sont responsables d’une grande morbidité dans les localisations cervicofaciales. Elles sont caractérisées par une capacité infiltrante très invasive et un taux de récidives locales très élevé. Leur prise en charge constitue un véritable défi.
Nous rapportons le cas d’un enfant âgé de dix ans présentant une fibromatose agressive de la lèvre supérieure. Chez ce patient, le traitement médical a été décidé pour éviter une chirurgie mutilante. Il a eu une excellente réponse à la chimiothérapie à base de méthotrexate et vinblastine.
La principale arme thérapeutique efficace contre les fibromatoses agressives de l’extrémité cervicofaciale reste la résection chirurgicale complète. Mais, dans le cas particulier de l’enfant, le traitement ne se discute pas de la même façon, en effet le pronostic esthétique et fonctionnel, dans ce cas, est primordial. La chimiothérapie à base de méthotrexate et vinblastine constitue une bonne alternative.
Although fibromatosis is considered as benign tumor, it can have significant morbidity, particularly when it occurs in the head and neck. Their propensity for infiltrative local growth with encroachment on vital structures and their tendency to recur make fibromatoses of the head and neck extremely challenging lesions.
We report the case of a 10-year-old boy with desmoid fibromatosis of the upper lip. For this patient, the medical treatment has been decided to avoid a mutilating surgery. He had a good result with chemotherapy using methotrexate and vinblastine.
According to the literature, surgery is the most common treatment of fibromatosis in the head and neck region. However, particularly in children, alternative modes of therapy must be considered because of the high recurrence rate and to avoid mutilating operations. Chemotherapy using methotrexate and vinblastine may be a reasonable choice.
Spindle cell lesions of the head and neck: an overview and diagnostic approach
2009, Diagnostic Histopathology
Citation Excerpt :
It is especially seen in children and young adults, most frequently in the supraclavicular region of the neck. It may also involve the oral and perioral region.72 Erosion of bone is a frequent finding in lesions arising in soft tissue around the jaws.
The diagnosis of lesions with spindle cell morphology is often difficult. For biopsies from the head and neck assessment is further complicated by the range of tissues present at this site, and the limited amount of material which may be available for study. We provide an overview of the lesions with a spindle cell morphology that may be encountered in this region and detail a histological approach to these biopsies which can aid the pathologist with classification.
Pediatric aggressive fibromatosis of the head and neck: a 20-year retrospective review
2008, Journal of Pediatric Surgery
Citation Excerpt :
Chemotherapy may be a reasonable alternative to radiotherapy in a growing child [32], though there is an inherent risk of secondary malignancies, fertility problems, cardiotoxicity [11,50], and neutropenia [51]. Chemotherapy has been considered for delay of progression of aggressive fibromatosis until children are considered old enough for radiotherapy [43] or surgery [32]. There is no strong evidence that chemotherapy agents are efficacious, and possible role may be with a rapid growing tumor to obtain local control [17].
Aggressive fibromatosis in children is a rare, benign condition that is locally infiltrative and destructive. It often presents as a rapidly growing, painless lump in the head and neck region. To date, only small series and case reports have been reported, and the management of the condition remains unclear. Recently, nuclear β-catenin expression has been suggested as a tumor-specific marker for aggressive fibromatosis (desmoid).
The aims of the study were to review our experience of the presentation, management, and treatment outcome of pediatric aggressive fibromatosis in the head and neck and to identify the presence of the desmoid tumor marker β-catenin within this population.
The study was conducted as a retrospective case review of children diagnosed with aggressive fibromatosis in the head and neck for a period of 20 years and a review of the literature. Pathologic review of the original tumor specimens was undertaken for evidence of positive tumor margins and presence of nuclear β-catenin expression.
A total of 10 patients (6 males, 4 females) were identified. The age at presentation ranged from 12 months to 14 years. In total, 8 patients were treated with surgery alone. This included 7 patients with extension of the tumor to the resection margin; all had good long-term outcomes with no disease progression. Two patients received chemoradiotherapy, one as primary treatment, and the other as adjuvant treatment after gross incomplete resection. Both resulted in poor outcomes requiring further treatments.
Within our series of pediatric fibromatosis, only 4 cases (40%) had positive results for any nuclear β-catenin expression, and 6 (60%) of 10 patients had negative results for β-catenin.
Our experience is that total gross resection and preservation of form and function is of higher priority than achieving a negative resection margin. Pediatric fibromatosis though aggressive is still a benign condition, and careful thought should be taken before considering adjuvant chemoradiotherapy. Nuclear β-catenin expression should not be considered a specific tumor marker for pediatric aggressive fibromatosis of the head and neck. Pediatric aggressive fibromatosis in this region may be a distinct subtype of desmoid tumor from its adult form.

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Case reportSix cases of fibromatosis of the head and neck in children

Abstract

Brit. J. Oral Maxillofacial Surg.

J. Paed.

Int. J. Rad. Oncol., Biol., Phys.

Int. J. Paed. Otorhinolaryngol.

Infantile fibromatosis

J. Laryngol. Otol.

Juvenile fibromatoses

Cancer

The fibromatoses: a clinicopathological classification based on 140 cases

Am. J. Surg. Pathol.

Desmoid tumours of the head and neck

Am. J. Surg.

Aggressive fibromatosis of the head and neck

Am. J. Surg.

Aggressive fibromatosis is a monoclonal disorder

Diag. Mol. Pathol.

Case report
Six cases of fibromatosis of the head and neck in children