Agressive fibromatosis involving the mandible—Case report and review of the literature

doi:10.1016/j.tripleo.2004.03.026

Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology

Volume 99, Issue 1, January 2005, Pages 30-38

https://doi.org/10.1016/j.tripleo.2004.03.026 Get rights and content

Abstract

Background

Aggressive fibromatosis (AF) involving the mandible is rare, and surgery is often complicated by a high recurrence rate.

Case report

A 4-year-old boy was referred because of a fast growing painless mass which involved the entire left angle of the mandible. Excisional biopsy revealed AF, and local excision of the tumor was performed. Six months after surgery a recurrence was detected. The tumor was determined to be unresectable and the boy was treated with low-dose chemotherapy including methotrexate and vinblastine for 1 year. With combined chemotherapy and surgical debulking, mutilating surgical resection will be delayed as long as possible or until completion of facial growth.

Conclusion

According to the literature, surgery is the most common treatment of AF 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.

Section snippets

Case report

A 4-year-old boy was referred with a painless hard swelling of the left angle and body of the mandible. The lesion was first detected 3 weeks earlier but referral was delayed owing to a concurrent viral infection. The boy was asymptomatic and his medical history was without pathological findings. Physical examination showed a firm mass around the mandible involving the entire body and angle. No mucosal surface ulceration was present, and the lesion covered the first permanent molar.

Literature review

This review includes all case reports of AF involving the mandible that were published between 1960 and 2003. The reports were collected using the PubMed database of the National Center for Biotechnology Information, National Library of Medicine (Bethesda, Md) using the key words “aggressive fibromatosis,” “desmoid tumors,” “AF,” and “DT.” The search was completed by the cross-references, using the references of all identified cases. Only those case reports with clear classification as

Age and sex

AF of the mandible was reported in 22 males and 14 females. The average age of the patients was 9 years; however, there was a wide range from 8 months to 63 years. Of the patients, 23 (62%) were between 5 and 15 years of age, and 8 patients (21%) were under the age of 5 years. Thus, the tumors were classified as aggressive juvenile fibromatosis or as aggressive infantile fibromatosis.

Signs and symptoms

Each patient with fibromatosis of the mandible was initially seen with hard masses or swellings. Most of the

Discussion

Fibromatoses are a group of fibrous connective tissue lesions that are morphologically classified as benign neoplasms. They do not develop distant metastases, however, locally they show an aggressive and infiltrative behavior. The low incidence of this rather rare tumor presents problems in both diagnosis and management.

The histologic differentiation between locally aggressive AF and fibrosarcoma is difficult but important owing to the different treatment strategies.1., 6., 7., 11. Whereas

Acknowledgements

The authors thank Dr. C. August for his valuable opinion regarding the histology and Professor G. Jundt (University of Basel) for confirmation of the diagnosis.

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Desmoplastic fibroma (DF) is an intraosseous counterpart of desmoid-type soft tissue fibromatosis. It is most frequently seen in the jawbones. The clinical and radiological features of the present cases were nonspecific. The accumulation of beta-catenin in the nuclei of neoplastic cells which is a diagnostic feature of desmoid-type soft tissue fibromatosis could not be detectED in the present DF series. The aim of this study is to report a series of 22 cases of DF involving either mandible or maxilla. A retrospective evaluation of desmoplastic fibroma and beta-catenin, smooth muscle actin, nestin, cyclin D1 immunostaining’s patterns.
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Oral and Maxillofacial SurgeryAgressive fibromatosis involving the mandible—Case report and review of the literature

Abstract

Background

Case report

Conclusion

Section snippets

Case report

Literature review

Age and sex

Signs and symptoms

Discussion

Acknowledgements

Am J Surg

Br J Oral Maxillofac Surg

Ann Onc

Am J Otol

Int J Radiat Oncol Biol Phys

Oral Surg Oral Med Oral Pathol Oral Radiol Endod

Oral Surg Oral Med Oral Pathol Oral Radiol Endod

Oral Surg

J Oral Maxillofac Surg

J Oral Maxillofac Surg

AORN

Br J Oral Maxillofac Surg

J Oral Maxillofac Surg

J Oral Maxillofac Surg

Int J Radiat Oncol Biol Phys

Int J Radiat Oncol Biol Phys

Pathology of soft tissue sarcomas

The fibromatoses: A clinicopathological classification based on 140 cases

Am J Surg Path

The desmoid problem

Chirurg

The fibromatoses: a clinicopathological concept

Br Med J

Soft tissue tumors

Fibromatoses in childhood: the desmoid/fibromatosis complex

Med Pediatr Oncol

Fibromatosis of the head and neck

Am J Surg

Juvenile fibromatoses

Cancer

Extra-abdominal desmoid fibromatosis

Ann Otol Rhinol Laryngol

Juvenile fibromatosis. A case report showing invasion of the bone

Arch Dis Chil

Oral and Maxillofacial Surgery
Agressive fibromatosis involving the mandible—Case report and review of the literature