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| Year : 2012 | Volume
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| Issue : 3 | Page : 435 |
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| Aggressive fibromatosis of the oral cavity |
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Santhosh Rao1, BS Dinesh2
1 Department of Oral and Maxillofacial Surgery, Rishiraj College of Dental Sciences, Bhopal, Madhya Pradesh, India
2 Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Soorman Zawiyah University, Zawiyah, Libya
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| Date of Submission | 05-Jan-2011 |
| Date of Decision | 05-Apr-2011 |
| Date of Acceptance | 01-Nov-2011 |
| Date of Web Publication | 11-Oct-2012 |
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 Abstract | | |
Fibromatosis are proliferations of highly differentiated fibrous tissue. The deep or musculoaponeurotic types of fibromatosis are known as desmoid tumors, aggressive fibromatosis, or desmoid-type fibrosarcoma. It is commonly seen as abdominal tumors; the occurrence of these tumors in maxillofacial region is a rare entity. We report a case of aggressive fibromatosis in a 50-year-old female, who presented with a swelling of the left side of the face. The tumor was extending in to the maxillary antrum which was confirmed as aggressive fibromatosis on histopathological examination. The detailed discussion of etiology, incidence, management and prognosis of the same in corelation with the literature is presented in the article. Keywords: Aggressive fibromatosis, desmoid tumor, low grade fibrosarcoma
How to cite this article:
Rao S, Dinesh B S. Aggressive fibromatosis of the oral cavity. Indian J Dent Res 2012;23:435 |
Aggressive fibromatosis is a rare condition which is benign, slow-growing tumors without any metastatic potential, but is locally aggressive. Despite being benign, they can damage nearby structures causing organ dysfunction. Histologically, they resemble low grade fibrosarcomas, but locally, they are very aggressive and have a tendency to recur even after complete resection, therefore, it has to be adjuvanted with post-op radiotherapy.
| Case Report | |  |
A 50-year-old female visited our hospital with a chief complaint of swelling on the left side of the face, which she noticed 2 months ago. Clinical examination revealed an ill-defined swelling of the left infraorbital area with normal overlying skin [Figure 1]. Intra-orally, the swelling obliterated the buccal vestibule on the right side extending from the maxillary first premolar to the second molar. The associated teeth were normal. The swelling was non-tender on palpation. There was no associated cervical lymphadenopathy.  | Figure 1: Pre-operative appearance of the patient with swelling in the left infraorbital area of the face (published with permission of the patient)
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Radiographic findings showed an intraosseous, mixed radiolucent-radioopaque lesion in the maxilla. CT revealed it to be a soft-tissue lesion of the buccal mucosa, extending into the anterior wall of the maxillary antrum [Figure 2].  | Figure 2: CT scan of the face revealing the soft tissue lesion extending into the maxillary sinus.
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Aspiration yielded frank blood. Incisional biopsy under the clinical diagnosis of an odontogenic tumor was performed, but no definitive diagnosis was obtained.
On surgical exploration, the lesion had an extensive involvement with an erosion of lateral and anterior walls of the maxillary sinus. Surgical excision of the lesion with left partial maxillectomy was performed and submitted for histopathological examination. An unusually large amount of bleeding was encountered during the excision of the tumor.
The histopathology of the surgical specimen revealed interlacing bundles of elongated spindle-shaped cells, showing moderate anisonucleosis, with capillary channels in between. Focal areas of hyalinization with peripheral arrangement of cells were observed [Figure 3]. Mitotic activity was 2-4/10 hpf. Few foci of giant cells were also seen. It was finally diagnosed to be a low grade malignant fibrosarcoma - aggressive fibromatosis. | Figure 3: Histopathology showing interlacing bundles of elongated spindle-shaped cells showing moderate anisonucleosis with focal areas of hyalinization (H and E, 10×).
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The patient was sent for a post-operative radiotherapy and followed periodically for 4 years. Radiation dose of 40 Gy at a fractionation of 4x 2.5 Gy/week.
| Discussion | | |
Extra-abdominal desmoid tumors or aggressive fibromatosis are rare tumors of fibroblastic origin, with variable biological behavior. [1] Aggressive fibromatosis account for about 0.03% of all neoplasms, >3% of all soft tissue tumors. An incidence is 2 to 4 per million populations per year. [1] Despite their benign appearance, they are locally aggressive and invasive to surrounding anatomic structures. Because of these characteristics, aggressive fibromatosis are often classified as low-grade fibrosarcomas.
Although etiology is unknown, a connective tissue growth disorder has been supported. [2] Aggressive fibromatosis have been associated with hereditary syndrome like Gardner's syndrome. Based on X-chromosome inactivation pattern, aggressive fibromatosis appear to be monoclonal disorder, indicative of a neoplastic rather than an inflammatory fibrous reactive process. [2]
Aggressive fibromatosis often recur after surgery, particularly after marginal or intralesional excision. Distant hematogenous or lymph nodes metastases have not been observed. Head and neck lesions are more aggressive than extra-abdominal fibromatosis arising elsewhere and are capable of massive destruction of the adjacent bone, erosion of the base of the skull, and occasionally encroach on the trachea, sometimes with fatal outcome. [3],[4]
Current management of aggressive fibromatosis involves a multidisciplinary approach. Although often difficult to achieve because of the tumor's extent and invasiveness, wide margin surgical resection offers the best chance of avoiding local recurrence. [3],[5],[6],[7],[8] Because of the infiltrative nature of these tumors, local recurrence rates after excision may be high. External-beam radiotherapy has been shown to improve local control of aggressive fibromatosis, both in the adjuvant and primary settings. [3],[6],[7],[9] Same observation was obtained in this study as 4 year follow-up has shown no reoccurrence or unusual consequences.
Non-steroid anti-inflammatory drugs such as indomethacin or sulindac as single agents or in combination with antioestrogens [6],[10] have also been used in the treatment of desmoid tumors, with variable response rates. Chemotherapy has been used in inoperable or unresectable tumors, progressive or residual disease. [5],[6],[9]
Although neither hematogenous nor lymphatic metastases have been observed, most lesions will progress refractory to the treatment. Mortality is rare in extra-abdominal desmoid tumors, however, has been reported in head and neck lesions, thus, a continuous follow-up protocol is maintained with the current case.
| Conclusion | | |
In regard with the rarity of the disease and literature on the treatment of the aggressive fibromatosis, we have followed the protocol of Nuyttens et al. [7] in managing the lesion. Nuyttens recommends aggressive surgery, followed by postoperative radiotherapy. Presently the case is under follow-up for the past 4 years, and there is no evidence of local recurrence and other unusual consequences.
| References | | |
| 1. | Leithner A, Schnack B, Katterschafka T, Wiltschke C, Amann G, Windhager R, et al. Treatment of extra-abdominal desmoid tumors with interferon-alpha with or without tretinoin. J Surg Oncol 2000;73:21-5. 
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| 2. | Alman BA, Pajerski ME, Diaz-Cano S, Corboy K, Wolfe HJ. Aggressive fibromatosis (desmoid tumor) is a monoclonal disorder. Diagn Mol Pathol 1997;6:98-101.
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| 3. | Posner MC, Shiu MH, Newsome JL, Hadju SI, Gaynor JJ, Brennan MF. The desmoid tumor: Not a benign disease. Arch Surg 1989;124:191-6.
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| 4. | Sweis IE, McHenry CR, Jordan RB. Limb and life threatening desmoid tumor of the neck. Plast Reconstr Surg 1993;92:335-9.
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| 7. | Nuyttens JJ, Rust PF, Thomas CR Jr, Turrisi AT 3rd. Surgery versus Radiation Therapy for Patients with Aggressive Fibromatosis or Desmoid Tumors A Comparative Review of 22 Articles. Cancer 2000;88:1517-23.
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| 8. | Patel SR, Benjamin RS. Desmoid Tumors Respond to Chemotherapy: Defying the Dogma in Oncology. J Clin Oncol 2006;24:11-2.
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| 9. | Kiel KD, Suit HD. Radiation therapy in the treatment of aggressive fibromatoses (desmoid tumors). Cancer 1984;54:2051-5.
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| 10. | Klein WA, Miller HH, Anderson M, DeCosse JJ. Use of indomethacin, suldinac, and tamoxifen for the treatment of desmoid tumors associated with familial polyposis. Cancer 1987;60:2863-8.
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Correspondence Address:
Santhosh Rao
Department of Oral and Maxillofacial Surgery, Rishiraj College of Dental Sciences, Bhopal, Madhya Pradesh
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0970-9290.102256
[Figure 1], [Figure 2], [Figure 3] |
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