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Aggressive fibromatosis in the maxilla
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British Journal of Oral and Maxillofacial Surgery 47 (2009) 129–131
Short Communication
Aggressive fibromatosis in the maxilla Tenglong Hu a,b,∗ , Guangping Jing b , Kewen Lv a a b
The Second Department of Oral & Maxillofacial Surgery, The First Affiliated Hospital, Harbin Medical University, Harbin, China Department of Oral Anatomy & Physiology, Stomatology School, Harbin Medical University, Harbin, China
Accepted 12 June 2008 Available online 18 July 2008
Abstract We present a rare case of recurrent aggressive fibromatosis of the maxilla in a 61-year-old woman, who was treated by resection of the left maxilla. Adjuvant treatments, particularly radiotherapy, are valuable if the tumour recurs. © 2008 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Keywords: Aggressive fibromatosis; Desmoid tumor; Maxilla
Introduction Aggressive fibromatosis (or desmoid tumour) is characterised by infiltrating growth of well-differentiated fibroblasts or myofibroblasts, and often recurs, in particular if the resection was incomplete, but it has no metastatic potential.1 The most common sites of these lesions are the mesentery, the retroperitoneal space, and the abdominal wall, or in extraabdominal sites such as the trunk and the extremities.2 They are rare in the head and neck region, particularly the maxilla. We present the case of aggressive fibromatosis in an adult woman which recurred twice postoperatively.
Case report A 61-year-old woman with a large painless mass on the left side of her face was referred to us in July 2000. Radiographs showed an area of low bone density roughly 2 × 3 cm in size with an area sharply defined at the roots in the left maxilla. The provisional clinical diagnosis was a maxillary cyst. The ∗ Corresponding author at: 23 You Zheng Street, Nan Gang District, The Second Department of Oral & Maxillofacial Surgery, The First Affiliated Hospital, Harbin Medical University, Harbin, People’s Republic of China, 150001. Tel.: +86 451 53643849 3928; fax: +86 451 53650087. E-mail addresses: flylong [email protected] (T. Hu), [email protected] (G. Jing), [email protected] (K. Lv).
mass was removed and a diagnosis of fibroma made. No further treatment was given. One year after the operation the painless swelling had recurred and was gradually increasing in size. A computed tomogram showed a low density soft tissue image with uneven boundaries. After reevaluation of the previous microscopic section, the diagnosis was changed to aggressive fibromatosis. In April, 2002, we did an open biopsy and the diagnosis was confirmed (Fig. 1, 2). This time she had a total excision of the lesion including the left upper dental alveolar and left hard palate with less than 1 cm free margin. She was followed up for 2 years with no further intervention. In September 2004, she complained of discomfort at the left maxilla and orbit, and on this occasion the computed tomogram showed a mass located in the left maxillary sinus and invading the orbital cavity. We therefore excised the whole left maxilla (Fig. 3). Histological diagnosis from both frozen and paraffin sections confirmed the diagnosis.. Local radiotherapy with doses of 50 Gy was given two weeks after the operation. She has been followedup regularly and there has been no recurrence so far.
Discussion Patients with aggressive fibromatosis often present with a deep, firm mass with or without pain. Genetic abnormalities,
0266-4356/$ – see front matter © 2008 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.bjoms.2008.06.010
130
T. Hu et al. / British Journal of Oral and Maxillofacial Surgery 47 (2009) 129–131
Fig. 1. Histological examination of the resected tumour (haematoxylin and eosin, original magnification × 200) showed proliferation of uniform spindle cells in densely collagenized stroma. Cells and collagen fibres were arranged in sweeping bundle that intersected at various angles.
physical and endocrine factors, and particularly steroid sex hormones seem to be important in the development of the disease. There are high incidences of oestrogen and antioestrogen binding sites in the disease, and collagen can be produced by culture of cells from the tumour in response to oestrogen.3 Diagnosis is difficult because the tumours are often classified as different types of fibromatosis or confused with low grade fibrosarcoma.4 Other differential diagnoses are fibroma (including the present study), fibrous histiocytoma, granuloma, cyst, ameloblastama, and fibrous dysplasia.5 The tumour is rare in the maxilla. We know of only 15 cases (including the present one) that have been reported from1980 to now.1,4,5 The standard treatment is resection with wide free margins, but in the head and neck the operation is difficult because many important structures are concentrated in the area. Radiotherapy can be used alone when resection might lead to serious morbidity and loss of function, and when the tumour is inoperable or there is gross residual disease after operative debulking.6 Chemotherapy has also been suggested.
Fig. 3. The lesion during operation.
Despite the use of resection and radiochemotherapy, 20%–35% of these tumours recur locally.7 The recurrence rate of a tumor treated by operation alone is reported to be around 40%.8 The potential morbidity from resection and radiochemotherapy, and the high local recurrence rates, have led investigators to evaluate the role of systemic treatment with drugs such as tamoxifen, toremifene, non-steroidal anti-inflammatory drugs, or biological agents such as interferon or retinoic acid. 1, 25-(OH)2 -vitamin D3 has also been shown effective in cases of progression and local recurrence.9
Acknowledgements This study was supported by the Science and Technology Bureau, Harbin, China (No. 2005AA9CS116-3). We thank Eryang Zhao for excellent technical assistance.
References
Fig. 2. Immunohistochemical staining showed the tumour cells stained diffusely for beta-catenin (original magnification × 400).
1. Bertrand JC, Plautier D, Chanterelle A. Mazza. Maxillary and mandibular desmoid fibromas (author’s transl). Rev Stomatol Chir Maxillofac 1981;82:127–31. 2. Loccufier A, Vanhulle A, Moreels R, Deruyter L, Legley W. Gardner syndrome and desmoid tumors. Acta Chir Belg 1993;93:230–2.
T. Hu et al. / British Journal of Oral and Maxillofacial Surgery 47 (2009) 129–131 3. Tonelli F, Valanzano R, Brandi ML. Pharmacologic treatment of desmoid tumors in familial adenomatous polyposis: results of an in vitro study. Surgery 1994;115:473–9. 4. Mascres C, Vauclair R. The maxillo-mandibular desmoid fibroma. Synthesis of knowledge about a rare entity. Apropos of 3 cases. Ann Pathol 1989;9:182–8. 5. Donohue WB, Malexos D, Pham H. Aggressive fibromatosis of the maxilla. Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol 1990;69:420–6. 6. Pritchard DJ, Nascimento AG, Perersen IA. Local control of extraabdominal desmoid tumors. J Bone Joint Surg 1996;78A:848–54.
131
7. Janinis J, Patriki M, Vini L, Aravantinos G, Whelan JS. The pharmacological treatment of aggressive fibromatosis: a systematic review. Ann Oncol 2003;14:181–90. 8. Shields CJ, Winter WO, Kirwan WO, Redmond HP. Desmoid tumors. Eur J Surg Oncol 2001;27:701–6. 9. Yildiz F, Kars A, Cengiz M, Selek U, Gurkaynak M, Atahan I. Possible therapeutic role of vitamin D3 in aggressive fibromatosis. Japanese Journal of Clinical Oncology 2004;34: 472–5.
British Journal of Oral and Maxillofacial Surgery 47 (2009) 129–131
Short Communication
Aggressive fibromatosis in the maxilla Tenglong Hu a,b,∗ , Guangping Jing b , Kewen Lv a a b
The Second Department of Oral & Maxillofacial Surgery, The First Affiliated Hospital, Harbin Medical University, Harbin, China Department of Oral Anatomy & Physiology, Stomatology School, Harbin Medical University, Harbin, China
Accepted 12 June 2008 Available online 18 July 2008
Abstract We present a rare case of recurrent aggressive fibromatosis of the maxilla in a 61-year-old woman, who was treated by resection of the left maxilla. Adjuvant treatments, particularly radiotherapy, are valuable if the tumour recurs. © 2008 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Keywords: Aggressive fibromatosis; Desmoid tumor; Maxilla
Introduction Aggressive fibromatosis (or desmoid tumour) is characterised by infiltrating growth of well-differentiated fibroblasts or myofibroblasts, and often recurs, in particular if the resection was incomplete, but it has no metastatic potential.1 The most common sites of these lesions are the mesentery, the retroperitoneal space, and the abdominal wall, or in extraabdominal sites such as the trunk and the extremities.2 They are rare in the head and neck region, particularly the maxilla. We present the case of aggressive fibromatosis in an adult woman which recurred twice postoperatively.
Case report A 61-year-old woman with a large painless mass on the left side of her face was referred to us in July 2000. Radiographs showed an area of low bone density roughly 2 × 3 cm in size with an area sharply defined at the roots in the left maxilla. The provisional clinical diagnosis was a maxillary cyst. The ∗ Corresponding author at: 23 You Zheng Street, Nan Gang District, The Second Department of Oral & Maxillofacial Surgery, The First Affiliated Hospital, Harbin Medical University, Harbin, People’s Republic of China, 150001. Tel.: +86 451 53643849 3928; fax: +86 451 53650087. E-mail addresses: flylong [email protected] (T. Hu), [email protected] (G. Jing), [email protected] (K. Lv).
mass was removed and a diagnosis of fibroma made. No further treatment was given. One year after the operation the painless swelling had recurred and was gradually increasing in size. A computed tomogram showed a low density soft tissue image with uneven boundaries. After reevaluation of the previous microscopic section, the diagnosis was changed to aggressive fibromatosis. In April, 2002, we did an open biopsy and the diagnosis was confirmed (Fig. 1, 2). This time she had a total excision of the lesion including the left upper dental alveolar and left hard palate with less than 1 cm free margin. She was followed up for 2 years with no further intervention. In September 2004, she complained of discomfort at the left maxilla and orbit, and on this occasion the computed tomogram showed a mass located in the left maxillary sinus and invading the orbital cavity. We therefore excised the whole left maxilla (Fig. 3). Histological diagnosis from both frozen and paraffin sections confirmed the diagnosis.. Local radiotherapy with doses of 50 Gy was given two weeks after the operation. She has been followedup regularly and there has been no recurrence so far.
Discussion Patients with aggressive fibromatosis often present with a deep, firm mass with or without pain. Genetic abnormalities,
0266-4356/$ – see front matter © 2008 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.bjoms.2008.06.010
130
T. Hu et al. / British Journal of Oral and Maxillofacial Surgery 47 (2009) 129–131
Fig. 1. Histological examination of the resected tumour (haematoxylin and eosin, original magnification × 200) showed proliferation of uniform spindle cells in densely collagenized stroma. Cells and collagen fibres were arranged in sweeping bundle that intersected at various angles.
physical and endocrine factors, and particularly steroid sex hormones seem to be important in the development of the disease. There are high incidences of oestrogen and antioestrogen binding sites in the disease, and collagen can be produced by culture of cells from the tumour in response to oestrogen.3 Diagnosis is difficult because the tumours are often classified as different types of fibromatosis or confused with low grade fibrosarcoma.4 Other differential diagnoses are fibroma (including the present study), fibrous histiocytoma, granuloma, cyst, ameloblastama, and fibrous dysplasia.5 The tumour is rare in the maxilla. We know of only 15 cases (including the present one) that have been reported from1980 to now.1,4,5 The standard treatment is resection with wide free margins, but in the head and neck the operation is difficult because many important structures are concentrated in the area. Radiotherapy can be used alone when resection might lead to serious morbidity and loss of function, and when the tumour is inoperable or there is gross residual disease after operative debulking.6 Chemotherapy has also been suggested.
Fig. 3. The lesion during operation.
Despite the use of resection and radiochemotherapy, 20%–35% of these tumours recur locally.7 The recurrence rate of a tumor treated by operation alone is reported to be around 40%.8 The potential morbidity from resection and radiochemotherapy, and the high local recurrence rates, have led investigators to evaluate the role of systemic treatment with drugs such as tamoxifen, toremifene, non-steroidal anti-inflammatory drugs, or biological agents such as interferon or retinoic acid. 1, 25-(OH)2 -vitamin D3 has also been shown effective in cases of progression and local recurrence.9
Acknowledgements This study was supported by the Science and Technology Bureau, Harbin, China (No. 2005AA9CS116-3). We thank Eryang Zhao for excellent technical assistance.
References
Fig. 2. Immunohistochemical staining showed the tumour cells stained diffusely for beta-catenin (original magnification × 400).
1. Bertrand JC, Plautier D, Chanterelle A. Mazza. Maxillary and mandibular desmoid fibromas (author’s transl). Rev Stomatol Chir Maxillofac 1981;82:127–31. 2. Loccufier A, Vanhulle A, Moreels R, Deruyter L, Legley W. Gardner syndrome and desmoid tumors. Acta Chir Belg 1993;93:230–2.
T. Hu et al. / British Journal of Oral and Maxillofacial Surgery 47 (2009) 129–131 3. Tonelli F, Valanzano R, Brandi ML. Pharmacologic treatment of desmoid tumors in familial adenomatous polyposis: results of an in vitro study. Surgery 1994;115:473–9. 4. Mascres C, Vauclair R. The maxillo-mandibular desmoid fibroma. Synthesis of knowledge about a rare entity. Apropos of 3 cases. Ann Pathol 1989;9:182–8. 5. Donohue WB, Malexos D, Pham H. Aggressive fibromatosis of the maxilla. Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol 1990;69:420–6. 6. Pritchard DJ, Nascimento AG, Perersen IA. Local control of extraabdominal desmoid tumors. J Bone Joint Surg 1996;78A:848–54.
131
7. Janinis J, Patriki M, Vini L, Aravantinos G, Whelan JS. The pharmacological treatment of aggressive fibromatosis: a systematic review. Ann Oncol 2003;14:181–90. 8. Shields CJ, Winter WO, Kirwan WO, Redmond HP. Desmoid tumors. Eur J Surg Oncol 2001;27:701–6. 9. Yildiz F, Kars A, Cengiz M, Selek U, Gurkaynak M, Atahan I. Possible therapeutic role of vitamin D3 in aggressive fibromatosis. Japanese Journal of Clinical Oncology 2004;34: 472–5.