Aggressive fibromatosis in the head and neck region: Benign tumor with often mutilating effects

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Title:	Aggressive fibromatosis in the head and neck region: Benign tumor with often mutilating effects
Author(s):	Hendriks, M.P. ; Driessen, C.M.L.; Laarhoven, H.W.M. van ; Janssens, G.O.R.J.; Verbist, B.M.; Graaf, W.T.A. van der ; Slootweg, P.J. ; Merkx, M.A.W. ; Herpen, C.M.L. van
Publication year:	2013
Source:	Head and Neck-Journal for the Sciences and Specialties of the Head and Neck, vol. 35, iss. 8, (2013), pp. E246-50
ISSN:	1043-3074
DOI:	https://doi.org/10.1002/hed.23092
Publication type:	Article / Letter to editor
Please use this identifier to cite or link to this item : https://hdl.handle.net/2066/119191
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Subject:	NCEBP 2: Evaluation of complex medical interventions ONCOL 3: Translational research NCEBP 7: Effective primary care and public health NCMLS 2: Immune Regulation ONCOL 3: Translational research ONCOL 2: Age-related aspects of cancer NCEBP 4: Quality of hospital and integrated care ONCOL 5: Aetiology, screening and detection Tijdelijke code tbv inlezen publicaties Radboudumc - Alleen voor gebruik door Radboudumc
Organization:	Anesthesiology Medical Oncology Radiation Oncology Radiology Pathology Oral and Maxillofacial Surgery
Journal title:	Head and Neck-Journal for the Sciences and Specialties of the Head and Neck
Volume:	vol. 35
Issue:	iss. 8
Page start:	p. E246
Page end:	p. 50
Abstract:	BACKGROUND: Aggressive fibromatosis (AF) or desmoid tumor of the head and neck region is a rare, usually unresectable, benign soft tissue tumor with locally aggressive behavior. METHODS AND RESULTS: A 31-year-old woman presented with a progressive trismus, a swelling in the retromandibular area, as well as loss of sensibility of the maxillary and mandibular branch of the trigeminal nerve. MRI of the head and neck revealed an infiltrative mass involving the masticator, parapharyngeal, and prevertebral and paravertebral space on the left with intracranial extension through the orbital fissure. After the fifth biopsy, 15 months after presentation, the diagnosis of AF was made. The tumor was unresectable, so intensity-modulated radiotherapy was given with curative intent using a total dose of 60 Gy in 30 fractions of 2 Gy. After 16 months, she showed progressive disease, for which tamoxifen 40 mg twice daily was started with a good response for 2 years. After that, she started with sorafinib, on which she has stable disease now. CONCLUSION: The often long delay in proper diagnosis and the treatment challenges of a desmoid tumor are illustrated in this case. Furthermore, this article reviews the literature concerning AF, especially of the head and neck region. Head Neck, 2013.