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Reported cases of pediatric desmoid fibromatosis of the paranasal sinuses. |
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| Age |
Location |
Presentation |
Pathology |
Therapy |
Response |
Reference |
|
|
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| 2 year old male |
Right maxillary sinus |
Nasal obstruction |
Aggressive fibromatosis |
Surgical resection |
Lost to follow up |
[1] |
| 14 year old female |
Right parotid/mandible |
Right facial deformity |
Aggressive fibromatosis |
Surgical resection (positive margins) |
No recurrence at < 1 year |
[6] |
| 15 month old male |
Nasal cavity/anterior maxilla |
Facial deformity |
Aggressive fibromatosis |
1. Surgical resection (positive margins) 2. Surgical resection (negative margins) |
Recurrence in 1 month, no recurrence |
[5] |
| 2 year old male |
Left maxillary sinus |
Nasal deformity |
Desmoid fibromatosis |
Surgical resection (twice), followed by adjuvant tamoxifen |
No recurrence at 2 years |
[4] |
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Conley et al. [2] reported a series of 40 different cases, three cases between the ages 1–10. One of these cases involved the ethmoid sinus. Fu [3] reported two cases of juvenile fibromatosis ages 2 and 10. One of these cases involved the maxillary sinus. | ||||||
Lakhan et al. Journal of Hematology & Oncology 2008 1:3 doi:10.1186/1756-8722-1-3 |
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