Aggressive fibromatosis of the oral cavity.

Desmoid fibromatosis is a benign fibroblastic neoplasm with high recurrence rates predominantly observed in pediatric and adolescent patients. The use of wide resection margins has been discussed controversially in literature. In addition, data on non-surgical treatment is limited as phase III studies are still missing. Nineteen patients under the age of 18years were identified. The mean age at diagnosis was 6.6years, with a mean follow-up of 114 months. Recurrences were detected in four out of nineteen patients. Surprisingly, the recurrence rate was not influenced by type of resection used (R0, R1/2). All samples were tested negative for ER α, somatostatin, and progesterone receptor. In contrast, a majority of tumors showed positive results for PDGFR α and β and β-catenin. No correlation between positive immunohistochemical markers and tumor recurrences was detectable. In conclusion, recurrence rates are not depending on resection type and immunohistochemical markers seem to behave differently in children and adolescents in contrast to adult patients.

The authors report a case of a 31-year-old pregnant woman with a large desmoid tumor, which increased rapidly in size and caused worsening symptom of dyspnea. The tumor was successfully removed during a caesarian section, which resulted in an anterior abdominal wall defect. Reconstruction of the abdominal wall defect was performed with a polypropylene mesh. The postoperative recovery of the patient was uneventful. After a follow-up of 44 months, the patient was found to be well and there was no evidence of local recurrence. The authors also reviewed the literature on the world’s experience with this tumor and its management during pregnancy.Twelve desmoid tumors arising during pregnancy were reported in the existing literature; the managements were varied and has yet to be defined.