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Background: Plexiform fibrohistiocytic tumor is a soft-tissue tumor of interm ediate malignancy occurring in children and young adults but is only rarely foun d in infants. The tumor usually involves the upper limbs and is slow growing and painless. Recurrence rate is high. Lymph node and systemic metastases can occur , but have never been reported in infants. Clinical behavior in infancy is not k nown. Histologically, the tumor is characterized by nodules of histiocyte-like and multinucleated cells and fascicles of spindle cells arranged in a plexiform pattern. Mitosis, atypia, and nuclear pleomorphism are common but not pronounced . Methods and Results: We report three cases in infants, one of which is congeni tal, having an unusual topography and a broad histological spectrum. Conclusion: In infants, wide excision with large safety margins should be performed as the behaviour of this tumor remains uncertain.
Background: Plexiform fibrohistiocytic tumor is a soft-tissue tumor of interm ediate malignancy occurring in children and young adults but is only rarely foun d in infants. The tumor usually involves the upper limbs and is slow growing and painless. Recurrence rate is high. Lymph node and systemic metastases can occur, but have have been reported in infants. Clinical behavior in infancy is not kown. Histologically, the tumor is characterized by nodules of histiocyte-like and multinucleated cells and fascicles of spindle cells arranged in a plexiform pattern. Mitosis, atypia, and nuclear pleomorphism are common but not pronounced. Methods and Results: We report three cases in infants, one of which is congeni tal, having an unusual topography and a broad histological spectrum. Conclusion: In infants, wide excision with large safety margins should be performed as the behavior of this tumor remains uncertain.