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Plexiform fibrohistiocytic tumor: three unusual cases occurring in infancy
Author(s) -
Leclerc Stéphanie,
HamelTeillac Dominique,
Oger Patrice,
Brousse Nicole,
Fraitag Sylvie
Publication year - 2005
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/j.0303-6987.2005.00334.x
Subject(s) - pathology , medicine , malignancy , atypia , pleomorphism (cytology) , nuclear atypia , giant cell , histiocyte , soft tissue , anatomy , immunohistochemistry
Background: Plexiform fibrohistiocytic tumor is a soft‐tissue tumor of intermediate malignancy occurring in children and young adults but is only rarely found 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 known. 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 congenital, 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.