Plexiform fibrohistiocytic tumor: three unusual cases occurring in infancy.
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.


RESULTS
We report three cases in infants, one of which is congenital, having an unusual topography and a broad histological spectrum.


CONCLUSIONS
In infants, wide excision with large safety margins should be performed as the behaviour of this tumor remains uncertain. Leclerc S, Hamel-Teillac D, Oger P, Brousse N, Fraitag S. Plexiform fibrohistiocytic tumor: three unusual cases occurring in infancy.
Leclerc
Stéphanie
S
Department of Pathology, Necker-Enfants Malades Hospital, Paris, France.
Hamel-Teillac
Dominique
D
Oger
Patrice
P
Brousse
Nicole
N
Fraitag
Sylvie
S
D014408:Biomarkers, Tumor / Q000032:analysis; D005260:Female; D018219:Histiocytoma, Benign Fibrous / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007124:Immunoenzyme Techniques; D007223:Infant; D008297:Male; D012983:Soft Tissue Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D016896:Treatment Outcome
D014408:Biomarkers, Tumor