Synopsis

 multinodular or plexiform proliferation of histiocyte- and fibroblast-like cells associated with multinuclear giant cells.
 +/- multinucleated giant cells, osteoclast-like giant cells (#12357200#)
 rare myxoid changes

Variants

 myxoid plexiform fibrohistiocytic tumour (#12428852#)
 metastatic forms (#9130995#)

Cytogenetics

 t(8;?) (p22;?) (#2164878#)
 t(4;15)(q21;q15) (#9973942#)

Differential diagnosis

 granuloma
 fibrous hamartoma
 fibrous histiocytoma
 giant cell tumor
 fibromatoses

Prognosis

 frequent recurrence (12.5-40%) within 1-2 years after original diagnosis
 extremely rare lymph node metastases
 extremely rare pulmonary metatstases

References

 Remstein ED, Arndt CA, Nascimento AG. Plexiform fibrohistiocytic tumor: clinicopathologic analysis of 22 cases. Am J Surg Pathol. 1999 Jun;23(6):662-70. PMID: #10366148#

 Thomazy V, Nagy A, Gal I, Nemes Z. Plexiform fibrohistiocytic tumour with novel phenotypic features. Histopathology. 1994 Aug;25(2):165-9. PMID: #7982679#

 Enzinger FM, Zhang RY. Plexiform fibrohistiocytic tumor presenting in children and young adults. An analysis of 65 cases. Am J Surg Pathol. 1988 Nov;12(11):818-26. PMID: #2847569#