Ray-like extension of fibrous tissue into surrounding fat
Infiltrates between and around individual fat cells
Lacks pseudovascular spaces
Pseudovascular spaces present
Nodules of histiocytes and giant cells in most cases
Scattered individual giant cells
CD34 stains lesional cells
Mean age 15-20 years
May occur at any age
Most frequent in upper extremity
May involve wide variety of other sites
Approximately 1/3 recur
Not destructive or uncontrolled
Regional lymph nodes
Kempson RL, Fletcher CDM, Evans HL, Henrickson MR, Sibley RS. Tumors of the Soft Tissues, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 30, 2001
Fletcher CDM, Unni KK, Mertens F. Pathology and Genetics of Tumours of Soft Tissue and Bone, World Health Organization Classification of Tumours 2002
Weiss SW, Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors, 5th edition, 2008
Moosavi C, Jha P, Fanburg-Smith JC. An update on plexiform fibrohistiocytic tumor and addition of 66 new cases from the Armed Forces Institute of Pathology, in honor of Franz M. Enzinger, MD. Ann Diagn Pathol. 2007 Oct;11(5):313-9.
Remstein ED, Arndt CA, Nascimento AG. Plexiform fibrohistiocytic tumor: clinicopathologic analysis of 22 cases. Am J Surg Pathol. 1999 Jun;23(6):662-70.
Salomao DR, Nascimento AG. Plexiform fibrohistiocytic tumor with systemic metastases: a case report. Am J Surg Pathol. 1997 Apr;21(4):469-76.
Zelger B, Weinlich G, Steiner H, Zelger BG, Egarter-Vigl E. Dermal and subcutaneous variants of plexiform fibrohistiocytic tumor. Am J Surg Pathol. 1997 Feb;21(2):235-41.
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.
Billings SD, Folpe AL. Cutaneous and subcutaneous fibrohistiocytic tumors of intermediate malignancy: an update. Am J Dermatopathol. 2004 Apr;26(2):141-55.
Jaffer S, Ambrosini-Spaltro A, Mancini AM, Eusebi V, Rosai J. Neurothekeoma and plexiform fibrohistiocytic tumor: mere histologic resemblance or histogenetic relationship? Am J Surg Pathol. 2009 Jun;33(6):905-13.