Plexiform Fibrohistiocytic Tumor Without Multinucleated Giant Cells

Abstract

Plexiform fibrohistiocytic tumor (PFT) is a rare but distinctive soft tissue tumor of children and young adults characterized by a mixture of histiocyte-, myofibroblast-, and osteoclast-like giant cells arranged in a plexiform pattern. We report the clinicopathologic and immunohistochemical features of an apparently unique case of PFT without multinucleated giant cells presenting in a 3-year-old child. Light microscopy revealed a subcutaneous tumor composed of a plexiform proliferation of histiocyte- and myofibroblast-like cells. Multinucleated osteoclast-like giant cells, the third classic cellular component of this mesenchymal neoplasm, were not observed. The differential diagnosis is also discussed. Emphasis is placed on the importance of the recognition of PFT, because it may exhibit an aggressive behavior.

Primary giant cell tumor of soft tissue of the groin – a case of 46 years duration

“…Plexiform fibrohistiocytic tumor should also be considered in the differential diagnosis; this neoplasm mainly affects the distal upper extremities of female children and adolescents and shows a plexiform pattern with smaller nodules usually centred by multinucleated cells 5,15–17 …”

Morphological variations of scar-related and spontaneous endometriosis of the skin and superficial soft tissue: A study of 71 cases with emphasis on atypical features and types of müllerian differentiations

“…When embedded in a fibrous tissue in scar-related cases, these stromal islands devoid of the glandular epithelium sometimes had a resemblance to tumorous elements seen in plexiform fibrohistiocytic tumor, especially to the areas in the latter entity in which there are no giant cells, a situation encountered at times. 12 Smooth muscle metaplasia was seen in 22 cases and appeared either as well-formed, well-demarcated fascicles of well-differentiated smooth muscle cells, often with cigar-shaped nuclei (Fig 6, A) or the appearance was that of a fascicular myofibroblastic proliferation with a similarity to desmoid (Fig 6, B).…”

Plexiform fibrohistiocytic tumor: three unusual cases occurring in infancy

“…The nodular aggregates are made of histiocyte‐like cells admixed with multinucleated osteoclast‐like cells, surrounding cells of intermediate morphology. Two cases 5,6 were reported without multinucleated osteoclast‐like cells. Foci of microhemorrhage are frequent, with hemosiderin deposits in some of the nodular aggregates.…”