Plexiform fibrohistiocytic tumor explained

Plexiform fibrohistiocytic tumor is a rare tumor that arises primarily on the upper extremities of children and young adults.^[1]

Signs and symptoms

Plexiform fibrohistiocytic tumor appears as a painless, slowly expanding soft tissue mass located in the dermis and subcutis.^[2] The skin layer above is somewhat elevated and occasionally has a central depression.^[3] The upper extremities were more commonly affected by plexiform fibrohistiocytic tumors. It was most frequently found on the fingers, hand, or wrist.^[4] The tumour's dimensions vary from 0.3 to 8.5 cm.^[2]

Causes

Plexiform fibrohistiocytic tumor's pathogenesis is unclear. A few cases of prior trauma have been documented. Two series of congenital instances have been documented.^{[5] [6]}

Diagnosis

From a histological perspective, plexiform fibrohistiocytic tumor is made up of several tiny nodules or long fascicles that are primarily arranged in a plexiform pattern. Variable amounts of three distinct cell types are found. These are multinucleate large cells, mononuclear histiocyte-like cells, and spindle fibroblast-like cells.^[7]

There are three main well-documented histologic patterns that have been identified: a fibrohistiocytic subtype that consists of plexiform clusters of mononuclear histiocyte-like cells and multinucleated giant cells; a fibroblastic subtype that consists primarily of elongated clusters and short fascicles of spindle fibroblast-like cells; and a mixed subtype that consists of equal parts of both patterns.^[8]

The fibroblastic cells show uniform reactivity to vimentin and at least focal reactivity for smooth muscle actin, while the histiocytic cells of plexiform fibrohistiocytic tumor stain for CD68 (KP-1).

Differential diagnoses of plexiform fibrohistiocytic tumor include plexiform schwannoma, plexiform neurofibroma, cellular neurothekeoma, deep benign fibrous histiocytoma, fibrous hamartoma of infancy, dermatofibroma, benign and malignant soft tissue giant cell tumor, and myofibromatosis.

Treatment

The treatment for plexiform fibrohistiocytic tumor is complete surgical resection.^[9]

See also

• Sarcoma

Further reading

• Thangaiah . Judith Jebastin . Dashti . Nooshin K. . Agaimy . Abbas . Fritchie . Karen . Folpe . Andrew L. . Plexiform fibrohistiocytic tumor: a clinicopathological and immunohistochemical study of 39 tumors, with evidence for a CSF1-producing “null cell” population . Virchows Archiv . 481 . 5 . 2022 . 0945-6317 . 10.1007/s00428-022-03408-2 . 739–750 . none.
• Chen . Yi-Chun . Hsiao . Cheng-Hsiang . Chen . Jau-Shiuh . Liao . Yi-Hua . Plexiform fibrohistiocytic tumor—report of one case with regional lymph node metastasis . Dermatologica Sinica . Medknow . 28 . 3 . 2010 . 1027-8117 . 10.1016/s1027-8117(10)60025-0 . 117–120 . none.

External links

• National Cancer Institute
• Pathology Outlines

Notes and References

1. James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. .
2. Taher . Altaf . Pushpanathan . Chitra . Plexiform Fibrohistiocytic Tumor: A Brief Review . Archives of Pathology & Laboratory Medicine . 131 . 7 . 2007-07-01 . 1543-2165 . 10.5858/2007-131-1135-PFTABR . 1135–1138.
3. Enzinger . Franz M. . Zhang . Renyuan . Plexiform Fibrohistiocytic Tumor Presenting in Children and Young Adults . The American Journal of Surgical Pathology . Ovid Technologies (Wolters Kluwer Health) . 12 . 11 . 1988 . 0147-5185 . 10.1097/00000478-198811000-00002 . 818–826.
4. Remstein . Ellen D. . Arndt . Carola A.S. . Nascimento . Antonio G. . Plexiform Fibrohistiocytic Tumor: Clinicopathologic Analysis of 22 Cases . The American Journal of Surgical Pathology . Ovid Technologies (Wolters Kluwer Health) . 23 . 6 . 1999 . 0147-5185 . 10.1097/00000478-199906000-00005 . 662–670.
5. HOLLOWOOD . K. . HOLLEY . M.P. . FLETCHER . C.D.M. . Plexiform fibrohistiocytic tumour: clinicopathological, immunohistochemical and ultrastructural analysis in favour of a myofibroblastic lesion . Histopathology . Wiley . 19 . 6 . 1991 . 0309-0167 . 10.1111/j.1365-2559.1991.tb01498.x . 503–513.
6. Leclerc . Stéphanie . Hamel‐Teillac . Dominique . Oger . Patrice . Brousse . Nicole . Fraitag . Sylvie . Plexiform fibrohistiocytic tumor: three unusual cases occurring in infancy . Journal of Cutaneous Pathology . Wiley . 32 . 8 . 2005-08-18 . 0303-6987 . 10.1111/j.0303-6987.2005.00334.x . 572–576.
7. Zemheri . Ebru . Özkanlı . Şeyma . Şenol . Serkan . Ozen . Filiz . Ulukaya Durakbaşa . Cigdem . Zindancı . İlkin . Okur . Hamit . Mitotically Active Plexiform Fibrohistiocytic Tumor . Case Reports in Pathology . Hindawi Limited . 2013 . 2013 . 2090-6781 . 10.1155/2013/547372 . free . 1–3.
8. Cho . S . Chang . S‐E . Choi . J‐H . Sung . K‐J . Moon . K‐C . Koh . J‐K . Myxoid plexiform fibrohistiocytic tumour . Journal of the European Academy of Dermatology and Venereology . Wiley . 16 . 5 . 2002 . 0926-9959 . 10.1046/j.1468-3083.2002.00499.x . 519–521.
9. Ghuman . Marcus . Hwang . Sinchun . Antonescu . Cristina R. . Panicek . David M. . Plexiform fibrohistiocytic tumor: imaging features and clinical findings . Skeletal Radiology . Springer Science and Business Media LLC . 48 . 3 . 2018-08-25 . 0364-2348 . 10.1007/s00256-018-3050-1 . 437–443. 6344299 .