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calcifying fibrous tumor

Monday 27 April 2009

calcifying fibrous pseudotumor

PO

Definition: Calcifying fibrous tumor (CFT) is a rare benign mesenchymal tumor composed of hyalinized fibrous tissue with interspersed bland fibroblastic spindled cells, scattered psammomatous, and/or dystrophic calcifications and variably prominent mononuclear inflammatory infiltrate. CFTs show a predilection for the abdominal cavity and soft tissue.

Calcifying fibrous tumor (CFT) is a rare benign lesion that is composed of abundant dense well-circumscribed hyalinized collagen with lymphoplasmatic infiltrate, spindle cells, lymphoid aggregates, and psammomatous or dystrophic calcifications.

This tumor was first described in the deep soft tissue in children, but subsequent reports revealed the occurrence of this lesion also in adults.

Common sites that CFT arises from are pleura, abdominal cavity, mediastinum, heart, lung, neck, mandible, oral, inguinal, paratesticular, intrascrotal, spine, back, arm, and thigh. The etiology and pathogenesis of the tumor are controversial.

CFTs are rare, benign lesions, which show a predilection for soft tissue. They are characterized by the presence of hypocellular, densely hyalinized collagen with psammomatous or dystrophic calcification and mononuclear inflammatory infiltrate.

Images

- Calcifying fibrous pseudo/tumor

Calcifying fibrous tumor, formerly known as calcifying fibrous pseudotumor, is now recognized to occur at a wide variety of anatomical sites, mainly in children and young adults.

It which may recur locally and consists of hypocellular/hyalinized fibrous tissue containing scattered lymphocytes, plasma cells and stromal calcifications which are often psammomatous in appearance.

Differential diagnosis

- In the neck , the pleura , and the thoracic cavity :

  • fibromatosis
  • solitary fibrous tumor (SFT)
  • calcified granulomas
    • Calcified granulomas contain residual histiocytes and multinuclear giant cells, whereas calcified pleural plaques and chronic fibrous pleuritis are characterized by diffuse pleural thickening.
  • calcified pleural plaques
  • chronic fibrous pleuritis
    • The cells in chronic fibrous pleuritis are more adjacent to the pleura and the capillaries are vertically aligned.
  • intrapulmonary tumors
    • Intrapulmonary tumors are most commonly childhood lung tumors and there is no specific method (laboratory check or imaging) in order that these tumors are distinguished from CFT.
  • desmoplastic mesothelioma
    • Malignant mesothelioma has got sarcomatous areas and a weak positive staining for osteopontin, but is strongly positive for cytokeratin.
  • amyloid tumor
  • monophasic synovial sarcoma
    • This type of tumor consists of spindle cells in dense cellular sheets or vague fascicles; hyalinization and calcifications can occasionally be seen and the cells are positive for cytokeratin and epithelial membrane antigen (EMA), which distinguish this tumor from CFT.

- musculoskeletal system

  • leiomyoma
    • Leiomyoma has undergone degenerative changes, has calcifications and ossifications, and its cells are positive or smooth muscle actin (SMA), desmin, caldesmon, and XIIIa factor.
  • desmoid fibromatosis
    • Desmoid fibromatosis is a locally aggressive fibrogenic neoplasm, characterized by lack of psammoma bodies and by the positive expression of β-catanin.
  • calcifying aponeurotic fibroma
    • Calcifying aponeurotic fibroma belongs to the same category of tumors as CFT, but occurs typically in hand and feet, it is less circumscribed, and has metaplastic cartilage and multinuclear giant cells.

Open references

- Calcifying Fibrous Tumor. 2016. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4902420/

Paywall References

- Calcifying fibrous tumor of the stomach: clinicopathologic and molecular study of seven cases with literature review and reappraisal of histogenesis. Agaimy A, Bihl MP, Tornillo L, Wünsch PH, Hartmann A, Michal M. Am J Surg Pathol. 2010 Feb;34(2):271-8. PMID: 20090503

- The evolving classification of soft tissue tumours: an update based on the new WHO classification. Fletcher CD. Histopathology. 2006 Jan;48(1):3-12. PMID: 16359532