Soft tissue

Fibroblastic / myofibroblastic

Calcifying fibrous tumor

Editor-in-Chief: Debra L. Zynger, M.D.
Smitha Mruthyunjayappa, M.D.
Deepti Dhall, M.D.

Last author update: 9 April 2020
Last staff update: 9 April 2020

Copyright: 2002-2024,, Inc.

PubMed Search: Calcifying fibrous tumor[TI]

Smitha Mruthyunjayappa, M.D.
Deepti Dhall, M.D.
Page views in 2023: 9,429
Page views in 2024 to date: 2,756
Cite this page: Mruthyunjayappa S, Dhall D. Calcifying fibrous tumor. website. Accessed April 14th, 2024.
Definition / general
  • Benign fibrous lesion with abundant hyalinized collagen, psammomatous or dystrophic calcifications and lymphoplasmacytic infiltration
Essential features
  • Paucicellular
  • Bland spindle cells in a collagenous tissue
  • Calcifications and inflammatory infiltrate
ICD coding
  • ICD-10: D21.9 - benign neoplasm of connective and other soft tissue, unspecified
  • Adolescents / young adults
  • M:F = 1:1.27
  • Most common sites: tubular gastrointestinal tract, solid organs, peritoneal and pleural surfaces but can occur anywhere
  • Calcifying fibrous tumor may represent different stages of IgG4 related disease and fits with the unifying concept of IgG4 related pseudotumor
  • Recently, gastrointestinal calcifying fibrous tumor has been thought to be a gastrointestinal lesion of immunoglobulin 4 (IgG4) related disease (Surg Case Rep 2019;5:150)
  • Previous infection, history of trauma and surgical intervention
  • No definitive mechanisms or causes have been confirmed
Diagrams / tables

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Clinical features
  • Patients can present with various symptoms depending on the location of the tumor
  • Histologic examination of tissue
Radiology images

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CT scan

Gastrohepatic ligament tumor

Esophageal tumor

Prognostic factors
  • Local recurrence rate is ~10%
Case reports
  • Excision
Clinical images

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Endoscopy of gastric tumor

Endoscopy of ileum tumor

Gross description
  • Single or multiple
  • Well circumscribed but unencapsulated
  • Spherical to lobulated mass with a solid, white to gray, gritty cut surface
  • Variable size, may infiltrate into surrounding tissue (Biomed Res Int 2019;2019:5026860)
Gross images

Contributed by Mary Wong, M.D.

Ileum tumor

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Gastrohepatic ligament tumor

Ileum tumor

Microscopic (histologic) description
  • Paucicellular fibroblastic proliferation with bland spindle cells embedded in dense collagenous tissue
  • Varying degrees of lymphocytes (possibly lymphoid follicles), plasma cells
  • Scattered dystrophic or psammomatous calcification (Biomed Res Int 2019;2019:5026860)
Microscopic (histologic) images

Contributed by Deepti Dhall, M.D.

Bland spindle cells and inflammation

Bland spindle cells and calcification

Case #249

Omental tumor

Electron microscopy description
  • Immature fibroblastic cells, collagen fibrils, dystrophic and psammomatous calcifications
Sample pathology report
  • Ileum, resection:
    • Calcifying fibrous tumor, 3.5 cm in greatest dimension
    • Surgical margins are negative
Differential diagnosis
Board review style question #1
A 30 year old man presents with abdominal pain and a CT reveals a mass within the gastrointestinal system, which is resected. Histopathologic examination reveals paucicellular spindle cells with dystrophic calcifications and lymphoid aggregates. Immunohistochemical stains for CD34 and vimentin are positive; CD117, DOG1, SMA and ALK1 are negative. Which of the following is the diagnosis for this patient?

  1. Calcifying fibrous tumor
  2. Desmoplastic fibroblastoma
  3. Gastrointestinal stromal tumor
  4. Inflammatory myofibroblastic tumor
Board review style answer #1
A. Calcifying fibrous tumor

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