Soft tissue
Fibroblastic / myofibroblastic
Calcifying fibrous tumor


Topic Completed: 9 April 2020

Minor changes: 9 April 2020

Copyright: 2002-2020, PathologyOutlines.com, Inc.

PubMed Search: Calcifying fibrous tumor[TI]

Smitha Mruthyunjayappa, M.D.
Deepti Dhall, M.D.
Page views in 2019: 4,596
Page views in 2020 to date: 3,621
Cite this page: Mruthyunjayappa S, Dhall D. Calcifying fibrous tumor. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissuecalcifyingfibrous.html. Accessed September 23rd, 2020.
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
Terminology
ICD coding
  • ICD-10: D21.9 - benign neoplasm of connective and other soft tissue, unspecified
Epidemiology
  • Adolescents / young adults
  • M:F = 1:1.27
Sites
  • Most common sites: tubular gastrointestinal tract, solid organs, peritoneal and pleural surfaces but can occur anywhere
Pathophysiology
  • 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)
Etiology
  • Previous infection, history of trauma and surgical intervention
  • No definitive mechanisms or causes have been confirmed
Diagrams / tables

Images hosted on other servers:

Sites

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

Images hosted on other servers:

CT scan

Gastrohepatic ligament tumor

Esophageal tumor

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

Images hosted on other servers:

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



Images hosted on other servers:

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 answer #1
A. Calcifying fibrous tumor

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