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Soft tissue Tumors

Fibroblastic / myofibroblastic tumors

Calcifying fibrous tumor


Reviewer: Komal Arora, M.D. (see Reviewers page)
Revised: 5 September 2012, last major update July 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Benign fibrous lesion with abundant hyalinized collagen, psammomatous or dystrophic calcifications and lymphocytic infiltration
● Also called calcifying fibrous pseudotumor
● First described in 1993 (Am J Surg Pathol 1993;17:502)

Clinical description
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● Adolescents/young adults, all sites
● Not related to inflammatory myofibroblastic tumor (Mod Pathol 2001;14:784, Int J Surg Pathol 2002;10:189)

Treatment
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● Excision, rarely recurs

Case reports
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● 24 year old woman with neck mass (Arch Pathol Lab Med 2000;124:435)
● 32 year old woman with omental tumor (Case of Week #249)
● Tumors of small intestine (Ann Diagn Pathol 2008;12:138)
● Multiple peritoneal tumors (Ann Diagn Pathol 2007;11:460, Am J Clin Pathol 2003;119:811)
● Multiple pleural tumors (Virchows Arch 2005;446:78, J Thorac Oncol 2008;3:1356)
● Mesentry tumors (J Cancer Res Ther 2011;7:500, Int J Surg Pathol 2001;9:249)
● Stomach tumor (Am J Surg Pathol 2010;34:271)

Gross description
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● Well circumscribed but unencapsulated, variable size, may infiltrate into surrounding tissue, may have gritty cut surface

Gross images
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Gray-white tumor with whorled cut surface


Well circumscribed tumor (adrenal gland)

Micro description
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● Paucicellular fibroblastic proliferation with lymphocytes (possibly lymphoid follicles), plasma cells, eosinophils, mast cells, dense collagenous tissue, varying degrees of calcification
● Border is often at least partially infiltrative

Micro images
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Psammoma body and adjacent lymphoplasmacytic infiltrate; hypocellular stroma with psammomatous calcifications


Adrenal gland tumor has paucicellular fibrous proliferation with focal microcalcifications


Microcalcifications vary in size


Lymphoplasmacytic inflammation is characteristic


Figures A, B, E, F (comparison with inflammatory myofibroblastic tumor)


Hyalinized collagen with psammoma body

           
Omental tumor


Various images (adrenal gland)

Positive stains
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● Factor XIIIa, vimentin
● Also CD34

Negative stains
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● Smooth muscle actin, muscle specific actin, ALK, desmin, S100, keratin

Electron microscopy description
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● Immature fibroblastic cells, collagen fibrils, dystrophic and psammomatous calcifications

Electron microscopy images
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Spindle cells with intermediate filaments and collagen fibrils showing the fibroblastic nature of the cells

Differential diagnosis
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● Inflammatory myofibroblastic tumor: more cellular, no calcifications, ALK+, actin+, CD34+ and focally Factor XIIIa+ (Mod Pathol 2001;14:784, Int J Surg Pathol 2002;10:189)
● Idiopathic retroperitoneal fibrosis and related sclerosing fibroinflammatory lesions: more inflammation, especially plasma cells and eosinophils
● Desmoplastic fibroblastoma: older patients, low cellularity, larger prominent fibroblasts, no microcalcifications, no prominent inflammatory infiltrate
● Calcifying aponeurotic fibroma: more cellular, usually distal location, usually smaller lesion

Additional references
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Stanford University

End of Soft Tissue Tumors > Fibroblastic / myofibroblastic tumors > Calcifying fibrous tumor


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