Fibromatoses

Fibromatoses are a group of benign stromal lesions that may be seen in pediatric pathology and may or may not be associated with syndromes.

Syndromes associated with fibromatoses

Familial adenomatous polyposis.^[1]
- Gardner syndrome.
  - Desmoid-type fibromatosis.
Hereditary desmoid syndrome.

Types

Superficial

Palmar (Dupuytren's contracture).
Plantar.
Penile (Peyronie's disease).

Deep

Desmoid-type fibromatosis.
Others.

Gross

Firm.
Infiltrative borders.

Image:

Microscopic

Features:

Bland spindle cells - typically in fascicles.
- Pale eosinophilic cytoplasm.^[2]
- Nucleus may be ovoid.^[3]
Small thin blood vessels that are parallel to one another.
- Nuclei of blood vessels are typically darker staining than those of the lesion.

DDx:

Metaplastic carcinoma, e.g. metaplastic breast carcinoma.
Nodular fasciitis.
Dermatofibroma.
GIST.
Desmoplastic fibroblastoma - esp. shoulder region.

Images:

IHC

Beta-catenin +ve -- nuclear stain.
CD117 -ve.

Image:

Fibromatosis (wikia.com).

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SOFT TISSUE LESION, LEFT SHOULDER, CORE BIOPSY:
- FIBROMATOSIS, SEE COMMENT.

COMMENT:
The lesion was evaluated with immunostains:
Positive: vimentin, SMA (rare), beta-catenin.
Negative: pankeratin, S-100, desmin, CD34 (stains vessels).

Micro

The sections show a lesion with bland spindle cells. There is no apparent nuclear atypia. Mitotic figures are not identified. No myxoid areas are apparent. No necrosis is identified.

References

↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1092. ISBN 978-1416031215.
↑ URL: http://www.histopathology-india.net/Fibromatosis.htm. Accessed on: 18 September 2012.
↑ ^3.0 ^3.1 ^3.2 Murphey, MD.; Ruble, CM.; Tyszko, SM.; Zbojniewicz, AM.; Potter, BK.; Miettinen, M. (Nov 2009). "From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation.". Radiographics 29 (7): 2143-73. doi:10.1148/rg.297095138. PMID 19926768.

External links

Fibromatoses (histopathology-india.net).

[Ref_PBoD8_1092-1] Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1092. ISBN 978-1416031215.

[2] URL: http://www.histopathology-india.net/Fibromatosis.htm. Accessed on: 18 September 2012.

[pmid19926768-3] 3.0 ^3.1 ^3.2 Murphey, MD.; Ruble, CM.; Tyszko, SM.; Zbojniewicz, AM.; Potter, BK.; Miettinen, M. (Nov 2009). "From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation.". Radiographics 29 (7): 2143-73. doi:10.1148/rg.297095138. PMID 19926768.

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