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.
  • 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:

• Desmoid tumour (flickr.com).
• Mesenteric fibromatosis (flickr.com).

Microscopic

Features:

• Bland spindle cells - typically in fascicles.
• 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.

Image:

• Plantar fibromatosis (sarcomaimages.com).
• Mesenteric fibromatosis (flickr.com).

IHC

• Beta-catenin +ve.^{[citation needed]}
• CD117 -ve.

See also

• Soft tissue lesions.
• Pediatric pathology.

External links

• Fibromatoses (histopathology-india.net).