Palmar fibromatosis

Palmar fibromatosis
Diagnosis in short
Palmar fibromatosis. H&E stain.
LM	poorly demarcated, usu. multiple lesions/multiple nodules, composed of bland spindle cells in dense collagen; spindle cells have pale grey cytoplasm and a pale ovoid nucleus with small round nucleolus, +/-giant cells (rare)
LM DDx	calcifying aponeurotic fibroma, extra-abdominal desmoid fibromatosis, inclusion body fibromatosis, fibrosarcoma, synovial sarcoma, epithelioid sarcoma, metastatic metaplastic carcinoma
IHC	beta-catenin +ve
Site	hand - palmar fascia, classically ring finger
Clinical history	male > female, +/-family history
Prognosis	benign

Palmar fibromatosis, also Dupuytren's contracture and Dupuytren disease, is a common pathology of the hand.

The bigger topic of fibromatoses is covered in the article fibromatosis.

Palmar fascia directs to this article.

General

Clinical:

• Usually older - 60s or 70s (years old).
• Male > female.
• Associated with:
  • Alcohol abuse.
• May be familial.

Gross

• Ring finger - classic location.^{[citation needed]}
• Nodular.
• Poorly demarcated.

Image

• 

  Palmar fibromatosis. (WC)

Microscopic

Features:

• Poorly demarcated, usu. multiple lesions/multiple nodules.
• Composed of bland spindle cells in dense collagen.
  • Pale grey cytoplasm (moderate quantity).
  • Pale ovoid nuclei with small round nucleoli.
• Giant cells - rare.

Note:

• No nuclear atypia.
• Mitotic figures - rarely present, none atypical.
• May see Pacinian corpuscle (AKA lamellar corpuscle) as an incidental finding.

DDx:

• Calcifying aponeurotic fibroma - calcification, chondroid differentiation, usu. < 20 years old.
• Extra-abdominal desmoid fibromatosis - extremely rare on hands and feet, muscle infiltrative.
• Inclusion body fibromatosis.
• Fibrosarcoma - atypia, necrosis, herring bone pattern.
• Synovial sarcoma - one mass.
• Epithelioid sarcoma - necrosis, atypia.
• Metastatic metaplastic carcinoma, e.g. metaplastic breast carcinoma - extremely uncommon.

Images

• 

  PF - intermed. mag.

• 

  PF - high mag.

• 

  PF - high mag.

• 

  PF - very high mag.

• 

  Pacinian corpuscle. (WC)

• 

  Tendon without fibromatosis - intermed. mag. (WC)

www

• Palmer fibromatosis (biomedsearch.com).
• Palmar fibromatosis (unibas.ch).

IHC

• Beta-catenin +ve (cytoplasmic & nuclear).

Sign out

Left

Palmar Fascia, Left Ring Finger, Fasciectomy:
- Palmar fibromatosis.

Palmar Fascia, Left Long Finger, Fasciectomy:
- Palmar fibromatosis (Dupuytren's contracture).

Block letters

PALMAR FASCIA, LEFT, FASCIECTOMY:
- PALMAR FIBROMATOSIS.

Right

PALMAR FASCIA, RIGHT, FASCIECTOMY:
- PALMAR FIBROMATOSIS.

Micro

The sections show multiple poorly-demarcated nodules composed of bland spindle cells with moderate pale grey cytoplasm and pale ellipsoid nuclei with small round nucleoli. The poorly-demarcated nodules are completely surrounded by dense collagen. Calcification is not present. There is no nuclear atypia or necrosis. Mitotic activity is not identified. Benign fibroadipose tissue is present.

Missed fibromatosis

Palmar Fascia, Left Hand, Fasciectomy: 
     - Palmar fascia within normal limits and benign fibroadipose tissue - in an
       individual with a preoperative diagnosis of palmar fibromatosis.
     - NEGATIVE for malignancy.

Comment:
A beta-catenin is NEGATIVE. Deepers were cut (x6); they are noncontributory.

Alternate

Palmar Fascia, Left, Fasciectomy:
- Palmar fascia within normal limits and benign fibroadipose tissue - in an
  individual with a clinical history of palmar fibromatosis.
- NEGATIVE for malignancy.

History not provided

Palmar Fascia, Left, Fasciectomy:
- Palmar fascia within normal limits and benign fibroadipose tissue, in an
  individual clinically described as having palmar fibromatosis.
- NEGATIVE for malignancy.

Block letters

PALMAR FASCIA, LEFT, FASCIECTOMY:
- PALMAR FASCIA WITHIN NORMAL LIMITS AND BENIGN FIBROADIPOSE TISSUE -- IN AN
  INDIVIDUAL WITH A CLINICAL HISTORY OF PALMAR FIBROMATOSIS.
- NEGATIVE FOR MALIGNANCY.

Micro

The sections show dense collagen (tendon) without an apparent pathology. Calcification is not present. There is no nuclear atypia or necrosis. Mitotic activity is not identified. Benign fibroadipose tissue is present. Lamellar corpuscles are present. The tissue was submitted in toto and levels were cut.

Straight

PALMAR FASCIA, RIGHT, FASCIECTOMY:
- PALMAR FASCIA WITHIN NORMAL LIMITS.
- NEGATIVE FOR FIBROMATOSIS.

COMMENT:
The tissue was submitted in toto and levels were cut.

Micro

The sections show dense collagen (tendon) without an apparent pathology. Calcification is not present. There is no nuclear atypia or necrosis. Mitotic activity is not identified. Benign fibroadipose tissue is present. Lamellar corpuscles are present.

See also

• Non-malignant skin disease.
• Fibromatosis.