Palmar fibromatosis

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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:[1]

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

Gross

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

Image

Microscopic

Features:[2][3]

  • 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:[3]

Images

www

IHC

  • Beta-catenin +ve (cytoplasmic & nuclear).[5]

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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, Fasciectomy:
- Palmar fascia within normal limits and benign fibroadipose tissue - in an
  individual with a clinical history of 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

References

  1. URL: http://www.humpath.com/palmar-fibromatosis. Accessed on: 6 January 2011.
  2. URL: http://surgpathcriteria.stanford.edu/softfib/plantar_fibromatosis/printable.html. Accessed on: 6 January 2011.
  3. 3.0 3.1 URL: http://surgpathcriteria.stanford.edu/softfib/palmar_fibromatosis/differentialdiagnosis.html. Accessed on: 9 November 2012.
  4. Wang L, Zhu H (2006). "Clonal analysis of palmar fibromatosis: a study whether palmar fibromatosis is a real tumor". J Transl Med 4: 21. doi:10.1186/1479-5876-4-21. PMC 1488873. PMID 16696857. http://www.biomedsearch.com/nih/Clonal-analysis-palmar-fibromatosis-study/16696857.html.
  5. Degreef, I.; De Smet, L.; Sciot, R.; Cassiman, JJ.; Tejpar, S. (Mar 2009). "Beta-catenin overexpression in Dupuytren's disease is unrelated to disease recurrence.". Clin Orthop Relat Res 467 (3): 838-45. doi:10.1007/s11999-008-0590-z. PMID 18958538.