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palmar fibromatosis

Wednesday 8 September 2004

Definition: Dupuytren’s disease is a superficial fibromatosis of the hand. It is a nodular, cytologically bland fibroproliferative process that involves the palmar aponeuroses.

Tumor histology was characteristically bland with dense collagen deposition and little or no mitotic activity in the absence of a clinical history suggestive of wounding or scarring. Pathologic examination revealed identical histology on both the palmar and the dorsal aspects of the hand.

Epidemiology

- Peak age 60-70 years
- Rare under 20
- May be bilateral
- Predominantly seen in patients of northwest European origin
- In some populations may affect as many as 20% of males and 4% of females
- Familial predisposition
- Familial form: Generally appears to be autosomal dominant
- May be associated with other fibromatoses

  • Plantar fibromatosis in 10-20% of cases
  • Penile fibromatosis in 1% of cases

Clinical synopsis

- May cause contractures
- With or without pain
- Knuckle pads

Clinical associations

- Epilepsy
- Diabetes
- Alcohol abuse and cirrhosis
- Smoking
- Manual labor

Localization

- Most cases palmar
- Rare cases involve dorsum of hand or wrist
- Multinodular
- Most often involves palm
- May rarely involve dorsum of hand or wrist
- Involves aponeurosis and rarely overlying dermis
- No infiltration of deeper tissues

Synopsis

- Grossly forms multiple nodules 1 cm or less
- Infrequently forms a single dominant nodule
- Cellularity ranges from low to high
- Older lesions are densely collagenous
- Bland spindle cells
- Nuclei small and dark to moderate sized and vesicular
- Scant cytoplasm
- Mitotic figures may be frequent but not atypical
- Small foci of perivascular hemorrhage and hemosiderin may be present
- Metaplastic cartilage and bone are seen rarely
- Necrosis very rare

Immunochemistry

- Actin: Variable positivity
- Desmin: Variable positivity
- S-100: negative
- Beta-catenin: 50% positive

Differential diagnosis

- calcifying aponeurotic fibroma
- extra-abdominal desmoid fibromatosis
- adult-type fibrosarcoma
- synovial sarcoma
- epithelioid sarcoma
- inclusion body fibromatosis (infantile fibromatosis)

Prognosis

- Never metastasizes
- May regress with age
- Recurrences do occur but are not destructive

CGH

- No gene copy number changes in Dupuytren’s contracture by array comparative genomic hybridization (18474290).

Credits

- Stanford Medical School

See also

- fibromatoses (fibromatosis)

Book sections

- Kempson RL, Fletcher CDM, Evans HL, Henrickson MR, Sibley RS. Tumors of the Soft Tissues, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 30, 2001

- Fletcher CDM, Unni KK, Mertens F. Pathology and Genetics of Tumours of Soft Tissue and Bone, World Health Organization Classification of Tumours 2002

- Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft Tissue Tumors, 4th edition, 2001

References

- Kaur S, Forsman M, Ryh√§nen J, Knuutila S, Larramendy ML. No gene copy number changes in Dupuytren’s contracture by array comparative genomic hybridization. Cancer Genet Cytogenet. 2008 May;183(1):6-8. PMID: 18474290

- Gudmundsson KG, Arngrimsson R, Sigfusson N, Bjornsson A, Jonsson T. Epidemiology of Dupuytren’s disease: clinical, serological, and social assessment. The Reykjavik Study. J Clin Epidemiol 2000 Mar 1;53(3):291-6

- Ushijima M, Tsuneyoshi M, Enjoji M. Dupuytren type fibromatoses. A clinicopathologic study of 62 cases. Acta Pathol Jpn 1984 Sep;34(5):991-1001

- Iwasaki H, Muller H, Stutte HJ, Brennscheidt U. Palmar fibromatosis (Dupuytren’s contracture). Ultrastructural and enzyme histochemical studies of 43 cases. Virchows Arch A Pathol Anat Histopathol 1984;405(1):41-53

- Montgomery E, Lee JH, Abraham SC, Wu TT. Superficial fibromatoses are genetically distinct from deep fibromatoses. Mod Pathol 2001 Jul;14(7):695-701

- Mikkelsen OA. Dupuytren’s disease—initial symptoms, age of onset and spontaneous course. Hand 1977 Feb;9(1):11-5

- Allen PW. The fibromatoses: a clinicopathologic classification based on 140 cases. Am J Surg Pathol. 1977 Sep;1(3):255-70.