Stanford School of Medicine
Surgical Pathology Criteria
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Palmar Fibromatosis

Definition

  • A nodular, cytologically bland fibroproliferative process that involves the palmar aponeuroses

Alternate / Historical Names

  • Dupuytren contracture

Diagnostic Criteria

  • Most often involves palm
    • May rarely involve dorsum of hand or wrist
  • Involves aponeurosis and rarely overlying dermis
    • No infiltration of deeper tissues
  • 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

Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting: November 29, 2007

Supplemental studies

Immunohistology

Actin Variable positivity
Desmin Variable positivity
S100 negative
Beta-catenin 50% positive

Differential diagnosis

Palmar or Plantar Fibromatosis Calcifying Aponeurotic Fibroma
Rare under 20 Predominantly <20
Not calcified Calcified
Rarely chondroid Chondroid
No muscle or nerve infiltration May infiltrate muscle and nerve

 

Extra-abdominal Desmoid Fibromatosis Palmar and Plantar Fibromatosis
Rare on hands and feet Occurs on hands and feet
Primarily one large mass Multiple small nodules
Infiltrates muscle No muscle infiltration

 

Palmar or Plantar Fibromatosis Adult Fibrosarcoma
Occurs in hands Rare in hands
Multiple small nodules Single large mass
Involves fascia Deep to fascia
Haphazard pattern Herringbone pattern
Cytologically bland Cytologically atypical
No atypical mitotic figures Atypical mitotic figures may be seen
Rarely necrotic Frequent necrosis

 

Palmar or Plantar Fibromatosis Synovial Sarcoma
Rare under age 20 Frequently under age 20
Multiple small nodules Single large mass
Not biphasic May be biphasic
Variable cellularity Uniformly hypercellular
Lacks ropy collagen, calcification and stag horn vessels May have ropy collagen, calcification and stag horn vessels
No SYT-SSX fusion SYT-SSX fusion present

 

Palmar or Plantar Fibromatosis Epithelioid Sarcoma
Necrosis absent Necrosis common
No epithelioid cells Epithelioid cells present
Cytologicially bland Cytologically atypical
Keratin negative Keratin positive
CD34 negative CD34 50% positive

 

Palmar or Plantar Fibromatosis Inclusion Body Fibromatosis
Usually in palm or sole Usually on digits
Uncommon in children Vast majority very young
No inclusions Inclusions

Clinical

  • Peak age 60-70 years
    • Rare under 20
  • Most cases palmar
    • Rare cases involve dorsum of hand or wrist
  • Multinodular
  • May cause contractures
    • With or without pain
  • May be bilateral
  • May be associated with other fibromatoses
    • Plantar fibromatosis in 10-20% of cases
    • Penile fibromatosis in 1% of cases
    • Knuckle pads
  • Clinical associations
    • Epilepsy
    • Diabetes
    • Alcohol abuse and cirrhosis
    • Smoking
    • Manual labor
  • 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
    • Generally appears to be autosomal dominant
  • Recurrences do occur but are not destructive
    • Never metastasizes
    • May regress with age

Lists

Fibromatoses

Bibliography

  • 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
  • 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.
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