Difference between revisions of "Palmar fibromatosis"

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#redirect [[Non-malignant_skin_disease#Palmar_fibromatosis]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Palmar fibromatosis -- high mag.jpg
| Width      =
| Caption    = Palmar fibromatosis. [[H&E stain]].
| Micro      = 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)
| Subtypes  =
| LMDDx      = [[calcifying aponeurotic fibroma]], extra-abdominal desmoid [[fibromatosis]], inclusion body [[fibromatosis]], [[fibrosarcoma]], [[synovial sarcoma]], [[epithelioid sarcoma]], [[metastasis|metastatic]] [[metaplastic carcinoma]]
| Stains    =
| IHC        = beta-catenin +ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = hand - palmar fascia, classically ring finger
| Assdx      =
| Syndromes  =
| Clinicalhx = male > female, +/-family history
| Signs      =
| Symptoms  =
| Prevalence =
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = benign
| Other      =
| ClinDDx    =
}}
'''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:<ref>URL: [http://www.humpath.com/palmar-fibromatosis http://www.humpath.com/palmar-fibromatosis]. Accessed on: 6 January 2011.</ref>
*Usually older - 60s or 70s (years old).
*Male > female.
*Associated with:
**[[Alcohol abuse]].
*May be familial.
 
==Gross==
*Ring finger - classic location.{{fact}}
*Nodular.
*Poorly demarcated.
 
===Image===
<gallery>
Image:Morbus dupuytren fcm.jpg | Palmar fibromatosis. (WC)
</gallery>
 
==Microscopic==
Features:<ref>URL: [http://surgpathcriteria.stanford.edu/softfib/plantar_fibromatosis/printable.html http://surgpathcriteria.stanford.edu/softfib/plantar_fibromatosis/printable.html]. Accessed on: 6 January 2011.</ref><ref name=standford_pf_ddx>URL: [http://surgpathcriteria.stanford.edu/softfib/palmar_fibromatosis/differentialdiagnosis.html http://surgpathcriteria.stanford.edu/softfib/palmar_fibromatosis/differentialdiagnosis.html]. Accessed on: 9 November 2012.</ref>
*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:<ref name=standford_pf_ddx>URL: [http://surgpathcriteria.stanford.edu/softfib/palmar_fibromatosis/differentialdiagnosis.html http://surgpathcriteria.stanford.edu/softfib/palmar_fibromatosis/differentialdiagnosis.html]. Accessed on: 9 November 2012.</ref>
*[[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.
*[[metastasis|Metastatic]] metaplastic carcinoma, e.g. [[metaplastic breast carcinoma]] - extremely uncommon.
 
===Images===
<gallery>
Image: Palmar fibromatosis -- intermed mag.jpg | PF - intermed. mag.
Image: Palmar fibromatosis -- high mag.jpg | PF - high mag.
Image: Palmar fibromatosis - alt -- high mag.jpg | PF - high mag.
Image: Palmar fibromatosis -- very high mag.jpg | PF - very high mag.
</gallery>
<gallery>
Image:WVSOM_Pacinian_Corpuscle.JPG | Pacinian corpuscle. (WC)
Image:Tendon_-_intermed_mag.jpg | Tendon without fibromatosis - intermed. mag. (WC)
</gallery>
====www====
*[http://www.biomedsearch.com/attachments/display/00/16/69/68/16696857/1479-5876-4-21-2.jpg Palmer fibromatosis (biomedsearch.com)].<ref name=pmid16696857>{{cite journal |author=Wang L, Zhu H |title=Clonal analysis of palmar fibromatosis: a study whether palmar fibromatosis is a real tumor |journal=J Transl Med |volume=4 |issue= |pages=21 |year=2006 |pmid=16696857 |pmc=1488873 |doi=10.1186/1479-5876-4-21 |url=http://www.biomedsearch.com/nih/Clonal-analysis-palmar-fibromatosis-study/16696857.html}}</ref>
*[http://alf3.urz.unibas.ch/pathopic/e/getpic-fra.cfm?id=8235 Palmar fibromatosis (unibas.ch)].
 
==IHC==
*Beta-catenin +ve (cytoplasmic & nuclear).<ref name=pmid18958538>{{Cite journal  | last1 = Degreef | first1 = I. | last2 = De Smet | first2 = L. | last3 = Sciot | first3 = R. | last4 = Cassiman | first4 = JJ. | last5 = Tejpar | first5 = S. | title = Beta-catenin overexpression in Dupuytren's disease is unrelated to disease recurrence. | journal = Clin Orthop Relat Res | volume = 467 | issue = 3 | pages = 838-45 | month = Mar | year = 2009 | doi = 10.1007/s11999-008-0590-z | PMID = 18958538 }}</ref>
 
==Sign out==
===Left===
<pre>
Palmar Fascia, Left Ring Finger, Fasciectomy:
- Palmar fibromatosis.
</pre>
 
<pre>
Palmar Fascia, Left Long Finger, Fasciectomy:
- Palmar fibromatosis (Dupuytren's contracture).
</pre>
 
====Block letters====
<pre>
PALMAR FASCIA, LEFT, FASCIECTOMY:
- PALMAR FIBROMATOSIS.
</pre>
 
===Right===
<pre>
PALMAR FASCIA, RIGHT, FASCIECTOMY:
- PALMAR FIBROMATOSIS.
</pre>
 
====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===
<pre>
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.
</pre>
 
=====Alternate=====
<pre>
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.
</pre>
 
=====History not provided=====
<pre>
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.
</pre>
====Block letters====
<pre>
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.
</pre>
 
=====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====
<pre>
PALMAR FASCIA, RIGHT, FASCIECTOMY:
- PALMAR FASCIA WITHIN NORMAL LIMITS.
- NEGATIVE FOR FIBROMATOSIS.
 
COMMENT:
The tissue was submitted in toto and levels were cut.
</pre>
 
=====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]].
 
==References==
{{Reflist|2}}
 
[[Category:Diagnosis]]
[[Category:Non-malignant skin disease]]

Latest revision as of 22:02, 9 November 2022

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]

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

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.