Difference between revisions of "Atypical fibroxanthoma"

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#redirect [[Dermatologic neoplasms#Atypical_fibroxanthoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = SkinTumors-P9280874.jpg
| Width      =
| Caption    = Atypical fibroxanthoma. [[H&E stain]].
| Synonyms  =
| Micro      = dermal lesion with marked nuclear atypia, mitoses, mulitnucleated cells, cell have foamy cytoplasm
| Subtypes  =
| LMDDx      = [[malignant melanoma]], [[pleomorphic undifferentiated sarcoma]], [[leiomyosarcoma]], sarcomatoid [[squamous carcinoma]]
| Stains    =
| IHC        = S-100 -ve, CK34betaE12 -ve, p63 -ve, desmin -ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[skin]] - see [[skin tumours]], usu. head & neck
| Assdx      =
| Syndromes  =
| Clinicalhx = rapid growth, elderly
| Signs      =
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = good
| Other      =
| ClinDDx    = malignant [[skin tumours]]
| Tx        = complete excision
}}
'''Atypical fibroxanthoma''', abbreviated '''AFX''', is poorly differentiated [[skin tumour]] with likeness to [[undifferentiated pleomorphic sarcoma]].
 
==General==
*Typically head & neck region.<ref>URL: [http://emedicine.medscape.com/article/1056204-overview http://emedicine.medscape.com/article/1056204-overview]. Accessed on 2 September 2011.</ref>
*Thought to be related to [[pleomorphic undifferentiated sarcoma]];<ref name=pmid21664889>{{Cite journal  | last1 = Withers | first1 = AH. | last2 = Brougham | first2 = ND. | last3 = Barber | first3 = RM. | last4 = Tan | first4 = ST. | title = Atypical fibroxanthoma and malignant fibrous histiocytoma. | journal = J Plast Reconstr Aesthet Surg | volume =  | issue =  | pages =  | month = Jun | year = 2011 | doi = 10.1016/j.bjps.2011.05.004 | PMID = 21664889 }}</ref><ref name=pmid23319144>{{Cite journal  | last1 = Tchernev | first1 = G. | last2 = Tronnier | first2 = M. | last3 = Ananiev | first3 = J. | last4 = Taneva | first4 = T. | last5 = Patterson | first5 = JW. | last6 = Gulubova | first6 = M. | last7 = Trafeli | first7 = JP. | last8 = Gegova | first8 = A. | last9 = Harrell | first9 = M. | title = Atypical fibroxanthoma-a diagnosis of exclusion! | journal = Wien Med Wochenschr | volume = 163 | issue = 15-16 | pages = 380-386 | month = Aug | year = 2013 | doi = 10.1007/s10354-012-0173-1 | PMID = 23319144 }}</ref> some say it is the same thing.<ref name=danny>Ghazarian, Danny; 16 September 2011.</ref>
*Usually benign.
**May metastasize - case report-type of occurrence.<ref>{{Cite journal  | last1 = New | first1 = D. | last2 = Bahrami | first2 = S. | last3 = Malone | first3 = J. | last4 = Callen | first4 = JP. | title = Atypical fibroxanthoma with regional lymph node metastasis: report of a case and review of the literature. | journal = Arch Dermatol | volume = 146 | issue = 12 | pages = 1399-404 | month = Dec | year = 2010 | doi = 10.1001/archdermatol.2010.206 | PMID = 20713774 | URL = http://archderm.jamanetwork.com/article.aspx?articleid=422416 }}</ref>
 
Clinical:
*Rapid growth.
*Elderly.
*Good prognosis.<ref name=pmid20526171>{{Cite journal  | last1 = Beer | first1 = TW. | last2 = Drury | first2 = P. | last3 = Heenan | first3 = PJ. | title = Atypical fibroxanthoma: a histological and immunohistochemical review of 171 cases. | journal = Am J Dermatopathol | volume = 32 | issue = 6 | pages = 533-40 | month = Aug | year = 2010 | doi = 10.1097/DAD.0b013e3181c80b97 | PMID = 20526171 }}</ref>
 
==Microscopic==
Features:<ref name=Ref_Derm521>{{Ref Derm|521}}</ref>
*Dermal lesion - '''key point'''.
*Marked nuclear atypia.
*Mitoses.
*Mulitnucleated cells.
*Foamy cytoplasm - '''key feature'''.
 
DDx:
*[[Malignant melanoma]].
*[[Pleomorphic undifferentiated sarcoma]] (MFH) - deeper than the dermis.
*[[Leiomyosarcoma]].
*Sarcomatoid [[squamous carcinoma]].
 
Notes:
*No Grenz zone. (???)
 
===Images===
<gallery>
Image: SkinTumors-P9280873.jpg | AFX. (WC)
Image: SkinTumors-P9280874.jpg | AFX. (WC)
Image: SkinTumors-P9280875.jpg | AFX. (WC)
</gallery>
www:
*[http://dermatology.cdlib.org/141/case_reports/afx/1.jpg AFX (cdlib.org)].<ref name=pmid18319023>{{Cite journal  | last1 = Vandergriff | first1 = TW. | last2 = Reed | first2 = JA. | last3 = Orengo | first3 = IF. | title = An unusual presentation of atypical fibroxanthoma. | journal = Dermatol Online J | volume = 14 | issue = 1 | pages = 6 | month =  | year = 2008 | doi =  | PMID = 18319023 }}</ref>
 
A markedly atypical lesion of far less worrisome significance is the pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis).
[[File:DP27AP17 sl1.png| Pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis).]]
[[File:DP27AP17 sl2.png| Pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis).]]
[[File:DP27AP17 sl3.png| Pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis).]]
[[File:DP27AP17 sl4.png| Pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis).]]
[[File:DP27AP17 sl5.png| Pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis).]]
[[File:DP27AP17 sl6.png| Pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis).]]
<br>
Pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis). A. The key distinction is at low power, wherein cluters of thin-walled, indented (ectatic) blood vessels are seen. B.  The ectatic vessels have a thin endothelial lining and a thick subjacent rim of amorphous eosinophilia. Note the multinucleated cells in the stroma. C. Hyaline material extends beyond vessels to entrap stromal cells. D. Cellularity in part raises the possibility of a sarcoma. E. High power further raises the suspicion of malignancy, given the aberrant nuclei with prominent nucleoli. F. Reassurance occurs because 1) there are virtually no mitoses, 2) there is no necrosis, and 3) there are readily identifiable nuclear inclusions as seen in these tumor cells.
 
==IHC==
Features:<ref name=Ref_Derm521>{{Ref Derm|521}}</ref>
*S100 -ve (done to r/o melanoma).
*[[CK34betaE12]] -ve.
*[[p63]] -ve (done to exclude [[squamous cell carcinoma of the skin|SCC]]).
**Scant staining not considered +ve.
*Desmin -ve (done to r/o leiomyosarcoma).
*[[CD99]] +ve.<ref name=pmid11789717>{{Cite journal  | last1 = Monteagudo | first1 = C. | last2 = Calduch | first2 = L. | last3 = Navarro | first3 = S. | last4 = Joan-Figueroa | first4 = A. | last5 = Llombart-Bosch | first5 = A. | title = CD99 immunoreactivity in atypical fibroxanthoma: a common feature of diagnostic value. | journal = Am J Clin Pathol | volume = 117 | issue = 1 | pages = 126-31 | month = Jan | year = 2002 | doi = 10.1309/2EXB-70CW-3U6P-VQ6H | PMID = 11789717 }}</ref>
**Usually +ve in [[melanoma]]... but negative in [[squamous carcinoma]].
 
Others:
*Vimentin +ve.
*SMA +ve.{{fact}}
 
New ''et al.'' suggests:<ref name=pmid20713774>{{Cite journal  | last1 = New | first1 = D. | last2 = Bahrami | first2 = S. | last3 = Malone | first3 = J. | last4 = Callen | first4 = JP. | title = Atypical fibroxanthoma with regional lymph node metastasis: report of a case and review of the literature. | journal = Arch Dermatol | volume = 146 | issue = 12 | pages = 1399-404 | month = Dec | year = 2010 | doi = 10.1001/archdermatol.2010.206 | PMID = 20713774 }}</ref>
*Vimentin, SMA, CD68, cytokeratins, p63, desmin, CD99, CD10, S100, CD117, LN-2, procollagen I.
 
A panel:
*Vimentin, SMA, CD68, CK18, [[EMA]], [[CAM5.2]], CK34betaE12, [[CK5/6]], [[p63]], desmin, CD99, CD10, S100, CD34.
 
==Sign out==
===Incompletely excised===
<pre>
SKIN LESION, MID BACK, SHAVE BIOPSY:
- ATYPICAL SPINDLE CELL NEOPLASM, SEE MICRO AND COMMENT.
 
COMMENT:
The diagnosis of atypical fibroxanthoma (AFX) is favoured.  The main differential
diagnosis is pleomorphic undifferentiated sarcoma.
 
The extent of the lesion cannot be determined, as it is present at the deep margin.
 
This lesion should be re-excised, as it could represent an aggressive malignancy.
</pre>
 
==See also==
*[[Dermatologic neoplasms]].
*[[Undifferentiated pleomorphic sarcoma]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Dermatologic neoplasms]]

Latest revision as of 14:51, 4 August 2022

Atypical fibroxanthoma
Diagnosis in short

Atypical fibroxanthoma. H&E stain.
LM dermal lesion with marked nuclear atypia, mitoses, mulitnucleated cells, cell have foamy cytoplasm
LM DDx malignant melanoma, pleomorphic undifferentiated sarcoma, leiomyosarcoma, sarcomatoid squamous carcinoma
IHC S-100 -ve, CK34betaE12 -ve, p63 -ve, desmin -ve
Site skin - see skin tumours, usu. head & neck
Clinical history rapid growth, elderly
Prevalence uncommon
Prognosis good
Clin. DDx malignant skin tumours
Treatment complete excision

Atypical fibroxanthoma, abbreviated AFX, is poorly differentiated skin tumour with likeness to undifferentiated pleomorphic sarcoma.

General

Clinical:

  • Rapid growth.
  • Elderly.
  • Good prognosis.[6]

Microscopic

Features:[7]

  • Dermal lesion - key point.
  • Marked nuclear atypia.
  • Mitoses.
  • Mulitnucleated cells.
  • Foamy cytoplasm - key feature.

DDx:

Notes:

  • No Grenz zone. (???)

Images

  • AFX. (WC)

  • AFX. (WC)

  • AFX. (WC)

www:

A markedly atypical lesion of far less worrisome significance is the pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis). Pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis). Pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis). Pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis). Pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis). Pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis). Pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis).
Pleomorphic hyalinizing angioectatic tumor of soft parts (subcutis). A. The key distinction is at low power, wherein cluters of thin-walled, indented (ectatic) blood vessels are seen. B. The ectatic vessels have a thin endothelial lining and a thick subjacent rim of amorphous eosinophilia. Note the multinucleated cells in the stroma. C. Hyaline material extends beyond vessels to entrap stromal cells. D. Cellularity in part raises the possibility of a sarcoma. E. High power further raises the suspicion of malignancy, given the aberrant nuclei with prominent nucleoli. F. Reassurance occurs because 1) there are virtually no mitoses, 2) there is no necrosis, and 3) there are readily identifiable nuclear inclusions as seen in these tumor cells.

IHC

Features:[7]

Others:

New et al. suggests:[10]

  • Vimentin, SMA, CD68, cytokeratins, p63, desmin, CD99, CD10, S100, CD117, LN-2, procollagen I.

A panel:

  • Vimentin, SMA, CD68, CK18, EMA, CAM5.2, CK34betaE12, CK5/6, p63, desmin, CD99, CD10, S100, CD34.

Sign out

Incompletely excised

SKIN LESION, MID BACK, SHAVE BIOPSY:
- ATYPICAL SPINDLE CELL NEOPLASM, SEE MICRO AND COMMENT.

COMMENT:
The diagnosis of atypical fibroxanthoma (AFX) is favoured.  The main differential
diagnosis is pleomorphic undifferentiated sarcoma.

The extent of the lesion cannot be determined, as it is present at the deep margin.

This lesion should be re-excised, as it could represent an aggressive malignancy.

See also

References

  1. ↑ URL: http://emedicine.medscape.com/article/1056204-overview. Accessed on 2 September 2011.
  2. ↑ Withers, AH.; Brougham, ND.; Barber, RM.; Tan, ST. (Jun 2011). "Atypical fibroxanthoma and malignant fibrous histiocytoma.". J Plast Reconstr Aesthet Surg. doi:10.1016/j.bjps.2011.05.004. PMID 21664889.
  3. ↑ Tchernev, G.; Tronnier, M.; Ananiev, J.; Taneva, T.; Patterson, JW.; Gulubova, M.; Trafeli, JP.; Gegova, A. et al. (Aug 2013). "Atypical fibroxanthoma-a diagnosis of exclusion!". Wien Med Wochenschr 163 (15-16): 380-386. doi:10.1007/s10354-012-0173-1. PMID 23319144.
  4. ↑ Ghazarian, Danny; 16 September 2011.
  5. ↑ New, D.; Bahrami, S.; Malone, J.; Callen, JP. (Dec 2010). "Atypical fibroxanthoma with regional lymph node metastasis: report of a case and review of the literature.". Arch Dermatol 146 (12): 1399-404. doi:10.1001/archdermatol.2010.206. PMID 20713774.
  6. ↑ Beer, TW.; Drury, P.; Heenan, PJ. (Aug 2010). "Atypical fibroxanthoma: a histological and immunohistochemical review of 171 cases.". Am J Dermatopathol 32 (6): 533-40. doi:10.1097/DAD.0b013e3181c80b97. PMID 20526171.
  7. ↑ 7.0 7.1 Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 521. ISBN 978-0443066542.
  8. ↑ Vandergriff, TW.; Reed, JA.; Orengo, IF. (2008). "An unusual presentation of atypical fibroxanthoma.". Dermatol Online J 14 (1): 6. PMID 18319023.
  9. ↑ Monteagudo, C.; Calduch, L.; Navarro, S.; Joan-Figueroa, A.; Llombart-Bosch, A. (Jan 2002). "CD99 immunoreactivity in atypical fibroxanthoma: a common feature of diagnostic value.". Am J Clin Pathol 117 (1): 126-31. doi:10.1309/2EXB-70CW-3U6P-VQ6H. PMID 11789717.
  10. ↑ New, D.; Bahrami, S.; Malone, J.; Callen, JP. (Dec 2010). "Atypical fibroxanthoma with regional lymph node metastasis: report of a case and review of the literature.". Arch Dermatol 146 (12): 1399-404. doi:10.1001/archdermatol.2010.206. PMID 20713774.
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