Difference between revisions of "Atypical fibroxanthoma"

From Libre Pathology
Jump to navigation Jump to search
(split out)
Line 1: Line 1:
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      =
| Width      =
| Caption    =
| 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]].
'''Atypical fibroxanthoma''', abbreviated '''AFX''', is poorly differentiated [[skin tumour]] with likeness to [[undifferentiated pleomorphic sarcoma]].


Line 21: Line 52:


DDx:
DDx:
*[[Melanoma]].
*[[Malignant melanoma]].
*[[Pleomorphic undifferentiated sarcoma]] (MFH) - deeper than the dermis.
*[[Pleomorphic undifferentiated sarcoma]] (MFH) - deeper than the dermis.
*[[Leiomyosarcoma]].
*[[Leiomyosarcoma]].
Line 35: Line 66:
Features:<ref name=Ref_Derm521>{{Ref Derm|521}}</ref>
Features:<ref name=Ref_Derm521>{{Ref Derm|521}}</ref>
*S100 -ve (done to r/o melanoma).
*S100 -ve (done to r/o melanoma).
*34betaE12 -ve.
*CK34betaE12 -ve.
*p63 -ve (done to exclude SCC)  
*p63 -ve (done to exclude [[squamous cell carcinoma of the skin|SCC]]).
**Scant staining not considered +ve.
**Scant staining not considered +ve.
*Desmin -ve (done to r/o leiomyosarcoma).
*Desmin -ve (done to r/o leiomyosarcoma).

Revision as of 02:56, 22 December 2013

Atypical fibroxanthoma
Diagnosis in short

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. (???)

Image:

IHC

Features:[7]

  • S100 -ve (done to r/o melanoma).
  • CK34betaE12 -ve.
  • p63 -ve (done to exclude SCC).
    • Scant staining not considered +ve.
  • Desmin -ve (done to r/o leiomyosarcoma).

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.