Extra-mammary cases are more common in men and have a predilection for the groin but have also been reported at other sites (posterior vaginal wall, buttock, anterior abdominal wall and mid-back).
These tumours are generally well circumscribed but not encapsulated.
|
mammary cases |
extra-mammary cases |
usually positive |
9/9 |
|
usually positive |
8/9 |
|
some cases positive |
3/9, focal positivity |
|
variably positive (both sexes) |
|
|
variably positive (both sexes) |
|
|
|
1/9 (in a case with epithelioid foci) |
|
cytokeratin |
|
0/9 |
|
shared features |
differentiating features |
Positive for CD34 SCL and myofibroblastoma are probably related entities with shared cytogenetic abnormalities (partial deletions of 13q and of 16q) |
Restricted to shoulder/head/neck. Spindle cells are more delicate and are more haphazardly arranged. Usually more fat. Lack thick, keloid-like bands of collagen. |
|
cellular angiofibroma (angiomyofibroblastoma-like tumour) (occurs in the groin) |
Scattered intralesional adipocytes common. CD34 may be positive. |
This lesion is distinguished by striking hyalinised vessels. |
(Mainly affects females) |
May have intralesional adipocytes. |
Prominent small blood vessels with small rounded, usually perivascular, cells which are desmin-positive. |
CD34 may be focally positive |
The myofibroblastic cells are plumper. There is usually focal microcystic myxoid change and red cell extravasation. Usually diffusely positive for SMA. Desmin is usually negative. |
|
Fat is rarely present. CD34 may be positive |
Long spindle cells show a storiform pattern. There is positivity for EMA and negativity for desmin. |
|
spindle cell well-differentiated liposarcoma |
CD34 is likely to be positive. |
Less well circumscribed. There is nuclear atypia and lipoblasts are present. S-100 are likely to be positive. Desmin is negative except in a few dendritic cells. |
CD34 is positive. |
Prominent haemangiopericytomatous vessels are present. Desmin is usually negative. |
|
lipomatous haemangiopericytoma (this may be a variant of solitary fibrous tumour) |
Admixed fatty component. CD34 is positive. |
Prominent haemangiopericytomatous vessels are present. Desmin is usually negative. |
low-grade MPNST: |
|
Desmin is negative. In 50% of cases, S-100 is positive.
|
Positive for CD34 |
More superficial and more infiltrative. Negative for desmin. |
Management
This page last revised 26.12.2001.