Sclerosing epithelioid fibrosarcoma: a study of five cases emphasizing diagnostic criteria.
OBJECTIVE
To study the clinical and histopathological features of sclerosing epithelioid fibrosarcoma, and to define diagnostic criteria for this uncommon soft-tissue tumour.


RESULTS
Standard histological, immunohistochemical and ultrastructural techniques were applied to five tumours from head and neck, chest wall and groin. Tumours consisted of groups of monomorphic rounded/epithelioid cells surrounded by a prominent collagenous stroma. Tumour cells showed positive vimentin staining but were negative for other markers. They contained prominent rough endoplasmic reticulum and a large Golgi apparatus which in one case was producing collagen secretion granules, an ultrastructural marker for collagen production. Three patients had medium to long-term survival (3-7 years). Of these, one was disease-free for 3 years, and two experienced multiple recurrences: one of the latter died of metastatic disease.


CONCLUSIONS
Criteria for diagnosing this uncommon tumour include: small to medium cell size, clear or pale cytoplasm, cellular arrangement in cords and strands, dense collagenous stroma; vimentin staining; rough endoplasmic reticulum and a Golgi apparatus producing, in well preserved examples, collagen secretion granules. The paper emphasizes the value of electron microscopy, supporting an appropriate histological picture and immunophenotype, in identifying these relatively low-grade sarcomas.
Eyden
B P
BP
Christie Hospital NHS Trust, Manchester, UK.
Manson
C
C
Banerjee
S S
SS
Roberts
I S
IS
Harris
M
M
D000328:Adult; D000368:Aged; D015622:Epithelioid Cells / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure; D005260:Female; D005354:Fibrosarcoma / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure; D006651:Histocytochemistry; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D012155:Reticulin / Q000032:analysis; D012598:Sclerosis; D012983:Soft Tissue Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure
D012155:Reticulin