Summary
We present a case series of sclerosing epithelioid fibrosarcoma (SEF) to further characterise
its clinical and pathological features.
Twenty-one patients with SEF were included in this study. There were 12 males and
nine females (range 25–63 years; median 38 years). Tumours were located in the kidney
(n=5), thigh (n=3), chest wall (n=3), head and neck (n=2), bone (n=2), abdominal wall (n=1), psoas major (n=1), retroperitoneum (n=1), omentum (n=1), popliteal space (n=1) and lung (n=1). Tumour sizes ranged from 2.5 to 16 cm (median 7 cm). Microscopically, epithelioid
tumour cells were arranged in nests and cords and embedded in a dense sclerotic stroma.
Some tumours showed myxoid areas, fibroma-like areas, acinar growth patterns and haemangiopericytoma-like
appearance. A few tumour cells presented a rhabdomyoid shape. Calcification, ossification,
cystic and necrosis were observed in some cases. The diagnosis was confirmed by immunoreactivity
for MUC4, and by further fluorescence in situ hybridisation (FISH) or next generation sequencing (NGS) analysis. Clinical follow-up
was available for 16 cases (median, 24 months; range 6–62 months). Seven patients
developed metastases to lung (n=3), bone (n=3), brain (n=2) and back (n=1). Four patients developed a local recurrence. Three patients died of disease. Overall
survival (OS) of SEF was related to patient age (p=0.001) and progression-free survival (PFS) was related to tumour size (p=0.046).
In addition to soft tissue, SEF is more likely to involve the viscera and the abdominal
cavity and has morphological variants. Familiarity with its distinctive clinical and
pathological features helps avoid misdiagnosis.
Key words
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Article info
Publication history
Published online: January 22, 2023
Accepted:
October 17,
2022
Received in revised form:
October 7,
2022
Received:
May 16,
2022
Identification
Copyright
© 2022 Royal College of Pathologists of Australasia. Published by Elsevier B.V. All rights reserved.
