Sarcoma (1997) 1, 103± 108
Desmoplastic small round cell tumour of childhood: a report of four
cases demonstrating wider clinical features and variable outcome
RUMA RAY,1 HEIDI C. L. TRAUNECKER,2 FARO RAAFAT1
& MICHAEL C. G. STEVENS2
Departments of 1Histopathology and 2Oncology, Birmingham Children’ s Hospital, UK
Purpose. Four further cases of desmoplastic small round cell tumour with multi phenotypic differentiation are described.
Subjects. Two patients were typical (male, adolescent with peritoneal tumour and, in one case, liver metastases) and
did not respond well to treatment. Two other patients showed less usual features (young, female with retroperitoneal
disease, both with intraspinal extension and renal tract obstruction). Both responded favourably to multi-modal treatment
regimens including extensive and invasive supportive care.
Results. Histologically, all tumours showed clear features of this diagnosis, namely angulated nests of small cells in a
background of ® brovascular stroma. Immunohistochemistry typically showed divergent differentiation with neural, muscle
and epithelial marker positivity. All four tumours stained positive for the Wilms’ tumour 1 suppressor gene product.
Electron microscopy showed intercellular tight junctions, cytoplasmatic intermediate ®
laments and absence of microvilli.
Rare neurosecretory-type granules were observed.
Discussion. These cases illustrate a broader spectrum of clinical presentation than previously associated with this
Key words: desmoplastic small round cell tumour, clinical presentation.
round cell tumour with desmoplastic reaction’ is a
relatively recent addition to the group of small blue
round cell tumours of childhood.1 Its clinical pres-
entation is characterized by its adolescent male pre-
dominance and by its rare occurrence outside the
show two distinct elementsÐ
a population of small
malignant cells which demon