Abstract
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 fibrovascular 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 filaments 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 diagnosis.
Highlights
The so-calledmalignant intra-abdom inal sm all 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 presentation is characterized by its adolescent male predominance and by its rare occurrence outside the peritoneal cavity
No peritoneal seeding was found at surgery but microscopically the margins were not clear and within 3 months of commencing chemotherapy the tumour recurred widely within the peritoneal cavity
Summary
The so-calledmalignant intra-abdom inal sm all round cell tumour with desmoplastic reaction’ is a relatively recent addition to the group of small blue round cell tumours of childhood.[1]. Alive at 14 months without evidence of disease current abdominal pain but was otherwise well underwent primary surgical resection of the intraperitoneal tumour at diagnosis and macroscopically complete resection was thought to have been achieved.
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