Peritoneal desmoplastic small round cell tumors with divergent differentiation: a review.
Ultrastruct Pathol
; 17(3-4): 295-306, 1993.
Article
em En
| MEDLINE
| ID: mdl-8266595
ABSTRACT
Peritoneal desmoplastic small round cell tumors with divergent differentiation are recently described highly aggressive neoplasms with characteristic clinical, morphologic, and immunohistochemical features. This review covers 38 cases that have been reported in the literature. The average age of patients is 18.4 years, and males are affected twice as frequently as females. Tumors generally present as multiple peritoneal nodules without obvious organ involvement. Histology shows islands of small cells set in dense desmoplastic stroma. Immunohistochemical stains are usually positive for cytokeratins, epithelial membrane antigen, desmin, and vimentin. Many cases also stain for neuron-specific enolase but rarely for other neuroepithelial markers. Ultrastructural appearances range from undifferentiated small cells to larger epithelial elements. Paranuclear aggregates of intermediate filaments are characteristic. Dense-core granules and other neuroendocrine features have been described in a minority of cases. Some tumors respond to chemotherapy, but most patients die within months to a few years. The histogenesis of these tumors is uncertain.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Peritoneais
/
Carcinoma de Células Pequenas
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
Limite:
Adolescent
/
Female
/
Humans
/
Male
Idioma:
En
Revista:
Ultrastruct Pathol
Ano de publicação:
1993
Tipo de documento:
Article