Highlighting the Diversity of Desmoplastic Small Round Cell Tumor: A Case Series.
Int J Surg Pathol
; 31(2): 142-151, 2023 Apr.
Article
en En
| MEDLINE
| ID: mdl-35466756
ABSTRACT
Desmoplastic small round cell tumor (DSRCT) is a rare malignant tumor that occurs mainly in the retroperitoneum of children and young adults. In its prototypical form, DSCRT displays characteristic morphology with nested primitive small round cells in a desmoplastic stroma and a distinctive immunophenotype with polyphenotypic differentiation. However, DSCRT can also exhibit a broader clinical, histologic and immunohistochemical spectrum and, therefore, cause diagnostic difficulties. Given that DSCRT is an aggressive and nearly universally fatal disease, making the correct diagnosis is critically important. Herein, we report three patients with DSRCT and unusual clinical, morphologic or immunohistochemical characteristics, in order to highlight its remarkable diversity and increase awareness of this unusual, distinctive neoplasm.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Tumor Desmoplásico de Células Pequeñas Redondas
Límite:
Adult
/
Child
/
Humans
Idioma:
En
Revista:
Int J Surg Pathol
Asunto de la revista:
PATOLOGIA
Año:
2023
Tipo del documento:
Article
País de afiliación:
Estados Unidos