Desmoplastic small round cell tumor of the kidney: a case report.
Diagn Pathol
; 15(1): 95, 2020 Jul 23.
Article
em En
| MEDLINE
| ID: mdl-32703229
ABSTRACT
BACKGROUND:
Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive neoplasm seen in children and young adults, usually manifested by involvement of abdominal serosa. Here, we present an unusual case of primary DSRCT of kidney. CASE PRESENTATION The patient was an 8-year-old girl with a large renal mass which was confused with primitive neuroectodermal tumor (PNET) in the needle biopsy. The tumor had a variegated histology revealing frequent pseudo-rosette formations, pseudopapillary architecture, rhabdoid, clear or pleomorphic cells in addition to typical small round cell morphology and desmoplasia. It showed immunohistochemical features of DSRCT, and EWSR1 re-arrangement.CONCLUSIONS:
Proffering this diagnosis is particularly difficult for tumors of viscera because of the incognizance of the entity in these locations. Moreover, DSRCT is a great mimicker and may get easily confused with more common kidney malignancies of childhood such as Wilms tumor, PNET/EWS, rhabdoid tumor, clear cell sarcoma, and other small round cell tumors as well as renal cell carcinomas. The distinction is critical as the accurate therapeutic approach will require correct diagnosis.Palavras-chave
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Base de dados:
MEDLINE
Assunto principal:
Tumores Neuroectodérmicos Primitivos
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Tumor de Wilms
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Tumor Desmoplásico de Pequenas Células Redondas
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Neoplasias Renais
Tipo de estudo:
Diagnostic_studies
Limite:
Child
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Female
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Humans
Idioma:
En
Ano de publicação:
2020
Tipo de documento:
Article