Alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation: report of 3 cases.
Int J Surg Pathol
; 17(2): 135-41, 2009 Apr.
Article
en En
| MEDLINE
| ID: mdl-18611935
ABSTRACT
The aim of this study is to report the clinicopathologic characteristics of 3 cases of alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation. Specimens of 3 cases of alveolar rhabdomyosarcoma were studied using histologic, immunohistochemical, ultrastructural, and molecular genetic techniques. The patients were a 19-year-old man with metastatic alveolar rhabdomyosarcoma in a groin lymph node, a 16-year-old girl with alveolar rhabdomyosarcoma of the perineum, and a 20-year-old man with recurrent orbital alveolar rhabdomyosarcoma. Microscopically, case 1 was composed of compact sheets of medium to large tumor cells. Cases 2 and 3 were small blue round cell tumors. Cases 1 and 3 were solid throughout, whereas case 2 demonstrated alveolar and solid architecture. By immunohistochemistry, the following markers were positive desmin (3/3), myogenin (3/3), synaptophysin (3/3), and chromogranin (2/3). Ultrastructurally, sarcomeric filaments were seen in all cases, while neuroendocrine granules were detected only in case 1. PAXFKHR fusion transcript was identified in case 2, case 3 had a variant PAX3 transcript, and case 1 was negative. The data presented expands the known differentiation of alveolar rhabdomyosarcoma.
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Neoplasias Orbitales
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Diferenciación Celular
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Rabdomiosarcoma Alveolar
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Células Neuroendocrinas
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Neoplasias de los Genitales Femeninos
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Linfoma
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Neuronas
Tipo de estudio:
Diagnostic_studies
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Prognostic_studies
Límite:
Adolescent
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Adult
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Female
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Humans
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Male
Idioma:
En
Año:
2009
Tipo del documento:
Article