tumor de wilms & neuroblastoma
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TUMOR DE WILMSNEUROBLASTOMA
DRA. PAOLA ROMERO PG. RADIOLOGIA UNL
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Tumor de Wilms (1867-1918)
Neoplasia maligna del riñón Nefroblastoma Cáncer abdominal en niños mas frecuente.[1] Se presenta en (1 de cada 200.000 a 250.000
niños) Es infrecuente en 8 años y recién nacidos.[2]
75% de los casos en niños sanos, 25% asociados a malformaciones: tracto urinario (hipospadias, criptorquidea, aniridia y hemihipertrofia 2]
Tasa de supervivencia es 90% al cabo de 5 años.[1
[1]Pediatrics: General Medicine > Oncology. eMedicine.com. Con. 29-01-2009.2 Enciclopedia médica en español. Con. 29-01- 2009.
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Fisiopatología
La mayoría son unilaterales, bilaterales (-5% )
Ubican: polo superior del riñón, Tumor maligno:
› Encapsulado, vascularizado y friable› No rebasa la línea media hacia el lado contra
lateral.› Si existe metástasis son en el pulmón. 3
› Puede presentar hemorragia y necrosis
3American Academy of Family Physicians (abril, 2001). Am Fam Physician; 61:2144-54.
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Patogenia
Contiene: blastema metanéfrico, epitelio y estroma.
Es característica su presencia en los glomérulos y túbulos renales rodeado por un estroma celular.
El estroma puede incluir › Musculo estriado› Cartílago› Hueso› Tejido Graso
Suele comprimir el parénquima renal normal.
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Cuadro Clínico
Un 95% de los casos : una masa palpable.
La mayoría de pacientes: presenta poca afectación general.
Síntomas inespecíficos: Dolor abdominal, hematuria, fiebre, anorexia, nausea, vomito, estreñimiento, TA elevada
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METASTASIS
METS
INVASION DIRECTA
DISEMINACION LINFATICA
DISEMINACION AL UROTELIO DEL T.U.
DISTAL
HEMATOGENA
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CLASIFICACION
GRADO I TUMOR LIMITADO AL RIÑON QUE PUEDE EXTIRPARSE COMPLETAMENTE
GRADO II TUMOR QUE SE EXTIENDE MAS ALLA DEL RIÑON. EXTIRPACION COMPLETA
GRADO III TUMOR RESIDUAL NO HEMATOGENO LIMITADO AL ABDOMEN
GRADO IV METASTASIS HEMATOGENAS PULMON, HIGADO, HUESO, CEREBRO
GRADO V AFECTACION RENAL BILATERAL. APARECEN INICIAL O DURANTE AL TRATAMIENTO
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Neuroblastoma
Derivado de la cresta neural Formación inicial Tejido Nervioso y
Glándulas Suprarrenales .[1] Cuello, pecho o médula espinal. Frecuente en 1er -3 años. antes del
nacimiento. Tumor agresivo: alta mortalidad Se detecta cuando existen metástasis[2]
1. Howman-Giles R, Shaw PJ, Uren RF, Chung DK (2007). «Neuroblastoma and other neuroendocrine tumors». Semin Nucl Med 37 (4): pp. 286–302. doi:10.1053/j.semnuclmed.2007.02.009. 2.Tsubono Y, Hisamichi S. A Halt to Neuroblastoma Screening in Japan. New England Journal of Medicine. 350. 19: 2010-2011 (2004).
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Cuadro Clínico
Ubicación SNS y en sitios de metástasis.[3] Los síntomas aparece 1er año 40% de los
casos en diversos tejidos, especial el hueso:[1] [2]
Dolor, síntomas de insuficiencia medular, anemia y púrpura
Masa palpable en el abdomen , cuello y tórax Signos paraneoplásicos : Hipertensión arterial
diarrea, Fiebre, debilidad o parálisis, Anorexia, distensión y dolor abdominal en
tumores del abdomen.
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Diagnostico
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Computed tomographic evaluation of Wilms tumor andneuroblastoma
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BIBLIOGRAFIA
1. Arger PH, Mulhem CB, Littman PS, Meadows AT, Coleman BG, Jarrett PT. Management of solid tumors in children: Contribution of computed tomography. AJR 1981; 137: 251-255.
2. Armstrong EA, Harwood-Nash DCF, Ritz CR, Chuang SH, Petterson H, Martin DJ. CT of neuroblastomas and ganglioneuromas in children. AJR 1982; 139:571-576.
3. Berger PE, Kuhn JP, Munschauer RW. Computed tomography and ultrasound in the diagnosis and management of neuroblastoma. Radiology 1978; 128:663-667.
4. Boldt DW, Reilly BJ. Computed tomography of abdominal mass lesions in children. Radiology 1977; 124:371-378.
5. Bra.sch RC. Computed tomography in the evaluation of pediatric genitouninary disease. Urol Clin North Am 1980; 7: 223-230.
6. Brasch RC, Randel SB, Gould RG. Follow-up of Wilms tumor: Comparison of CT with other imaging procedures. AJR 1981; 137:1005-1009.
7. Kirks DR, Merten DF, Grossman H, Bowie JD. Diagnostic imaging of pediatric abdominal masses: An overview. Radiol
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RX
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ECO
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RM
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Patogenia
Neuroblastos (remanentes de fase embrionaria de desarrollo).[9]
Células de Schwann pueden secretar FC inductores de neoplasia.[17]
El 25-35% brazo corto del cromosoma 1 (1p35-36).[18] La presencia de esta delección se asocia con mal pronóstico.
El pronóstico desfavorable : proto-oncogen N-myc.[19]
El proto-oncogen normal se encuentra en el brazo corto del cromosoma 2.