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HEPATOBLASTOMA
Lic. Rosa Gasco Garcia
El hepatoblastoma es el tumor hepático maligno más frecuente en la infancia. Afecta sobre todo a niños
menores de 3 años. Se trata de un tumor infrecuente, cuya incidencia parece aumentar en los últimos
años. Puede aparecer de forma aislada o integrarse en el contexto de un síndrome de predisposición.
DEFINICIÓN
EPIDEMIOLOGÍA
El hepatoblastoma (HB) supone el
79% de los tumores hepáticos
malignos pediátricos, pero solo el
2% de todos los tumores malignos
infantiles
01
La etiología se desconoce,
aunque se han descrito
factores de riesgo como la
prematuridad y un muy bajo
peso al nacer (< 1.500 g)
02
03
El promedio de edad en el
momento del diagnóstico varía
de 12 a 21 meses. El 4%
corresponde a formas
congénitas. La incidencia es
más elevada en los niños
portadores de anomalías
congénitas
04
La proporción en función del
sexo (varones/mujeres) es de
alrededor de 1,5 y la incidencia
se estima en 0,5-1,5 casos por
millón
Clasificación
SIGNOS CLINICOS
El signo revelador suele ser un
aumento de volumen del abdomen sin
otros signos asociados, salvo en los
tumores muy voluminosos (trastornos
del tránsito o dolor)
El diagnóstico puede
sospecharse en caso de
pubertad precoz con
secreción anormal de -HCG
(subunidad de la
gonadotropina coriónica
humana)
Puede manifestarse por una
ruptura tumoral o una
hemorragia intraabdominal
causante de un síndrome
abdominal agudo
La fiebre, el dolor, la ictericia
y la alteración del estado
general
Se han descrito varios
casos de asociación con
una osteoporosis
generalizada con fracturas.
DIAGNÓSTICO
Venus has a beautiful
name and is the second
planet from the Sun
VENUS
Concentración sérica de alfa-fetoproteína
La AFP es un marcador
muy sensible, pero
inespecífico de los tumores
hepáticos. Su
concentración debe
interpretarse en función de
la edad, porque existe una
secreción fisiológica en
recién nacidos
En todos los casos de
secreción no tumoral,
la concentración no es
tan elevada como en
los hepatoblastomas,
donde la
concentración sérica
media es mayor de 1,1
× 104ng/ml en el 90%
de los casos
Se puede observar una gran masa en el hígado
y áreas de hemorragia interna dentro del
tumor.
Ecografía abdominal
tomografía computarizada
(TC)
multifásica o la resonancia magnética nuclear (RMN).
BIOPSIA
HEPATICA
Aunque el diagnóstico de HB es muy
probable basándose en la asociación
de un tumor hepático con el aumento
de la AFP por encima de 104ng/ml,
la biopsia es necesaria para precisar
la biología del tumor y avanzar en
las posibilidades terapéuticas, al
permitir una mejor estratificación de
los pacientes antes de cualquier
tratamiento. La biopsia con aguja de
tipo Tru-Cut bajo anestesia general y
guiada por ecografía es el método
de elección. Debe realizarse a través
del hígado sano, dando prioridad al
método coaxial para evitar el riesgo
de diseminación tumoral
intraperitoneal
El diagnóstico histológico
de una muestra de tumor
es esencial, aunque
algunos investigadores
creen que la biopsia
puede no ser necesaria
en niños muy pequeños
(6 meses a 3 años) y así
evitar la probabilidad de
crecimiento o
diseminación del tumor
CLASIFICACION PRETEX
En Europa el Childhood Liver
Tumor Study Group de la Sociedad
Universal de Oncología Pediátrica
(SIOPEL) ha desarrollado la
evaluación preoperatoria del
sistema de estadificación de la
expansión tumoral (PRETEXT), que
parece ser un instrumento preciado
para la estratificación del peligro.
TRATAMIENTO
QUIMIOTERAPIA
CIRUGIA
PROTOCOLO HB
ALTO RIESGO
SIOPEL 4
SIOPEL 4
leucopenia, trombocitopenia y
anemia dosis-dependientes,
ototoxicidad (más grave en niños),
anorexia, náuseas, vómitos, diarrea y
fiebre.
BLOQUE B Y C
CIRUGIA
MANEJO QUIRURGICO DEL HB
CUIDADOS DE ENFERMERIA
CUIDADOS DE ENFERMERIA
“This is a quote, words full of
wisdom that someone
important said and can
make the reader get
inspired.”
—SOMEONE FAMOUS
CLINICAL TRIAL
PRECLINICA
L
Mars is a actually a
very cold place
Human safety
PHASE 1
Venus is the
second planet from
the Sun
Expanded safety
PHASE 2
Mercury is the
smallest planet
Efficacy & safety
PHASE 3
Lab studies
TRIAL TIMELINE
1
Mercury is the closest
planet to the Sun and the
smallest one in the Solar
System
RESEARCH
2
Despite being red, Mars is
actually a cold place. It's
full of iron oxide dust
EXPERIMENTAL
3
Venus has a beautiful
name and is the second
planet from the Sun. It’s
terribly hot
RESULTS
4
Neptune is the farthest
planet from the Sun. It's
also the fourth-largest
object
CONCLUSIONS
80%
PHASE 1
20%
MERCUR
Y
It’s the closest
planet to the
Sun
80%
MARS
Mars is a very
cold place
Success rate
Despite being red, Mars is
actually a cold place. It's
full of iron oxide dust
RESULTS
Young healthy people
20 - 80 Participants
TREND
PHASE 1
Despite being red, Mars is a
cold place. It's full of iron oxide
dust
PHASE 2
Neptune is the farthest planet
from the Sun. It's really cold
there
PHASE 3
Saturn is a gas giant. It’s
composed of hydrogen and
helium
Follow the link in the graph to modify its data and then paste
the new one here. For more info, click here
RESULTS
OUTCOME
TREATME
NT
123 42 397
GROUP 1 256 - 423
GROUP 2 90 89 190
GROUP 3 - 245 99
EXPERIMENT A
OUTCOME
TREATME
NT
- 36 93
GROUP 1 315 70 150
GROUP 2 777 - -
GROUP 3 375 - 36
EXPERIMENT B
A PICTURE IS
WORTH A
THOUSAND
WORDS
RESULTS ANALYSIS
JUPITER
Jupiter is the biggest planet in
the entire Solar System
15%
Venus has a
very beautiful
name
30%
Mercury is the
smallest planet
55%
Mars is a very
cold place
SATURN
Saturn is a gas giant and is
composed of hydrogen and
helium
EARTH
Earth is the third planet from
the Sun and the only one with
life
SUCCESS RATE
Neptune is the farthest
planet from the Sun. It's
really cold there
NEPTUNE
10%
Despite being red, Mars
is actually a cold place.
It's full of iron oxide
dust
MARS
30%
Jupiter is the biggest
planet in the Solar
System. It’s a gas giant
JUPITER
60%
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“Despite being red,
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HEPATOBLASTOMA.pptx

  • 2. El hepatoblastoma es el tumor hepático maligno más frecuente en la infancia. Afecta sobre todo a niños menores de 3 años. Se trata de un tumor infrecuente, cuya incidencia parece aumentar en los últimos años. Puede aparecer de forma aislada o integrarse en el contexto de un síndrome de predisposición. DEFINICIÓN
  • 3. EPIDEMIOLOGÍA El hepatoblastoma (HB) supone el 79% de los tumores hepáticos malignos pediátricos, pero solo el 2% de todos los tumores malignos infantiles 01 La etiología se desconoce, aunque se han descrito factores de riesgo como la prematuridad y un muy bajo peso al nacer (< 1.500 g) 02 03 El promedio de edad en el momento del diagnóstico varía de 12 a 21 meses. El 4% corresponde a formas congénitas. La incidencia es más elevada en los niños portadores de anomalías congénitas 04 La proporción en función del sexo (varones/mujeres) es de alrededor de 1,5 y la incidencia se estima en 0,5-1,5 casos por millón
  • 5.
  • 6. SIGNOS CLINICOS El signo revelador suele ser un aumento de volumen del abdomen sin otros signos asociados, salvo en los tumores muy voluminosos (trastornos del tránsito o dolor) El diagnóstico puede sospecharse en caso de pubertad precoz con secreción anormal de -HCG (subunidad de la gonadotropina coriónica humana) Puede manifestarse por una ruptura tumoral o una hemorragia intraabdominal causante de un síndrome abdominal agudo La fiebre, el dolor, la ictericia y la alteración del estado general Se han descrito varios casos de asociación con una osteoporosis generalizada con fracturas.
  • 8. Venus has a beautiful name and is the second planet from the Sun VENUS Concentración sérica de alfa-fetoproteína La AFP es un marcador muy sensible, pero inespecífico de los tumores hepáticos. Su concentración debe interpretarse en función de la edad, porque existe una secreción fisiológica en recién nacidos En todos los casos de secreción no tumoral, la concentración no es tan elevada como en los hepatoblastomas, donde la concentración sérica media es mayor de 1,1 × 104ng/ml en el 90% de los casos
  • 9. Se puede observar una gran masa en el hígado y áreas de hemorragia interna dentro del tumor. Ecografía abdominal tomografía computarizada (TC) multifásica o la resonancia magnética nuclear (RMN).
  • 10. BIOPSIA HEPATICA Aunque el diagnóstico de HB es muy probable basándose en la asociación de un tumor hepático con el aumento de la AFP por encima de 104ng/ml, la biopsia es necesaria para precisar la biología del tumor y avanzar en las posibilidades terapéuticas, al permitir una mejor estratificación de los pacientes antes de cualquier tratamiento. La biopsia con aguja de tipo Tru-Cut bajo anestesia general y guiada por ecografía es el método de elección. Debe realizarse a través del hígado sano, dando prioridad al método coaxial para evitar el riesgo de diseminación tumoral intraperitoneal El diagnóstico histológico de una muestra de tumor es esencial, aunque algunos investigadores creen que la biopsia puede no ser necesaria en niños muy pequeños (6 meses a 3 años) y así evitar la probabilidad de crecimiento o diseminación del tumor
  • 11. CLASIFICACION PRETEX En Europa el Childhood Liver Tumor Study Group de la Sociedad Universal de Oncología Pediátrica (SIOPEL) ha desarrollado la evaluación preoperatoria del sistema de estadificación de la expansión tumoral (PRETEXT), que parece ser un instrumento preciado para la estratificación del peligro.
  • 14. SIOPEL 4 leucopenia, trombocitopenia y anemia dosis-dependientes, ototoxicidad (más grave en niños), anorexia, náuseas, vómitos, diarrea y fiebre.
  • 19.
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  • 22. “This is a quote, words full of wisdom that someone important said and can make the reader get inspired.” —SOMEONE FAMOUS
  • 23. CLINICAL TRIAL PRECLINICA L Mars is a actually a very cold place Human safety PHASE 1 Venus is the second planet from the Sun Expanded safety PHASE 2 Mercury is the smallest planet Efficacy & safety PHASE 3 Lab studies
  • 24. TRIAL TIMELINE 1 Mercury is the closest planet to the Sun and the smallest one in the Solar System RESEARCH 2 Despite being red, Mars is actually a cold place. It's full of iron oxide dust EXPERIMENTAL 3 Venus has a beautiful name and is the second planet from the Sun. It’s terribly hot RESULTS 4 Neptune is the farthest planet from the Sun. It's also the fourth-largest object CONCLUSIONS
  • 25. 80% PHASE 1 20% MERCUR Y It’s the closest planet to the Sun 80% MARS Mars is a very cold place Success rate Despite being red, Mars is actually a cold place. It's full of iron oxide dust RESULTS Young healthy people 20 - 80 Participants
  • 26. TREND PHASE 1 Despite being red, Mars is a cold place. It's full of iron oxide dust PHASE 2 Neptune is the farthest planet from the Sun. It's really cold there PHASE 3 Saturn is a gas giant. It’s composed of hydrogen and helium Follow the link in the graph to modify its data and then paste the new one here. For more info, click here
  • 27. RESULTS OUTCOME TREATME NT 123 42 397 GROUP 1 256 - 423 GROUP 2 90 89 190 GROUP 3 - 245 99 EXPERIMENT A OUTCOME TREATME NT - 36 93 GROUP 1 315 70 150 GROUP 2 777 - - GROUP 3 375 - 36 EXPERIMENT B
  • 28. A PICTURE IS WORTH A THOUSAND WORDS
  • 29. RESULTS ANALYSIS JUPITER Jupiter is the biggest planet in the entire Solar System 15% Venus has a very beautiful name 30% Mercury is the smallest planet 55% Mars is a very cold place SATURN Saturn is a gas giant and is composed of hydrogen and helium EARTH Earth is the third planet from the Sun and the only one with life
  • 30. SUCCESS RATE Neptune is the farthest planet from the Sun. It's really cold there NEPTUNE 10% Despite being red, Mars is actually a cold place. It's full of iron oxide dust MARS 30% Jupiter is the biggest planet in the Solar System. It’s a gas giant JUPITER 60%
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  • 45. JANUARY FEBRUARY MARCH APRIL MAY JUNE PHASE 1 PHASE 2 Task 1 Task 2 Task 1 Task 2 JANUARY FEBRUARY MARCH APRIL PHASE 1 Task 1 Task 2
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  • 58. Add the title here Text 1 Text 2 Text 3 Text 4 Text 5 Text 6 2016 2017 2018 2019 2020 “Despite being red, Mars is actually a very cold place” “Mercury is closest planet to the Sun and the smallest” “Neptune is the farthest-known planet from the Sun” “Saturn is a gas giant and has several rings” “Venus has a beautiful name and is the second planet” Premium infographics
  • 59. “Mercury is closest planet to the Sun and the smallest” TITLE 1 75% “Mercury is closest planet to the Sun and the smallest” “Mercury is closest planet to the Sun and the smallest” “Mercury is closest planet to the Sun and the smallest” 75% Premium infographics