Tratamiento del sarcoma de tejido blando infantil (PDQ®)–Versión para profesionales de salud - Instituto Nacional del Cáncer

Tratamiento del sarcoma de tejido blando infantil (PDQ®)–Versión para profesionales de salud

Sarcomas de células redondas indiferenciados con reordenamientos BCOR-CCNB3

Características moleculares

Los sarcomas de células redondas indiferenciados se caracterizan por presentar inversión paracéntrica del cromosoma X y un reordenamiento BCOR-CCNB3; también se han notificado empalmes alternativos BCOR, incluso MAMAL3 y ZC3H7B.[284] A pesar de las similitudes con el sarcoma de Ewing en la patología clínica, estos tumores son biológicamente diferentes en cuanto al análisis de la matriz del perfil de expresión y el polimorfismo de un solo nucleótido.

Cuadro clínico inicial

El sarcoma de células redondas indiferenciado representa 5 % de todos los sarcomas sin el reordenamiento en EWSR1 y, por lo general, afecta a los varones. Más de 70 % de los casos se encuentran en pacientes menores de 18 años (mediana de edad en el momento del diagnóstico, 13–15 años).[285,286][Grado de comprobación: 3iiA] Estos tumores aparecen con mayor frecuencia en los huesos de la pelvis y las extremidades, y se observan metástasis en alrededor de 30 % de los casos.

Tratamiento

Cuando se los trata con terapias similares a las del sarcoma de Ewing, 75 % de los pacientes exhiben respuestas patológicas significativas relacionadas con el tratamiento. En una serie de 36 casos, la tasa de supervivencia a 3 y 5 años, fue de 93 y 72 %, respectivamente.[285][Grado de comprobación: 3iiA] En otra serie de 26 pacientes, la SG a 5 años fue de 76,5 % y la supervivencia fue superior en el caso de los pacientes que recibieron terapia de inducción con un régimen de tipo sarcoma de Ewing.[287][Grado de comprobación: 3iiA] La mayoría de los tumores en estas series se originaron en el hueso.

(Para mayor información, consultar el sumario del PDQ sobre Tratamiento del sarcoma de Ewing).

Sarcomas de células redondas indiferenciados con reordenamientos CIC-DUX

Características moleculares

Estos tumores se caracterizan por presentar una fusión CIC-DUX que resulta de una t(4;19) o t(10;19) recurrente y son los sarcomas de células redondas indiferenciados sin la fusión EWSR1-FUS más frecuentes.[288]

Cuadro clínico inicial

Afectan por lo general a adultos jóvenes; 50 % de los casos se presentan entre los 21 y los 40 años de edad. En una serie de 115 casos, la mediana de edad en el momento del diagnóstico fue de 32 años y 22 % de los casos se presentaron en pacientes menores de 18 años.[286,288] Esta entidad por lo general afecta a varones y se origina en el tejido blando del tronco y las extremidades.

Tratamiento

En una serie de 115 casos de sarcoma de células redondas con el reordenamiento CIC, se obtuvo información de seguimiento adecuada de 57 pacientes.[288] Nueve pacientes presentaron metástasis y 53 % de los pacientes con enfermedad localizada experimentaron una recidiva que, por lo general, comprometió el pulmón. Los pacientes que se trataron con quimioterapia neoadyuvante tuvieron una supervivencia inferior en comparación con los pacientes tratados con resección quirúrgica inicial; sin embargo, esta diferencia podría haberse relacionado con un tamaño de tumor mayor en el cuadro clínico inicial del primer grupo. Las tasas de supervivencia a 2 y 5 años fueron de 53 y 43 %, respectivamente. Estas tasas de supervivencia son significativamente más bajas que las tasas observadas en pacientes de sarcoma de Ewing. Se necesitan más estudios para identificar el tratamiento óptimo de esta enfermedad.

Tumores vasculares

Los tumores vasculares varían desde los hemangiomas, que siempre se consideran benignos, hasta los angiosarcomas, que son muy malignos.[289] Los tumores vasculares malignos incluyen los siguientes subtipos:

Hemangioendotelioma epitelioide

Incidencia y desenlace

En 1982, Weiss y Enzinger describieron por primera vez este tumor de tejido blando. Los hemangioendoteliomas epitelioides a veces se presentan a una edad más temprana, pero la incidencia máxima se ubica en la cuarta y quinta décadas de vida. Los tumores pueden tener una evolución poco activa o de gran malignidad; la supervivencia general a 5 años es de 73 %. Hay informes de casos de pacientes con lesiones múltiples que no recibieron tratamiento y que presentaron una evolución muy benigna comparada con la de otros pacientes con una evolución muy maligna. Si bien algunos patólogos han tratado de estratificar a los pacientes para evaluar los riesgos y ajustar el tratamiento, se necesita llevar a cabo más investigación sobre este tema.[290-296]

La presencia de efusiones, un tamaño tumoral mayor de 3 cm y un índice mitótico alto (>3 mitosis/50 campos de gran aumento) se han relacionado con desenlaces desfavorables.[292]

Características histopatológicas y moleculares

Se encontró la fusión génica WWTR1-CAMTA1 en un gran porcentaje de pacientes; con menos frecuencia, se notificó la fusión génica YAP1-TFE3.[290] No es posible dirigir el efecto de los medicamentos actuales de forma directa a estas fusiones. Se ha descrito monoclonalidad en las lesiones hepáticas múltiples, lo que indicaría un proceso metastásico.

Desde el punto de vista histológico, estas lesiones se caracterizan como lesiones epitelioides dispuestas en nidos, hebras y trabéculas, con pocos espacios vasculares. Las características que quizá se relacionen con un comportamiento clínico muy maligno incluyen atipia celular, una o más mitosis por 10 campos de gran aumento, un incremento en la proporción de células fusiformes, necrosis focal y formación ósea metaplásica.[292]

El número de pacientes pediátricos notificados en la bibliografía es limitado.

Cuadro clínico inicial y evaluación diagnóstica

Los sitios comunes de compromiso son: solo el hígado (21 %), hígado y pulmón (18 %), solo el pulmón (12 %) y solo el hueso (14 %).[292,297,298] El cuadro clínico inicial depende del sitio comprometido, como sigue:

Hígado: los nódulos hepáticos exhiben vascularización central en la ecografía, mejor contraste de las lesiones en la tomografía computarizada, además de señal T1 baja y señal T2 moderada en las imágenes por resonancia magnética.
Pulmón: el hemangioendotelioma epitelioide pulmonar a veces es un hallazgo asintomático en una radiografía de tórax o se relaciona con dolor pleural, hemoptisis, anemia y fibrosis.
Hueso: las metástasis óseas a veces se relacionan con una fractura patológica. En las radiografías, hay lesiones osteolíticas bien definidas, múltiples o solitarias.
Tejido blando: 30 % de los casos con compromiso de tejido blando se vinculan con metástasis y, cuando están presentes, tienen una evolución muy maligna con escasa respuesta a la quimioterapia.
Piel: las lesiones cutáneas se presentan en forma sobresaliente y nodular, o en placas calientes de color marrón rojizo.

Tratamiento del hemangioendotelioma epitelioide

Las opciones de tratamiento del hemangioendotelioma epitelioide son las siguientes:

Observación.
Cirugía.
Inmunoterapia.
Terapia dirigida.
Quimioterapia.

Para los casos de poca malignidad, se justifica la observación. Para los casos más malignos, se han utilizado múltiples medicamentos, como interferón, talidomida, sorafenib, pazopanib y rapamicina.[299] Los casos más malignos se tratan con quimioterapia como la que se utiliza para el angiosarcoma. La cirugía se utiliza siempre que sea posible. El trasplante de hígado se ha utilizado para lesiones hepáticas muy malignas, con metástasis o sin estas.[292,300-303]

Los pacientes o las familias que deseen una terapia adicional dirigida a la enfermedad deberían considerar la participación en ensayos sobre abordajes terapéuticos novedosos ya que con ninguno de los fármacos tradicionales se ha demostrado una actividad clínicamente significativa.

Independientemente de si se decide continuar con una terapia dirigida a la enfermedad en el momento de la progresión, el objetivo principal del tratamiento sigue siendo el cuidado paliativo. Esto garantiza que se aumenta al máximo la calidad de vida mientras se intenta reducir la tensión relacionada con la enfermedad terminal.

Opciones de tratamiento en evaluación clínica para el hemangioendotelioma epitelioide

La información en inglés sobre los ensayos clínicos patrocinados por el Instituto Nacional del Cáncer (NCI) se encuentra en el portal de Internet del NCI. Para obtener información en inglés sobre ensayos clínicos patrocinados por otras organizaciones, consultar el portal de Internet ClinicalTrials.gov.

A continuación se presentan ejemplos de ensayos clínicos nacionales o institucionales en curso:

NCT03148275 (Trametinib in Treating Patients with Epithelioid Hemangioendothelioma That Is Metastatic, Locally Advanced, or Cannot Be Removed by Surgery): este es un ensayo de fase II en el que se evalúa la eficacia de trametinib, con desenlaces notificados por los pacientes como objetivos secundarios.
NCT01532687 (Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma): en este ensayo aleatorizado de fase II se estudia qué tan eficaz es el clorhidrato de gemcitabina con el clorhidrato de pazopanib, o sin este, para tratar a los pacientes con sarcoma de tejido blanco resistente al tratamiento.

Ensayos clínicos en curso

Realizar una búsqueda avanzada en inglés de los ensayos clínicos sobre cáncer auspiciados por el NCI que ahora aceptan pacientes. La búsqueda se puede simplificar por ubicación del ensayo, tipo de tratamiento, nombre del fármaco y otros criterios. También se dispone de información general sobre los ensayos clínicos.

Angiosarcoma de tejido blando

Incidencia

El angiosarcoma es un tumor vascular poco frecuente (representa 2 % de los sarcomas) y de gran malignidad que puede surgir en cualquier parte del cuerpo, pero es más común en el tejido blando. Se calcula que la incidencia del angiosarcoma es de 2 casos por millón de personas; en los Estados Unidos, cada año afecta a alrededor de 600 personas que suelen tener entre 60 y 70 años.[304]

Los angiosarcomas son muy infrecuentes en los niños y no está claro si la fisiopatología de este tumor es diferente en la población pediátrica. Se notificaron casos en recién nacidos y lactantes mayores que presentaron al inicio lesiones cutáneas y hepáticas múltiples, algunas de las cuales expresan GLUT1.[305-308] La mayoría de los angiosarcomas comprometen la piel y el tejido blando superficial, aunque también afectan el hígado, el bazo y el pulmón; con poca frecuencia afectan el hueso.

Factores de riesgo

Los factores de riesgo establecidos incluyen los siguientes:[309]

Exposición al cloruro de vinilo.
Exposición a la radiación.
Linfedema crónico por cualquier causa, incluso el síndrome de Stewart-Treves.

Características histopatológicas y moleculares

Los angiosarcomas son tumores mayormente aneuploides. Los escasos casos de angiosarcoma que surgen de lesiones benignas, como los hemangiomas, siguen una vía distintiva que se necesita investigar. En el angiosarcoma inducido por radiación se observa amplificación de MYC. Las mutaciones KDR-VEGFR2 y las amplificaciones de FLT4-VEGFR3 se han observado con una frecuencia de menos de 50 %.[309]

Es posible que el diagnóstico histopatológico sea muy difícil porque a veces hay áreas de atipia variada. La característica común es una red irregular de canales en un modelo de disección a lo largo de haces de colágeno dérmico. Hay variación de forma celular, tamaño celular, mitosis, múltiples capas de endotelio y formación papilar. También puede haber células epitelioides. La necrosis y la hemorragia son comunes. Los tumores se tiñen para analizar el factor VIII, el CD31 y el CD34. Algunas lesiones hepáticas se parecen a los hemangiomas infantiles y tienen expresión focal de GLUT1. La nomenclatura de estas lesiones hepáticas ha sido difícil y confusa con el uso de la terminología de 1971 (por ejemplo, hemangioendotelioma tipo I: hemangioma infantil; hemangioendotelioma tipo II: angiosarcoma de grado bajo; hemangioendotelioma de tipo III: angiosarcoma de grado alto).[306]

Tratamiento del angiosarcoma del tejido blando

Las opciones de tratamiento del angiosarcoma de tejido blando son las siguientes:

Cirugía (enfermedad localizada).
Radioterapia (enfermedad cutánea localizada en adultos).
Cirugía, quimioterapia y radioterapia (enfermedad metastásica).

La enfermedad localizada se cura mediante cirugía radical. La resección quirúrgica completa parece ser fundamental para los angiosarcomas y los linfangiosarcomas pese a las pruebas de que el tumor se encoge en algunos pacientes tratados con terapia local o sistémica.[307,310-312] En una revisión de 222 pacientes (mediana de edad de 62 años; intervalo de edad de 15–90 años), se observó una tasa de supervivencia general específica para la enfermedad (SEE) de 38 % a los 5 años. La SEE a 5 años fue de 44 % en 138 pacientes con tumores localizados resecados, pero solo de 16 % en 43 pacientes con metástasis en el momento del diagnóstico.[312] Hay poca información sobre el uso del trasplante de hígado para el angiosarcoma localizado.[313][Grado de comprobación: 3iiA]

Es posible tratar con radioterapia la enfermedad localizada, en especial el angiosarcoma cutáneo. La mayoría de los casos notificados son de adultos.[314]

El tratamiento multimodal con cirugía, quimioterapia sistémica y radioterapia se utiliza para la enfermedad metastásica, a pesar de que rara vez es curativo.[315] El objetivo del tratamiento del angiosarcoma metastásico es el control de la enfermedad, con tasas de supervivencia sin progresión publicadas de 3 a 7 meses [316] y una mediana de supervivencia general (SG) de 14 a 18 meses.[317] Tanto en adultos como en niños, se notifican tasas de SG a 5 años de 20 a 35 %.[307,308,318]

En un niño con diagnóstico de angiosarcoma secundario a una transformación maligna de un hemangioma infantil, se notificó una respuesta al tratamiento con bevacizumab, un anticuerpo monoclonal contra el factor de crecimiento endotelial vascular, combinado con quimioterapia sistémica.[305,315] En un informe de ocho casos de angiosarcoma de hígado en niños se resaltó el uso indebido del término hemangioendotelioma así como la importancia del diagnóstico y tratamiento tempranos de estos tumores.[319]

Los inhibidores biológicos de la angiogénesis han mostrado actividad en adultos con angiosarcoma.[306,318]

Opciones de tratamiento en evaluación clínica para el angiosarcoma de tejido blando

A continuación se presenta un ejemplo de ensayo clínico nacional o institucional en curso:

NCT01532687 (Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma): En este ensayo aleatorizado de fase II se estudia qué tan eficaz es el clorhidrato de gemcitabina con el clorhidrato de pazopanib, o sin este, para tratar a los pacientes con sarcoma de tejido blanco resistente al tratamiento.

Ensayos clínicos en curso

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