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Vol. 22. Núm. 5.Enero 2011Páginas 381-460
DOI: 10.1016/S1130-1473(11)70035-5
Estudio retrospectivo de 11 casos de estesioneuroblastomas tratados en el Hospital Santa Creu i Sant Pau entre los años 2000 y 2008 más revisión de la literatura
A retrospective study about 11 cases of Esthesioneuroblastomas treated in Hospital Santa Creu i Sant Pau between 2000 and 2008 and literature review
Visitas
1654
F. Muñoz, P. Tresserras, F. Bartumeus
Servicios de Neurocirugía. Hospital Santa Creu i Sant Pau. Barcelona
J.R. Montserrat*, F.J. Sancho**
* Otorrinolaringología. Hospital Santa Creu i Sant Pau. Barcelona
** Anatomía Patológica. Hospital Santa Creu i Sant Pau. Barcelona
Visitas
1654
Información del artículo
Resumen
Introducción

El estesioneuroblastoma (ENB) es un tumor maligno de origen neuroectodérmico poco habitual que afecta principalmente a la fosa craneal anterior y cavidad nasal.

Objetivos

Revisar nuestra experiencia en el manejo de los ENB, evaluar la validez del diagnóstico anatomopatológico, el tipo de tratamiento y factores pronósticos de la enfermedad comparando nuestros hallazgos con los descritos en la literatura.

Material y métodos

Estudio retrospectivo de 11 casos de ENB tratados en nuestro hospital en el periodo comprendido entre 2000 y 2008. Se realiza análisis estadístico sobre la existencia de factores pronósticos. Se revisa la bibliografía publicada sobre el ENB entre 1990 y 2009.

Resultados

Se trataron 3 mujeres y 8 hombres, cuya edad media fue 42 años (rango 20–71 años). El síntoma inicial fue la obstrucción nasal (81%), epistaxis (27%), disminución de agudeza visual (18%), cefalea y otros. Según la clasificación de Kadish, 2 son estadío B y 9 estadío C; según la clasificación de Dulguerov, 2 son T2, 3 son T3 y 6 son T4. El resultado histológico según la clasificación de Hyams fue de 2 casos en grado I, 4 en grado II, 3 en grado III y 2 en grado IV. Dos de los casos inicialmente catalogados en grado IV cambiaron de diagnóstico en la segunda biopsia a tumor indiferenciado. Se realizó un abordaje subcraneal en 73% (8 casos) con comprobación de la exéresis completa mediante endoscopia endonasal. De ellos, tras radioterapia coadyuvante en todos y en uno quimioterapia, el 62% (5 pacientes) están vivos sin enfermedad, 12,5% (1) vivo con enfermedad controlada, 25% (2) muerte por progresión de la enfermedad. En 1 paciente se realizó resección subtotal por endoscopia endonasal más radioterapia y está vivo sin enfermedad. En los otros 2 casos, que se consideraron irresecables, se realizó biopsia endonasal más quimioterapia y radioterapia; uno está vivo con enfermedad controlada y el otro fue éxitus por progresión de la enfermedad. Se realizó RT coadyuvante en todos los casos y QT adyuvante en 5 de los pacientes. El grado histológico de Hyams se ha encontrado como factor pronóstico estadísticamente significativo en la predicción de menor periodo libre de enfermedad en nuestra serie.

Conclusiones

En el diagnóstico histopatológico del ENB, la gradación de Hyams puede ser válida considerando los grado IV como la variante más avanzada siendo en ocasiones difícil de diferenciar de otros tumores indiferenciados. El abordaje subcraneal o resección craneofacial en estadíos avanzados (Kadish C y algunos B) creemos que sigue siendo de elección como primer tratamiento. La radioterapia es coadyuvante en todos los casos y la quimioterapia en casos seleccionados. El único factor pronóstico que hemos podido extraer de nuestra serie es el grado histológico según la clasificación de Hyams.

Palabras clave:
Estesioneuroblastoma olfatorio
Tratamiento quirúrgico
Resultado
Supervivencia
Revisión de la literatura
Summary
Introduction

Esthesioneuroblastoma (ENB) is a very uncommon malignant tumor with a neuroectodermal origin that usually involves the anterior cranial fossa and nasal cavity.

Objectives

To review our experience in the management of ENB and assess the validity of the histopathological diagnosis, modality of treatment and prognostic factors of the disease comparing our findings with the literature.

Methods

A retrospective study of 11 cases with the diagnosis of esthesioneuroblastoma treated in our hospital between 2000 and 2008. Statistical analysis was performed in search for prognostic factors. The bibliography about ENB published between 1990 and 2009 was reviewed

Results

There were 3 women and 8 men, with a mean age of 42 years old (range 20–71y). Their symptoms upon admission were nasal obstruction (81%), epistaxis (27%), visual loss (18%), headache and others. According to the Kadish Stage, 2 were stage B and 9 were stage C. Dulguerov and Calcaterra Classification was also used: 2 were T2, 3 were T3 and 6 were T4. The hystopathological result according to the Hyams classification was: 2 cases in stage I, 4 in stage II, 3 in stage III and 2 in stage IV. The two cases classified in stage IV changed the diagnosis to undifferenciate tumor in the second biopsy. A subcranial approach was performed in 8 cases combined with endonasal endoscopy to confirm the total removal, followed by radiotherapy in all and chemotherapy in one case, resulting on 62% (5 patients) being alive without disease, 12,5% (1 p) alive with disease, and 25% (2 p) dead of disease. Another patient was operated by a single endonasal endoscopic approach and a subtotal removal was achieved. This patient is alive without disease. The other 2 patients were treated by biopsy plus radiotherapy and chemotherapy, because they were considered unresectable, and one of them is alive with disease and the other one is dead of disease. Radiotherapy was performed in all cases and chemotherapy in 5 cases. The hystopathological grading system of Hyams was considered statistically significant as a prognostic factor of disease-free survival.

Conclusions

When the hystopathological diagnosis of ENB is considered, the Hyams classification can be valid considering grade IV as an advanced stage that is sometimes difficult to differentiate from other undiferentiated tumors.

The subcranial approach or craneofacial resection in advanced stages (Kadish C and some B) should be considered as the first treatment of choice. Radiotherapy is indicated in all cases and chemotherapy in selected cases. Hyams’ classification was the only staging system that proved useful as a prognostic factor in our series.

Key words:
Olfactory esthesioneuroblastoma
Surgery
Treatment outcome
Survival
Review
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Muñoz, F.; Tresserras, P.; Montserrat, J.R.; Sancho, F.J.; Bartumeus, F.: Estudio retrospectivo de 11 casos de estesioneuroblastomas tratados en el Hospital Santa Creu i Sant Pau entre los años 2000 y 2008 más revisión de la literatura. Neurocirugía 2011; 22: 401–418.

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