[A retrospective study about 11 cases of Esthesioneuroblastomas treated in Hospital Santa Creu i Sant Pau between 2000 and 2008 and literature review].

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.