Esthesioneuroblastoma outcome

A review by Jethanamest et al ¹⁾ 22 identified a cohort of 311 patients diagnosed with esthesioneuroblastoma from the Surveillance, Epidemiology, and End Results tumor registry. In this group of patients, the overall survival was 62% at 5 years and 45% at 10 years.

Median overall survival is typically 7.2 ± 0.7 years ²⁾.

Mean progression-free survival is 4.8 ± 0.7 years. The 5 and 10-year survivals are 63% and 40% ³⁾.

Population-based analysis of the Surveillance Epidemiology and End Results (SEER) database confirms that Kadish staging, lymph node involvement, and age at diagnosis have significant prognostic value. These findings have been confirmed in a large meta-analysis recently published by Kane et al in 2010. Furthermore, higher Hyams grading (grades 3 and 4) correlate with a poorer prognosis. ^{4) 5)}.

Salvage treatment: For patients with recurrent disease, this typically occurs in 2 patterns: that of intracranial recurrence or those with distant metastases ^{6) 7)}.

Intracranial recurrence is typically treated with repeat transcranial resection; however, stereotactic radiosurgery is a viable option. ^{8) 9) 10)}.

In patients with distant metastases, those with cervical lymph node metastases should undergo modified radical neck dissection to understand the extent of the disease. This typically leads to chemotherapy, of which platinum-based therapies remain the standard of therapy at this time ^{11) 12) 13)}

In children overall, 5-year survival ranged from 44 to 91% with a median follow-up of 3-13 years. Children with esthesioneuroblastoma usually present at an advanced stage and undergo multi-modality therapy at a higher rate than adult patients. There is a wide range of documented overall survival though this lack of precision could be due to a paucity of patients ¹⁴⁾.

Vuong et al. verified that the Hyams grading system appears to be a reliable prognostic indicator to assess esthesioneuroblastoma outcomes. Consolidating the Hyams grading system into a three-tier system based on similar clinical outcomes of grades I and II may simplify this classification schema ¹⁵⁾.

In the era of intensity-modulated radiation therapy (IMRT), surgery followed by radiotherapy or chemoradiotherapy (S+R/RC) failed to improve the outcomes of patients with ENB. neoadjuvant chemotherapy followed by chemoradiotherapy (C+RC) may be a feasible treatment option for patients with ENB ¹⁶⁾.

The development of cervical nodal metastases and distant metastases had a significant adverse impact on prognosis. The value of the Kadish staging system was confirmed in a study, significantly correlating with prognosis. Tumor grade according to the Hyams staging system also seems to be an important factor in determining prognosis for tumor recurrence and survival. Aggressive multimodality therapeutic strategies, particularly CFR and adjuvant RT, yielded the best treatment outcome ¹⁷⁾.