Long-Term Survival Outcomes and Treatment Experience of 64 Patients With Esthesioneuroblastoma

Abstract

Background: Esthesioneuroblastoma (ENB) is a rare sinonasal malignancy, lacking a unified staging system and treatment. Management at a single center was retrospectively evaluated to inform future treatment options and prognostic factors.Methods: Clinical data of 64 consecutive ENB patients, including prognostic factors and treatment methods, were reviewed retrospectively. Data were collected to calculate overall survival (OS) and progression free survival (PFS).Results: The majority of tumors 84.4% were within Kadish C stage, 79.7% were within T3 or T4, and 64.0% were within Hyams grade III or IV. A total of 50 (78.1%) patients received surgery and combined radiotherapy with or without chemotherapy, 10 (15.6%) received surgery with or without chemotherapy alone, and 4 (6.3%) received radiotherapy with or without chemotherapy alone. The majority of patients (79.7%) underwent endoscopic resection (endoscopic and endoscopically assisted). Surgery combined with radiotherapy with or without chemotherapy resulted in significantly better OS (84.4 vs. 50.6%, 84.4 vs. 37.5%) compared to surgery alone and radiotherapy alone (P = 0.0064). Endoscopic surgery group (endoscopic and endoscopically assisted) resulted in significantly better 5-year PFS (61.7 vs. 22.2%) compared to the open surgery group (P < 0.001). Although endoscopic surgery group was not a statistically significant predictor of 5-year OS (P = 0.54), the 5-year OS was 79.3% for the endoscopic surgery group and 76.2% for the open surgery group. A Cox regression analysis identified intracranial extension and surgery combined with radiotherapy as independent factors affecting 5-year OS while cervical lymph node metastasis and Hyams grade IV as independent factors affecting 5-year PFS.Conclusion: Our findings suggest that surgery combined with radiotherapy is the best treatment approach for ENB. For advanced tumors, endoscopic surgery is an effective treatment, and its survival rate is equal to or better than open surgery.

Long‐term oncologic outcomes in esthesioneuroblastoma: An institutional experience of 143 patients

“…As demonstrated on multivariable analysis, high Hyams grade was independently associated with worse OS, PFS, and DMFS; and high mKadish staging was independently associated with worse OS and PFS. This strengthens previous large database studies reported by Tajudeen et al and Zeng et al that have demonstrated a negative prognostic value of high Hyams grade on oncologic outcomes 9,18,25 . Additionally, Sun et al, Song et al, Van Gompel et al, and Tajudeen et al demonstrated a negative prognostic value of high mKadish stage on oncologic outcomes 9,18,25,27 .…”

“…This strengthens previous large database studies reported by Tajudeen et al and Zeng et al that have demonstrated a negative prognostic value of high Hyams grade on oncologic outcomes. 9,18,25 Additionally, Sun et al, Song et al, Van Gompel et al, and Tajudeen et al demonstrated a negative prognostic value of high mKadish stage on oncologic outcomes. 9,18,25,27 This is in contrast to a study of 124 patients by Bell et al that did not demonstrate a relationship between mKadish stage and OS, but higher Hyams grade was associated with worsened DFS.…”

“…9,18,25 Additionally, Sun et al, Song et al, Van Gompel et al, and Tajudeen et al demonstrated a negative prognostic value of high mKadish stage on oncologic outcomes. 9,18,25,27 This is in contrast to a study of 124 patients by Bell et al that did not demonstrate a relationship between mKadish stage and OS, but higher Hyams grade was associated with worsened DFS. 28 On multivariable analysis, negative margin status was a positive prognostic factor for PFS.…”

Lymph node metastasis from olfactory neuroblastoma at presentation and as disease relapse: A systematic review and proportion meta‐analysis of prevalence data and variables influencing regional control

“…All studies except three 24,29,33 reported Kadish C as the most frequent stage at diagnosis. Similarly, pT3 22,28,32 and pT4 2,25,35 were most frequently reported as the prevalent T‐categories at presentation. Details on Hyams grade were provided only by four 8,28,32,35 studies, with three 8,28,32 showing the Grade II as the most frequent.…”

“…Similarly, pT3 22,28,32 and pT4 2,25,35 were most frequently reported as the prevalent T‐categories at presentation. Details on Hyams grade were provided only by four 8,28,32,35 studies, with three 8,28,32 showing the Grade II as the most frequent. Four major treatment protocols were applied throughout the studies included.…”