Esthesioneuroblastoma: Summary of Single-center Experiences with Focus on Adjuvant Therapy and Overall Survival

Abstract

Esthesioneuroblastoma (ENB) is a rare malignant tumor of the nasal cavity. The genetic basis of its development is still under study and has not been fully delineated. It has varying symptoms depending on the lesion’s location within the nasal cavity. The most commonly used systems for such lesions are the Kadish staging and Haymes grading systems. The objectives are to review the most recent published literature evaluating the different treatments/ combination of treatment and assess the most appropriate treatment modality that can provide the longest progression-free survival and overall survival for ENB patients. Moreover, a look at what the latest literature suggests when it comes to adjuvant treatments and their effect on survival is also key to further the body of knowledge for neurosurgeons, Ears, Nose, and Throat (ENT) physicians and all the different subspecialties that deal and serve these population of patients. The published literature was reviewed starting from 1990. The focus was made on single-center experiences given their availability and easy access. The most recently published systematic review was used as the benchmark; research published after that was included in this study. The database search in OVID was conducted using the following keywords: “Esthesioneuroblastoma”, “ENB”, “Olfactory Neuroblastoma”, Nose neoplasm”, skull base neoplasm”, “radiation”, and “resection”. The database search found 17 papers which included 14 single-center reports, one systematic review, and two nationwide multi-center reviews. Surgery plus adjuvant radiation therapy appears to provide the best overall survival and progression-free survival especially in patients with high Kadesh grade. On the other hand, surgery alone or biopsy followed with radiation therapy provided the lower progression-free survival and overall survival from time of diagnosis. The role of chemotherapy, however, requires further investigation to assess its potentially harmful effects. The use of surgery as a stand-alone modality of treatment should be cautiously and rarely used in patients with lower staging scores and multiple negative resection margins.

Survival Associations between Patient Age and Treatment Modality in Olfactory Neuroblastoma: A Retrospective Population-Based Study

“…However, the efficacy of CT treatment on ONB patient outcomes is largely speculated [ 8 ]. Some retrospective studies have reported positive patient outcomes in response to CT [ 17 , 18 ], while others have observed that CT is associated with poor patient outcomes [ 19 , 20 , 21 ], In a recent epidemiological survey, CT treatment was associated with poor ONB patient outcomes and reduced survival [ 21 ]. However, the long-term effects associated with CT and other treatment modalities on patient age groups remain unknown.…”

Radiographic predictors of occult intracranial involvement in olfactory neuroblastoma patients

“…Currently, small, low-grade tumors resected with negative margins can be managed without adjuvant radiation therapy; however, tumors with higher Hyam's grade or positive dura, olfactory bulb, and/or brain would typically be offered at least local radiation therapy. [38][39][40][41] If surgeons defer elective skull base dissection, approximately 12% of patients may be inadvertently downstaged, and adjuvant therapy may not be offered. Although neither the involvement of intracranial structure on imaging or pathology nor the specific treatment modalities were significant predictors of OS or DFS, 83% of our multicenter cohort did receive radiation therapy, which introduces potential selection bias.…”

“…There are additional treatment implications to consider if surgeons decide to defer elective skull base resection, specifically regarding adjuvant therapy. Currently, small, low‐grade tumors resected with negative margins can be managed without adjuvant radiation therapy; however, tumors with higher Hyam's grade or positive dura, olfactory bulb, and/or brain would typically be offered at least local radiation therapy 38–41 . If surgeons defer elective skull base dissection, approximately 12% of patients may be inadvertently downstaged, and adjuvant therapy may not be offered.…”

Facial disfigurement due to olfactory neuroblastoma: beauty regained with chemotherapy

“…A review of 17 studies published from 1990 until 2019 showed that surgery and adjuvant radiation therapy provided the best overall survival and progression‐free survival especially in patients with high Kadish stage. The role of chemotherapy was not clear 6 . A recent review of 149 articles also suggested surgical resection followed by radiotherapy as the standard approach for higher‐grade tumors 7 …”

“…The role of chemotherapy was not clear. 6 A recent review of 149 articles also suggested surgical resection followed by radiotherapy as the standard approach for higher-grade tumors. 7 A beneficial role of chemotherapy in ONB has been suggested owing to its biological behavior similar to other chemosensitive neural crest tumors like high-grade neuroendocrine tumors.…”