Esthesioneuroblastoma
Esthesioneuroblastoma | |
---|---|
Other names | Neuroblastoma |
Esthesioneuroblastoma | |
Specialty | Neuro-oncology |
Esthesioneuroblastoma is a rare cancer of the nasal cavity. Arising from the upper nasal tract, esthesioneuroblastoma is believed to originate from sensory neuroepithelial cells, also known as neuroectodermal olfactory cells.[1]
Due to the location of the tumor and its proximity to the cranial cavity, esthesioneuroblastoma can be highly invasive and challenging to treat. There is no consensus on an appropriate treatment approach of esthesioneuroblastoma because of the rarity of the disease. Most studies reported cranial surgical resection with radiotherapy or chemotherapy to target the tumor.[2]
Signs and symptoms
Esthesioneuroblastoma frequently first presents as a nasal mass.
Genetics
There is limited research on the genetic role in esthesioneuroblastoma development. Of the research to date, the
Mutations in
Pathophysiology
Esthesioneuroblastoma is of neurocrest origin, arising from olfactory sensory cells in the
Hyam's histopathological grades for esthesioneuroblastoma[11]
Grade | Lobular architexture preservation | Mitotic index | Nuclear polymorphism | Fibrillary matrix | Rosettes | Necrosis |
---|---|---|---|---|---|---|
I | + | none | none | prominent | Homer Wright (HW) rosettes |
none |
II | + | low | moderate | present | HW rosettes | none |
III | +/- | moderate | prominent | low | Flexner-Wintersteiner rosettes |
rare |
IV | +/- | high | marked | absent | none | frequent |
Diagnosis
Esthesioneuroblastoma can resemble small blue cell tumors like
Staging
The Kadish classification is used for clinical classification of sinonasal tumors including esthesioneuroblastoma. Subsequent research articles have been published to determine prognosis based on tumor grade.
Modified Kadish classification[13][4]
Stage | Description | 5 year survival |
---|---|---|
A | Tumor confined to nasal cavity | 75–91 |
B | Nasal cavity and paranasal sinuses | 68–71 |
C | Tumor extends beyond nasal cavity and paranasal sinuses, including skull base, orbit or cribiform plate | 41–47 |
D | Tumor metastasizes to cervical lymph nodes and beyond | <40 |
Dulguerov classification[14]
Stage | Characteristics |
---|---|
T1 | Tumour involving the ethmoidal cells
|
T2 | Tumour involving the nasal cavity and/or paranasal sinuses (including the sphenoid) with extension to or erosion of the cribriform plate |
T3 | Tumour extending into the orbit or protruding into the anterior cranial fossa, without dural invasion |
T4 | Tumour involving the brain |
N0 | No cervical lymph-node metastasis |
N1 | Any form of cervical lymph-node metastasis |
M0 | No metastases |
M1 | Distant metastasis |
Treatment
The preferred treatment for esthesioneuroblastoma is surgery followed by
Surgical approaches
Several surgical approaches have been described,
Radiotherapy
Radiotherapy alone is reserved only for small lesions not appropriate for either surgery or chemotherapy.[4] Both photon and proton radiotherapy have been used effectively to treat esthesioneuroblastoma.[14][21] Proton radiotherapy has recently been shown to be effective in a 10-person study with Kadish C tumors, while delivering less toxicity to the nervous system.[21]
Chemotherapy
Chemotherapy is used in a multimodality treatment plan generally for more advanced, unresectable or reoccurring tumors.[4] Cyclophosphamide, vincristine and doxorubicin have been used as neoadjuvant chemotherapy drugs for grade C esthesioneuroblastoma before surgical resection, producing fair outcomes. Cisplatin and etoposide are often used to treat esthesioneuroblastoma as neoadjuvants or adjuvants with radiotherapy or surgery.[22][23][24]
Study results are promising. In advanced stage esthesioneuroblastoma in pediatric patients, where surgery is no longer possible, aggressive chemotherapy and radiotherapy has resulted in some tumor control and long-term survival.[25]
Prognosis
Esthesioneuroblastoma is a slow developing but malignant tumor with high recurrence rates because of its anatomical position.
Survival rates for treated esthesioneuroblastoma are best for surgery with radiotherapy (65%), then for radiotherapy and chemotherapy (51%), just surgery (48%), surgery, radiotherapy and chemotherapy (47%) and finally just radiotherapy (37%).[14] From the literature, radiotherapy and surgery seem to boast the best outcome for patients. However, it is important to understand that to some degree, prognosis is related to tumor severity. More progressed, higher grade tumors would result in chemotherapy or radiotherapy as the only treatment. It is no surprise that the prognosis would be worse in these cases.[citation needed]
Incidence
Esthesioneuroblastoma accounts for 2% of all intranasal tumors with an incidence of 0.4 cases per million people.[1] Fewer than 700 cases have been documented in the United States.[12] Fewer than 400 unique cases have been reported globally.[4][1] Esthesioneuroblastoma can occur at any time, with peak occurrence reported in the second and sixth decades of life.[1]
History
Esthesioneuroblastoma was first characterized in 1924.[27][28]
Notable cases
The disease was brought into prominence by the case of Chantal Sébire, who was suffering from the disease and ended her life after being denied euthanasia.[29]
References
- ^ a b c d e f g h i j k Thompson LD (2009). Olfactory neuroblastoma. Head and neck pathology. 3: 252–259
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- ^ Fordice JO (3 March 1994). "Esthesioneuroblastoma". Baylor College of Medicine. Archived from the original on April 9, 2007. Retrieved 2008-03-22.
- ^ Berger L, Luc G, Richard D (1924). "L'esthésioneuroépithéliome olfactif". Bull Assoc Franç Étude Cancer. 13: 410–421.
- ^ "BBC NEWS - Europe - Tumour woman's death not natural". BBC. 21 March 2008. Retrieved 1 November 2016.