The first 60 days: Esthesioneuroblastoma (olfactory neuroblastoma)
Esthesioneuroblastoma is a rare cancer of the nasal cavity and sinuses that arises from the smell-sensing olfactory nerve lining at the roof of the nose, next to the brain. It is treated with surgery through the nose or skull base followed by radiotherapy, with chemotherapy added for high-grade or widespread tumours, and because it can return a decade or more later patients are followed for life. Below, week by week, is what OnCo's record of Esthesioneuroblastoma (olfactory neuroblastoma) says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Endoscopic biopsy with immunohistochemistry, MRI and CT of the sinuses and skull base, neck imaging, DOTATATE PET where available.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- PathologistNamed in the standard of care for: Diagnosis and staging.
- RadiologistNamed in the standard of care for: Diagnosis and staging, Follow-up.
- SurgeonNamed in the standard of care for: Resectable disease, High-grade or advanced disease (Hyams III to IV, Kadish C to D), Recurrent or metastatic disease.
- Medical oncologistNamed in the standard of care for: Resectable disease, High-grade or advanced disease (Hyams III to IV, Kadish C to D), Recurrent or metastatic disease.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Resectable disease, High-grade or advanced disease (Hyams III to IV, Kadish C to D), Recurrent or metastatic disease.
- Transplant and cell therapy teamNamed in the standard of care for: High-grade or advanced disease (Hyams III to IV, Kadish C to D).
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
Endoscopic endonasal or craniofacial resection with skull base reconstruction by a skull base team, followed by postoperative radiotherapy (intensity-modulated or proton) for nearly all patients.
- 2.High-grade or advanced disease (Hyams III to IV, Kadish C to D)NCCN Guidelines: Head and Neck Cancers (ethmoid and maxillary sinus tumours)
Induction cisplatin and etoposide, then surgery and radiotherapy or definitive chemoradiotherapy; elective neck irradiation considered.
- 3.Recurrent or metastatic diseaseNCCN Guidelines: Head and Neck Cancers (ethmoid and maxillary sinus tumours)
Repeat surgery or re-irradiation for local relapse; lutetium-177 dotatate for somatostatin-receptor-positive disease; platinum-etoposide or temozolomide; trials.
MRI surveillance indefinitely because of late relapse; management of anosmia, cerebrospinal fluid leak and visual effects.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Hyams grade, Kadish or modified Kadish stage, Synaptophysin, chromogranin and S100 sustentacular cell pattern, Somatostatin receptor expression and DOTATATE PET uptake, IDH2 mutation), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Low-grade esthesioneuroblastoma, High-grade esthesioneuroblastoma, Kadish A to B esthesioneuroblastoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Diagnosis and staging
- For my situation (diagnosis and staging), which of the standard options do you recommend and why?Guideline options include: Endoscopic biopsy with immunohistochemistry, MRI and CT of the sinuses and skull base, neck imaging, DOTATATE PET where available.
Resectable disease
- For my situation (resectable disease), which of the standard options do you recommend and why?Guideline options include: Endoscopic endonasal or craniofacial resection with skull base reconstruction by a skull base team, followed by postoperative radiotherapy (intensity-modulated or proton) for nearly all patients.
High-grade or advanced disease (Hyams III to IV, Kadish C to D)
- For my situation (high-grade or advanced disease (hyams iii to iv, kadish c to d)), which of the standard options do you recommend and why?Guideline options include: Induction cisplatin and etoposide, then surgery and radiotherapy or definitive chemoradiotherapy; elective neck irradiation considered.
- Am I a candidate for Cisplatin, Etoposide, Platinum + etoposide (EP / CE), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Recurrent or metastatic disease
- For my situation (recurrent or metastatic disease), which of the standard options do you recommend and why?Guideline options include: Repeat surgery or re-irradiation for local relapse; lutetium-177 dotatate for somatostatin-receptor-positive disease; platinum-etoposide or temozolomide; trials.
- Am I a candidate for Lutetium-177 dotatate, Platinum + etoposide (EP / CE), Temozolomide, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Follow-up
- For my situation (follow-up), which of the standard options do you recommend and why?Guideline options include: MRI surveillance indefinitely because of late relapse; management of anosmia, cerebrospinal fluid leak and visual effects.
Any stage
- Are there clinical trials I could join, for example of Proton therapy, Lutetium-177 dotatate, Peptide receptor radionuclide therapy (PRRT)?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “No prospective trial has ever been conducted; every recommendation rests on series”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Late relapse after ten or more years makes cure hard to define”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Esthesioneuroblastoma (olfactory neuroblastoma): the full pageEsthesioneuroblastoma is a rare cancer of the nasal cavity and sinuses that arises from the smell-sensing olfactory nerve lining at the roof of the nose, next to the brain. It is treated with surgery through the nose or skull base followed by radiotherapy, with chemotherapy added for high-grade or widespread tumours, and because it can return a decade or more later patients are followed for life.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- Head and neck subsites (oral cavity, oropharynx, larynx): Head and neck cancer is really several cancers named by exact location: mouth (oral cavity), back of the throat (oropharynx, where HPV cancers arise), voice box (larynx), lower throat (hypopharynx) and behind the nose (nasopharynx).
- Disease-specific staging and risk systems (FIGO, Ann Arbor, IPI, R-ISS, ELN, IMDC): Beyond the generic TNM system, gynaecological cancers (FIGO), lymphoma (Ann Arbor, IPI), myeloma (R-ISS), AML (ELN), kidney cancer (IMDC), neuroblastoma (INRG) and CLL (Rai, Binet) each have their own system that combines stage, blood tests, genetics and fitness into risk groups.
- Re-irradiation: Giving radiotherapy again to a region that has already been treated, once thought impossible because normal tissues remember the first dose.
- Rare cancers: Rare cancers are those with fewer than about 6 new cases per 100,000 people per year.
- Chemoradiation (chemoradiotherapy, CRT): Radiotherapy given at the same time as chemotherapy, which sensitises the cancer to radiation.
Every term links to the glossary.