3/1/19

What?
• Esthesioneuroblastoma (ENB)
• Olfactory neuroblastoma (ONB)

Esthesioneuroblastoma • Rare malignant neuroectodermal cancer

• Arises from olfactory neuroepithelium surrounding
the olfactory nerve
• Accounts for ~5% of all nasal cavity & sinus cancers
CARLY DUGAN
9/14/2018

http://www.theantiseptic.in/uploads/medicine/
Olfactory%20Neuroblastoma%20-
%20A%20Rare%20Sinonasal%20Malignancy.jpg

Olfactory Nerve Histopathology

• Four main cells in the nasal cavity
• Squamous epithelial cells
• Glandular cells
• Immune system cells
• Nerve cells

•Neuroectodermal Tumors
• Small, blue round cells
• Group I: With epithelial differentiation, i.e. sinonasal undifferentiated carcinoma
• Group II: Without epithelial differentiation, i .e. olfactory neuroblastoma

• Olfactory neuroblastoma cells present as sheets of small, round nuclei

http://histonano.com/books/Junqueira%27s%2
https://www.semanticscholar.org/paper/HEAD-AND-NECK-PRE-
0Basic%20Histology%20PDF%20WHOLE%20BO MEETING-OF-THE-22-N-D-EUROPEAN-OF-
OK/17.%20The%20Respiratory%20System.htm Thompson/552e82d429b20c47a6733a0053f2c658360c159a/figure/8

Lymph Nodes & Spread Etiology & Epidemiology

• Often spreads by direct invasion
• Will metastasize to regional lymph nodes • Four cases per ten million people
• Exact cause of olfactory neuroblastoma is • 55:45 male to female ratio
unknown
• IA: Submental nodes
• IB: Submandibular nodes
• Race is not a factor
• II: Internal jugular chain (upper) • Most common in adults 40– 70 years old
• Exposure to harmful chemicals/ particles
• III: Internal jugular chain (middle) • Has been seen in ages 3 – 90 years old
• Tobacco smoke
• IV: Internal jugular chain (lower)
• Median age 53
• V: Spinal accessory nodes
•VI: Pretracheal nodes
•VII: Mediastinal nodes

Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org, rID: 9618

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Our Patient Presentation

• 55 year old African-American female • Diabetes Mellitus (DM) • Right neck mass following dental implants • Typical Presentation:
• Treated at James Vault 6 (2250) • Gastrointestinal Reflux Disease (GERD) • Nasal congestion that inhibited sleep • Stuffiness & congestion
• Epistaxis
• Severe epistaxis • Nasal drainage in the throat
• Former cigarette smoker • Hysterectomy • Ear pain & pressure
• Headaches
• Occasional alcohol use • Right knee arthroscopy • Enlarged lymph nodes in neck
• Decreased change of smell
• Married
• Loss or change of vision
• Two children • Father had colorectal cancer
• Mother had diabetes

https://www.istockphoto.com/photos/stacked-
files?sort=mostpopular&mediatype=photograp
hy&phrase=stacked%20files

Diagnostic Workup
•Clinical Exam
• Revealed a “polypoid lesion medial to the right turbinate”
•Ultrasound
• Lymph nodes evaluated- found three nodules in the right neck, largest at 3 cm
•Biopsy
• Fine needle aspiration (FNA) of right lymph node was positive for neuroendocrine
carcinoma
• Diagnostic endoscopic sinus surgery (ESS) performed- showed a large sinonasal tumor
on the right side that was ”destructive in nature and vascular in appearance”
• Ruled out undifferentiated sinonasal carcinoma, and confirmed a high grade olfactory
neuroblastoma
•M RI & CT
• Revealed a large “bilobed mass” centered on the cribriform plate, extension into
inferior right frontal lobe and sinonasal cavity, and also extensive nodal disease

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Kadish/ Morita Staging Hyams Grading

• Stage A: • Grade I:
• Tumor is confined to the nasal cavity • No mitotic figures
• Stage B: • Grade II:
• Tumor is confined to the nasal cavity and paranasal sinuses Kadish Stage D • Some mitotic figures Hyams Grade III-IV
• Stage C: • Grade III:
• Tumor extends beyond the nasal cavity and paranasal sinuses • Prominent mitotic figures
• Stage D: • Grade IV:
• Regional lymph node or distant metastasis • Marked mitotic figures

Disease Prognosis & Survival Treatment Options

• Surgery
• Primary treatment
• Tend to present at later stages
• Endoscopic sinus surgery (ESS)
• Grade is the greatest prognostic factor
• Radiation Therapy
• Average five-year survival: ~75% • Often used to maintain local control
• Low grade ENB: 86% • IMRT to avoid critical structures
• High grade ENB: 53%
• Metastatic ENB declines • Chemotherapy
• Unsure if Cisplatin-based chemotherapy is useful

https://www.alamy.com/stock-photo/endoscopic.html

Treatment Plan Overview Treatment Prescription

• Tx Energy: 6 MV
ü Chemotherapy • Technique: IMRT/VMAT with SIB
ü Induction cisplatin-etoposide
ü Four cycles ü Surgery • Total Dose: 6600 cGy (PTV66)
ü Concurrent carboplatin-paclitaxel ü Endoscopic sinus surgery 6000 cGy (PTV60)
5600 cGy (PTV56)
ü Weekly ü Definitive resection and craniotomy
ü Negative margins were unable to be obtained
• Fractions: 33 fractions
ü External Beam Radiation Therapy
ü VMAT/ IMRT • Dose/ Fraction: 200 cGy/ fx
ü 6 MV photons • Frequency: 5 days/ week
• Imaging: MV/ kV orthogs daily
CBCT/ daily

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Treatment Parameters Simulation & Patient Set-Up

• Extends from anterior cranial fossa to supraclav • Supine on conformal board • Had to take out both shims in the first week
• 6600 cGy: • Long Aquaplast mask • Dental stent made things difficult
• right nasal cavity, left retropharyngeal nodes, &
• ”C” headrest • Oxygen prescription of 2L helped with anxiety
right level II nodes
• 2 x 1 mm shims
• 6000 cGy:
• anterior cranial fossa, nasopharynx, nasal cavity, • Arms at sides holding pegs
bilateral level IB nodes, bilateral level II nodes, right
level III nodes, and level V • Custom dental stent
• 5600 cGy • Knee sponge
• left level III nodes, left level IV nodes, left level V
nodes, and bilateral supraclav

https://www.amazon.com/60pcs-Untreated-Unflavored-Disposable-Sponge/dp/B01E5A4W9A

http://www.karibuadventure.com/oxygen/

Imaging & Alignment Treatment Portals

• CBCT/ daily
• Shifted 3 cm superior daily
• M V/kV orthogs 2x/ weekly
• Turned into MV/kV daily

Treatment Portals Critical Structures

• Lung: 1750 cGy
• Parotid glands: 2300 cGy
• Spinal cord: 4700 cGy
• Esophagus: 5500 cGy
• Lens: 1000 cGy
• Brain: 4500 cGy
• Optic nerves: 5000 cGy
• Optic chiasm: 5000 cGy
• Brainstem: 5000 cGy

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Treatment Side Effects

• Short Term: •Long Term:
• Fatigue • Blood vessel damage
• Skin erythema • Nerve damage
• Managed with moisturizers and Silvadene • Decreased hearing
• Dysphagia/ Odynophagia • Spinal cord injury
• Managed with narcotics
• Thyroid problems
• Xerostomia
• Scar tissue in irradiated areas
• Managed with antiemetics and Mucinex
• Mucositis
• Managed with salt/ soda rinses & mouthwash
• Impaired vision
• Weight loss • Migraines
• Patient had a PEG tube placed • Memory problems
• Alopecia • Impaired speech

This Patient Works Cited

Agarwal A. Olfactory Nerve. Know Your Body. March 2018.
Bell D, Saade R, Roberts D, et al. Prognostic Utility of Hyams Histological Grading and
Kadish-Morita Staging Systems for Esthesioneuroblastoma Outcomes. Head and Neck
Pathology. 2015;9(1):51-59. doi:10.1007/s12105-014-0547-3.
Kennedy D, Hwang P. Rhinology: disease of the nose, sinuses, and skull base. Thieme. New
York. 2012.
Khademi B, Safari S, Hosseini S, Mohammadianpanah M. Olfactory Neuroblastoma: A 15-
Year Single Institution Experience. Radiotherapy Oncology. 2015 ;2(3):e4472
Olfactory Neuroblastoma. Johns Hopkins Medicine. 2018.
Gaillard F. Lymph node levels in the neck. Radiopaedia. 2018.