Version 3.2021, 04/27/21 © 2021 National Comprehensive Cancer Network® (NCCN®), All rights reserved. NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN.
Note: All recommendations are category 2A unless otherwise indicated.
Clinical Trials: NCCN believes that the best management of any patient with cancer is in a clinical trial. Participation in clinical trials is especially encouraged.
Version 3.2021, 04/27/21 © 2021 National Comprehensive Cancer Network® (NCCN®), All rights reserved. NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN.
Note: All recommendations are category 2A unless otherwise indicated.
Clinical Trials: NCCN believes that the best management of any patient with cancer is in a clinical trial. Participation in clinical trials is especially encouraged.
Ethmoid Sinus Tumors
ETHM-2 CLINICAL
PRESENTATION PRIMARY TREATMENT ADJUVANT TREATMENT FOLLOW-UP
Follow-up (See FOLL-A)
Recurrent or persistent disease (See ADV-3) RTl
or Observeo for T1 only (category 2B) orConsider systemic therapy/RTl,m (category 2B) if adverse featuresp Resectionj,k (preferred)
or
Definitive RTl Newly diagnosed T1,T2
RTl
or Consider systemic therapy/RTl,m (category 2B) if adverse featuresp Resectionj,k
(preferred) or
Concurrent systemic therapy/RTl,m
or
Systemic therapyn (category 2B) Newly diagnosed T3,T4ai
Newly diagnosed T4b or patient declines surgery
i For SNUC with neuroendocrine features, small cell, high-grade olfactory
esthesioneuroblastoma, or SNEC histologies, systemic therapy should be a part of the overall treatment. Consider a clinical trial and referral to a major medical center that specializes in these diseases. See SYST-A.
j N+ neck disease is uncommon in ethmoid cancers, but, if present, requires neck dissection and appropriate risk-based adjuvant therapy.
k See Principles of Surgery (SURG-A).
l See Principles of Radiation Therapy (ETHM-A). For minor salivary gland tumors, see SALI-A.
m See Principles of Systemic Therapy for Non-Nasopharyngeal Cancers (SYST-A).
n Primary systemic therapy options for newly diagnosed T3,T4a ethmoid sinus tumor include etoposide/cisplatin (category 2B), or docetaxel/
cisplatin/fluorouracil (category 2B).
o Pathologic features: negative margins, favorable histology (including low grade), not located along the cribriform plate or medial wall of the orbit, no perineural invasion or lymphovascular space invasion.
p Adverse features include positive margins, close margins (tumors
adjacent to the cribriform plate and/or medial wall of the orbit), unfavorable histology (high grade, adenoid cystic), intracranial and/or intraorbital extension, cribriform plate location, medial wall of orbit location, perineural invasion, and lymphovascular space invasion. (See Discussion).
See ADV-1
See Post Systemic Therapy/RT or RT Neck Evaluation (FOLL-A, 2 of 2)
See Post Systemic Therapy/RT or RT Neck Evaluation (FOLL-A, 2 of 2) CR
Consider systemic therapy/RTl,m if adverse featuresp
orRTl (category 2B)
Consider systemic therapy/RTl,m orRTl (category 2B)
<CR Resectionk
Version 3.2021, 04/27/21 © 2021 National Comprehensive Cancer Network® (NCCN®), All rights reserved. NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN.
Note: All recommendations are category 2A unless otherwise indicated.
Clinical Trials: NCCN believes that the best management of any patient with cancer is in a clinical trial. Participation in clinical trials is especially encouraged.
Ethmoid Sinus Tumors
ETHM-3
i For SNUC with neuroendocrine features, small cell, high-grade olfactory esthesioneuroblastoma, or SNEC histologies, systemic therapy should be a part of the overall treatment. Consider a clinical trial and referral to a major medical center that specializes in these diseases. See SYST-A.
j N+ neck disease is uncommon in ethmoid cancers, but, if present, requires neck dissection and appropriate risk-based adjuvant therapy.
k See Principles of Surgery (SURG-A).
l See Principles of Radiation Therapy (ETHM-A). For minor salivary gland tumors, see SALI-A.
CLINICAL
PRESENTATION PRIMARY TREATMENTi ADJUVANT TREATMENTi FOLLOW-UP
Follow-up (See FOLL-A)
Recurrent or persistent disease (See ADV-3) Diagnosed after incomplete
resection (eg, polypectomy) and gross residual disease
Diagnosed after incomplete resection (eg, polypectomy) and no residual disease on physical exam, imaging, and/or endoscopy
Surgeryj,k (preferred), if feasible orRTl,q
orConcurrent systemic therapy/RTl,m
RTi,l
or Consider systemic therapy/RTl,m (category 2B) if adverse featuresp
RTl or
Surgery,j,k if feasible
RTl
or Observeo for T1 only (category 2B) orConsider systemic therapy/RTl,m (category 2B) if adverse featuresp
m See Principles of Systemic Therapy for Non-Nasopharyngeal Cancers (SYST-A).
o Pathologic features: negative margins, favorable histology (including low grade), not located along the cribriform plate or medial wall of the orbit, no perineural invasion or lymphovascular space invasion.
p Adverse features include positive margins, close margins (tumors adjacent to the cribriform plate and/or medial wall of the orbit), unfavorable histology (high grade, adenoid cystic), intracranial and/or intraorbital extension, cribriform plate location, medial wall of orbit location, perineural invasion, and lymphovascular space invasion.
(See Discussion).
q Primary RT is an option for minimal residual squamous cell carcinoma.
Version 3.2021, 04/27/21 © 2021 National Comprehensive Cancer Network® (NCCN®), All rights reserved. NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN.
Note: All recommendations are category 2A unless otherwise indicated.
Clinical Trials: NCCN believes that the best management of any patient with cancer is in a clinical trial. Participation in clinical trials is especially encouraged.
Ethmoid Sinus Tumors
ETHM-A
1 See Principles of Radiation Techniques (RAD-A) and Discussion.
2 In the paranasal sinus area, care should be taken to avoid critical neural structures;
therefore, 1.8 Gy/fraction can be considered.
3 For doses >70 Gy, some clinicians feel that the fractionation should be slightly modified (eg, <2.0 Gy/fraction for at least some of the treatment) to minimize toxicity. An additional 2–3 doses can be added depending on clinical circumstances.
4 Suggest 44–50 Gy in sequentially planned IMRT or 54–63 Gy with IMRT dose painting technique (dependent on dose per fraction).
PRINCIPLES OF RADIATION THERAPY1 DEFINITIVE:
RT Alone
• PTV
High risk: Primary tumor and involved lymph nodes [this includes possible local subclinical infiltration at the primary site and at the high-risk level lymph node(s)]
◊Fractionation:
– 66 Gy (2.2 Gy/fraction) to 70–70.2 Gy (1.8–2.0 Gy/fraction);
daily Monday–Friday in 6–7 weeks2,3 – Concomitant boost accelerated RT:
▪72 Gy/6 weeks (2 Gy once daily and then 1.8 Gy/fraction, large field; 1.5 Gy boost as second daily fraction during last 12 treatment days)
▪66–70 Gy (2.0 Gy/fraction; 6 fractions/wk accelerated) – Hyperfractionation: 81.6 Gy/7 weeks (1.2 Gy/fraction, twice
daily)
Low to intermediate risk: Sites of suspected subclinical spread
◊44–50 Gy (2.0 Gy/fraction) to 54–63 Gy (1.6–1.8 Gy/fraction)4,5 CONCURRENT SYSTEMIC THERAPY/RT:6
• PTV
High risk: typically 70–70.2 Gy (1.8–2.0 Gy/fraction); daily Monday–
Friday in 7 weeks2
Low to intermediate risk: 44–50 Gy (2.0 Gy/fraction) to 54–63 Gy (1.6–1.8 Gy/fraction)4,5
POSTOPERATIVE:
RT or Concurrent Systemic Therapy/RT6
• Preferred interval between resection and postoperative RT is ≤6 weeks
• PTV
High risk: Adverse features such as positive margins7
◊60–66 Gy (1.8–2.0 Gy/fraction); daily Monday–Friday in 6–6.5 weeks2
Low to intermediate risk: sites of suspected subclinical spread
◊44–50 Gy (2.0 Gy/fraction) to 54–63 Gy (1.6–1.8 Gy/fraction)4,5
Either IMRT or proton therapy is recommended for maxillary sinus or paranasal/ethmoid sinus tumors to minimize dose to critical structures.
5 Treatment to sites of suspected subclinical spread is not consistently performed at all institutions. (Le QT, Fu KK, Kaplan MJ, et al. Lymph node metastasis in maxillary sinus carcinoma. Int J Radiat Oncol Biol Phys 2000;46:541-549.)
6 See Principles of Systemic Therapy for Non-Nasopharyngeal Cancers (SYST-A).
7 Adverse features include positive margins, close margins (tumors adjacent to the cribriform plate and/or medial wall of the orbit), unfavorable histology (high grade, adenoid cystic), intracranial and/or intraorbital extension, cribriform plate location, medial wall of orbit location, perineural invasion, and lymphovascular space invasion. (See Discussion).
Version 3.2021, 04/27/21 © 2021 National Comprehensive Cancer Network® (NCCN®), All rights reserved. NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN.
Note: All recommendations are category 2A unless otherwise indicated.
Clinical Trials: NCCN believes that the best management of any patient with cancer is in a clinical trial. Participation in clinical trials is especially encouraged.