General Information About Paranasal Sinus and Nasal Cavity Cancer

Incidence and Mortality

Most tumors of the paranasal sinuses present with advanced disease, and cure rates are generally poor (≤50%). Squamous cell carcinoma (SCC) is the most frequent type of malignant tumor in the nose and paranasal sinuses (70%–80%). Papillomas are distinct entities that may undergo malignant degeneration. The cancers grow within the bony confines of the sinuses and are often asymptomatic until they erode and invade adjacent structures.[1,2,3]

Nodal involvement is infrequent. Metastases from both the nasal cavity and paranasal sinuses may occur, and distant metastases are found in 20% to 40% of patients who do not respond to treatment. However, locoregional recurrence accounts for most cancer deaths because most patients die of direct extension into vital areas of the skull or of rapidly recurring local disease.

Cancers of the maxillary sinus are the most common of the paranasal sinus cancers. Tumors of the ethmoid sinuses, nasal vestibule, and nasal cavity are less common, and tumors of the sphenoid and frontal sinuses are rare.

Anatomy

The major lymphatic drainage route of the maxillary antrum is through the lateral and inferior collecting trunks to the first station submandibular, parotid, and jugulodigastric nodes and through the superoposterior trunk to retropharyngeal and jugular nodes.

Clinical Evaluation and Follow-Up

Pretreatment evaluation and staging, as well as the need for multidisciplinary planning of treatment, is very important. Generally, the first opportunity to treat patients with head and neck cancers is the most effective, although salvage surgery or salvage radiation therapy, as appropriate, may occasionally be successful.

Because most treatment failures occur within 2 years, patients must be monitored frequently and meticulously during this period. Lifetime follow-up is essential because nearly 33% of these patients develop second primary cancers in the aerodigestive tract.

Carcinogenesis and Risk Factors

Data indicate that various industrial exposures may be related to cancer of the paranasal sinus and nasal cavity. The risk of a second primary head and neck tumor is considerably increased.[4] A study has shown that a subgroup of paranasal sinus and nasal cavity SCCs are associated with human papilloma virus (HPV) infection and that HPV-positive patients may have a better prognosis than those who are HPV negative.[5]

References:

1. Mendenhall WM, Werning JW, Pfister DG: Treatment of head and neck cancer. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Lippincott Williams & Wilkins, 2011, pp 729-80.
2. Laramore GE, ed.: Radiation Therapy of Head and Neck Cancer. Springer-Verlag, 1989.
3. Thawley SE, Panje WR, Batsakis JG, et al., eds.: Comprehensive Management of Head and Neck Tumors. 2nd ed. WB Saunders, 1999.
4. Johns ME, Kaplan MJ: Advances in the management of paranasal sinus tumors. In: Wolf GT, ed.: Head and Neck Oncology. Martinus Nijhoff Publishers, 1984, pp 27-52.
5. Alos L, Moyano S, Nadal A, et al.: Human papillomaviruses are identified in a subgroup of sinonasal squamous cell carcinomas with favorable outcome. Cancer 115 (12): 2701-9, 2009.

Cellular Classification of Paranasal Sinus and Nasal Cavity Cancer

The most common cell type for paranasal sinus and nasal cavity cancers is squamous cell carcinoma. Minor salivary gland tumors comprise 10% to 15% of these neoplasms. Malignant melanoma presents in less than 1% of neoplasms in this region. Some 5% of cases are malignant lymphomas.[1,2]

Esthesioneuroepithelioma, sometimes confused with undifferentiated carcinoma or undifferentiated lymphoma, arises from the olfactory nerves.[3]

Chondrosarcoma, osteosarcoma, Ewing sarcoma, and most soft tissue sarcomas have been reported for this region.

Inverting papilloma is considered a low-grade benign tumor with a tendency to recur and, in a small percentage of cases, to transform into a malignant tumor.

Midline granuloma, a progressively destructive condition, also involves this region.

References:

1. Mendenhall WM, Werning JW, Pfister DG: Treatment of head and neck cancer. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Lippincott Williams & Wilkins, 2011, pp 729-80.
2. Goldenberg D, Golz A, Fradis M, et al.: Malignant tumors of the nose and paranasal sinuses: a retrospective review of 291 cases. Ear Nose Throat J 80 (4): 272-7, 2001.
3. Jethanamest D, Morris LG, Sikora AG, et al.: Esthesioneuroblastoma: a population-based analysis of survival and prognostic factors. Arch Otolaryngol Head Neck Surg 133 (3): 276-80, 2007.

Stage Information for Paranasal Sinus and Nasal Cavity Cancer

The staging systems are clinical estimates of the extent of disease. The assessment of the tumor is based on inspection, palpation, and direct endoscopy when necessary. The tumor must be confirmed histologically, and any other pathological data obtained on biopsy may be included. The appropriate nodal drainage areas are examined by careful palpation. Computed tomographic and/or magnetic resonance imaging studies are generally required to adequately evaluate tumor extent before surgical resection or definitive radiation therapy. If a patient's disease relapses, complete restaging must be done to select the appropriate additional therapy.[1,2]

American Joint Committee on Cancer (AJCC) Stage Groupings and TNM Definitions

Staging for nasal cavity and paranasal sinus carcinomas is not as well established as staging for other head and neck tumors. For cancer of the maxillary sinus, the nasal cavity, and the ethmoid sinus, the AJCC has designated staging by TNM (tumor, node, metastasis) classification. Lymphomas, sarcomas, and mucosal melanomas of the paranasal sinuses and nasal cavity are not staged using this system.[3] The staging described below is used only for patients who have not had a lymph node dissection of the neck.

Table 1. Definition of Primary Tumor (T)^a

T Category	Maxillary Sinus T Criteria	Nasal Cavity and Ethmoid Sinus T Criteria
a Reprinted with permission from AJCC: Nasal cavity and paranasal sinuses. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 137–47.
TX	Primary tumor cannot be assessed.	Primary tumor cannot be assessed.
Tis	Carcinomain situ.	Carcinomain situ.
T1	Tumor limited to maxillary sinus mucosa with no erosion or destruction of bone.	Tumor restricted to any one subsite, with or without bony invasion.
T2	Tumor causing bone erosion or destruction including extension into the hard palate and/or middle nasal meatus, except extension to posterior wall of maxillary sinus and pterygoid plates.	Tumor invading two subsites in a single region or extending to involve an adjacent region within the nasoethmoidal complex, with or without bony invasion.
T3	Tumor invades any of the following: bone of the posterior wall of maxillary sinus, subcutaneous tissues, floor or medial wall of orbit, pterygoid fossa, ethmoid sinuses.	Tumor extends to invade the medial wall or floor of the orbit, maxillary sinus, palate, or cribriform plate.
T4	Moderately advanced or very advanced local disease.	Moderately advanced or very advanced local disease.
–T4a	Moderately advanced local disease. Tumor invades anterior orbital contents, skin of cheek, pterygoid plates, infratemporal fossa, cribriform plate, sphenoid or frontal sinuses.	Moderately advanced local disease. Tumor invades any of the following: anterior orbital contents, skin of nose or cheek, minimal extension to anterior cranial fossa, pterygoid plates, sphenoid or frontal sinuses.
–T4b	Very advanced local disease. Tumor invades any of the following: orbital apex, dura, brain, middle cranial fossa, cranial nerves other than maxillary division of trigeminal nerve (V2), nasopharynx, or clivus.	Very advanced local disease. Tumor invades any of the following: orbital apex, dura, brain, middle cranial fossa, cranial nerves other than V2, nasopharynx, or clivus.

Table 2. Definition of Regional Lymph Node (N)^a

N Category	Clinical Node (cN) Criteria
ENE = extranodal extension.
a Reprinted with permission from AJCC: Nasal cavity and paranasal sinuses. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 137–47.
Note: A designation of "U" or "L" may be used for any N category to indicate metastasis above the lower border of the cricoid (U) or below the lower border of the cricoid (L).
NX	Regional lymph nodes cannot be assessed.
N0	No regional lymph node metastasis.
N1	Metastasis in a single ipsilateral lymph node, ≤3 cm in greatest dimension and ENE(‒).
N2	Metastasis in a single ipsilateral node >3 cm but ≤6 cm in greatest dimension and ENE(‒);or metastases in multiple ipsilateral lymph nodes, none >6 cm in greatest dimension and ENE(‒);or in bilateral or contralateral lymph nodes, none >6 cm in greatest dimension and ENE(‒).
–N2a	Metastasis in a single ipsilateral node >3 cm but ≤6 cm in greatest dimension and ENE(‒).
–N2b	Metastases in multiple ipsilateral nodes, none >6 cm in greatest dimension and ENE(‒).
–N2c	Metastases in bilateral or contralateral lymph nodes, none >6 cm in greatest dimension and ENE(‒).
N3	Metastasis in a lymph node >6 cm in greatest dimension and ENE(‒);or metastasis in any node(s) with clinically overt ENE(+).
–N3a	Metastasis in a lymph node >6 cm in greatest dimension and ENE(‒).
–N3b	Metastasis in any node(s) with clinically overt ENE (ENEc).

Table 3. Definition of Distant Metastasis (M)^a

M Category	M Criteria
a Reprinted with permission from AJCC: Nasal cavity and paranasal sinuses. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 137–47.
M0	No distant metastasis (no pathologic M0; use clinical M to complete stage group).
M1	Distant metastasis.

Table 4. Definition of TNM Stage 0^a

Stage	TNM	Description
T = primary tumor; N = regional lymph node; M = distant metastasis.
a Reprinted with permission from AJCC: Nasal cavity and paranasal sinuses. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 137–47.
0	Tis, N0, M0	Tis = See Table 1.
		N0 = No regional lymph node metastasis.
		M0 = No distant metastasis (no pathological M0; use clinical M to complete stage group).

Table 5. Definition of TNM Stage I^a

Stage	TNM	Description
T = primary tumor; N = regional lymph node; M = distant metastasis.
a Reprinted with permission from AJCC: Nasal cavity and paranasal sinuses. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 137–47.
I	T1, N0, M0	T1 = See Table 1.
		N0 = No regional lymph node metastasis.
		M0 = No distant metastasis (no pathological M0; use clinical M to complete stage group).

Table 6. Definition of TNM Stage II^a

Stage	TNM	Description
T = primary tumor; N = regional lymph node; M = distant metastasis.
a Reprinted with permission from AJCC: Nasal cavity and paranasal sinuses. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 137–47.
II	T2, N0, M0	T2 = See Table 1.
		N0 = No regional lymph node metastasis.
		M0 = No distant metastasis (no pathological M0; use clinical M to complete stage group).

Table 7. Definitions of TNM Stage III^a

Stage	TNM	Description
T = primary tumor; N = regional lymph node; M = distant metastasis; ENE = extranodal extension.
a Reprinted with permission from AJCC: Nasal cavity and paranasal sinuses. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 137–47.
III	T3, N0, M0	T3 = See Table 1.
		N0 = No regional lymph node metastasis.
		M0 = No distant metastasis (no pathological M0; use clinical M to complete stage group).
	T1, T2, T3; N1, M0	T1, T2, T3 = See Table 1.
		N1 = Metastasis in a single ipsilateral lymph node, ≤3 cm in greatest dimension and ENE(‒).
		M0 = No distant metastasis (no pathological M0; use clinical M to complete stage group).

Table 8. Definitions of TNM Stage IVA, IVB, and IVC^a

Stage	TNM	Description
T = primary tumor; N = regional lymph node; M = distant metastasis.
a Reprinted with permission from AJCC: Nasal cavity and paranasal sinuses. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 137–47.
IVA	T4a; N0, N1; M0	T4a = See Table 1.
		N0, N1 = See Table 2.
		M0 = No distant metastasis (no pathological M0; use clinical M to complete stage group).
	T1, T2, T3, T4a; N2, M0	T1, T2, T3, T4a = See Table 1.
		N2 = See Table 2.
		M0 = No distant metastasis (no pathological M0; use clinical M to complete stage group).
IVB	Any T, N3, M0	Any T = See Table 1.
		N3 = See Table 2.
		M0 = No distant metastasis (no pathological M0; use clinical M to complete stage group).
	T4b, Any N, M0	T4b = See Table 1.
		Any N = See Table 2.
		M0 = No distant metastasis (no pathological M0; use clinical M to complete stage group).
IVC	Any T, Any N, M1	Any T = See Table 1.
		Any N = See Table 2.
		M1 = Distant metastasis.

References:

1. Mendenhall WM, Werning JW, Pfister DG: Treatment of head and neck cancer. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Lippincott Williams & Wilkins, 2011, pp 729-80.
2. Laramore GE, ed.: Radiation Therapy of Head and Neck Cancer. Springer-Verlag, 1989.
3. Nasal cavity and paranasal sinuses. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. Springer; 2017, pp 137-47.

Treatment Option Overview for Paranasal Sinus and Nasal Cavity Cancer

Except for patients with T1 mucosal carcinomas, the accepted method of treatment is a combination of radiation therapy and surgery. The incidence of lymph node metastases is generally low (approximately 20% of cases). Thus, routine radical neck dissection or elective neck radiation therapy is recommended only for patients presenting with positive nodes.

For patients with operable tumors, radical surgery is generally performed first to remove the bulk of the tumor and to establish drainage of the affected sinus(es). This is followed by postoperative radiation therapy. Some institutions continue to give a full dose of radiation therapy preoperatively for all patients with stage II and stage III tumors and operate 4 to 6 weeks later.[1,2,3] A review of published clinical results of radical radiation therapy for head and neck cancer suggested a significant loss of local control with prolonged radiation therapy; therefore, lengthening of standard treatment schedules should be avoided whenever possible.[4]

Surgery

Surgical exploration may be required to determine operability.

Relative contraindications to surgery include destruction of the base of the skull (i.e., anterior cranial fossa), cavernous sinus, or the pterygoid process; infiltration of the mucous membranes of the nasopharynx; or nonresectable lymph node metastases. Surgical approaches include fenestration with removal of the bulk tumor, which is usually followed by radiation therapy or block resection of the upper jaw. A combined craniofacial approach, including resection of the floor of the anterior cranial fossa, has been used with success in selected patients.[5] Removal of the eye is performed if the orbit is extensively invaded by cancer. Clinically positive nodes, if resectable, may be treated with radical neck dissection.

Radiation Therapy

Radiation therapy must be carried to high doses for any significant probability of permanent control. The treatment volume must include all of the maxillary antrum and involved hemiparanasal sinus and contiguous areas. The orbit and its contents are excluded except under unusual circumstances. Lymph nodes of the neck, when palpable, should be treated in conjunction with treatment of advanced carcinomas of the antrum. This may be unnecessary for early tumors.

Accumulating evidence has demonstrated a high incidence (>30%–40%) of hypothyroidism in patients who have received external-beam radiation therapy to the entire thyroid gland or to the pituitary gland. Thyroid function testing of patients should be considered prior to therapy and as part of posttreatment follow-up.[6,7]

Recurrent Disease

Patients with recurrent disease should consider chemotherapy clinical trials. Chemotherapy for recurrent squamous cell cancer of the head and neck has been shown to be efficacious as palliation and may improve a patient's quality of life and length of survival. Various drug combinations, including cisplatin, fluorouracil, and methotrexate, are effective.[8,9]

Treatment of tumors of the paranasal sinuses and of the nasal cavity should be planned on an individual basis because of the complexity involved.

Fluorouracil dosing

The DPYD gene encodes an enzyme that catabolizes pyrimidines and fluoropyrimidines, like capecitabine and fluorouracil. An estimated 1% to 2% of the population has germline pathogenic variants in DPYD, which lead to reduced DPD protein function and an accumulation of pyrimidines and fluoropyrimidines in the body.[10,11] Patients with the DPYD*2A variant who receive fluoropyrimidines may experience severe, life-threatening toxicities that are sometimes fatal. Many other DPYD variants have been identified, with a range of clinical effects.[10,11,12] Fluoropyrimidine avoidance or a dose reduction of 50% may be recommended based on the patient's DPYD genotype and number of functioning DPYD alleles.[13,14,15]DPYD genetic testing costs less than $200, but insurance coverage varies due to a lack of national guidelines.[16] In addition, testing may delay therapy by 2 weeks, which would not be advisable in urgent situations. This controversial issue requires further evaluation.[17]

References:

1. Mendenhall WM, Werning JW, Pfister DG: Treatment of head and neck cancer. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Lippincott Williams & Wilkins, 2011, pp 729-80.
2. Laramore GE, ed.: Radiation Therapy of Head and Neck Cancer. Springer-Verlag, 1989.
3. Thawley SE, Panje WR, Batsakis JG, et al., eds.: Comprehensive Management of Head and Neck Tumors. 2nd ed. WB Saunders, 1999.
4. Fowler JF, Lindstrom MJ: Loss of local control with prolongation in radiotherapy. Int J Radiat Oncol Biol Phys 23 (2): 457-67, 1992.
5. Ganly I, Patel SG, Singh B, et al.: Craniofacial resection for malignant paranasal sinus tumors: Report of an International Collaborative Study. Head Neck 27 (7): 575-84, 2005.
6. Turner SL, Tiver KW, Boyages SC: Thyroid dysfunction following radiotherapy for head and neck cancer. Int J Radiat Oncol Biol Phys 31 (2): 279-83, 1995.
7. Constine LS: What else don't we know about the late effects of radiation in patients treated for head and neck cancer? Int J Radiat Oncol Biol Phys 31 (2): 427-9, 1995.
8. Jacobs C, Lyman G, Velez-García E, et al.: A phase III randomized study comparing cisplatin and fluorouracil as single agents and in combination for advanced squamous cell carcinoma of the head and neck. J Clin Oncol 10 (2): 257-63, 1992.
9. Schornagel JH, Verweij J, de Mulder PH, et al.: Randomized phase III trial of edatrexate versus methotrexate in patients with metastatic and/or recurrent squamous cell carcinoma of the head and neck: a European Organization for Research and Treatment of Cancer Head and Neck Cancer Cooperative Group study. J Clin Oncol 13 (7): 1649-55, 1995.
10. Sharma BB, Rai K, Blunt H, et al.: Pathogenic DPYD Variants and Treatment-Related Mortality in Patients Receiving Fluoropyrimidine Chemotherapy: A Systematic Review and Meta-Analysis. Oncologist 26 (12): 1008-1016, 2021.
11. Lam SW, Guchelaar HJ, Boven E: The role of pharmacogenetics in capecitabine efficacy and toxicity. Cancer Treat Rev 50: 9-22, 2016.
12. Shakeel F, Fang F, Kwon JW, et al.: Patients carrying DPYD variant alleles have increased risk of severe toxicity and related treatment modifications during fluoropyrimidine chemotherapy. Pharmacogenomics 22 (3): 145-155, 2021.
13. Amstutz U, Henricks LM, Offer SM, et al.: Clinical Pharmacogenetics Implementation Consortium (CPIC) Guideline for Dihydropyrimidine Dehydrogenase Genotype and Fluoropyrimidine Dosing: 2017 Update. Clin Pharmacol Ther 103 (2): 210-216, 2018.
14. Henricks LM, Lunenburg CATC, de Man FM, et al.: DPYD genotype-guided dose individualisation of fluoropyrimidine therapy in patients with cancer: a prospective safety analysis. Lancet Oncol 19 (11): 1459-1467, 2018.
15. Lau-Min KS, Varughese LA, Nelson MN, et al.: Preemptive pharmacogenetic testing to guide chemotherapy dosing in patients with gastrointestinal malignancies: a qualitative study of barriers to implementation. BMC Cancer 22 (1): 47, 2022.
16. Brooks GA, Tapp S, Daly AT, et al.: Cost-effectiveness of DPYD Genotyping Prior to Fluoropyrimidine-based Adjuvant Chemotherapy for Colon Cancer. Clin Colorectal Cancer 21 (3): e189-e195, 2022.
17. Baker SD, Bates SE, Brooks GA, et al.: DPYD Testing: Time to Put Patient Safety First. J Clin Oncol 41 (15): 2701-2705, 2023.

Treatment of Stage I Paranasal Sinus and Nasal Cavity Cancer

Stage I disease includes small lesions.

Maxillary Sinus Tumors

Maxillary sinus tumors are small lesions of the infrastructure.

Treatment options for stage I maxillary sinus tumors include the following:

1. Surgical resection.
2. Postoperative radiation therapy should be considered for close margins (particularly in tumors of the suprastructure).

Ethmoid Sinus Tumors

Ethmoid sinus tumors are usually extensive when diagnosed.[1,2,3]

Treatment options for stage I ethmoid sinus tumors include the following:

1. External-beam radiation therapy alone is generally used for unresectable lesions.
2. Well-localized lesions can be resected, but resection of the ethmoids, maxilla, and orbit, with consideration for a craniofacial approach, is generally required.
3. If surgery can be done with good functional and cosmetic results, postoperative radiation therapy should be given even with clear surgical margins.

Sphenoid Sinus Tumors

Treatment options for stage I sphenoid sinus tumors include the following:

1. Treatment is the same as for nasopharyngeal cancers, primarily radiation therapy. For more information, see the Treatment of Stage I Nasopharyngeal Carcinoma section in Nasopharyngeal Carcinoma Treatment.

Nasal Cavity Tumors

For nasal cavity tumors (squamous cell carcinomas), treatment preferences are either surgery or radiation therapy, which have equal cure rates.

Treatment options for stage I nasal cavity tumors include the following:

1. Surgery for tumors of the septum.
2. Radiation therapy for tumors of the lateral and superior walls.[4]
3. Surgery plus radiation therapy for tumors of the septal and lateral walls.[5]

Inverting Papillomas

Treatment options for stage I inverting papillomas include the following:

1. Surgical excision.
2. Re-excision for surgery failures.
3. Radical surgery may eventually be necessary.
4. Radiation therapy has been used successfully for surgical failures.

Melanomas and Sarcomas

Treatment options for stage I melanomas and sarcomas include the following:

1. Surgical excision if possible.
2. Combined surgery, radiation therapy, and chemotherapy are recommended for rhabdomyosarcoma.

Midline Granulomas

Treatment options for stage I midline granulomas include the following:

1. Radiation therapy to nasal cavity and paranasal sinuses.

Nasal Vestibule Tumors

Treatment options for stage I nasal vestibule tumors include the following:

1. Surgery or radiation therapy may be performed. If lesions are extremely small, surgery is preferred, provided that no deformity is expected and a need for reconstruction is not anticipated. Radiation therapy is preferred for other small lesions.[6,7] Treatment of the ipsilateral neck should be considered.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References:

1. Kraus DH, Sterman BM, Levine HL, et al.: Factors influencing survival in ethmoid sinus cancer. Arch Otolaryngol Head Neck Surg 118 (4): 367-72, 1992.
2. Shah JP: Surgery of the anterior skull base for malignant tumors. Acta Otorhinolaryngol Belg 53 (3): 191-4, 1999.
3. Cantù G, Solero CL, Mariani L, et al.: Anterior craniofacial resection for malignant ethmoid tumors--a series of 91 patients. Head Neck 21 (3): 185-91, 1999.
4. Hawkins RB, Wynstra JH, Pilepich MV, et al.: Carcinoma of the nasal cavity--results of primary and adjuvant radiotherapy. Int J Radiat Oncol Biol Phys 15 (5): 1129-33, 1988.
5. Ang KK, Jiang GL, Frankenthaler RA, et al.: Carcinomas of the nasal cavity. Radiother Oncol 24 (3): 163-8, 1992.
6. Levendag PC, Pomp J: Radiation therapy of squamous cell carcinoma of the nasal vestibule. Int J Radiat Oncol Biol Phys 19 (6): 1363-7, 1990.
7. Wong CS, Cummings BJ: The place of radiation therapy in the treatment of squamous cell carcinoma of the nasal vestibule. A review. Acta Oncol 27 (3): 203-8, 1988.

Treatment of Stage II Paranasal Sinus and Nasal Cavity Cancer

Stage II disease includes small and moderately advanced lesions.

Maxillary Sinus Tumors

Treatment options for stage II maxillary sinus tumors include the following:

1. Surgical resection with high-dose preoperative or postoperative radiation therapy.

Ethmoid Sinus Tumors

Ethmoid sinus tumors are usually extensive when diagnosed.[1,2,3]

Treatment options for stage II ethmoid sinus tumors include the following:

1. External-beam radiation therapy alone is generally used and produces better overall results than surgery.
2. Well-localized lesions can be resected, but resection of the ethmoids, maxilla, and orbit, often with a combined neurosurgical sinus craniofacial approach, is generally required.
3. If surgery can be done with good functional and cosmetic results, postoperative radiation therapy should be given, even when surgical margins are clear.

Sphenoid Sinus Tumors

Treatment options for stage II sphenoid sinus tumors include the following:

1. Treatment is the same as for nasopharyngeal cancers, primarily radiation therapy. Concurrent chemotherapy and radiation therapy may be considered. For more information, see the Treatment of Stages II, III, and IV Nonmetastatic Nasopharyngeal Carcinoma section in Nasopharyngeal Carcinoma Treatment.

Nasal Cavity Tumors

For nasal cavity tumors (squamous cell carcinomas), treatment preferences are either surgery or radiation therapy, which have equal cure rates.[4]

Treatment options for stage II nasal cavity tumors include the following:

1. Surgery or radiation therapy for tumors of the septum.
2. Radiation therapy for tumors of the lateral and superior walls. Concurrent chemotherapy and radiation therapy may be considered.
3. Surgery plus radiation therapy for tumors of the septal and lateral walls.[5]

Inverting Papillomas

Treatment options for stage II inverting papillomas include the following:

1. Surgical excision.
2. Re-excision for surgery failures.
3. Radiation therapy for radical surgery failures may eventually be necessary.

Melanomas and Sarcomas

Treatment options for stage II melanomas and sarcomas include the following:

1. Surgical excision if possible.
2. Combined surgery, radiation therapy, and chemotherapy are recommended for rhabdomyosarcoma.

Midline Granulomas

Treatment options for stage II midline granulomas include the following:

1. Radiation therapy to nasal cavity and paranasal sinuses.

Nasal Vestibule Tumors

Treatment options for stage II nasal vestibule tumors include the following:

1. Surgery or radiation therapy may be performed. If tumors are extremely small, surgery is preferred, provided that no deformity is expected and a need for reconstruction is not anticipated. Radiation therapy is preferred for other small lesions.[6,7] Treatment of the neck should be considered.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References:

1. Kraus DH, Sterman BM, Levine HL, et al.: Factors influencing survival in ethmoid sinus cancer. Arch Otolaryngol Head Neck Surg 118 (4): 367-72, 1992.
2. Cantù G, Solero CL, Mariani L, et al.: Anterior craniofacial resection for malignant ethmoid tumors--a series of 91 patients. Head Neck 21 (3): 185-91, 1999.
3. Shah JP: Surgery of the anterior skull base for malignant tumors. Acta Otorhinolaryngol Belg 53 (3): 191-4, 1999.
4. Hawkins RB, Wynstra JH, Pilepich MV, et al.: Carcinoma of the nasal cavity--results of primary and adjuvant radiotherapy. Int J Radiat Oncol Biol Phys 15 (5): 1129-33, 1988.
5. Ang KK, Jiang GL, Frankenthaler RA, et al.: Carcinomas of the nasal cavity. Radiother Oncol 24 (3): 163-8, 1992.
6. Levendag PC, Pomp J: Radiation therapy of squamous cell carcinoma of the nasal vestibule. Int J Radiat Oncol Biol Phys 19 (6): 1363-7, 1990.
7. Wong CS, Cummings BJ: The place of radiation therapy in the treatment of squamous cell carcinoma of the nasal vestibule. A review. Acta Oncol 27 (3): 203-8, 1988.

Treatment of Stage III Paranasal Sinus and Nasal Cavity Cancer

Stage III disease includes small and moderately advanced lesions.

Maxillary Sinus Tumors

Treatment options for stage III maxillary sinus tumors include the following:

1. Surgical resection with high-dose preoperative or postoperative radiation therapy.
2. Superfractionated preoperative or postoperative radiation therapy (under clinical evaluation).[1]

Ethmoid Sinus Tumors

Treatment options for stage III ethmoid sinus tumors include the following:

1. Generally, a craniofacial resection in combination with postoperative radiation therapy.[2,3,4]
2. Clinical trials using new drug combinations for advanced tumors should be considered to evaluate chemotherapy preoperatively or before radiation therapy. Adjuvant therapy after surgery or after combined-modality therapy should also be considered.

Sphenoid Sinus Tumors

Treatment options for stage III sphenoid sinus tumors include the following:

1. Treatment is the same as for nasopharyngeal cancers, primarily radiation therapy. For more information, see the Treatment of Stages II, III, and IV Nonmetastatic Nasopharyngeal Carcinoma section in Nasopharyngeal Carcinoma Treatment.
2. Concurrent chemotherapy and radiation therapy may be considered.

Nasal Cavity Tumors

Nasal cavity tumors are squamous cell carcinomas.

Treatment options for stage III nasal cavity tumors include the following:

1. Surgery alone.
2. Radiation therapy alone.[5] Concurrent chemotherapy and radiation therapy may be considered.
3. Combined surgery and radiation therapy (postoperative radiation therapy is preferred).[5,6]
4. Clinical trials using new drug combinations for advanced tumors should be considered to evaluate chemotherapy preoperatively or before radiation therapy. Adjuvant therapy after surgery or after combined-modality therapy should also be considered.

Inverting Papillomas

Treatment options for stage III inverting papillomas include the following:

1. Surgical excision.
2. Re-excision for surgery failures.
3. Radiation therapy or radical surgery may eventually be necessary.

Melanomas and Sarcomas

Treatment options for stage III melanomas and sarcomas include the following:

1. Surgical excision if possible; otherwise, consider radiation therapy.
2. Combined surgery, radiation therapy, and chemotherapy are recommended for rhabdomyosarcoma.

Midline Granulomas

Treatment options for stage III midline granulomas include the following:

1. Radiation therapy to nasal cavity and paranasal sinuses.

Nasal Vestibule Tumors

Treatment options for stage III nasal vestibule tumors include the following:

1. Generally, radiation is preferred to minimize deformity.[7] External-beam (photons or electrons) and/or interstitial implantation can be used. Surgery is reserved for salvage.
2. Clinical trials using new drug combinations for advanced tumors should be considered to evaluate chemotherapy preoperatively or before radiation therapy. Adjuvant therapy after surgery or after combined-modality therapy should also be considered.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References:

1. Johnson CR, Schmidt-Ullrich RK, Wazer DE: Concomitant boost technique using accelerated superfractionated radiation therapy for advanced squamous cell carcinoma of the head and neck. Cancer 69 (11): 2749-54, 1992.
2. Kraus DH, Sterman BM, Levine HL, et al.: Factors influencing survival in ethmoid sinus cancer. Arch Otolaryngol Head Neck Surg 118 (4): 367-72, 1992.
3. Cantù G, Solero CL, Mariani L, et al.: Anterior craniofacial resection for malignant ethmoid tumors--a series of 91 patients. Head Neck 21 (3): 185-91, 1999.
4. Shah JP: Surgery of the anterior skull base for malignant tumors. Acta Otorhinolaryngol Belg 53 (3): 191-4, 1999.
5. Hawkins RB, Wynstra JH, Pilepich MV, et al.: Carcinoma of the nasal cavity--results of primary and adjuvant radiotherapy. Int J Radiat Oncol Biol Phys 15 (5): 1129-33, 1988.
6. Ang KK, Jiang GL, Frankenthaler RA, et al.: Carcinomas of the nasal cavity. Radiother Oncol 24 (3): 163-8, 1992.
7. Wong CS, Cummings BJ: The place of radiation therapy in the treatment of squamous cell carcinoma of the nasal vestibule. A review. Acta Oncol 27 (3): 203-8, 1988.

Treatment of Stage IV Paranasal Sinus and Nasal Cavity Cancer

Stage IV disease includes advanced lesions.

Maxillary Sinus Tumors

Treatment options for stage IV maxillary sinus tumors include the following:

1. High-dose radiation therapy is used because extension to the base of the skull and nasopharynx is a potential, but not absolute, contraindication to surgery. If radiation therapy is to be used alone, localized drainage of the sinus(es) must be established before radiation therapy treatments are initiated.
2. Superfractionated radiation therapy (under clinical evaluation).[1]
3. Clinical trials for advanced tumors should be considered to evaluate chemotherapy preoperatively or before radiation therapy. Adjuvant therapy after surgery or after combined-modality therapy should also be considered.
4. Concurrent chemotherapy and radiation therapy may be considered.

Ethmoid Sinus Tumors

Treatment options for stage IV ethmoid sinus tumors include the following:

1. Generally, a craniofacial resection in combination with preoperative or postoperative radiation therapy is performed.[2,3,4]
2. Concurrent chemotherapy and radiation therapy may be considered for patients with inoperable tumors.
3. Clinical trials for advanced tumors should be considered to evaluate chemotherapy preoperatively or before radiation therapy. Adjuvant therapy after surgery or after combined-modality therapy should also be considered.

Sphenoid Sinus Tumors

Treatment options for stage IV sphenoid sinus tumors include the following:

1. Treatment is the same as for nasopharyngeal cancers, primarily radiation therapy. For more information, see the Treatment of Stages II, III, and IV Nonmetastatic Nasopharyngeal Carcinoma and Treatment of Metastatic and Recurrent Nasopharyngeal Carcinoma sections in Nasopharyngeal Carcinoma Treatment.
2. Concurrent chemotherapy and radiation therapy may be considered.

Nasal Cavity Tumors

Nasal cavity tumors are squamous cell carcinomas.

Treatment options for stage IV nasal cavity tumors include the following:

1. Surgery alone.
2. Radiation therapy alone.[5] Concurrent chemotherapy and radiation therapy may be considered.
3. Combined surgery and radiation therapy (postoperative radiation therapy is preferred).[5]
4. Clinical trials for advanced tumors should be considered to evaluate chemotherapy preoperatively or before radiation therapy. Adjuvant therapy after surgery or after combined-modality therapy should also be considered.

Inverting Papillomas

Treatment options for stage IV inverting papillomas include the following:

1. Surgical excision.
2. Re-excision for surgery failures.
3. Radiation therapy or radical surgery may eventually be necessary.

Melanomas and Sarcomas

Treatment options for stage IV melanomas and sarcomas include the following:

1. Surgical excision if possible.
2. Appropriate radiation therapy and various chemotherapy agents should be considered.

Midline Granulomas

Treatment options for stage IV midline granulomas include the following:

1. Radiation therapy to nasal cavity and paranasal sinuses.

Nasal Vestibule Tumors

Treatment options for stage IV nasal vestibule tumors include the following:

1. Generally, radiation therapy is preferred to minimize deformity. External-beam (i.e., photons or electrons) and/or interstitial implantation can be used. Surgery is reserved for salvage. Treatment of the neck should be considered.
2. Clinical trials for advanced tumors should be considered to evaluate chemotherapy preoperatively or before radiation therapy. Adjuvant therapy after surgery or after combined-modality therapy should also be considered.
3. Concurrent chemotherapy and radiation therapy may be considered.

Neoadjuvant chemotherapy as used in clinical trials has been used to shrink tumors and to render them more definitively treatable with either surgery or radiation therapy. This chemotherapy is given before the other modalities; therefore, the designation of neoadjuvant is used to distinguish it from standard adjuvant therapy, which is given after or during definitive therapy with radiation or after surgery. Many drug combinations have been used in neoadjuvant chemotherapy.[6,7,8]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References:

1. Johnson CR, Schmidt-Ullrich RK, Wazer DE: Concomitant boost technique using accelerated superfractionated radiation therapy for advanced squamous cell carcinoma of the head and neck. Cancer 69 (11): 2749-54, 1992.
2. Kraus DH, Sterman BM, Levine HL, et al.: Factors influencing survival in ethmoid sinus cancer. Arch Otolaryngol Head Neck Surg 118 (4): 367-72, 1992.
3. Cantù G, Solero CL, Mariani L, et al.: Anterior craniofacial resection for malignant ethmoid tumors--a series of 91 patients. Head Neck 21 (3): 185-91, 1999.
4. Shah JP: Surgery of the anterior skull base for malignant tumors. Acta Otorhinolaryngol Belg 53 (3): 191-4, 1999.
5. Hawkins RB, Wynstra JH, Pilepich MV, et al.: Carcinoma of the nasal cavity--results of primary and adjuvant radiotherapy. Int J Radiat Oncol Biol Phys 15 (5): 1129-33, 1988.
6. Stupp R, Weichselbaum RR, Vokes EE: Combined modality therapy of head and neck cancer. Semin Oncol 21 (3): 349-58, 1994.
7. Al-Sarraf M: Head and neck cancer: chemotherapy concepts. Semin Oncol 15 (1): 70-85, 1988.
8. Dimery IW, Hong WK: Overview of combined modality therapies for head and neck cancer. J Natl Cancer Inst 85 (2): 95-111, 1993.

Treatment of Recurrent Paranasal Sinus and Nasal Cavity Cancer

Chemotherapy for recurrent head and neck squamous cell cancer has shown promise. Chemotherapy may be indicated when there is recurrence in either distant or local disease after primary surgery or radiation therapy, and when there is residual disease after primary treatment.[1,2] Survival may be improved in those achieving a complete response to chemotherapy.[3] Combined-modality therapy with platinum and radiation therapy has been used in clinical trials such as UMCC-8810.[4]

Maxillary Sinus Tumors

Treatment options for recurrent maxillary sinus tumors include the following:

1. After surgery, radiation therapy or craniofacial resection with postoperative radiation therapy.
2. After radiation therapy, craniofacial resection if indicated.
3. Chemotherapy should be considered for patients with disease that does not respond to other treatments.
4. Clinical trials using chemotherapy should be considered.[5,6]

Ethmoid Sinus Tumors

Treatment options for recurrent ethmoid sinus tumors include the following:

1. After limited surgery, craniofacial resection, radiation therapy, or both.[7,8,9]
2. After radiation therapy, craniofacial resection.
3. Chemotherapy should be considered for patients with disease that does not respond to other treatments.
4. Clinical trials using chemotherapy should be considered.[5,6]

Sphenoid Sinus Tumors

Treatment options for recurrent sphenoid sinus tumors include the following:

1. Treatment is the same as for nasopharyngeal cancers, primarily radiation therapy. For more information, see the Treatment of Metastatic and Recurrent Nasopharyngeal Carcinoma section in Nasopharyngeal Carcinoma Treatment.
2. Chemotherapy should be considered for patients with disease that does not respond to other treatments.

Nasal Cavity Tumors

For nasal cavity tumors (squamous cell carcinomas), salvage is possible in approximately 25% of patients.

Treatment options for recurrent nasal cavity tumors include the following:

1. For disease that does not respond to radiation therapy, craniofacial resection.
2. For disease that does not respond to surgery, radiation therapy.
3. Chemotherapy should be considered for patients with disease that does not respond to radiation therapy or surgery.
4. Clinical trials using chemotherapy should be considered.[5,6]

Inverting Papillomas

Treatment options for recurrent inverting papillomas include the following:

1. Surgical excision.
2. Re-excision for surgery failures.
3. Radical surgery or radiation therapy may eventually be necessary.

Melanomas and Sarcomas

Treatment options for recurrent melanomas and sarcomas include the following:

1. Surgical excision if possible.
2. Appropriate chemotherapy geared specifically to cell type. For more information, see the Treatment of Metastatic and Recurrent Nasopharyngeal Carcinoma section in Nasopharyngeal Carcinoma Treatment and the Treatment of Recurrent Major Salivary Gland Cancer section in Salivary Gland Cancer Treatment.

Midline Granulomas

Treatment options for recurrent midline granulomas include the following:

1. Radiation therapy to nasal cavity and paranasal sinuses.

Nasal Vestibule Tumors

Treatment options for recurrent nasal vestibule tumors include the following:

1. For disease that does not respond to radiation therapy, surgery.
2. For disease that does not respond to surgery, radiation therapy or a combination of surgery and radiation therapy.
3. Chemotherapy should be considered for patients with disease that does not respond to radiation therapy or surgery.
4. Clinical trials using chemotherapy should be considered.[5,6]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References:

1. Kies MS, Levitan N, Hong WK: Chemotherapy of head and neck cancer. Otolaryngol Clin North Am 18 (3): 533-41, 1985.
2. LoRusso P, Tapazoglou E, Kish JA, et al.: Chemotherapy for paranasal sinus carcinoma. A 10-year experience at Wayne State University. Cancer 62 (1): 1-5, 1988.
3. Al-Kourainy K, Kish J, Ensley J, et al.: Achievement of superior survival for histologically negative versus histologically positive clinically complete responders to cisplatin combination in patients with locally advanced head and neck cancer. Cancer 59 (2): 233-8, 1987.
4. Al-Sarraf M, Pajak TF, Marcial VA, et al.: Concurrent radiotherapy and chemotherapy with cisplatin in inoperable squamous cell carcinoma of the head and neck. An RTOG Study. Cancer 59 (2): 259-65, 1987.
5. Brasnu D, Laccourreye O, Bassot V, et al.: Cisplatin-based neoadjuvant chemotherapy and combined resection for ethmoid sinus adenocarcinoma reaching and/or invading the skull base. Arch Otolaryngol Head Neck Surg 122 (7): 765-8, 1996.
6. Licitra L, Locati LD, Cavina R, et al.: Primary chemotherapy followed by anterior craniofacial resection and radiotherapy for paranasal cancer. Ann Oncol 14 (3): 367-72, 2003.
7. Kraus DH, Sterman BM, Levine HL, et al.: Factors influencing survival in ethmoid sinus cancer. Arch Otolaryngol Head Neck Surg 118 (4): 367-72, 1992.
8. Cantù G, Solero CL, Mariani L, et al.: Anterior craniofacial resection for malignant ethmoid tumors--a series of 91 patients. Head Neck 21 (3): 185-91, 1999.
9. Shah JP: Surgery of the anterior skull base for malignant tumors. Acta Otorhinolaryngol Belg 53 (3): 191-4, 1999.

Latest Updates to This Summary (07 / 05 / 2024)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Treatment Option Overview for Paranasal Sinus and Nasal Cavity Cancer

Added Fluorouracil dosing as a new subsection.

This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® Cancer Information for Health Professionals pages.

About This PDQ Summary

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of adult paranasal sinus and nasal cavity cancer. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

• be discussed at a meeting,
• be cited with text, or
• replace or update an existing article that is already cited.

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Paranasal Sinus and Nasal Cavity Cancer Treatment are:

• Andrea Bonetti, MD (Azienda ULSS 9 of the Veneto Region)
• Minh Tam Truong, MD (Boston University Medical Center)

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

Permission to Use This Summary

PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as "NCI's PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary]."

The preferred citation for this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Paranasal Sinus and Nasal Cavity Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/head-and-neck/hp/adult/paranasal-sinus-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389272]

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Based on the strength of the available evidence, treatment options may be described as either "standard" or "under clinical evaluation." These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

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Last Revised: 2024-07-05