Paranasal Sinus and Nasal Cavity Cancer Treatment 1/2 - National Cancer Institute

Paranasal Sinus and Nasal Cavity Cancer Treatment - National Cancer Institute

Paranasal Sinus and Nasal Cavity Cancer Treatment (Adult) (PDQ®)–Health Professional Version

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ON THIS PAGE

• General Information About Paranasal Sinus and Nasal Cavity Cancer
• Cellular Classification of Paranasal Sinus and Nasal Cavity Cancer
• Stage Information for Paranasal Sinus and Nasal Cavity Cancer
• Treatment Option Overview
• Stage I Paranasal Sinus and Nasal Cavity Cancer
• Stage II Paranasal Sinus and Nasal Cavity Cancer
• Stage III Paranasal Sinus and Nasal Cavity Cancer
• Stage IV Paranasal Sinus and Nasal Cavity Cancer
• Recurrent Paranasal Sinus and Nasal Cavity Cancer
• Changes to This Summary (07/26/2019)
• About This PDQ Summary

General Information About Paranasal Sinus and Nasal Cavity Cancer

In This Section

• Incidence and Mortality
• Anatomy
• Clinical Evaluation and Follow-up
• Carcinogenesis and Risk Factors

Incidence and Mortality

The majority of 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.[1-3]

Nodal involvement is infrequent. Although 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, locoregional recurrence accounts for the majority of cancer deaths since 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, though occasionally salvage surgery or salvage radiation therapy, as appropriate, may be successful. Since most treatment failures occur within 2 years, the follow-up of patients must be frequent and meticulous during this period. In addition, because nearly 33% of these patients develop second primary cancers in the aerodigestive tract, a lifetime of follow-up is essential.

Carcinogenesis and Risk Factors

Some 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 subgroup has shown that paranasal sinus and nasal cavity SCC 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. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 729-80.
2. Laramore GE, ed.: Radiation Therapy of Head and Neck Cancer. Berlin: Springer-Verlag, 1989.
3. Thawley SE, Panje WR, Batsakis JG, et al., eds.: Comprehensive Management of Head and Neck Tumors. 2nd ed. Philadelphia, Pa: WB Saunders, 1999.
4. Johns ME, Kaplan MJ: Advances in the management of paranasal sinus tumors. In: Wolf GT, ed.: Head and Neck Oncology. Boston, Mass: 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. [PUBMED Abstract]

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 <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, involves this region as well.

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. Philadelphia, Pa: 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. [PUBMED Abstract]
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. [PUBMED Abstract]

Stage Information for Paranasal Sinus and Nasal Cavity Cancer

In This Section

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

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 prior to attempted surgical resection or definitive radiation therapy. If a patient 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 of nasal cavity and paranasal sinus carcinomas is not as well established as 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
aReprinted 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	Carcinoma in situ.	Carcinoma in 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.
aReprinted 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
aReprinted 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 0a

Stage	TNM	Description
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted 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 Ia

Stage	TNM	Description
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted 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 IIa

Stage	TNM	Description
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted 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 IIIa

Stage	TNM	Description
T = primary tumor; N = regional lymph node; M = distant metastasis; ENE = extranodal extension.
aReprinted 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 IVCa

Stage	TNM	Description
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted 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. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 729-80.
2. Laramore GE, ed.: Radiation Therapy of Head and Neck Cancer. Berlin: 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. New York, NY: Springer, 2017, pp 137-47.

Treatment Option Overview

In This Section

• Surgery
• Radiation Therapy
• Recurrent Disease

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 all 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 to operate 4 to 6 weeks later.[1-3] A review of published clinical results of radical radiation therapy for head and neck cancer suggested a significant loss of local control when the administration of radiation therapy was prolonged; therefore, lengthening of standard treatment schedules should be avoided whenever possible.[4]

Surgery

Surgical exploration may be required to determine operability.

Destruction of the base of 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 are relative contraindications to surgery. 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

For patients with recurrent disease, chemotherapy trials should be considered. Chemotherapy for recurrent squamous cell cancer of the head and neck has been shown to be efficacious as palliation and may improve 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.

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. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 729-80.
2. Laramore GE, ed.: Radiation Therapy of Head and Neck Cancer. Berlin: Springer-Verlag, 1989.
3. Thawley SE, Panje WR, Batsakis JG, et al., eds.: Comprehensive Management of Head and Neck Tumors. 2nd ed. Philadelphia, Pa: 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. [PUBMED Abstract]
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. [PUBMED Abstract]
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. [PUBMED Abstract]
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. [PUBMED Abstract]
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. [PUBMED Abstract]
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. [PUBMED Abstract]