Purpose of This PDQ Summary
General Information
Cellular Classification
Stage Information

TNM Definitions AJCC Stage Groupings

Treatment Option Overview

Surgery Radiation Therapy Recurrent Disease

Stage I Paranasal Sinus and Nasal Cavity Cancer

Current Clinical Trials

Stage II Paranasal Sinus and Nasal Cavity Cancer

Current Clinical Trials

Stage III Paranasal Sinus and Nasal Cavity Cancer

Current Clinical Trials

Stage IV Paranasal Sinus and Nasal Cavity Cancer

Current Clinical Trials

Recurrent Paranasal Sinus and Nasal Cavity Cancer

Current Clinical Trials

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Changes to This Summary (07/11/2008)
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Purpose of This PDQ Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of paranasal sinus and nasal cavity cancer. This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board ¹.

Information about the following is included in this summary:

• Anatomy and pathology.
• Cellular classification.
• Staging.
• Treatment options by cancer stage.

This summary is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.

Some of the reference citations in the 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 ² in developing its level-of-evidence designations. Based on the strength of the available evidence, treatment options are described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for reimbursement determinations.

This summary is available in a patient version ³, written in less technical language, and in Spanish ⁴.

General Information

The majority of tumors of the paranasal sinuses present with advanced disease, and cure rates are generally poor (≤50%). Squamous cell carcinoma 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]

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.

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.

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.

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]

References

1. Mendenhall WM, Riggs CE Jr, Cassisi NJ: Treatment of head and neck cancers. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds.: Cancer: Principles and Practice of Oncology. 7th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2005, pp 662-732. 
   
   
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. 
   
   

Cellular Classification

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, Riggs CE Jr, Cassisi NJ: Treatment of head and neck cancers. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds.: Cancer: Principles and Practice of Oncology. 7th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2005, pp 662-732. 
   
   
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

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]

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 American Joint Committee on Cancer (AJCC) has designated staging by TNM classification.[3]

Maxillary sinus

Primary tumor (T)

• TX: Primary tumor cannot be assessed
• T0: No evidence of primary tumor
• Tis: Carcinoma in situ
• T1: Tumor limited to maxillary sinus mucosa with no erosion or destruction of bone
• T2: Tumor causing bone erosion or destruction including extension into the hard palate and/or the middle of the nasal meatus, except extension to the posterior wall of maxillary sinus and pterygoid plates
• 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
• T4a: Tumor invades anterior orbital contents, skin of cheek, pterygoid plates, infratemporal fossa, cribriform plate, sphenoid or frontal sinuses
• T4b: 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

Nasal cavity and ethmoid sinus

Primary tumor (T)

• TX: Primary tumor cannot be assessed
• T0: No evidence of primary tumor
• Tis: Carcinoma in situ
• T1: Tumor restricted to any one subsite, with or without bony invasion
• T2: 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 extends to invade the medial wall or floor of the orbit, maxillary sinus, palate, or cribriform plate
• T4a: 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: Tumor invades any of the following: orbital apex, dura, brain, middle cranial fossa, cranial nerves other than (V₂), nasopharynx, or clivus

Regional lymph nodes (N)

• NX: Regional lymph nodes cannot be assessed
• N0: No regional lymph node metastasis
• N1: Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension
• N2: Metastasis in a single ipsilateral lymph node, more than 3 cm but 6 cm or less in greatest dimension, or in multiple ipsilateral lymph nodes, 6 cm or less in greatest dimension, or in bilateral or contralateral lymph nodes, 6 cm or less in greatest dimension
  • N2a: Metastasis in a single ipsilateral lymph node more than 3 cm but 6 cm or less in greatest dimension
  • N2b: Metastasis in multiple ipsilateral lymph nodes, 6 cm or less in greatest dimension
  • N2c: Metastasis in bilateral or contralateral lymph nodes, 6 cm or less in greatest dimension
• N3: Metastasis in a lymph node more than 6 cm in greatest dimension

In clinical evaluation, the actual size of the nodal mass should be measured, and allowance should be made for intervening soft tissues. Most masses more than 3 cm in diameter are not single nodes but confluent nodes or tumors in soft tissues of the neck. There are three stages of clinically positive nodes: N1, N2, and N3. The use of subgroups a, b, and c is not required but is recommended. Midline nodes are considered homolateral nodes.

Distant metastasis (M)

• MX: Distant metastasis cannot be assessed
• M0: No distant metastasis
• M1: Distant metastasis

Stage 0

• Tis, N0, M0

Stage I

• T1, N0, M0

Stage II

• T2, N0, M0

Stage III

• T3, N0, M0
• T1, N1, M0
• T2, N1, M0
• T3, N1, M0

Stage IVA

• T4a, N0, M0
• T4a, N1, M0
• T1, N2, M0
• T2, N2, M0
• T3, N2, M0
• T4a, N2, M0

Stage IVB

• T4b, any N, M0
• Any T, N3, M0

Stage IVC

• Any T, any N, M1

References

1. Mendenhall WM, Riggs CE Jr, Cassisi NJ: Treatment of head and neck cancers. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds.: Cancer: Principles and Practice of Oncology. 7th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2005, pp 662-732. 
   
   
2. Laramore GE, ed.: Radiation Therapy of Head and Neck Cancer. Berlin: Springer-Verlag, 1989. 
   
   
3. Nasal cavity and paranasal sinuses. In: American Joint Committee on Cancer.: AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002, pp 59-67. 
   
   

Treatment Option Overview

Except for 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 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 suggests 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]

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 is 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 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]

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, Riggs CE Jr, Cassisi NJ: Treatment of head and neck cancers. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds.: Cancer: Principles and Practice of Oncology. 7th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2005, pp 662-732. 
   
   
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]
   
   

Stage I Paranasal Sinus and Nasal Cavity Cancer

Stage I disease includes small lesions.

Standard treatment options:

1. For maxillary sinus tumors (small lesions of the infrastructure):
   • Surgical resection.
   • Postoperative radiation therapy should be considered for close margins (particularly in tumors of the suprastructure).



2. For ethmoid sinus tumors (lesions are usually extensive when diagnosed):[1-3]
   • Generally, external-beam radiation therapy alone is used for unresectable lesions.
   • Well-localized lesions can be resected, but it generally requires resection of the ethmoids, maxilla, and orbit with consideration for a craniofacial approach.
   • If surgery can be done with good functional and cosmetic results, postoperative radiation therapy should be given even with clear surgical margins.



3. For sphenoid sinus tumors:
   • Treatment is the same as for nasopharyngeal cancers, primarily radiation therapy. (Refer to the PDQ summary on Nasopharyngeal Cancer ⁵ for more information.)



4. For nasal cavity tumors (squamous cell carcinomas), treatment preferences are either surgery or radiation therapy with equal cure rates:
   • Surgery for tumors of the septum.
   • Radiation therapy for tumors of the lateral and superior walls.[4]
   • Surgery plus radiation therapy for tumors of the septal and lateral walls.[5]



5. For inverting papilloma:
   • Surgical excision.
   • Re-excision for surgery failures.
   • Radical surgery may eventually be necessary.
   • Radiation has been used successfully for surgical failures.



6. For melanomas and sarcomas:
   • Surgical excision if possible.
   • Combined surgery, radiation, and chemotherapy are recommended for rhabdomyosarcoma.



7. For midline granuloma:
   • Radiation therapy to nasal cavity and paranasal sinuses.



8. For nasal vestibule tumors:
   • Surgery or radiation 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.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage I paranasal sinus and nasal cavity cancer ⁶. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site ⁷.

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.  [PUBMED Abstract]
   
   
2. Shah JP: Surgery of the anterior skull base for malignant tumors. Acta Otorhinolaryngol Belg 53 (3): 191-4, 1999.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   
5. Ang KK, Jiang GL, Frankenthaler RA, et al.: Carcinomas of the nasal cavity. Radiother Oncol 24 (3): 163-8, 1992.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   

Stage II Paranasal Sinus and Nasal Cavity Cancer

Stage II disease includes small and moderately advanced lesions.

Standard treatment options:

1. For maxillary sinus tumors:
   • Surgical resection with high-dose preoperative or postoperative radiation therapy.



2. For ethmoid sinus tumors (lesions are usually extensive when diagnosed):[1-3]
   • Generally, external-beam radiation therapy alone is used and produces better overall results than surgery.
   • 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.
   • If surgery can be done with good functional and cosmetic results, postoperative radiation therapy should be given even with clear surgical margins.



3. For sphenoid sinus tumors:
   • Treatment is the same as for nasopharyngeal cancers, primarily radiation therapy. (Refer to the PDQ summary on Nasopharyngeal Cancer ⁵ for more information.) Concomitant chemotherapy and radiation therapy may be considered.



4. For nasal cavity tumors (squamous cell carcinomas), treatment preferences are either surgery or radiation therapy, which have equal cure rates:[4]
   • Surgery or radiation therapy for tumors of the septum.
   • Radiation therapy for tumors of the lateral and superior walls. Concomitant chemotherapy and radiation therapy may be considered.
   • Surgery plus radiation therapy for tumors of the septal and lateral walls.[5]



5. For inverting papilloma:
   • Surgical excision.
   • Re-excision for surgery failures.
   • Radiation therapy for radical surgery failures may eventually be necessary.



6. For melanomas and sarcomas:
   • Surgical excision if possible.
   • Combined surgery, radiation, and chemotherapy are recommended for rhabdomyosarcoma.



7. For midline granuloma:
   • Radiation therapy to nasal cavity and paranasal sinuses.



8. For nasal vestibule tumors:
   • 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.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage II paranasal sinus and nasal cavity cancer ⁸. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site ⁷.

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.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   
3. Shah JP: Surgery of the anterior skull base for malignant tumors. Acta Otorhinolaryngol Belg 53 (3): 191-4, 1999.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   
5. Ang KK, Jiang GL, Frankenthaler RA, et al.: Carcinomas of the nasal cavity. Radiother Oncol 24 (3): 163-8, 1992.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   

Stage III Paranasal Sinus and Nasal Cavity Cancer

Stage III disease includes small and moderately advanced lesions.

Standard treatment options:

1. For maxillary sinus tumors:
   • Surgical resection with high-dose preoperative or postoperative radiation therapy.



2. For ethmoid sinus tumors:[1-3]
   • Generally a craniofacial resection in combination with postoperative radiation therapy.



3. For sphenoid sinus tumors:
   • Treatment is the same as for nasopharyngeal cancers, primarily radiation therapy. (Refer to the PDQ summary on Nasopharyngeal Cancer ⁵ for more information.)
   • Concomitant chemotherapy and radiation therapy may be considered.



4. For nasal cavity tumors (squamous cell carcinomas):
   • Surgery alone.
   • Radiation therapy alone.[4] Concomitant chemotherapy and radiation therapy may be considered.
   • Combined surgery and radiation therapy (postoperative radiation therapy is preferred).[4,5]



5. For inverting papilloma:
   • Surgical excision.
   • Re-excision for surgery failures.
   • Radiation therapy or radical surgery may eventually be necessary.



6. For melanomas and sarcomas:
   • Surgical excision if possible, otherwise consider radiation therapy.
   • Combined surgery, radiation, and chemotherapy are recommended for rhabdomyosarcoma.



7. For midline granuloma:
   • Radiation therapy to nasal cavity and paranasal sinuses.



8. For nasal vestibule tumors:
   • Generally, radiation is preferred to minimize deformity.[6] External-beam (photons or electrons) and/or interstitial implantation can be used. Surgery is reserved for salvage.

Treatment options under clinical evaluation:

1. For maxillary sinus tumors:
   • Superfractionated preoperative or postoperative radiation therapy.[7]



2. For ethmoid sinus tumors, nasal cavity tumors (squamous cell carcinomas), and nasal vestibule tumors:
   • Clinical trials using new drug combinations for advanced tumors should be considered to evaluate chemotherapy preoperatively or before radiation therapy, or as adjuvant therapy after surgery or after combined modality therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage III paranasal sinus and nasal cavity cancer ⁹. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site ⁷.

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.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   
3. Shah JP: Surgery of the anterior skull base for malignant tumors. Acta Otorhinolaryngol Belg 53 (3): 191-4, 1999.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   
5. Ang KK, Jiang GL, Frankenthaler RA, et al.: Carcinomas of the nasal cavity. Radiother Oncol 24 (3): 163-8, 1992.  [PUBMED Abstract]
   
   
6. 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.  [PUBMED Abstract]
   
   
7. 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.  [PUBMED Abstract]
   
   

Stage IV Paranasal Sinus and Nasal Cavity Cancer

Stage IV disease includes advanced lesions.

Standard treatment options:

1. For maxillary sinus tumors:
   • High-dose radiation therapy is used because extension to base of 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 initiating radiation therapy treatments.



2. For ethmoid sinus tumors:[1-3]
   • Generally a craniofacial resection in combination with preoperative or postoperative radiation therapy.
   • Concomitant chemotherapy and radiation therapy may be considered for patients with inoperable tumors.



3. For sphenoid sinus tumors:
   • Treatment is the same as for nasopharyngeal cancers, primarily radiation therapy. (Refer to the PDQ summary on Nasopharyngeal Cancer ⁵ for more information.)
   • Concomitant chemotherapy and radiation therapy may be considered.



4. For nasal cavity tumors (squamous cell carcinomas):
   • Surgery alone.
   • Radiation alone.[4] Concomitant chemotherapy and radiation therapy may be considered.
   • Combined surgery and radiation therapy (postoperative radiation therapy is preferred).[4]



5. For inverting papilloma:
   • Surgical excision.
   • Re-excision for surgery failures.
   • Radiation therapy or radical surgery may eventually be necessary.



6. For melanomas and sarcomas:
   • Surgical excision if possible.
   • Appropriate radiation and various chemotherapy agents should be considered.



7. For midline granuloma:
   • Radiation therapy to nasal cavity and paranasal sinuses.



8. For nasal vestibule tumors:
   • Generally, radiation 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.

Treatment options under clinical evaluation:

1. For maxillary sinus tumors:
   • Superfractionated radiation therapy.[5]



2. For maxillary sinus tumors, ethmoid sinus tumors, nasal cavity tumors, and nasal vestibule tumors:
   • Clinical trials for advanced tumors should be considered to evaluate chemotherapy preoperatively or before radiation therapy, as is adjuvant therapy after surgery or after combined modality therapy.
   • Concomitant chemotherapy and radiation therapy may be considered.

Neoadjuvant chemotherapy as employed in clinical trials has been used to shrink tumors and to render them more definitively treatable with either surgery or radiation. This chemotherapy is given prior to 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.[6-8]

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage IV paranasal sinus and nasal cavity cancer ¹⁰. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site ⁷.

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.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   
3. Shah JP: Surgery of the anterior skull base for malignant tumors. Acta Otorhinolaryngol Belg 53 (3): 191-4, 1999.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   
5. 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.  [PUBMED Abstract]
   
   
6. Stupp R, Weichselbaum RR, Vokes EE: Combined modality therapy of head and neck cancer. Semin Oncol 21 (3): 349-58, 1994.  [PUBMED Abstract]
   
   
7. Al-Sarraf M: Head and neck cancer: chemotherapy concepts. Semin Oncol 15 (1): 70-85, 1988.  [PUBMED Abstract]
   
   
8. Dimery IW, Hong WK: Overview of combined modality therapies for head and neck cancer. J Natl Cancer Inst 85 (2): 95-111, 1993.  [PUBMED Abstract]
   
   

Recurrent Paranasal Sinus and Nasal Cavity Cancer

Chemotherapy for recurrent head and neck squamous cell cancer has shown promise. Chemotherapy may be indicated where there is recurrence in either distant or local disease after primary surgery or radiation, 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 trials such as UMCC-8810 ¹¹.[4]

Standard treatment options:

1. For maxillary sinus tumors:
   • After surgery, radiation therapy or craniofacial resection with postoperative radiation therapy.
   • After radiation therapy, craniofacial resection if indicated.
   • Chemotherapy should be considered after failure of the above.



2. For ethmoid sinus tumors:[5-7]
   • After limited surgery, craniofacial resection or radiation therapy or both.
   • After radiation therapy, craniofacial resection.
   • Chemotherapy should be considered after failure of the above.



3. For sphenoid sinus tumors:
   • Treatment is the same as for nasopharyngeal cancers, primarily radiation therapy.
   • Chemotherapy should be considered after failure of the above.



4. For nasal cavity tumors (squamous cell carcinomas) salvage is possible in approximately 25% of patients:
   • For failure after radiation therapy, craniofacial resection.
   • For failure after surgery, radiation therapy.
   • Chemotherapy should be considered after failure of the above.



5. For inverting papilloma:
   • Surgical excision.
   • Re-excision for surgery failures.
   • Radical surgery or radiation therapy may eventually be necessary.



6. For melanomas and sarcomas:
   • Surgical excision if possible.
   • Appropriate chemotherapy geared specifically to cell type. (See specific sections elsewhere in PDQ.)



7. For midline granuloma:
   • Radiation therapy to nasal cavity and paranasal sinuses.



8. For nasal vestibule tumors:
   • For radiation therapy failures, surgery.
   • For surgery failures, radiation therapy or a combination of surgery and radiation therapy.
   • Chemotherapy should be considered after failure of the above.

Treatment options under clinical evaluation:

• For maxillary sinus tumors, ethmoid sinus tumors, nasal cavity tumors, and nasal vestibule tumors, clinical trials using chemotherapy should be considered.[8,9]

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent paranasal sinus and nasal cavity cancer ¹². The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site ⁷.

References

1. Kies MS, Levitan N, Hong WK: Chemotherapy of head and neck cancer. Otolaryngol Clin North Am 18 (3): 533-41, 1985.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   
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.  [PUBMED Abstract]
   
   
5. 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.  [PUBMED Abstract]
   
   
6. 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.  [PUBMED Abstract]
   
   
7. Shah JP: Surgery of the anterior skull base for malignant tumors. Acta Otorhinolaryngol Belg 53 (3): 191-4, 1999.  [PUBMED Abstract]
   
   
8. 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.  [PUBMED Abstract]
   
   
9. 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.  [PUBMED Abstract]
   
   

Get More Information From NCI

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The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator ¹⁵. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

Changes to This Summary (07/11/2008)

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.

Cellular Classification ¹⁶

Added Jethanamest et al. as reference 3 ¹⁷.

More Information

About PDQ

• PDQ® - NCI's Comprehensive Cancer Database ¹⁸.

  Full description of the NCI PDQ database.

Additional PDQ Summaries

• PDQ® Cancer Information Summaries: Adult Treatment ¹⁹

  Treatment options for adult cancers.

• PDQ® Cancer Information Summaries: Pediatric Treatment ²⁰

  Treatment options for childhood cancers.

• PDQ® Cancer Information Summaries: Supportive and Palliative Care ²¹

  Side effects of cancer treatment, management of cancer-related complications and pain, and psychosocial concerns.

• PDQ® Cancer Information Summaries: Screening/Detection (Testing for Cancer) ²²

  Tests or procedures that detect specific types of cancer.

• PDQ® Cancer Information Summaries: Prevention ²³

  Risk factors and methods to increase chances of preventing specific types of cancer.

• PDQ® Cancer Information Summaries: Genetics ²⁴

  Genetics of specific cancers and inherited cancer syndromes, and ethical, legal, and social concerns.

• PDQ® Cancer Information Summaries: Complementary and Alternative Medicine ²⁵

  Information about complementary and alternative forms of treatment for patients with cancer.

Important:

This information is intended mainly for use by doctors and other health care professionals. If you have questions about this topic, you can ask your doctor, or call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).