Malignant Tumors of the Nasal Cavity
- Author: Ricardo Luis Carrau, MD; Chief Editor: Arlen D Meyers, MD, MBA more...
Background
Sinonasal malignant neoplasms are rare tumors that constitute about 3% of tumors in the upper respiratory tract. Only a fraction arises at the nasal cavity.[1] Due to the contiguity of the nasal cavities with the paranasal sinuses, identifying the specific site of origin of large sinonasal tumors is often difficult. Henceforth, malignant tumors of the nasal cavities are often grouped with those in the paranasal sinuses. Their proximity to vital structures such as the brain, optic nerves, and internal carotid artery pose significant challenges for their treatment and may be the source of significant morbidity to the patients. Malignant tumors of the sinonasal tract are derived from diverse histologic elements within the nasal cavity. They include the following:
Epithelial
- Transitional cell carcinoma
- Adenocarcinoma
- Adenoid cystic carcinoma
- Olfactory neuroblastoma
- Undifferentiated carcinoma
Nonepithelial
- Soft-tissue sarcoma
- Leiomyosarcoma
- Myxosarcoma
- Hemangiopericytoma
- Connective tissue sarcoma
- Synovial sarcoma
Lymphoreticular tumors
- Lymphoma
- Plasmacytoma
- Metastatic carcinoma
Epidemiology
Frequency
The annual incidence of nasal tumors in the United States is estimated to be less than 1 in 100,000 people per year. These tumors occur most commonly in whites, and the incidence in males is twice that of females.[2] Epithelial tumors most commonly present in the fifth and sixth decades of age.
Although tumors of the nasal cavities are equally divided between benign and malignant types, most tumors of the paranasal sinuses are malignant. Approximately 55% of sinonasal tumors originate from the maxillary sinuses, 35% from the nasal cavities, 9% from the ethmoid sinuses, and the remainder from the frontal and sphenoid sinuses. Squamous cell carcinoma is the most common malignant histologic type (approximately 70-80%) followed by adenoid cystic carcinoma and adenocarcinoma (approximately 10% each).[3]
Etiology
Exposures to industrial fumes, wood dust, nickel refining, and leather tanning have all been implicated in the carcinogenesis of various types of sinonasal malignant tumors. In particular, wood dust and leather tanning exposures are well associated with increased risk for adenocarcinoma.[4] Other etiologic agents have been reported including mineral oils, chromium and chromium compounds, isopropyl oils, lacquer paint, soldering and welding, and radium dial painting. Tobacco smoking is not considered to be a significant etiologic factor; however, recent studies demonstrated a higher incidence of nasal cancers in cigarette smokers.[2, 5]
Presentation
Tumors of the sinonasal tract commonly present with symptoms that are identical to those caused by inflammatory sinus disease, such as nasal obstruction, nasal discharge, epistaxis, headache, facial pain, and nasal discharge. Tumors of nasal cavities, however, tend to be diagnosed earlier than those of the paranasal sinuses because of the earlier presentation of obstructive symptoms and epistaxis.
To further complicate this issue, 9-12% of patients are frequently asymptomatic.[6] These factors contribute to a delay in diagnoses, and, hence, an advanced stage of disease at the time of diagnosis. Patients with unilateral sinonasal symptoms or those that are associated with unilateral facial swelling, diplopia or blurred vision, unilateral proptosis, and cranial neuropathies should raise a high index of suspicion for sinonasal cancer and warrant urgent evaluation.
Regional and distant metastases are infrequent even in the presence of advanced stage tumors. The incidence of cervical metastases on initial presentation varies from 1-26%, with most large series reporting less than 10%. Distant metastasis on initial presentation is even less common, with most series presenting an incidence of less than 7%.[1] The presence of regional or distant metastases is a poor prognostic sign.
A thorough head and neck examination, cranial nerve assessment, and a nasal endoscopy should be performed in all patients. Physical examination may reveal proptosis, extraocular muscle impairment, mass effect of the cheek, gingival or gingivobuccal sulcus, (eg, ill-fitting dentures) and loose dentition. Numbness or hyperesthesia of the infraorbital (V2) branch of the maxillary nerve strongly suggests malignant invasion (as in the images below).
A nasal cavity tumor has eroded through the hard palate and is causing difficulty with fitting a denture.
A nasal tumor that has eroded through the nasal bone and causing deformity of the nasal bridge. Relevant Anatomy
By examining the close relationships of the nasal cavities to the oral cavity, paranasal sinuses, orbit, nasopharynx, pterygomaxillary fissure and pterygopalatine fossa, infratemporal fossa, skull base, and intracranial fossa, one can better understand the myriad signs and symptoms caused by sinonasal tumors.
Local tumor invasion can breach the boundaries of the nasal cavity invading and destroying structures and/or following preformed pathways. The paired nasal cavities are separated by the nasal septum. Their lateral walls comprise the medial wall of the maxillary sinus and the inferior, middle, and superior turbinates. Lateral extension of tumor can infiltrate the maxillary sinus, ethmoid air cells, or even the orbit (through the lamina papyracea). Eventually, orbital involvement manifests as ocular pain, fullness of the eyelid, unilateral epiphora, diplopia, extraocular muscle limitation/diplopia, or proptosis. The floor of the nasal cavity corresponds to the hard palate of the oral cavity; thus, caudal extension of the tumor can present as palatal fullness, pain, and ulceration.
The roof of the nasal cavities is formed by the cribriform plate, which separates the dura of the anterior cranial fossa from the nasal cavity. The cribriform plate, as implied by its name, has multiple openings to accommodate the passage of olfactory filaments. Tumor can spread to the anterior cranial fossa using these openings or by perineural spread. Violation of this barrier during surgery is likely to produce a cerebrospinal fluid (CSF) leak, increasing the risk for meningitis and intracranial abscess. The nasal cavities open externally via the nares and communicate posteriorly with the nasopharynx via the choanae. The eustachian tubes open into the nasopharynx just behind the infero-lateral aspect of the choanae. Tumor extension into the nasopharynx may cause eustachian tube obstruction and secondary serous otitis media that manifests as hearing loss.
Except in the nasal vestibule, the nasal cavity is lined with pseudostratified columnar ciliated epithelium. The nasal vestibule, which corresponds to the ala of the nose, is lined with squamous epithelium containing vibrissae and sweat and sebaceous glands. A small part of the superior portion of the nasal cavity (bound by the superior turbinate laterally and the nasal septum medially) is lined by olfactory epithelium.
The pterygopalatine and infratemporal fossae are important anatomical considerations, as they are densely populated by the mastication muscles, various sensory and motor nerves, and by the blood vessels that supply the nasal cavity, oral cavity, maxillary teeth, pharynx, and ICAs. Tumor extension into these areas can cause a myriad of symptoms, such as the following:
1. Trismus (involvement of the pterygoid muscles or motor branches of the mandibular division of the trigeminal nerve)
2. Facial hypesthesia (involvement of the infraorbital nerve or other sensory branches from the maxillary and mandibular divisions of the trigeminal nerve)
3. Pain in the maxillary dentition (involvement of the anterior, middle, or posterior superior alveolar nerve branches of the maxillary division of the trigeminal nerve)
4. Severe epistaxis (involvement of the terminal branches of the internal maxillary artery)
The pterygopalatine and infratemporal fossae are also potential routes for intracranial tumor spread, via direct extension or hematogenous spread.
Zimmer LA, Carrau RL. Neoplasms of the nose and paranasal sinuses. In: Bailey BJ, Johnson JT, Newland SD, eds. Head & Neck Surgery - Otolaryngology. 4th. Lippincott, Williams & Wilkins; 2006.
Caplan LS, Hall I, Levine RS, Zhu K. Preventable risk factors for nasal cancer. Ann Epidemiol. 2000;10:186-91.
Weymuller EA, Gal TJ. Neoplasms of the nasal cavity. In: Cummings CW, Flint PW, Harker LA et al. eds. Otolaryngology - Head and Neck surgery. 4th. Mosby; 2005.
d'Errico A, Pasian S, Baratti A, et al. A case-controlled study on occupational risk factors for sino-nasal cancer. Occup Environ Med. 2009;66:448-55.
Benninger MS. The impact of cigarette smoking and environmental tobacco smoke on nasal and sinus disease: a review of the literature. Am J Rhinol. Nov-Dec 1999;13(6):435-8. [Medline].
Jackson RT, Fitz-Hugh GS, Constable WC. Malignant neoplasms of the nasal cavities and paranasal sinuses: (a retrospective study). Laryngoscope. May 1977;87(5 Pt 1):726-36. [Medline].
Sasaki M, Eida S, Sumi M, Nakamura T. Apparent diffusion coefficient mapping for sinonasal diseases: differentiation of benign and malignant lesions. AJNR Am J Neuroradiol. Jun 2011;32(6):1100-6. [Medline].
Snyderman CH, Carrau RL, deVries EJ. Johnson JT, Derkay CS, Mandell-Brown MK, Newman RK eds. Carotid artery resection: update on preoperative evaluation. 6. Mosby; 1993:341-4.
Mansell NJ, Bates GJ. The inverted Schneiderian papilloma: a review and literature report of 43 new cases. Rhinology. Sep 2000;38(3):97-101. [Medline].
Schlosser RJ, Mason JC, Gross CW. Aggressive endoscopic resection of inverted papilloma: an update. Otolaryngol Head Neck Surg. Jul 2001;125(1):49-53. [Medline].
Kraft M, Simmen D, Kaufmann T, Holzmann D. Long-term results of endonasal sinus surgery in sinonasal papillomas. Laryngoscope. Sep 2003;113(9):1541-7. [Medline].
Nicolai P, Villaret AB, Bottazzoli M, Rossi E, Valsecchi MG. Ethmoid Adenocarcinoma--From Craniofacial to Endoscopic Resections: A Single-Institution Experience over 25 Years. Otolaryngol Head Neck Surg. Aug 2011;145(2):330-7. [Medline].
Hanna EYN, Westfall C. Cancer of the nasal cavity, paranasal sinuses and orbit. In: Myers EN, Suen JY, Myers JN, Hanna EYN, eds. Cancer of the head and neck. 4th. Saunders; 2003.
Barnes L. Surgical pathology of the head and neck. 2nd. Marcel Dekker; 2001.
Kida A, Endo S, Iida H, et al. Clinical assessment of squamous cell carcinoma of the nasal cavity proper. Auris Nasus Larynx. 1995;22(3):172-7. [Medline].
Heffner DK, Hyams VJ, Hauck KW, Lingeman C. Low-grade adenocarcinoma of the nasal cavity and paranasal sinuses. Cancer. Jul 15 1982;50(2):312-22. [Medline].
Van Gerven L, Jorissen M, Nuyts S, Hermans R, Vander Poorten V. Long-term follow-up of 44 patients with adenocarcinoma of the nasal cavity and sinuses primarily treated with endoscopic resection followed by radiotherapy. Head Neck. Jun 2011;33(6):898-904. [Medline].
Claus F, Boterberg T, Ost P, et al. Postoperative radiotherapy for adenocarcinoma of the ethmoid sinuses: treatment results for 47 patients. Int J Radiat Oncol Biol Phys. Nov 15 2002;54(4):1089-94. [Medline].
Knegt PP, Ah-See KW, vd Velden LA, Kerrebijn J. Adenocarcinoma of the ethmoidal sinus complex: surgical debulking and topical fluorouracil may be the optimal treatment. Arch Otolaryngol Head Neck Surg. Feb 2001;127(2):141-6. [Medline].
Almeyda R, Capper J. Is surgical debridement and topical 5 fluorouracil the optimum treatment for woodworkers' adenocarcinoma of the ethmoid sinuses? A case-controlled study of a 20-year experience. Clin Otolaryngol. Oct 2008;33(5):435-41. [Medline].
Rhee CS, Won TB, Lee CH, et al. Adenoid cystic carcinoma of the sinonasal tract: treatment results. Laryngoscope. Jun 2006;116(6):982-6. [Medline].
Lupinetti AD, Roberts DB, Williams MD, et al. Sinonasal adenoid cystic carcinoma: the M. D. Anderson Cancer Center experience. Cancer. Dec 15 2007;110(12):2726-31. [Medline].
Szanto PA, Luna MA, Tortoledo ME, White RA. Histologic grading of adenoid cystic carcinoma of the salivary glands. Cancer. Sep 15 1984;54(6):1062-9. [Medline].
Spiro RH. Distant metastasis in adenoid cystic carcinoma of salivary origin. Am J Surg. Nov 1997;174(5):495-8. [Medline].
Frierson HF Jr, Mills SE, Fechner RE, Taxy JB, Levine PA. Sinonasal undifferentiated carcinoma. An aggressive neoplasm derived from schneiderian epithelium and distinct from olfactory neuroblastoma. Am J Surg Pathol. Nov 1986;10(11):771-9. [Medline].
Enepekides DJ. Sinonasal undifferentiated carcinoma: an update. Curr Opin Otolaryngol Head Neck Surg. Aug 2005;13(4):222-5. [Medline].
Cerilli LA, Holst VA, Brandwein MS, Stoler MH, Mills SE. Sinonasal undifferentiated carcinoma: immunohistochemical profile and lack of EBV association. Am J Surg Pathol. Feb 2001;25(2):156-63. [Medline].
Mendenhall WM, Mendenhall CM, Riggs CE Jr, Villaret DB, Mendenhall NP. Sinonasal undifferentiated carcinoma. Am J Clin Oncol. Feb 2006;29(1):27-31. [Medline].
Hyams VJ. Olfactory neuroblastoma (case 6). In: Batsakis JG, Hyams VJ, Morales AR, eds. Specifial tumors of the head and neck. Chicago: American Society of Clinical Pathologist Press; 1992:24-29.
Dulguerov P, Calcaterra T. Esthesioneuroblastoma: the UCLA experience 1970-1990. Laryngoscope. Aug 1992;102(8):843-9. [Medline].
Folbe A, Herzallah I, Duvvuri U, et al. Endoscopic endonasal resection of esthesioneuroblastoma: a multicenter study. Am J Rhinol Allergy. Jan-Feb 2009;23(1):91-4. [Medline].
Devaiah AK, Andreoli MT. Treatment of esthesioneuroblastoma: a 16-year meta-analysis of 361 patients. Laryngoscope. Jul 2009;119(7):1412-6. [Medline].
Dulguerov P, Allal AS, Calcaterra TC. Esthesioneuroblastoma: a meta-analysis and review. Lancet Oncol. Nov 2001;2(11):683-90. [Medline].
Loy AH, Reibel JF, Read PW, et al. Esthesioneuroblastoma: continued follow-up of a single institution's experience. Arch Otolaryngol Head Neck Surg. Feb 2006;132(2):134-8. [Medline].
Dauer EH, Lewis JE, Rohlinger AL, Weaver AL, Olsen KD. Sinonasal melanoma: a clinicopathologic review of 61 cases. Otolaryngol Head Neck Surg. Mar 2008;138(3):347-52. [Medline].
Mendenhall WM, Amdur RJ, Hinerman RW, Werning JW, Villaret DB, Mendenhall NP. Head and neck mucosal melanoma. Am J Clin Oncol. Dec 2005;28(6):626-30. [Medline].
Lund VJ, Howard DJ, Harding L, Wei WI. Management options and survival in malignant melanoma of the sinonasal mucosa. Laryngoscope. Feb 1999;109(2 Pt 1):208-11. [Medline].
Wagner M, Morris CG, Werning JW, Mendenhall WM. Mucosal melanoma of the head and neck. Am J Clin Oncol. Feb 2008;31(1):43-8. [Medline].
Hillstrom RP, Zarbo RJ, Jacobs JR. Nerve sheath tumors of the paranasal sinuses: electron microscopy and histopathologic diagnosis. Otolaryngol Head Neck Surg. Mar 1990;102(3):257-63. [Medline].
Hicks J, Flaitz C. Rhabdomyosarcoma of the head and neck in children. Oral Oncol. Jul 2002;38(5):450-9. [Medline].
Hawkins WG, Hoos A, Antonescu CR, et al. Clinicopathologic analysis of patients with adult rhabdomyosarcoma. Cancer. Feb 15 2001;91(4):794-803. [Medline].
Fyrmpas G, Wurm J, Athanassiadou F, et al. Management of paediatric sinonasal rhabdomyosarcoma. J Laryngol Otol. Sep 2009;123(9):990-6. [Medline].
Raney RB, Meza J, Anderson JR, et al. Treatment of children and adolescents with localized parameningeal sarcoma: experience of the Intergroup Rhabdomyosarcoma Study Group protocols IRS-II through -IV, 1978-1997. Med Pediatr Oncol. Jan 2002;38(1):22-32. [Medline].
Herve S, Abd Alsamad I, Beautru R, et al. Management of sinonasal hemangiopericytomas. Rhinology. Dec 1999;37(4):153-8. [Medline].
Vidal RW, Devaney K, Ferlito A, Rinaldo A, Carbone A. Sinonasal malignant lymphomas: a distinct clinicopathological category. Ann Otol Rhinol Laryngol. Apr 1999;108(4):411-9. [Medline].
Nasal cavity and paranasal sinues. In: Greene FL, Page DL, Fleming ID, et al. eds. AJCC Cancer Staging Manual. 6. New York: Springer - Verlga; 2002:59-67.
Hadad G, Bassagasteguy L, Carrau RL, et al. A novel reconstructive technique after endoscopic expanded endonasal approaches: vascular pedicle nasoseptal flap. Laryngoscope. Oct 2006;116(10):1882-6. [Medline].
Nicolai P, Battaglia P, Bignami M, et al. Endoscopic surgery for malignant tumors of the sinonasal tract and adjacent skull base: a 10-year experience. Am J Rhinol. May-Jun 2008;22(3):308-16. [Medline].
Lund V, Howard DJ, Wei WI. Endoscopic resection of malignant tumors of the nose and sinuses. Am J Rhinol. Jan-Feb 2007;21(1):89-94. [Medline].
Lee NY, Le QT. New developments in radiation therapy for head and neck cancer: intensity-modulated radiation therapy and hypoxia targeting. Semin Oncol. Jun 2008;35(3):236-50. [Medline].
Chen AM, Daly ME, Bucci MK, et al. Carcinomas of the paranasal sinuses and nasal cavity treated with radiotherapy at a single institution over five decades: are we making improvement?. Int J Radiat Oncol Biol Phys. Sep 1 2007;69(1):141-7. [Medline].
Hoppe BS, Stegman LD, Zelefsky MJ, et al. Treatment of nasal cavity and paranasal sinus cancer with modern radiotherapy techniques in the postoperative setting--the MSKCC experience. Int J Radiat Oncol Biol Phys. Mar 1 2007;67(3):691-702. [Medline].
Daly ME, Chen AM, Bucci MK, et al. Intensity-modulated radiation therapy for malignancies of the nasal cavity and paranasal sinuses. Int J Radiat Oncol Biol Phys. Jan 1 2007;67(1):151-7. [Medline].
Patel SG, Singh B, Polluri A, et al. Craniofacial surgery for malignant skull base tumors: report of an international collaborative study. Cancer. Sep 15 2003;98(6):1179-87. [Medline].
Suárez C, Ferlito A, Lund VJ, et al. Management of the orbit in malignant sinonasal tumors. Head Neck. Feb 2008;30(2):242-50. [Medline].

