You are in: eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > HEAD AND NECK ONCOLOGY Malignant Tumors of the Nasal CavityArticle Last Updated: Nov 16, 2007AUTHOR AND EDITOR INFORMATIONSection 1 of 10
  Author: Weiru Shao, MD, Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, Tufts University School of Medicine Weiru Shao is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery Coauthor(s): Adarsh Vasanth, MD, Resident Physician, Department of Otolaryngology, New England Medical Center Editors: William M Lydiatt, MD, Associate Professor, Department of Otolaryngology-Head and Neck Surgery, University of Nebraska Medical Center; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Nader Sadeghi, MD, FRCS(C), Associate Professor of Surgery, Director of Head and Neck Surgery, Department of Surgery, Division of Otolaryngology, George Washington University; Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders; Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine Author and Editor Disclosure Synonyms and related keywords: malignant tumors of the nasal cavity, epithelial tumors, squamous cell carcinoma, SCCA, glandular tumor, adenocarcinoma, AC, adenoid cystic carcinoma, ACC, undifferentiated carcinoma, soft-tissue tumors, malignant lymphoma, chondrosarcoma, osteosarcoma, hemangiopericytoma, metastatic carcinoma (kidney, lung, breast), miscellaneous tumors, malignant melanoma, esthesioneuroblastoma INTRODUCTIONSection 2 of 10
  Sinonasal malignant neoplasms are diverse and uncommon diseases. Large clinical studies with adequate patient population are scarce. Because of a lack of sufficient clinical data, many treatment regimens remain empirical and controversial. This article discusses the considerations in the evaluation and treatment of the malignancies that arise in the nasal cavity. Malignant tumors of the sinonasal tract include the following:
FrequencyEpidemiologic studies estimate that the annual incidence of nasal tumors in the United States is less than 1 per 100,000 people. Sinonasal neoplasms represent approximately 3% of all upper aerodigestive tract tumors, and only a fraction of these arise from the nasal cavity. Tumors of the nasal cavity are equally divided between benign and malignant types, while most paranasal sinus tumors are malignant. Approximately 55% of sinonasal tumors originate from the maxillary sinus, 35% from the nasal cavity, 9% from the ethmoid sinus, and the remainder from the frontal and sphenoid sinuses. The site of origin may be difficult to identify with large tumors.1 Sinonasal malignancies are seen predominantly in the fifth to sixth decade of life. The incidence in males is twice that in females. Most series document SCCA as the most common histologic type, with an incidence of roughly 80%. ACC and AC are next in frequency (approximately 10%). Numerous other tumors complete the list in small numbers.1 Except for nonepithelial neoplasms, malignant nasal tumors are diseases of adults. EtiologyMany environmental factors have been implicated in the carcinogenesis of certain types of sinonasal malignancies, including exposure to industrial fumes, wood dust, nickel-refining processes, and leather tanning. Exposure to mineral oils, chromium, lacquer paint, soldering, and welding has also been associated with an increased incidence of sinonasal malignant tumors. Although tobacco and alcohol are well known risk factors for head and neck malignancy, no significant association has been shown with sinonasal cancers (with the exception of cigarette smoking for SCCA). ClinicalThe clinical symptoms in most sinonasal tumors lack specificity for a particular tumor type and are often indistinguishable from benign sinonasal diseases. This can lead to a delay in diagnoses of a malignancy. Sinonasal cancer is especially challenging in a patient who has been diagnosed with chronic sinusitis with temporary improvement and recurrent symptoms. Common presenting symptoms include nasal obstruction, unilateral facial, cheek and nasal swelling or pain, diplopia or blurred vision, epistaxis, headache, nasal discharge or repeated infection, unilateral proptosis, and cranial neuropathies. Tumors of the nasal cavity tend to be diagnosed earlier than those of the paranasal sinuses because of the earlier presentation of obstructive symptoms and epistaxis. Patients who present with unilateral nasal symptoms, prolonged symptoms resistant to routine treatments, and radiologic evidence of bony erosion require a high index of suspicion for sinonasal cancer. Tumor invasion is frequently underestimated based on clinical evaluation. Knowledge of the relevant anatomy of the nasal cavity and surrounding structures can help in the proper evaluation of a patient, and this knowledge is paramount in the surgical treatment of such tumors. RELEVANT ANATOMYSection 3 of 10
By examining the close relationship of the nasal cavity to the oral cavity, paranasal sinuses, orbit, nasopharynx, pterygomaxillary fissure and pterygopalatine fossa, infratemporal fossa, skull base, and intracranial fossae, one can understand the myriad signs and symptoms caused by sinonasal tumors. The paired nasal cavities are separated by the nasal septum. Local tumor invasion can breach the boundaries of the nasal cavity. The lateral wall of each nasal cavity is made up of 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). Orbital involvement can present as ocular pain, fullness of the eyelid, unilateral epiphora, diplopia, exophthalmos, or proptosis. The floor of the nasal cavity is the hard palate of the oral cavity; inferior extension of nasal cavity tumors can present as palatal fullness and pain. The roof of the nasal cavities is formed by the cribriform plate, which shields the dura in the anterior cranial fossa from the nasal cavity. This is a potential area of intracranial tumor spread, and violation of this barrier during surgery can cause a cerebrospinal fluid (CSF) leak and an increased 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 at the level of the choanae. Tumor extension into the nasopharynx may cause eustachian tube obstruction and secondary serous otitis media that presents 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 and contains vibrissae and sweat and sebaceous glands. A small part of the superior portion of the nasal cavity (bound by the superior nasal concha laterally) is lined by olfactory epithelium. The pterygopalatine and infratemporal fossae are important considerations, as they contain various sensory and motor nerves and blood vessels that supply the nasal cavity, oral cavity, maxillary teeth, and pharynx. Tumor extension into these areas can cause myriad symptoms, such as the following:
The pterygopalatine and infratemporal fossae are also potential routes for intracranial tumor spread, via direct extension or hematogenous spread. CONTRAINDICATIONSSection 4 of 10
Contraindications to surgery include the following:
WORKUPSection 5 of 10
Imaging Studies
Diagnostic Procedures
Histologic FindingsStaging), and the 5-year survival rate for squamous carcinoma is 35-50%. The recommended management is combined therapy that includes radiotherapy and surgery, such as maxillectomy with or without orbital exenteration. Radiotherapy is often complicated by severe panophthalmopathy with the development of corneal ulceration within 2 years of treatment. With exposure to 28-54 Gy, most patients eventually experience visual disturbances and radiation-induced cataracts.1 Radiation exposure greater than 60 Gy. guarantees vision loss secondary to retinal damage. The rate of early and late lymph node metastasis is 18-23%. Nodal treatment is indicated when the nodes are clinically involved (see Staging).
Histologic grading also influences prognosis. Low-grade tumors are well differentiated with minimal mitotic activity. High-grade tumors are poorly differentiated with nuclear pleomorphism and higher mitotic activity. In one study of 50 cases of sinonasal tract AC (excluding ACC and mucoepidermoid carcinoma), 78% of the 23 patients diagnosed with low-grade ethmoid AC survived with no evidence of disease at a mean follow-up of 6.3 years. At the same time, only 7% of the 27 patients with high-grade lesions had no evidence of disease; 68% of them died of disease within 3 years of initial treatment.8 The management of nasal AC consists of complete surgical excision to achieve local control. Because of the intricate anatomy of the nasal cavity and the skull base, en bloc resection with negative margins might not be possible in difficult cases, even with craniofacial resection. Radiotherapy is recommended for positive margins after resection; for high-grade, aggressive, or large-volume lesions; and for disease that involves dura or cribriform plate.6 Radiotherapy or chemotherapy alone has a limited role in the initial treatment of primary nasal AC without metastases. Local recurrence, persistent tumor at the skull base, infratemporal fossa invasion, and dural involvement are the causes of death in most patients. Nodal involvement at presentation is not common. Elective neck dissection or radiotherapy for clinical N0 neck is not recommended.6 Lymphoma Malignant lymphomas that arise from the nasal cavity and paranasal sinuses are rare. They comprise 5.8-8% of the extranodal lymphomas in the head and neck area. They are the most common nonepithelial malignant tumors of the nose.9 Although they are typically observed in adults in their fourth and fifth decades, they are also observed in young children with a median age of 5 years. Recent advancement in immunohistochemistry has significantly improved the understanding and, hence, the diagnoses of nasal lymphomas. Prior to immunophenotyping of lymphocytes, clinical diagnoses such as lethal midline granuloma, idiopathic midline destructive disease, or polymorphic reticulosis were used to describe progressive nonhealing ulceration and necrosis in the nasal cavity. Now, many of these lesions are understood to be lymphomas, often of T-cell or natural killer (NK)–cell origin. T-cell lymphomas are widely prevalent in Further studies in the Asian population revealed that many of the T lymphomas possess markers of NK cells.11 These patients with sinonasal lymphoma had a preponderant incidence of Epstein-Barr virus (EBV) infection, which parallels the high prevalence of EBV in Asia and Once viral infection (EBV in this case) challenges the host, the NK cells are activated and recruited to the nasal mucosa. Some speculate that a single clone of NK cells, influenced by other possible molecular accidents, continues to proliferate and ultimately leads to NK-cell lymphoma. At the present time, many investigators agree that T-cell and NK-cell lymphomas of the sinonasal region are, almost without exception, associated with EBV. A similar association between EBV and NK-cell proliferation is also observed in posttransplant immunoproliferative disorders. Nevertheless, T-cell and NK-cell lymphomas from other sites are not well correlated with EBV infection.9 By contrast, 55-85% of sinonasal lymphomas in Western studies are described as being of B-cell origin. They are believed to arise from sinonasal mucosa–associated lymphoid tissue (MALT) that resides in the subepithelium. MALT contains specialized clusters of lymphocytes next to mucosal surfaces. It protects vulnerable mucosa in close contact with, or permeable to, the external environment. It is characterized by chronic inflammatory infiltrates. Acquired MALT can arise in the setting of chronic inflammation or autoimmune disease. In addition to the sinonasal region, MALT-derived B-cell lymphomas have been found in the larynx, thyroid gland, salivary gland, trachea, orbit, gastrointestinal tract, lung, breast, kidney, prostate, liver, gallbladder, and uterine cervix. Salivary MALT-derived lymphomas arise from myoepithelial sialadenitis, often in the setting of Sjögren disease. Similarly, in the stomach, MALT-derived lymphomas are associated with chronic Helicobacter pylori infection. In individuals with chronic sinusitis, well-developed submucosal lymphoid follicles may be identified. The MALT accumulation may result from inflammation in chronic sinusitis, which eventually transforms into MALT-derived lymphomas. These tumors are most likely antigen driven, and tumor regression is observed when the offending antigen is removed. For example, primary gastric MALT lymphomas may regress completely after eradication of H pylori. Clinically, T-cell and NK-cell lymphomas can be distinguished from B-cell lymphomas by location and appearance. T-cell and NK-cell lymphomas often cause severe destruction of the nasal septum and midline facial structures, which is why they are named lethal midline granuloma. They are characterized by unrelenting ulceration and necrosis. They frequently demonstrate angiocentricity or angiotropism, in which tumor cells infiltrate and destroy blood vessels, causing ischemic necrosis. An important NK-cell marker, CD56, is a cell adhesion molecule. Its expression in the tumor has been suggested to be responsible for such clinical features. Conversely, B-cell lymphomas tend to surround, but not invade, blood vessels. Extensive necrosis and ulceration are extremely rare. They arise most commonly from the paranasal sinuses in patients with a history of chronic sinusitis. During the diagnostic workup, a biopsy of adequate tissue must be taken to differentiate lymphoma from destructive nonneoplastic processes or other malignant tumors. Radiographically and grossly, they may be mistaken for SCCAs, which are far more prevalent in both Western and Asian populations than lymphomas. Because subtyping determines both therapy and prognosis, the most important role of the otolaryngologist is to provide adequate tissue in good condition for histologic processing. The 2 common mistakes of an unsuspecting surgeon include obtaining a biopsy sample that is (1) too small or (2) too superficial. As aforementioned, sinonasal lymphomas are subepithelial lesions, often with perfectly normal overlying mucosa. Superficial biopsies frequently miss deeper malignant lesions. Furthermore, immunophenotyping of lymphomas requires fresh tissue and more tissues than regular biopsy samples. For suspicious nasal lesions, submitting fresh and unfixed biopsy specimens immediately for possible lymphoma workup is best. Failure to determine the accurate diagnosis results in incorrect treatment. As with other lymphomas, surgical resection of sinonasal lymphomas is not recommended unless the tumor's spread to critical locations results in impending death. A good response is achieved with local radiotherapy alone, often with complete tumor regression. However, the incidence of metastasis and local recurrence is high (up to 49% in one study). With the addition of chemotherapy, such as cyclophosphamide, hydroxydaunomycin, Oncovin, and prednisone (CHOP), a reduction in recurrence and metastasis and improved survival rates are observed. Patients treated with chemotherapy alone have a higher risk of local recurrence. Bone marrow transplant has been attempted with mixed results in a few cases that are refractory to the combined treatment. For certain high-grade aggressive lymphomas, the CNS is at risk for tumor involvement. The current recommendation is to radiate the CNS only if there is disease involvement documented by lumbar puncture and MRI, not prophylactically. The 5-year overall survival rate for all subtypes together is 52%. Younger age, early stage of disease, and combined chemoradiotherapy correlate with a better prognosis. Tumor immunophenotype is not an independent prognostic factor based on multivariate analyses, although univariate analyses demonstrate that T-cell lymphomas are associated with a lower cure rate, a higher relapse rate, and a worse overall survival rate. Mucosal melanoma Mucosal melanomas of the head and neck are, in general, rapidly lethal neoplasms. Most nasal mucosal melanomas arise from the mucosa of the septum and the lateral nasal wall rather than from the sinuses. The overall incidence is low; mucosal melanomas account for less than 1% of all malignant melanomas.12 In addition to symptoms typical of a nasal mass, gross pigmentation is present with 50% of sinonasal melanomas. 13The cause of melanoma in solar-hidden mucosa is unclear, although smoking may have a role in the activation of preexisting melanocytes, leading to melanogenic metaplasia.14 Nasal and paranasal sinus melanomas are usually advanced when they are discovered. The possibility of regional or distant metastases must be considered, and metastases are frequently found at initial presentation. Involvement of regional lymph nodes strongly suggests distant spread, which is often not preceded by lymphatic spread. For both cutaneous and mucosal melanomas, the single most powerful predictor of survival is the status of regional lymph nodes. Once spread to regional lymph node has occurred, prognostic information derived from the primary melanoma, such as depth of invasion, helps little in comparison with the number of, not the size of, the involved lymph nodes.14 Clark and Breslow scales are of low prognostic value in mucosal melanoma. Mucosal melanoma tends to follow a more rapidly lethal course than its cutaneous counterpart. This rapid course is due in part to the advanced stage at discovery in terms of regional and distant metastases and deeper invasion at the primary site. Other factors include failure of early detection and possible unknown intrinsic tumor biology of mucosal melanoma. The overall prognosis and survival rates are poor. Close to one half of patients die within 3 years; of those alive at 5 years, 50% may have residual disease. Virtually all patients eventually succumb to the disease because of persistent local recurrence and disseminated disease.12 The disease course is variable, depending on the host-tumor relationship. Some patients die within a few weeks or months of presentation despite aggressive surgical excision; others have a long disease-free period followed by dramatic recurrence, suggesting a disturbance in host immune competency.12 Unfortunately, the probability of death does not decrease over time, as it does with other tumors, if a patient has no evidence of disease after 4-5 years. Surgical resection offers the best treatment, although its effectiveness is questionable. The incidence of local recurrence is high, even with fresh frozen section to ensure complete excision. More radical excision, which includes the removal of the eye, palate, or external portion of the nose, may not significantly decrease the incidence of local recurrence.1 Local recurrence and even metastasis do not necessarily imply death within the near future; some patients with both have survived for several years. If possible, vigorous secondary local excision may be worthwhile.1 Radiotherapy is often used as adjuvant therapy, although the tumor is relatively radio insensitive. Adjuvant therapies, which include interferons and tumor-specific melanoma vaccine, are under investigation. Chondrosarcoma Chondrosarcoma is a malignant, slowly growing, cartilaginous tumor most commonly found in the pelvis, ribs, and long bones. Approximately 5-10% are located in the head and neck, mostly in the maxilla and mandible. Nasal septal origin is rare. Most patients are male and in the fourth decade of life. CT scan demonstrates characteristic scattered ring-forming calcifications among a hypodense matrix and often-bony erosions.15 The ringlike calcifications are related to the pattern of calcification inside the tumor and can be observed microscopically. MRIs are of low intensity on T1, high intensity on T2, and inhomogeneous enhancement on T1 with contrast. Chondrosarcoma is classified into the following 3 types:
Hemangiopericytoma Hemangiopericytoma is an uncommon vascular tumor formed by the proliferation of vascular pericytes of Zimmerman. They should be considered malignant, not in terms of a 5-year survival rate, but over the patient's lifetime.1 Vascular pericytes are of mesenchymal origin that spiral around capillaries and postcapillary venules. They are believed to be capable of differentiating into smooth muscle cells. They possess contractile properties and are able to change the lumen of blood vessels that regulate blood flow. Hemangiopericytoma is a mysterious tumor. Histopathologic studies have yet to generate reliable and reproducible criteria to distinguish the malignant from the benign.18 The tumor's behavior is inconsistent with respect to its pathologic and ultrastructural characteristics. Lesions with a benign appearance have been found to recur and metastasize. The recurrence rate is 25-50%, and the metastasis rate is 11-65%.18 Hence, the benign or malignant nature of a hemangiopericytoma is determined not histologically, but clinically, with lifelong follow-up observation. At initial workup, hemangiopericytoma should be regarded as potentially malignant.19 The malignant behavior of the tumor is speculated to be closely related to tumor location and anatomic dimension, rather than to histologic feature.19 In the paranasal sinuses and nasal cavity, hemangiopericytoma tends to be indolent; conversely, those that arise around the cribriform plate are more likely to metastasize. Lesions smaller than 6.5 cm in greatest dimension have a low potential to metastasize. The tumor can appear in persons of any age, with 80-95% of patients older than 20 years. Most develop in the extremities, especially the lower limbs.19 Approximately 15-30% of hemangiopericytomas develop in the head and neck region; sinonasal presentation is rare.18 Nevertheless, it should be included in the differential diagnoses for a nasal mass. Its clinical appearance is a firm, usually well-circumscribed swelling of the mucosa.1 Approximally 10% of hemangiopericytomas are found in children. Their prognosis is more favorable than the prognosis in adults. Optimal treatment is wide excision with negative margins. Tumor cells may be left behind with enucleation. For unresectable or incompletely excised tumors, radiotherapy has an important role. Hemangiopericytomas metastasize via lymphatics and blood streams to the lung, bone, liver, and local lymph nodes. Regional lymph node involvement at presentation is unusual. Lung and bone are the most frequent sites for distant metastases.20 Esthesioneuroblastoma (olfactory neuroblastoma) Olfactory neuroblastoma is a rare neoplasm of neuroectodermal origin that arises from the olfactory epithelium at the cribriform plate level. Olfactory neuroblastomas have a bimodal distribution; incidence peaks in persons aged 11-20 years and again in individuals aged 50-60 years. The tumor originates from premature neuroblasts and spreads submucosally and intracranially. MRI demonstrates hypointensity or isointensity on T1 and hyperintensity in T2.1 Management is surgical resection, which often necessitates a craniofacial approach, followed by postoperative radiation. Although patients with early-stage diseases do better, the prognosis in patients with high-grade tumors is poor compared with the prognosis in patients with low-grade tumors. The addition of platinum-containing chemotherapy as adjuvant therapy to advanced esthesioneuroblastoma (T3 and T4) is reasonable to improve the survival rate, but not for cure. Despite this neuroblastoma's sensitivity to platinum-based chemotherapy, many patients experience recurrence or progression over time.21 The mean time for recurrence is reported as 4.67 years.22 Long-term follow-up is warranted. Regional metastasis to the neck and distant metastasis to the lung often occur. These patients may have a long and protracted disease course and can be treated with radiotherapy because the tumor is considered radiosensitive. Undifferentiated carcinoma Sinonasal undifferentiated carcinoma (SNUC) is an uncommon and aggressive malignancy with a mean survival time of less than 1 year after diagnosis.23 A significant number of patients smoke. Other unknown environmental carcinogens may also play a role in its development.1 The disease is often advanced locally at presentation. The tumor has a tendency to spread intracranially. More than 50% of patients initially exhibit ophthalmologic symptoms, such as proptosis, diplopia, and decreased visual acuity. Histologically, the cells have high nucleus-to-cytoplasm ratios and numerous mitoses. Virtually all of them are positive for cytokeratin, epithelial membrane antigen, or both.23 Whatever treatment modality is used, outcomes have remained grave. Some investigators recommend a multimodal approach that includes chemotherapy, radiotherapy, and surgical resection for patients without distant metastases and without extensive intracranial involvement. Dural involvement can be addressed with en bloc resection of the tumor. Patients with brain parenchymal invasion have exceptionally poor prognoses. To avoid surgical morbidity for these patients, surgical resection should be reserved until the intracranial disease demonstrates a response to chemotherapy and radiotherapy. Even in persons with significant tumor shrinkage, the entire pretherapy extent of tumor should be resected.23 Inverted papilloma The surgical management of the inverted papilloma, a potentially malignant sinonasal tumor, remains controversial. First described in 1854 by Ward and then in 1855 by Billroth (Billroth, 1855), the lesion was long considered in some way associated with simple nasal polyposis. Inverted papilloma is now known to be a distinct pathologic entity that should be distinguished from allergic or inflammatory polyps in diagnosis and treatment. The incidence of inverted papilloma has been estimated at 0.74 per 100,000 population per year.24 The male-to-female ratio is 2-4:1. On average, the pathologic condition presents early in the sixth decade of life. The tumor represents 0.5-4% of all primary nasal malignancies.25 The 1995 World Health Organization (WHO) classification of intranasal papilloma includes the following 3 histopathologic classes:26
The clinical behavior of inverted papilloma is closely associated with the location of the involved mucosa (ie, medial or lateral nasal wall). Papillomas on the medial nasal wall tend to have a well-defined localized attachment with early presentation, whereas lateral nasal wall lesions have a diffuse attachment and are more often advanced at presentation. Nearly 95% of the tumors arise on the lateral wall in the region of the middle turbinate and meatus.26 Extension into the maxillary, anterior, and posterior ethmoid sinus at initial presentation is frequent. Involvement of the frontal sinus, sphenoid sinus, and septum is much less common. Inverted papilloma can be locally aggressive, with bony erosion at the skull base and invasion into the orbit in addition to the adjacent sinuses. Malignant degeneration with SCCA is found in approximately 10% of inverted papillomas (range, 0-17%).28 It has been suggested to exist as a progression from the benign to the malignant because inverted papilloma, carcinoma in situ, and invasive squamous carcinomas are often found in the same pathologic specimen.29 Some evidence suggests that human papillomavirus (HPV) may be a factor in the etiology of inverted papillomas.11 In one study, HPV type 6b, HPV type 11, or both were found in 76% of inverted papillomas.30 In addition, a strong association exists between HPV type 16 and squamous neoplasm found in inverted papilloma.31, 32, 33 More recent findings suggest that HPV type 6 or HPV type 11 is associated with a low risk of malignant degeneration of inverted papilloma, whereas HPV type 16 or HPV type 18 is associated with high risk of local recurrence and progression to squamous carcinoma.26 Cigarette smoking is associated with higher risk of malignant degeneration of inverted papilloma.34 Most patients present with unilateral nasal obstruction, followed by (in decreasing frequency) nasal discharge and/or postnasal drainage, epistaxis, and headache and/or facial pain. Some patients also report symptoms such as proptosis, globus, otalgia, epiphora, and V2 hypoesthesia.28 Complete surgical excision of the diseased tissue is the treatment of choice. The main challenge in surgical excision of inverted papilloma is that the lesion tends to be more extensive than clinical examination suggests. Twelve to 30% of inverted papillomas have been demonstrated to be multicentric.26 Clean excision with true negative margin is difficult, especially around important structures such as the lamina papyracea. Incomplete removal of disease invariably leads to recurrence. The primary procedure should provide the lowest recurrence rate. Debate exists regarding the operative technique and whether surgery should be endonasal, endoscopic, or open. The open approach is more radical in the way of lateral rhinotomy with medial maxillectomy. It offers good exposure, total excision, easy postoperative examination, and, most importantly, low recurrence. In most series, the recurrence rate is 10-30% with aggressive open procedures.28 Nevertheless, the procedure is prone to complications such as epiphora, dacryocystitis, diplopia, and infraorbital hypesthesia. Later postoperative complications include epicanthal scar and/or web, crusting in the cavity with persistent pain, nasocutaneous fistula, mucocele, poor external scar, and nasal collapse. Early endonasal procedures for resection of inverted papilloma consisted primarily of intranasal ethmoidectomy and polypectomy with the use of headlights rather than endoscopes. The recurrence rate was high (up to 80%).24, 28 Since functional endoscopic sinus surgery (FESS) was used for the resection, the crude overall recurrence rate remains high (from 43%1 to 60%26). The main factor that precipitated the high recurrence rate was suggested to be piecemeal removal of inverted papilloma, which left behind diseased mucosa during endoscopic surgery. Interestingly, FESS is now beyond its infancy and many otolaryngology surgeons are skilled in its performance with good exposure and cosmetic and functional results. A new endoscopic resection of inverted papilloma has been developed. This procedure involves debulking large lesions, followed by precise identification of the site of origin.28 A generous margin of normal-appearing mucosa is removed in continuity with the diseased mucosa. Total ethmoidectomy and wide maxillary antrostomy are performed. Maxillary antrostomy is made flush with the roof and posterior walls of the maxillary sinus. Anteriorly, the resection is carried to the nasolacrimal duct. The inferior border is made flush with either (1) the superior attachment of the inferior turbinate if it is free of disease and is preserved, or (2) the nasal floor if the disease requires removal of inferior turbinate. Frozen sections are obtained if margins are unclear to the operating surgeons. Once all visible papilloma and wide mucosa margins are removed, residual mucosa remnants are further denuded with a diamond burr, which is used to polish bone deep to the site of mucosal involvement. This aggressive endoscopic surgery performed by experienced surgeons has produced promising early results, with recurrence reported in the range of 0-33%.28 The superior bony buttress that divides the maxillary sinus from the lamina papyracea was responsible for 60% of the recurrence in one study and deserves meticulous mucosal removal and diamond burring.28 Long-term endoscopic surveillance and frequent follow-up care are crucial, regardless of the surgical technique used. Treating disease recurrence early may prevent malignant transformation. Recurrence is usually discovered endoscopically in 12-20 months but has been reported as late as 30-56 months.28 Nasal metastatic tumors Metastatic tumors from other locations are rarely reported in the literature. The most common metastatic tumor is renal cell carcinoma, which can present as the initial finding. One recent patient of the senior author experienced nasal obstruction resistant to 4.5 years of treatment of chronic sinusitis. Nasal biopsy suggested renal cell carcinoma, and then the primary lesion was identified. Other metastatic tumors include the lung and the breast. More data are needed for the treatment of this particular type of cancer. StagingThe American Joint Committee on Cancer (AJCC) has recently proposed a staging system for nasal cavity and ethmoid sinus tumors. This new staging system differentiates resectable (T4a) from unresectable (T4b) tumors by recognizing recent surgical advances and limitations.35
Ellingwood and Million also devised a staging system for nasal, ethmoid, and sphenoid sinus tumors by examining tumor size, osteolysis, and extension beyond the sinus.6
Esthesioneuroblastomas tend to present in an advanced stage, and treatment is usually based on the Kadish or UCLA staging systems. The UCLA classification may provide prognostic information regarding local recurrences by considering factors such as intradural and orbital invasion.36
TREATMENTSection 6 of 10
Medical therapyRadiation is the primary treatment for lymphoreticular tumors (lymphoma) and sinonasal undifferentiated carcinoma (SNUC) and for patients who are poor surgical candidates, refuse surgical treatment, or have tumors that are deemed inoperable. Chemotherapy is adjunctive because of its cytoreductive effect. As mentioned above, most sinonasal tract tumors present in an advanced stage and are managed with combined treatment that consists of surgery and preoperative or postoperative radiation therapy, with or without chemotherapy. Preoperative and postoperative radiation therapies are associated with similar survival rates. Preoperative radiation can help to decrease tumor burden in cases in which surgical resection of the initial tumor would result in severe morbidity. Otherwise, postoperative radiation is favored because the volume of tumor cells to kill is smaller, nonradiated tumor margins are better defined intraoperatively, and postoperative wound healing is more predictable.36 The role of chemotherapy in survival, local control, and development of distant metastases in sinonasal tumors is poorly defined because of the paucity of data in the literature. The addition of chemotherapy in the treatment of most sinonasal tumors is usually palliative in that it is used to help debulk a massive lesion and to relieve pain or obstruction. Chemotherapy protocols should be considered for patients at high risk for recurrence (ie, tumors with positive margins, perinerual spread, or extracapsular spread in regional metastasis). Surgical therapyEn bloc surgical resection is the primary treatment for most nasal cavity tumors, with the addition of postoperative radiation for tumors that extend into the paranasal sinuses, tumors larger than 2 cm, and tumors associated with positive surgical margins.36 Sisson proposed specific criteria for unresectability: extension of tumor to the frontal lobes (superior extension), invasion of prevertebral fascia (posterior extension), bilateral optic nerve involvement, and cavernous sinus extension (lateral extension).37 The new American Joint Committee on Cancer (AJCC) staging system for nasal cavity neoplasms also identifies criteria for unresectability in T4b tumors. A discussion of the various surgical approaches is beyond the scope of this article. The type of surgical approach used depends on tumor size and local extension. Tumors confined to the nasal cavity can be accessed via transnasal endoscopic, sublabial, or lateral rhinotomy approaches. More advanced tumors may require partial or total maxillectomy via midfacial degloving, orbital exenteration, or anterior craniofacial resection (in the case of intracranial extension). A combination of some of the aforementioned approaches may be necessary to resect certain tumors adequately. Reconstruction can range from simple closure to the use of grafts (split-thickness skin or fascia), dental obturators, or other prostheses. Surgical treatment of the neck (neck dissection) is indicated only in the event of clinically evident nodal disease. Surgery may also have a palliative role in some patients to alleviate pain or obstruction. Follow-upRoutine, long-term follow-up is necessary for proper oncological surveillance. Examination of the treated site can help to identify recurrence or even a new primary tumor. Rigid or flexible endoscopy can help to facilitate this evaluation in a postoperative patient. Abnormal findings or new symptoms that are suspicious for recurrence warrant further radiological evaluation (CT scan, MRI, or both). COMPLICATIONSSection 7 of 10
The close proximity of many vital anatomical structures to the nasal cavity is responsible for the possible complications due to local extension of the primary tumor or treatment (surgical resection and radiation therapy). Advancement of disease into the orbits, nasopharynx, skull base, intracranial fossae, pterygomaxillary fissure and pterygopalatine fossa, or infratemporal space can produce various secondary signs and symptoms. Some examples include ocular pain, proptosis, diplopia, hearing loss secondary to serous otitis media, cranial neuropathies, cheek hypesthesia, pain in maxillary dentition, and trismus. Surgical complications include bleeding, cerebrospinal fluid (CSF) leak, infection (skin and soft tissue infections, meningitis, intracranial abscess, osteomyelitis), pneumocephalus, blindness, and facial disfiguration due to extensive resections. The orbit deserves special attention because of its functional and aesthetic importance. Sacrifice of the nasolacrimal duct during a maxillectomy or subsequent stenosis of the lacrimal sac opening can cause epiphora. This can be prevented with dacryocystorhinostomy during resection or cannulation of the lacrimal canaliculi in cases of recurrent stenosis or when the lacrimal sac is resected. Limitation of extraocular muscle movement can be caused by surgical trauma of the muscle or its motor innervation or by entrapment in the craniofacial osteotomies. Entrapment should be managed with urgent surgical release. the optic nerve can compress during mobilization of the specimen. High-dose steroids and emergent surgical decompression are recommended. Enophthalmos or hypophthalmos due to loss of orbital support can be prevented or minimized with appropriate reconstructive techniques. Radiation therapy causes orbital complications in most patients. Shields and lateral fields can spare the anterior orbital segment (eyelids, conjunctiva, lacrimal gland and apparatus, cornea, lens, the rest of the anterior chamber); however, delayed vision loss can still develop in 3-5 years secondary to postradiation retinopathy or optic neuropathy. The incidence of these complications appears to be dose-related: rare below 3500 cGy, 50% to 65% with 6000 cGy to 7000 cGy, > 85% with 8000 cGy.36 Although the retina and optic nerve are radioresistant, the microvasculature is not. Chemotherapy or medical conditions such as diabetes or atherosclerosis can potentiate this effect. Complications of the anterior globe occur with full-eye irradiation. Dryness of the eye leads to severe keratitis, panophthalmitis, and blindness within one year. Enucleation is recommended for uncontrolled panophthalmitis or a painful eye. Bilateral blindness is rare (up to 8%) and is related to the irradiation of the contralateral posterior segment.36 Conformal radiation therapy reduces the percentage of radiation received by normal tissues and can decrease the incidence of optic nerve and optic chiasm complications. Osteoradionecrosis is also possible and can be managed with antibiotics, selective debridement, and hyperbaric oxygen therapy. OUTCOME AND PROGNOSISSection 8 of 10
The prognosis of treatment outcome depends on the various factors, as follows:
FUTURE AND CONTROVERSIESSection 9 of 10
Treatment of the orbit Preservation of orbital contents during surgical resection of advanced sinonasal malignant tumors that have encroached on the orbit remains a controversial issue. The 2 main points of contention are oncological safety of orbital preservation and the functional outcome in preserved eyes. More than 30 years ago, the standard surgical treatment for such cancers involved radical excision with orbital exenteration. Radiation therapy was also added perioperatively in an effort to improve survival. Presently, planned combined surgical resection and radiation therapy is the standard of care for sinonasal carcinoma. However, the treatment failure rate is high because of local recurrence; this has not changed significantly since the early 1970s. Sisson first introduced orbital preservation surgery in 1970, and the indications for orbital exenteration have since evolved. Imola et al use the following indications for orbital exenteration:
Studies that examined the oncological safety of selective orbital preservation have largely shown no difference in local recurrence or actuarial survival rates when compared with orbital exenteration. Considerable debate regarding the functional outcome of the preserved eye has taken place. Studies have shown that up to 90% of patients treated with selective orbital preservation achieve a functional eye if appropriate reconstruction such as adequate orbital support, lacrimal stenting, and secondary correction of lid malposition is performed as necessary (Imola, 2002). A significant number of patients with preserved orbits (41% in Imola's series) have some form of tolerable ocular impairment or sequelae. REFERENCESSection 10 of 10
Malignant Tumors of the Nasal Cavity excerpt Article Last Updated: Nov 16, 2007 |