18 Sinonasal Tumors Flashcards
What are the important epidemiologic aspects of sinonasal cancer?
What are the important epidemiologic aspects of sinonasal cancer?
Sinonasal cancer is rare, accounting for only 3% of upper aerodigestive tract malignancies. There are varied histologic subtypes of sinonasal cancer, which at least in part explains the diverse behavior and presentation of these tumors. Sinonasal malignancies tend to be diagnosed in the fifth and sixth decades of life. The disease is most common in Caucasians, and men are affected at twice the rate of women. A number of occupational exposures are associated with these cancers, including industrial fumes, nickel, leather, and wood dust (more details later in this chapter). There is also a higher rate of sinonasal cancers in cigarette smokers and heavy alcohol users. The 5-year survival for all nasal and paranasal malignancy is 40%, although this varies based on the histopathology of the tumor.
Is there a difference in rates of malignancy between tumors found in the nasal cavity and paranasal sinuses?
Is there a difference in rates of malignancy between tumors found in the nasal cavity and paranasal sinuses?
Tumors of the nasal cavity are more likely to be benign than tumors found in the paranasal sinuses.
Tumors of the nasal cavity are approximately equally split between benign and malignant neoplasms. The most common benign tumor of the nasal cavity is inverted papilloma (IP), the majority of which arise along the lateral nasal wall. The most common malignant tumor of the nasal cavity is squamous cell carcinoma (SCC). Tumors of the paranasal sinuses are more likely to be malignant than benign, with SCC also representing the majority of these tumors.
What are the most common presenting symptoms of sinonasal tumors?
What symptoms are particularly concerning for malignancy?
- What are the most common presenting symptoms of sinonasal tumors?*
- What symptoms are particularly concerning for malignancy?*
Unilateral nasal symptoms are the most common presenting symptoms of sinonasal tumors, including obstruction, discharge, congestion, and epistaxis. These symptoms are often overlooked because they can mimic chronic sinusitis or allergies. However, persistent or worsening unilateral nasal symptoms or development of orbital symptoms, such as vision loss, tearing (epiphora), diplopia, or exophthalmos warrant a detailed examination.
Paresthesia or pain along V2 (maxillary nerve), cheek swelling, and numbness of the face or palate would be unusual for sinusitis, and are symptoms that are concerning for malignancy. Cavernous sinus invasion by sphenoid tumors can lead to dysfunction of cranial nerves III, IV, V1, V2, and VI. Thus, the most important indicators of malignancy include cranial neuropathies and orbital complications.
What are the different types of nasal papillomas?
What are the different types of nasal papillomas?
Nasal papillomas are characterized based on their histologic appearance.
- Exophytic (fungiform) papilloma: The most common subtype, accounting for 50% of nasal papillomas. These papillomas typically arise from the nasal septum and resemble papillomas found in other locations on the body in terms of histopathology. In contrast to the other types of nasal papillomas, the exophytic papilloma does not have malignant potential.
- Inverted (endophytic) papilloma: These arise from Schneiderian mucosa, most commonly located on the lateral nasal wall or maxillary sinus, however any paranasal sinus can be involved (Figure 18-1). These papillomas account for 47% of nasal papillomas, and are associated with high rates of recurrence if not completely resected. Inverted papillomas are associated with an 8% to 10% chance of malignant transformation to SCC. It is associated with HPV infection.
- Oncocytic (cylindrical) papilloma: Oncocytic papillomas usually arise from the lateral nasal wall* and are the rarest of the three papillomas types, accounting for *only 3%* of nasal papillomas. These tumors are thought to have *rare malignant potential, usually reported to be between 4% and 17% (Figure 18-1).
What is the standard treatment for inverting papilloma?
What is the standard treatment for inverting papilloma?
Complete surgical resection with clear margins is the treatment of choice for all sinonasal papillomas. Identification and removal of the tumor site of attachment (origin) gives the highest chance of cure. Radiation with or without chemotherapy is reserved for tumors with malignant transformation.
Traditional open surgery utilizes a lateral rhinotomy or midface degloving to provide access into the nasal cavity for tumor removal. Endoscopic or endoscopic-assisted approaches have largely replaced open approaches for most IPs, and have reduced the tumor recurrence rate from 20% to 12%.
What is a juvenile nasopharyngeal angiofibroma (JNA)?
What is a juvenile nasopharyngeal angiofibroma (JNA)?
JNA is a benign but aggressive vascular tumor. They are seen exclusively in adolescent males. These tumors are slow growing, locally invasive, and do not metastasize. However, these tumors can be quite large at presentation, and can involve the intracranial cavity, orbit, pterygopalatine fossa, or infratemporal fossa. JNAs often present with unilateral, recurrent epistaxis. CT/MRI adding angiography can be helpful to visualize the vascularity of the tumor and confirm the diagnosis. Biopsy carries a high risk of hemorrhage and is not recommended.
What is Fisch’s classification system for JNA?
What is Fisch’s classification system for JNA?
Fisch I: Limited to nasal cavity
Fisch II: Extends to pterygomaxillary fossa or sinuses with bony destruction
Fisch III: Invades orbit, infratemporal fossa, or parasellar area
Fisch IV: Extends to cavernous sinus, optic chiasm, or pituitary fossa
What is the treatment of JNA?
What is the treatment of JNA?
Tumors are typically embolized prior to surgical removal to reduce intraoperative bleeding. Endoscopic techniques are typically used for Fisch I and II, whereas more advanced lesions may require a craniofacial or endoscopic-assisted resection. Radiation therapy may be used for unresectable tumors.
What other benign tumors are found in the nasal cavity? What are the unique features of these tumors?
What other benign tumors are found in the nasal cavity? What are the unique features of these tumors?
Osteomas are the most common benign sinonasal tumors and are slow growing tumors of mature bone. Multiple osteomas can be associated with Gardner’s syndrome. These are most often incidentally discovered on CT scans of the sinus, although they can cause symptoms by obstruction of normal sinus drainage or through direct mass effect. The most common location of osteomas in the paranasal sinuses is in the frontal sinuses, with over 80% presenting in this location.
Hemangiomas are rare and most often present on the septum or inferior turbinate.
Pyogenic granulomas are benign, friable polypoid lesions often found on the septum that can be caused by irritation, physical trauma, and hormonal factors. There is a female predilection and increased incidence during the first trimester of pregnancy.
Hemangiopericytomas are vascular tumors derived from pericyte cells (Zimmerman pericytes) that surround capillaries and postcapillary venules; they account for about 1% of all vascular tumors. Hemangiopericytomas are usually well-differentiated tumors with a low potential for recurrence with complete resection. The treatment of choice is surgical resection.
Salivary gland tumors arising from minor salivary glands in the sinuses are rare. The most common is pleomorphic adenoma.
Chordomas—benign, locally aggressive tumors arising from notochord—are usually found in the clivus, and often present with cranial nerve palsy.
Describe the epidemiology of sinonasal malignancy.
Describe the epidemiology of sinonasal malignancy.
Sinonasal malignancies are rare and represent 3% of head and neck malignancies, typically presenting in the fifth to sixth decade of life.
What are the most common pathological types of sinonasal malignancy?
What are the most common pathological types of sinonasal malignancy?
Squamous cell carcinoma and adenocarcinoma are the most common histologic subtypes. Others include esthesioneuroblastoma, adenoid cystic, mucoepidermoid, mucosal melanoma, sinonasal undifferentiated carcinoma, sarcoma, and lymphoma.
What are the distinctive features of the following malignant sinonasal tumors?
- What are the distinctive features of the following malignant sinonasal tumors?*
- Squamous Cell Carcinoma: Most common sinonasal malignancy, representing approximately 80% of these tumors.
- Adenocarcinoma: Presents most commonly in the ethmoid sinuses, with increased incidence in wood and leather workers.
- Sinonasal Undifferentiated Carcinoma (SNUC): These tumors typically arise near the olfactory groove. These tumors portend a very poor prognosis because they are rapidly progressive, cause extensive local tissue destruction, and commonly metastasize.
-
Esthesioneuroblastoma: These tumors arise from olfactory epithelium and show bimodal distribution in teenage and the elderly populations. They frequently involve the skull base and orbit. There are two staging systems for these tumors: Kadish and Dulguerov-Calcaterra.
- Kadish
- A: Tumors confined to the nasal cavity
- B: Tumor in nasal cavity with extension to the paranasal sinuses
- C: Tumor extending to the orbit, skull base, brain, or with distant metastasis
- Dulguerov-Calcaterra
- T1: Tumor involving the nasal cavity or paranasal sinuses (excluding sphenoid or superior ethmoid air cells)
- T2: Tumor involving the nasal cavity or paranasal sinuses including the sphenoid, or with extension to the cribiform plate
- T3: Extension to orbit or anterior cranial fossa
- T4: Extension to the brain
- Kadish
- Mucoepidermoid: Salivary gland tumors that rarely present in the nasal cavity.
- Adenoid Cystic Carcinoma: Characterized by insidious growth, distant metastasis, and perineural invasion. Long-term surveillance is important due to a higher risk of late tumor recurrence.
Are there occupational exposures that increase the risk of certain sinonasal tumors?
Are there occupational exposures that increase the risk of certain sinonasal tumors?
Adenocarcinoma is associated with exposure to wood and leather dust, as well as organic solvents. Squamous cell carcinoma is associated with exposure to chromium, nickel, mustard gas, and aflatoxin. Exposure to tobacco smoke, alcohol, and salted or smoked foods increases the risk of all types of sinonasal malignancy.
What is the most common sinonasal tumor in the pediatric population?
What is the most common sinonasal tumor in the pediatric population?
Sinonasal tumors are rare in the pediatric population. Sarcomas represent approximately 75% of sinonasal malignancies in this demographic.
What is the prognosis of sinonasal malignancy?
What is the prognosis of sinonasal malignancy?
Five-year survival has been reported ranging from 20% to 50%, but this can vary based on location and histology. Tumors in the maxillary sinus located superior to Ohngren’s line (a line drawn from the medial canthus to the angle of the mandible) are associated with poorer survival (see Question 25 for more details).
What are the surgical approaches used to treat sinonasal malignancies?
- What are the surgical approaches used to treat sinonasal malignancies?*
- Endoscopic approach: Previously limited to benign tumors, now increasingly used in malignant disease and advanced disease. Advantages of the endoscopic approach include decreased morbidity, no external incisions, shorter hospitalizations, and improved visualization of structures and margins.
-
Transfacial open approaches
- Lateral rhinotomy: The incision begins at medial brow and extends along the lateral nasal side wall, around the alar cartilage to the philtrum and through the lip. This is the classic approach to a medial maxillectomy providing access to the maxillary sinus, medial orbital wall, nasal cavity, ethmoid and sphenoid sinuses. Lip split is performed to improve exposure of the hard palate. Can be combined with a sublabial or transpalatal approach for tumors involving the floor of nose or inferior portion of maxilla.
- Weber-Ferguson: Lateral rhinotomy approach in combination with a lip splitting incision that extends sublabially. A subciliary/transconjunctival incision is also made which provides improved access to the maxilla for a total maxillectomy.
- Midface degloving: Involves gingivobuccal incisions as well as bilateral intercartilaginous incisions. The advantage of this approach is avoiding an external scar, and adequate visualization of the inferior and medial maxillary walls.
- Facial translocation: Involves extensive facial flaps with sacrifice of the frontal branch of the facial nerve. The approach affords wide exposure of the skull base, infratemporal fossa, and pterygopalatine fossa.
- Infratemporal approach: Access though a preauricular or postauricular incision that extends coronally. High risk of damage to the frontal branch of the facial nerve.
- Craniofacial resection: Combined approach from above and below. Involves en bloc tumor removal of the anterior cranial base including the dura, cribiform plate, and ethmoid sinuses (see image).
What are the anatomic boundaries of the different types of maxillectomies?
What are the indications for these surgeries?
- What are the anatomic boundaries of the different types of maxillectomies?*
- What are the indications for these surgeries?*
Medial maxillectomy: Removal of lateral nasal wall and medial maxilla, with or without sphenoethmoidectomy. May be performed endoscopically or open. This technique is commonly utilized for tumors in the maxillary sinus.
Inferior maxillectomy: Involves removal of the inferior portion of the maxillary sinus. Indicated for tumors involving the maxillary alveolar process or limited hard palate lesions.
Total maxillectomy: Includes en bloc removal of the entire maxilla. Indicated for tumors involving the maxillary antrum.
Radical maxillectomy: Total maxillectomy with orbital exenteration.
What is Ohngren’s line? What is the importance of this anatomic marker?
What is Ohngren’s line? What is the importance of this anatomic marker?
Ohngren’s line (Figure 18-5) is an imaginary line drawn from the medial canthus to the angle of the mandible. The significance of this marker is that maxillary sinus tumors that are located above this line on presentation are associated with a poor prognosis, tend to spread superiorly and posteriorly, and are more prone to perineural invasion and skull base invasion.
