TABLE 25–1. Sinonasal Papilloma
• Pathophysiology:
1. More common in males (8:1), most common in fifth decade
2. Most arise from nasal septum; rarely involves vestibule, turbinates, or any of the meatus
3. Associated with HPV 6 and 11
4. Malignant transformation is rare.
• SSx:
1. Unilateral nasal congestion and epistaxis are the most common presenting symptoms followed by nasal drainage, nasal mass, sinusitis, headache, anosmia, proptosis, diplopia, and epiphora.
1. Usually exophytic, grey-tan, small and unilateral with a narrow stalk on nasal endoscopy
2. Diagnosis is made based on tissue biopsy.
3. Diagnostic approach is the same as for inverted papilloma.
• Histopathology:
1. Branching, exophytic proliferation with fibrovascular cores with well-differentiated squamous epithelium lining the core
2. Koilocytosis can be seen in superficial squamous cells.
3. Mitotic figures can be seen in some basal cells.
4. There may be microcysts with mucus seen in the intraepithelial regions
5. Subtypes include fungiform, septal, and squamous.
• Management:
1. Treatment is similar to treatment for inverted papilloma; complete resection with negative margins is necessary for long term control.
2. Site of attachment is important to address surgically to prevent recurrence.
3. Removal of microscopic foci is not usually needed for exophytic papillomas.
4. Radiation therapy is not needed unless associated with malignancy.
• Rarest of sinonasal papillomas
• Equally common in males and females
• Malignant transformation possible
• Not usually associated with HPV
• Pathophysiology:
1. Most commonly involves lateral nasal wall; some involve maxillary or ethmoid sinuses; very rarely affect the nasal septum
• SSx:
1. Unilateral nasal congestion and epistaxis are the most common presenting symptoms followed by nasal drainage, nasal mass, sinusitis, headache, anosmia, proptosis, diplopia, and epiphora.
• Dx:
1. Soft, pink, papillary tissue is seen on nasal endoscopy.
2. Diagnosis is made based on tissue biopsy.
3. Diagnostic approach is the same as for inverted papilloma.
• Histopathology:
1. Multilayered columnar or oncocytic epithelium lining thin fibrovascular cores
2. Endophytic segments with columnar or oncocytic epithelium extending into seromucinous glands
3. Cells have oncocytic cytoplasm with round nuclei
4. Intraepithelial mucous cysts can be seen
5. Needs to be distinguished from adenocarcinoma by presence of stratified epithelium, intact basement membrane, and absence of bony destruction and mitotic activity
6. Subtypes include cylindrical and columnar
• Management:
1. Treatment is similar to treatment for inverted papilloma; complete resection with negative margins is necessary for long-term control.
2. Site of attachment is important to address surgically to prevent recurrence.
3. Removal of microscopic foci may be necessary for complete resection.
4. Radiation therapy is not needed unless associated with malignancy.
• Also known as epithelial papilloma, papillary sinusitis, Schneiderian papilloma, inverted Schneiderian papilloma, polyp with inverting metaplasia, transitional cell papilloma
• Pathophysiology:
1. HPV (serotypes 6, 11, 16, 18 most common) thought to be important co-factor
– Serotype 16 and 18 most commonly associated with malignancy
2. Associated with mutations in tumor suppressor gene p53 and chronic inflammation
3. Possible correlation with exposure to diethylnitrosamine, smoke, dust, aerosols, and industrial occupations
4. Associated with malignant degeneration to squamous cell carcinoma in 5% to 10% of cases
5. Maxillary sinus is the most commonly involved paranasal sinus, followed by the ethmoid sinus.
6. IP usually arise from the lateral nasal wall, most commonly the osteomeatal complex, middle turbinate, and inferior turbinate.
• Most common epithelial tumor of sinonasal tract
• Second most common benign tumor of the sinonasal tract after osteomas
• More common in males, occurring most frequently in the 5th to 8th decade of life
1. Unilateral nasal congestion and epistaxis are the most common presenting symptoms followed by nasal drainage, nasal mass, sinusitis, headache, anosmia, proptosis, diplopia and epiphora.
2. Exophytic and polypoid appearance on nasal endoscopy
3. Attachment site is typically pedicled to a discrete site, meanwhile adjacent tissue/mucosa remains normal without invasion.
• Dx:
1. Definitive diagnosis is based on pathology and tissue biopsy.
2. Preoperative imaging help to evaluate extent of disease (involvement of orbit, skull base, internal carotid artery, etc).
– CT paranasal sinus with thin slices (0.625 mm): assess bony involvement (bowing/defects/remodeling of local bony structures); focal hyperostosis and osteitic changes suggest tumor attachment site (Figure 25–1)
– MRI: best for evaluating soft tissue anatomy and distinguishing IP from inspissated mucus; hypodense to isodense on T1 weighted images, isodense to hyperdense on T2 weighted images (Figure 25–2)
• Histopathology:
1. Digitiform proliferation of squamous or ciliated columnar epithelium with mucocytes
2. Inverts into underlying connective tissue
3. Lacks mucus secreting cells and eosinophils
4. Basement membrane usually remains intact
• Krause Classification:
1. Stage 1: confined to nasal cavity
2. Stage 2: involving ethmoid sinus, medial, and superior maxillary sinus
3. Stage 3: involving all paranasal sinuses but confined to nose and paranasal sinuses
4. Stage 4: not confined to nose or paranasal sinuses or having malignancy
• Complications: epistaxis, epiphora, temporary infraorbital hypoesthesia, periorbital ecchymosis, CSF leak (<6%)
• Management:
1. Complete resection with negative margins is necessary for long-term control.
2. Historically, lateral rhinotomy or sublabial degloving open approaches were used.
3. With advancements in visualization and miniaturization of endoscopic instrumentation, an increasing number of lesions are amenable to complete endonasal endoscopic approach, especially for Krause Stages 1 to 3.
FIGURE 25–1. CT scan with inverted papilloma occupying the right frontal sinus, with evidence of hyperostosis noted along the posterior table of the frontal sinus.
FIGURE 25–2. MRI scan characteristics of inverted papilloma with (a) T1-weighted image, (b) T2-weighted image, (c) T1 postcontrast image. The signal differentiation allows for distinguishing inspissated mucus versus IP.
4. More aggressive disease or malignancy may need a combined approach:
– Involving the lateral frontal sinus or supraorbital cell
– Transorbital extension
– Presence of malignancy involving critical areas
– Presence of scarring or anatomical distortion from previous surgeries
5. Regardless of the approach used, goals of surgery include complete resection and creation of a large sinonasal cavity that allow office endoscopic examinations.
6. Long-term endoscopic surveillance with or without MRI scans are needed to monitor for recurrence.
7. Adjuvant radiation therapy may be required for patients with malignant degeneration.
JUVENILE NASOPHARYNGEAL ANGIOFIBROMA (JNA)
• Pathophysiology:
1. Rare tumor (<1% of sinonasal tumors)
2. Most common in adolescent males
3. Increased risk in those with familial adenomatous polyposis gene
4. May develop from incomplete regression of a branchial artery
5. May be under hormonal regulation and undergo spontaneous regression after puberty
• SSx:
1. Unilateral nasal obstruction and recurrent epistaxis are the most common initial symptoms
2. Headaches, proptosis, or swelling of the cheek may develop in advanced disease
3. Purple-grey pedunculated mass, usually centered at sphenopalatine foramen
4. Holman-Miller sign: anterior bowing of posterior maxillary sinus wall on CT scan
• Dx:
1. Imaging with CT or MRI
2. Confirms origin at level of pterygopalatine fossa with anterior bowing of posterior maxillary wall; Holman-Miller sign
3. Hypervascularity seen with contrast administration
4. T1 and T2 images on MRI may show signal void within mass suggesting major vessels
5. Biopsy should not be taken in the office setting due to highly vascular nature
• Histopathology:
1. Thin-walled vessels of different sizes usually lacking a complete smooth muscle layer
2. Fibrinous stroma
• Complications:
1. Lateral extension through pterygomaxillary fissure into infratemporal fossa
2. Intracranial extension through inferior or superior orbital fissure
3. Bony involvement
4. Resorption by pressure from subperiosteal growth
5. Invasion of cancellous bone
6. Transdural growth is rare
• Andrews’ (Modified Fisch) Classification
1. Type I: limited to the nasopharynx and nasal cavity; bone destruction negligible or limited to the sphenopalatine foramen
2. Type II: invades the pterygopalatine fossa or the maxillary, ethmoid, or sphenoid sinus with bone destruction
3. Type IIIa: invades the infratemporal fossa or the orbital region without intracranial involvement
4. Type IIIb: invades the infratemporal fossa or orbit with intracranial extradural (parasellar) involvement
5. Type Iva: intracranial intradural tumor without infiltration of the cavernous sinus, pituitary fossa, or optic chiasm
6. Type IVb: intracranial intradural tumor with infiltration of the cavernous sinus, pituitary fossa, or optic chiasm
• Management:
1. Endoscopic removal for most tumors, even those involving middle cranial fossa
2. Larger tumors may be resected with endoscopically assisted combined approaches.
3. Disease involving cavernous sinus or encasing internal carotid artery may be treated with a combined approach.
4. Preoperative devascularization with arterial ligation or embolization is key to reduce intraoperative blood loss and increase chance of complete resection.
5. Subperiosteal dissection is essential.
6. Newer techniques are also available, including Gamma Knife radiosurgery.
7. Postoperative surveillance is needed for at least 3 years.
BENIGN FIBRO-OSSEOUS LESIONS (BFOL)
• Variable group of bony lesions, all with normal bone replaced with collagen fibers and fibroblasts
• Osseous dysplasia, fibro-osseous neoplasms, fibrous dysplasia
• Benign lesion but can be disfiguring or functionally obstructive to sinus outflow tract
• Pathophysiology:
1. Development theory: embryonic cells trapped at the junction of the endochondral ethmoid cells and membranous ossified frontal bone
2. Traumatic and infectious theory: inflammation results in excessive bony production
3. Most commonly occur in frontoethmoidal region
4. If multiple osteomas noted, consider Gardner’s syndrome (colon polyps, soft tissue tumors, multiple osteomas); typically need a gastroenterology referral
• Most common benign lesion of sinonasal tract
• SSx:
1. Appear hard, white, and multilobulated
2. Slow and steady growth often seen
3. Most osteomas are asymptomatic and diagnosed incidentally on imaging; sinusitis may occur if there is blockage of sinus drainage; cosmesis is affected if outer table of frontal bone is involved.
4. If osteoma extends to the anterior cranial fossa, it can cause CSF leak, meningitis, and brain abscess.
• Dx: CT resembles cortical bone or ground glass pattern
• Histopathology: proliferation of dense cancellous bone
• Classifications:
1. Ivory: lobulated, dense bone, minimal fibrous tissue, no haversian ducts
2. Mature: spongy, mature bone; divided by fibrous tissue; includes fibroblasts, collagen fibers, and connective tissue with thin-walled vessels
3. Mixed: include findings from both of the above
• Management: no treatment needed unless causing nasal obstruction, sinus outflow obstruction, or compression/invasion of orbit or skull base; endoscopic resection if symptomatic
• Pathophysiology:
1. Deformative lesion but rarely destructive
2. Typically monostotic ~75%
3. Polyostotic lesions can be present as part of McCune-Albright Syndrome (precocious puberty, café-au-lait spots, and other endocrine abnormalities)
4. Presents during childhood and growth stops by adulthood
5. Most commonly involve maxilla, but also involve skull base, zygoma, or mandible
• Dx: CT—ground-glass appearing lesions
• Histopathology: Woven bone with non-lamellar trabeculae
• Management:
1. Isolated lesions can be observed
2. Lesions can extend beyond sinuses; play a role for surgical plan of extirpation
3. Consider surgery for extension to orbit or cranial nerves
• Painless, expansile lesions, similar to fibrous dysplasia, but more aggressive and rapid growth, which can cause local bony erosion
• More common in mandible; more destructive when involving sinonasal cavity
• Management: Treatment usually involves surgical resection, even early in the disease process to prevent extensive bone destruction
1. May be resected endoscopically but associated with higher recurrence rate
RESPIRATORY EPITHELIAL ADENOMATOID HAMARTOMA (REAH)
• Pathophysiology:
1. Proliferative glandular lesions of the sinonasal ciliated respiratory epithelium
2. Commonly misdiagnosed as inflammatory polyps, inverted papilloma, sinonasal adenocarcinoma, and other malignant lesions
3. Can be associated with inflammatory polyps in 35% to 48% patients
4. Rare association with low-grade sinonasal adenocarcinoma reported
5. May arise from olfactory cleft, mimicking malignant lesions
• Most commonly found in fifth decade of life
• Possible sex predilection of 3:2 male to female ratio
• SSx: nasal obstruction, nasal discharge, facial pressure/pain, and hyposmia/anosmia; symptoms generally similar to that of chronic sinusitis with nasal polyposis
• Dx:
1. Nasal endoscopy shows unilateral or bilateral fleshy and pinkish/yellowish masses, firmer than inflammatory polypoid tissues, and commonly found medial to middle turbinate arising from the olfactory cleft or posterior septum
2. CT paranasal sinus may demonstrate homogenous masses without enhancement; possible widening of the olfactory cleft >10 to 12 mm may be seen, and skull base or lamina papyracea erosions generally not encountered
3. MRI paranasal sinus may reveal a homogenous mass that is hyper-intense on T1 sequence with contrast enhancement
• Histopathology: definitive diagnosis made on histopathology; lesion characterized by pseudoglandular proliferation with submucosal invagination; no specific immunohistochemistry markers have been found
• Complications: similar to that of all endoscopic sinus surgery; however, the risk of CSF leak may be higher due to location of REAH arising from olfactory cleft and close proximity to the cribriform plate
• Management:
1. Endoscopic resection while preserving vital structures is the treatment of choice.
2. Concurrent treatment for chronic sinusitis with nasal polyposis may be necessary.
3. Recurrence is rare (3.7%) in limited follow-up.
(Formerly Known as Sinonasal Hemangiopericytoma)
• Pathophysiology:
1. Rare tumor arising from pericytes surrounding capillary vessels, representing <1% of vascular neoplasms; WHO classification as tumor of perivascular myloid phenotype
2. Benign but can be locally invasive
3. Possibility of malignant transformation, more likely with tumor >6.5 cm or tumors with necrosis, nuclear atypia, and high number of mitosis on histology; regional lymphatic metastasis and distant metastasis to lung, liver, and bone have been described
4. More indolent course in sinonasal cavity and considered a distinct entity from soft tissue hemangiopericytoma; more closely related to glomus tumors
5. Most commonly found in nasal cavity but may extend to paranasal sinuses
• Peak incidence in third to fifth decade of life, with a slight female predominance
• Oncogenic osteomalacia may occur.
• SSx: patient typically presents with unilateral nasal obstruction and epistaxis; symptoms of sinusitis may occur as a result of drainage pathway obstruction
• Dx:
1. Endoscopy: firm, fleshy, hemorrhagic polypoid
2. CT: avidly enhancing mass, can evaluate bony anatomy; MRI: T1 isointense mass that enhances strongly with contrast
• Histopathology: characterized by subepithelial proliferation of bland spindle cells in a variably fibrotic stroma; rich vascular network may be seen with perivascular hyalinization; staghorn vessel formation may be seen; immunohistochemistry positive for vimentin and nuclear beta-catenin
• Management: complete surgical resection with recurrence rate of 20%; routine postoperative surveillance with nasal endoscopy to aid in early detection of recurrence
1. Preoperative embolization can decrease intraoperative blood loss
2. Larger lesions may require open approaches, such as Caldwell Luc or lateral rhinotomy approaches
3. Radiotherapy and chemotherapy have limited efficacy and are reserved for unresectable tumors for palliative care.
(Also Known as Lobular Capillary Hemangioma or Pyogenic Granuloma)
• Pathophysiology:
1. Benign vascular lesions
3. Associated with nasogastric tube placement and digital trauma
4. Commonly found on gingiva; maxillary more common than mandibular gingiva; rarely can occur in nasal cavity
5. More commonly found along anterior septum near the Kiesselbach’s plexus (Figure 25–3)
6. Neither infectious nor granulomatous features
7. More common in females in second decade of life; found in 2% of pregnant women
• SSx: recurrent epistaxis
• Dx: Rapidly growing nasal mass; red to purple coloration on nasal endoscopy
• Histopathology: endothelial hyperplasia, inflammatory infiltrate, fibrous tissue with perforating blood vessels, ulceration
• Management:
1. If associated with pregnancy, lesion may involute after pregnancy.
2. If lesion does not resolve, endoscopic surgical resection and cauterization is preferred mode of treatment.
3. Laser treatment and sclerotherapy offer an alternative to surgical treatment.
NEUROENDOCTRINE TUMORS (SCHWANNOMA)
• Pathophysiology: neoplasms arising from Schwann cells, affecting branches of the maxillary or ophthalmic branches of the trigeminal nerve and sympathetic/parasympathetic nerves in the head and neck region
• Schwannoma occurrence in sinonasal cavity: 4%
• May involve nasoethmoidal complex, paranasal sinuses, septum, pterygopalatine fossa, infratemporal fossa, or skull base
• Malignant degeneration has been reported in rare cases.
• Peak incidence between second and fifth decade of life
• No sex predilection
• SSx: patients may present with nasal obstruction, nasal drainage, facial/retro-orbital pain, headache, or epistaxis
• Dx:
1. Biopsy should be performed in the operating room to prepare for a vascular lesion.
2. Nasal endoscopy: highly vascular sinonasal mass or mass effect on surrounding structures
FIGURE 25–3. CT scan with contrast of pyogenic granuloma confined to the anterior septum on the right pedicle to the anterior septum near Kiesselbach’s plexus.
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