• Tx: surgery adequate for early-stage low-grade lesions (T1 to T2); postoperative radiotherapy recommended for high-grade and advanced-stage (T3 to T4) lesions; elective neck dissection not routine because low risk of regional metastasis (<10%)
• Prevalence: 3% to 6% of all sinonasal malignancies
• Arises from olfactory neuroepithelium in superior nasal vault
• Bimodal age distribution: ~20 and 50 years of age; no sex predilection
• Metastasis to cervical lymph node in ~10% of cases
• Histopathology: submucosal, sharply-demarcated nests of cells separated by richly vascular fibrous stroma; small round blue cells in Homer-Wright pseudorosettes and Flexner-Wintersteiner rosettes
• Kadish Staging System (based on exam)
1. Kadish Stage A: tumor limited to nasal cavity
2. Kadish Stage B: extension to paranasal sinuses
3. Kadish Stage C: extension beyond nasal cavity/paranasal sinuses
4. Kadish Stage D: regional lymph node or metastasis
• Dulguerov-Calcaterra Staging System (based on imaging)
1. T1: involves nasal cavity and/or paranasal sinuses (excluding sphenoid and superior ethmoidal cells)
2. T2: involves nasal cavity and/or paranasal sinuses with extension to or erosion of cribriform plate
3. T3: extends into orbit or protrudes into anterior cranial fossa without dural invasion
4. T4: involves brain
• Tx: removal of cribriform plate and bilateral olfactory bulbs; but for limited tumors, can perform unilateral resection with preservation of contralateral cribriform and olfactory bulb to preserve olfaction; postoperative radiotherapy recommended because of reduction in local recurrence rates and improved survival
• Prevalence: 5% to 10% of intracranial meningiomas
• Arises in midline along dura of cribriform plate and planum sphenoidale
• Slight female predominance
• Sx: behavioral and personality changes, headaches, anosmia, seizures, visual disturbances (late)
• Firm in consistency, well-demarcated with broad dural attachment
• Hyperostosis of adjacent bone leads to extension into ethmoid sinuses and nasal cavity in 15% of cases.
• Histopathology: varies based on variant but generally has syncytial and epithelial cells, indistinct cell borders, classic whorls, psammoma bodies
• World Health Organization Grading System
1. Grade I: benign tumor
2. Grade II: atypical meningioma
3. Grade III: anaplastic or malignant meningioma
• Imaging: CT—isodense; T1-weighted MRI—iso-intense +/− dural tail, contrast enhancing
• Simpson grading of resection (increasing rate of recurrence with increasing grade)
1. Grade 1: gross total resection + resection of dural attachments and pathologic bone
2. Grade 2: gross total resection + coagulation of dural attachments
3. Grade 3: gross total resection + coagulation/resection of dural attachment
4. Grade 4: subtotal/partial tumor resection
5. Grade 5: biopsy
• Tx: surgery; radiotherapy used for Grade III or very large meningiomas; can opt to observe with imaging follow-up in elderly patients with multiple comorbidities, asymptomatic patients with unproven tumor growth, and those with calcified meningiomas
Sinonasal Undifferentiated Carcinoma
• Rare malignancy of neuroendocrine origin
• Arises most commonly from the nasal cavity, followed by the ethmoid sinuses
• Highly aggressive with poor prognosis (5-year survival rate ~30% to 40%); ~65% to 80% of patients present with locally advanced disease
• Histopathology: