Paranasal sinus cancer Presenter: 張敬弘 Supervisor: 俞芹英 主任

Brief history Left peri-orbital swelling for 2 months Left nasal obstruction for 10+ days Epistaxis(+) for 3 episodes Proptosis(+) Purulent rhinorrhea(+) Lower visual field darkness(+) Bone scan: Suspicious left maxillary and ethmoid sinus involvement 2/22 2/26 2/27 3/03 3/05 3/09 3/12 CT OP: Lateral rhinotomy MBD CT

Introduction Incidence: 1/500,000 ~ 1/1,000,000 Males : Females = 2 : 1 Peak incidence at 50 to 59 years of age Maxillary > ethmoid > sphenoid > frontal sinuses

Risk factors Occupational or medical exposures Air pollution Tobacco smoke Viral exposure Chronic sinusitis

Pathology Squamous cell carcinomas (45~80%) Adenoid cystic carcinomas Adenocarcinomas Mucoepidermoid carcinomas Rare: Angiosarcomas, rhabdomyosarcomas, lymphomas, esthesioneuroblastomas (olfactory neuroblastomas), melanomas, and meningiomas Salivary gland cancers

Clinical presentation The most common symptoms Facial or dental pain Nasal obstruction Epistaxis

Clinical presentation Symptoms mimic sinusitis and allergic rhinitis Discharge Nasal obstruction Difficulty breathing through the nasal cavity Pressure sensation in the midface Epistaxis Symptoms that are specific to sinonasal cancers included Loosening and numbness of the upper teeth Eye bulging Double or blurred vision Tearing For very advanced lesions, loss of vision

Ethmoid sinus In the ethmoid sinus, locally advanced lesions may extend into the anterior cranial fossa via the cribriform plate or into the orbit through the lamina papyracea. This may result in anosmia or displacement (typically upward and/or outward) of the globe

Sphenoid sinus In the sphenoid sinus, disease may directly extend through the lateral bony wall into the cavernous sinus It may also invade the middle cranial fossa directly or via the infraorbital nerve Diplopia, blurred vision, proptosis, paresthesias in the distribution of the trigeminal nerve or trismus if the pterygoid musculature is invaded

Nodal metastases Regional nodal metastases are uncommon, occurring in only about 20 percent of patients The retropharyngeal nodes comprise the first echelon lymphatic drainage for sinus malignancies Other regional nodes that may be involved with lymphatic spread are the periparotid and level 1b nodes

Diagnosis PE/NE for orbital extension and/or cranial nerve involvement Endoscopic examination (rhinoscopy and nasopharyngoscopy) Both CT and MRI are utilized to further characterize sinonasal tumors

Imaging CT with thin section cuts (3 mm or less), obtained in both axial and coronal projections, is most useful for evaluating tumor invasion into bony structures such as the floor of the orbit and the infratemporal fossa MRI offers a better assessment of tumor infiltration into adjacent soft tissues such as dura and cavernous sinus. Sagittal MRI views permit optimal assessment of the cribriform plate, base of the skull, and floor of the middle cranial fossa

CT In general, tumors enhance more than any normal head and neck structures except mucosa, extraocular muscles, and blood vessels A radiologically heterogeneous appearance or the presence of central low attenuation may indicate pathologic lymphadenopathy, even in small lymph nodes

AJCC & UICC staging system

Treatment Surgery: en bloc craniofacial resection Management of the eye: preserved Reconstruction: improved quality of life Adjuvant RT: optimizes local control Preoperative RT: unclear The optimal management for paranasal sinus cancer is not known Currently, the majority of patients undergo surgery with postoperative radiation

Follow up Cavity cleaning 2-3 weeks postoperatively and again at 4-6 weeks After all treatment is completed a general formula which is modified according to the individual patient's characteristics is: 1st year post treatment: 1-3 months 2nd year post treatment: 2-4 months 3rd year post treatment: 3- 6 months 4th and 5th years: 4- 6 months After 5 years: Every 12 months

Image follow up Repeat CT/MRI scan at 3-6 months to establish a baseline exam and then at 6-12 month intervals depending on the clinical findings

Prognosis There are no prospective data demonstrating a survival benefit for any follow-up strategy in patients with treated head and neck cancer High-grade adenocarcinomas have a reported survival rate of less than 35% at 3 years. For low-grade adenocarcinoma, the 5-year survival rate is approximately 80%.

Sinonasal adenocarcinomas Most nonsalivary head and neck adenocarcinomas arise in the sinonasal tract Two main categories: One resembling seromucinous glands and the other showing enteric-type differentiation

Two main categories Low-grade tumors with fusion of glands composed of bland seromucinous cells, to those with cystic or tubulo-papillary growth patterns Intestinal-type adenocarcinomas (ITAC), resembles metastatic carcinoma from the gastrointestinal tract ITACs are reported to be more aggressive than seromucinous-type carcinomas

Well-differentiated sinonasal adenocarcinoma Intestinal-type sinonasal adenocarcinoma

Thanks for listening

Weber-Fergusson incision

OP findings Huge granular tumor with calcification occupying whole left maxillary, eyhmoid, sphenoid sinuses, nasal cavity and medial portion of orbital fossa Left side partial maxillectomy, anterior and posterior ethmoidectomy, and sphenoidectomy was done

Adenocarcinomas of the nasal cavity and paranasal sinuses Well-differentiated adenocarcinoma Papillary type of adenocarcinoma Solid type of adenocarcinoma Mucinous type of adenocarcinoma Colonic type of adenocarcinoma Mixed type of adenocarcinoma