Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Paranasal Sinus Carcinoma Malignant tumors of the sinonasal tract constitute less than 1% of all malignancies in the body and about 3% of head and neck cancers. The incidence is approximately 1 in 100,000 people per year. Generally, the incidence in males is twice that of females. These tumors are most frequently found during the fifth to the seventh decades. Most paranasal sinus cancers originate from the maxillary sinus. However, because a majority of them present at advanced stages, it is difficult to determine the primary site of the tumor. Although these tumors are rare, a great deal has been written concerning its etiology. Macbeth and Ascherson report a significantly high incidence of adenocarcinoma of the paranasal sinus in High Wycombe, England, among furniture workers. Nickel and chronic refining processes have been implicated in the development of squamous cell and anaplastic carcinoma. Snuff and thorium dioxide, a radiological contrast agent, also have been associated with an increased incidence of sinonasal cancer. Squamous cell carcinoma is the most common tumor of the sinonasal tract. The prognosis is related to the extent of the tumor and the site of origin. Adenocarcinomas make up 4% to 8% of all sinonasal cancer. They originate most commonly at the ethmoids and nasal cavity, and are locally aggressive. Adenocarcinoma of the sinus may also resemble moderately differentiated colon adenocarcinoma. Adenoid cystic carcinoma accounts for 6% of sinonasal tract cancer. It is notorious for recurrence, distant metastasis and perineural invasion. Mucoepidermoid carcinomas are extremely rare in the sinonasal tract. They tend to present in very advanced stages. Undifferentiated carcinomas are usually composed of small and medium-sized cells and must be differentiated from rhabdomyosarcoma, melanoma, olfactory neuroblastoma, lymphoma, and squamous cell carcinoma. The onset of symptoms is very rapid. Prognosis is poor, and trimodal therapy is recommended. In 1933 Ohngren described an early staging system for maxillary sinus cancer. He drew an oblique line through the cavity of the maxillary antrum that extended from the medial canthus of the eye to the angle of the mandible when the sinus was viewed in its lateral aspect. He found that tumors located above the line have worse prognoses than those below it. The currently used American Joint Committee TMN classification is based upon Ohngren's original description. It emphasizes the size, as well as the extension of tumors. A tumor involving the cribriform plate, sphenoid sinuses, nasopharynx, pterygomaxillary or temporal fossa, or base of skull has a poor prognosis. This uniform staging system is not perfect, but allows various different centers to report their experiences in a manner that lends itself to objective comparison and interpretation. The most common clinical presentation of tumors of the sinonasal tract includes nasal obstruction, pain, epistaxis, nasal discharge or swelling of the cheek. Because these symptoms are similar to those of chronic sinusitis, The average delay between the onset of symptoms and final diagnosis is 3 to 18 months. Furthermore, about 9% to 12% of patients with sinonasal tumors are asymptomatic. Most of paranasal sinus cancer initially present at stage T3 or T4. All patients with a suspicious nasal lesion or any findings suggestive of destructive disease or infratemporal fossa disease should undergo a thorough physical examination including direct fiberoptic endoscopy as well as radiologic imagining studies. CT scanning is excellent for demonstrating opacification, mass effect or bone destruction. It also allows an evaluation of the orbital apex, infratemporal fossa, posterior ethmoid sinus, cribriform and pterygoid plates, and sphenoid sinus. MRI differentiates an obstructed sinus with fluid collection from a space occupying lesion. MRI may also demonstrate perineural spread. These two modalities complement each other in evaluating and staging a sinonasal tract mass. Lund et al found 94% correlation between the histologic and imaging findings. Prior to a major exploration or resection, a tissue diagnosis must be made. This can be achieved by an intranasal biopsy using endoscopic sinus surgery techniques or by transoral or transcutansous procedures such as Caldwell-Luc or external ethmoidectomy. Currently, most of maxillary sinus carcinomas in T1 and T2 stages are treated by surgery alone, whereas the T3 and T4 lesions are treated by a combination therapy of surgery and radiation therapy. A study by St. Pierre and others demonstrated that a five-year survival rate was 58% for the combined therapy, whereas surgery or radiotherapy alone resulted in 20% and 15.8%, respectively. The issue as to whether radiation is more effective before or after surgery remains controversial. It appears that preoperative and postoperative radiation therapy produce similar results (Jesse and Sission). Orbital invasion is commonly associated with antral cancer with an incidence as high as 60% in a study by Gullane and Conley. Tumor extension of an orbital involvement can only be properly evaluated at the time of surgery. Sisson and Weymuller have shown that orbital preservation has not compromised the five-year survival rate when the periorbitum is not involved by a tumor. Weymuller observed that in the patients who received preoperative radiation therapy, if the tumor could be peeled off easily from the intact periosteum, their prognosis was not different from those with similar tumor who underwent an orbital exenteration. Current indications for orbital exenteration are a gross involvement of the periorbitum at presentation or an extensive involvement of the periorbitum even after radiotherapy. A locally involved periorbitum can be partially resected using frozen-section control. A failure of conventional total maxillectomy was frequently due to local recurrences in the remaining ethmoid sinus, cribriform plate, apex of the orbit, and pterygopalantine fossa. Smith and Ketcham introduced a technique of craniofacial resection to resect these previously nonresectable regions. Terz then extended the limits of the resection to include the middle cranial fossa and pterygoid plates. Most surgeons agree that a craniofacial resection is contraindicated when a patient is a poor surgical candidate or the tumor involves the brain parenchyma, prevertebral fascia, cavernous sinus, carotid artery, bilateral optic nerve or optic chiasm. Resection even beyond these limits is technically possible, but there is no evidence of survival advantage to warrant the extensive functional defects and morbidity from these procedures. The incidence of cervical nodal metastasis on an initial presentation varies from 3% to 16%. The incidence of nodal metastasis is higher if a tumor extends into the intraoral mucosa or cheek. A patient with nodal metastasis carries a poor prognosis. Because of the low incidence of nodal metastasis from sinonasal malignancies, an elective neck dissection or radiation is not recommended. However, a recent study by Yang et al at M.D. Anderson reported that, without previous treatment, the overall nodal metastasis rate was 38 % for squamous cell and undifferentiated carcinoma, and they recommend elective radiotherapy for the patients with these types of carcinoma. Chemotherapy is generally used as a palliative measure. However, in 1970, Sato reported a five-year survival rate of 55% and significantly low recurrence rates using a treatment modality combining radiotherapy, intra-arterial 5-Fluorouracil and antrostomy for cleaning necrotic debris. Sakata also reported a similar result using a trimodal therapy. Recently the M.D. Anderson Hospital reported that 19 out of the 21 patients with advanced paranasal sinus cancer, who were treated with combined super-selective intra-arterial and systemic chemotherapy, had immediate satisfactory response. They showed encouraging evidence that induction of such chemotherapy before definite surgery and/or radiotherapy might improve the immediate response rate and prolong survival. In conclusion, paranasal sinus cancer is not easily amenable to our current treatment. Advances in endoscopy and diagnostic imaging will help us to make a diagnosis at an earlier stage of the disease and to better plan treatment. A multicenter prospective randomized study is needed to determine the most effective way of treating these tumors. Case Presentation An 82 year-old white man developed a new-onset seizure in November, 1992. CT scanning of the head as part of his routine seizure work-up revealed a left frontal sinus mass eroding the anterior table. He denied previous history of paranasal or ocular problems. His past medical history was significant for bladder cancer requiring a cystectomy and ureteroileostomy in 1991. His physical examination revealed a small swelling in the left nasofrontal area and left proptosis. His vision was grossly intact and the extra-ocular muscles were intact. His nasal exam was unremarkable. MRI of the brain with contrast showed soft tissue which eroded through both the anterior and posterior wall of the frontal sinus, consistent with a malignant neoplasm with no involvement of the brain parenchyma. An open biopsy of the mass was performed, revealing a papillary, well-differentiated adenocarcinoma. Subsequently his metastatic evaluation was negative. The patient was then taken to an operating room for a combined procedure with a neurosurgeon on March 30, 1993. An anterior craniofacial resection via bifrontal craniotomy was performed, along with the frontal sinus obliteration and preservation of the left orbit. The anterior aspect of the left frontal lobe was invaded by the tumor, which was resected. His postoperative course was unremarkable. 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