Malignant ethmoid sinus tumor masquerading as chronic ethmoid sinusitis.

A 67-year-old woman had been treated for 6 months with antihistamines, nasal steroids, and antibiotics for suspected sinusitis. When she continued to complain of progressive headaches, right-sided epiphora, and anosmia, limited computed tomography (CT) of the paranasal sinuses was obtained (figure 1). A diagnosis of right-sided ethmoid sinusitis was made, and the patient was referred to us for endoscopic sinus surgery. Findings on our review of the patient's sinus CTs were not consistent with the range and severity of her symptoms, so we obtained further imaging.

[FIGURE 1 OMITTED]

CT of the orbits detected the presence of a mass that extended from the right ethmoid sinuses into the orbital apex, anterior cranial fossa, right frontal lobe, and sphenoid sinus (figure 2). Findings on endoscopic biopsy were consistent with squamous cell carcinoma (SCC). The patient underwent a craniofacial resection and postoperative radiotherapy.

[FIGURE 2 OMITTED]

Malignant tumors of the ethmoid sinus account for a low percentage of head and neck cancers. When they do occur, SCC is the most common histologic subtype, followed by adenocarcinoma, adenoid cystic carcinoma, and several others. (1)

Like other sinonasal malignancies, SCC of the ethmoid sinus is often diagnosed at a relatively late stage. This delay in diagnosis is largely attributable to the nonspecific nature of early symptoms, which are often consistent with benign nasal and sinus obstructions. The American Joint Committee on Cancer Staging (2) has classified the tumor component of sinonasal SCC thusly:

* TI: The tumor is confined to a single subsite with or without bony invasion.

* T2: The tumor involves 2 subsites or an adjacent area of the nasoethmoid complex with or without bony invasion.

* T3: The tumor has invaded the orbital floor, maxillary sinus, palate, or cribriform plate.

* T4a: The tumor has invaded the anterior orbit, anterior cranial fossa, overlying facial dermis, sphenoid sinus, or frontal sinus.

* T4b: The tumor has invaded the orbital apex, brain, middle cranial fossa, or overlying cranial nerves.

Because the SCC in our patient extended into the right orbital apex, the anterior cranial fossa, the brain, and the sphenoid sinus, it was staged as a T4b lesion.

The treatment of SCCs of the ethmoid sinus has evolved in a manner consistent with the evolution of the treatment of SCCs in other structure-sensitive areas of the head and neck. Surgical resection and intensity-modulated radiotherapy (IMRT) are now used in a complementary fashion for local tumor control. Moreover, a variety of cisplatin-based chemotherapeutic protocols can be added to the treatment regimen for patients with known or suspected regional or distant metastatic disease. IMRT is especially useful for patients with tumor-positive surgical margins, recurrent disease, and/or locally advanced disease.

Given the rarity of ethmoid sinus tumors, treatment-specific and overall outcomes data are derived mostly from small cohort studies, and results vary somewhat. The overall 5-year survival rate for ethmoid carcinomas of all histologic subtypes appears to be as high as 50 to 60%, (3) although several studies have reported much lower rates of 5-year survival for patients with SCC. For example, Uchida et al reported a 5-year survival rate of only 25% among 20 patients with ethmoid SCC who were treated with either radiotherapy plus surgery or radiotherapy alone. (1) Dulguerov et al reported a 5-year survival rate of 48% among 220 patients with ethmoid carcinoma, but they did not distinguish among multiple histologic subtypes. (3) They also suggested that survival was better among patients who underwent surgery or surgery plus radiation rather than radiation alone. Finally, they recommended orbit enucleation rather than preservation in the setting of orbital invasion.

The increasing availability of IMRT and our experience with it may well have a significant impact on improving local control and perhaps survival as more optimal doses of radiation are delivered to the desired area far more precisely than before.

References

(1.) Uchida D, Shirato H, Onimaru R, et al. Long-term results of ethmoid squamous cell or undifferentiated carcinoma treated with radiotherapy with or without surgery. Cancer J 2005;11:152-6.

(2.) Greene FL, Page DL, Fleming ID, et al, eds. AJCC Cancer Staging Manual. 6th ed. New York: Springer; 2002:73-80.

(3.) Dulguerov P, Jacobsen MS, Allal AS, et al. Nasal and paranasal sinus carcinoma: Are we making progress? A series of 220 patients and a systematic review. Cancer 2001 ;92:3012-29.

From the Department of Surgical Oncology, John Wayne Cancer Institute, Santa Monica, Calif. (Dr. Wright); the Osborne Head and Neck Institute, Los Angeles (Dr. Hamilton and Dr. Osborne); and the Head and Neck Cancer Center, Cedars-Sinai Medical Center, Los Angeles (Dr. Osborne).

Byron E. Wright, MD, FACS; Jason S. Hamilton, MD; Ryan F. Osborne, MD, FACS