Malignant Neoplasms Squamous Cell Carcinomas General Considerations Squamous cell carcinoma is the most common malignant neoplasm of the paranasal sinuses, accounting for 6080% of paranasal sinus tumors. The etiology and epidemiology of this tumor are poorly understood , although nickel workers are at a markedly increased risk of developing these tumors. Clinical Findings Squamous cell carcinomas arise from a silent location and grow insidiously with few to no symptoms. At the time of diagnosis, these tumors are very large and therefore bode a very poor prognosis. Only when these neoplasms invade adjacent structures, causing symptoms of oral, ocular, or facial involvement, are patients accurately diagnosed and treated (Figure 162). These symptoms include pain in the maxillary teeth, erosion of the palate, diplopia, proptosis, and cheek paresthesias. Squamous cell carcinomas arise most commonly in the maxillary antrum and account for up to 80% of all paranasal sinus squamous cell carcinoma. The ethmoid sinus is the second most common site of origin. Primary squamous cell carcinomas of the frontal and sphenoid sinuses are rare. Staging The revised staging system for maxillary sinus carcinoma, created by the American Joint Committee on Cancer (AJCC), is clinically more relevant and better at distinguishing survival results between T2T3 and T2T4 diseases than its previous staging system. The N stage designates regional lymph node involvement and is identical with staging of the neck in other head and neck cancers. The staging system for ethmoid sinus cancers is shown in Table 162. Table 162. Staging Criteria of Primary Malignant Maxillary and Ethmoid Sinus Tumors. | | Stage | Maxillary Sinus | Ethmoid Sinus | T X | Primary tumor cannot be assessed | T | No evidence of primary tumor | T IS | Carcinoma in situ | T 1 | Tumor confined to antral mucosa with no bony destruction | Tumor confined to ethmoid sinuses with no bony destruction | T 2 | Tumor causing bony destruction (except for posterior wall of maxillary sinus), including extension into the hard palate or middle meatus | Tumor extends into the nasal cavity | T 3 | Tumor invades any of the following: bone of the posterior wall of maxillary sinus, subcutaneous tissue, skin of cheek, floor of medial wall of orbit, infratemporal fossa, pterygoid plates, ethmoid sinuses | Tumor extends to the anterior orbit or the maxillary sinus | T 4 | Tumor invades orbital contents beyond the floor or medial wall, including any of the following: the orbital apex, cribriform plate, base of skull, nasopharynx, sphenoid, frontal sinus | Tumor with intracranial extension; orbital extension including the apex or the sphenoid, the frontal external nose, or the skin of the external nose | | Modified, with permission from Greene FL, Page DL, Fleming ID et al (eds): American Joint Committee on Cancer: AJCC Cancer Staging Manual, 6th ed. New York, Berlin, Heidelberg: Springer-Verlag; 2002. | Treatment & Prognosis For nearly all patients, the treatment is surgical resection followed by radiation therapy. Treatment with this combination of modalities has shown markedly improved results compared with radiation therapy alone. The 5-year survival rate for patients with maxillary sinus squamous cell carcinoma who are treated with combined surgery and radiation therapy is 4668% versus 919% for patients treated with radiation therapy alone. Surgical procedures typically start with a maxillectomy and can include orbital exenteration, infratemporal fossa dissection, and craniofacial resection. Postoperative radiation therapy is administered until at least 65 Gy is delivered. Intensity-modulated radiation therapy (IMRT) allows an even greater dosage delivery while sparing crucial structures such as the optic nerve and chiasm, the pituitary gland, and the brain. The addition of chemotherapy may improve locoregional control and 5-year disease-specific survival. Because of the rarity of ethmoid squamous cell carcinoma, all ethmoid tumors tend to be grouped together, despite different histologic features. Patients with ethmoid tumors fare no better than patients with maxillary sinus tumors; the 5-year local control and disease-specific survival rates for both are similar. Dulguerov P, Jacobsen MS, Allal AS, Lehmann W, Calcaterra T. Nasal and paranasal sinus carcinoma: are we making progress? Cancer. 2001;92:3012. (Meta-analysis suggesting an improvement in the prognosis for patients with paranasal sinus malignancies.) [PMID: 11753979] | Hayashi T, Nonaka S, Bandoh N, Kobayashi Y, Imada M, Harabuchi Y. Treatment outcome of maxillary sinus squamous cell carcinoma. Cancer. 2001;92:1495. (Multimodality therapy offers improved 5-year survival rates compared to radiotherapy alone.) [PMID: 11745227] | Le QT, Fu KK, Kaplan M, Terris DJ, Fee WE, Goffinet DR. Treatment of maxillary sinus carcinoma: a comparison of the 1997 and 1977 American Joint Committee on cancer staging systems. Cancer. 1999;86:1700. (The 1997 staging system is superior to the 1977 system in determining patient survival and local control. Combined modality therapy was superior to radiotherapy alone.) [PMID: 10547542] | Waldron JN, O'Sullivan B, Gullane P et al. Carcinoma of the maxillary antrum: a retrospective analysis of 110 cases. Radiother Oncol. 2000;57:167. (Local disease extension often determines the prognosis, despite the treatment modality. The best treatment modality is, as yet, elusive .) [PMID: 11054520] | Adenocarcinomas & Adenoid Cystic Carcinomas General Considerations Adenocarcinomas arise from the epithelial surface of the sinonasal mucosa and occur more frequently than adenoid cystic carcinomas, which arise from the minor salivary glands. Together, they represent the most common mucous gland malignant neoplasms of the paranasal sinuses. Adenoid cystic carcinomas tend to arise from the maxillary antrum and can infiltrate into the surrounding tissue. They demonstrate perineural spread into the maxillary and mandibular branches of the trigeminal nerve (CN V), with extension into the foramina ovale and rotundum. Adenoid cystic carcinomas have a low incidence of regional metastases but greater distant metastases. Clincal Findings Adenocarcinomas arise typically from the ethmoid sinuses. There has been no correlation with smoking in the development of adenocarcinomas, but there has been a documented association with woodworkers and leather workers. Several histologic types are seen with variability in mucin production and cellular differentiation. They are similar to adenoid cystic carcinomas in their growth behavior. Treatment The treatment for both adenocarcinomas and adenoid cystic carcinomas consists of multimodality therapy at the advanced stages of disease. For maxillary sinus tumors, the treatment usually consists of a maxillectomy. An anterior craniofacial resection is often the recommended treatment for advanced ethmoid cancers. Postoperative radiation therapy is frequently employed in treating patients with all of these tumors. Cantu G, Solero CL, Mariani L et al. Anterior craniofacial resection for malignant ethmoid tumors: a series of 91 patients. Head Neck. 1999;21:185. (Surgical resection for most ethmoid tumors, regardless of tumor histology encroaching the cribriform plate, necessitates an anterior craniofacial resection.) [PMID: 10208659] | Olfactory Esthesioneuroblastomas General Considerations Olfactory esthesioneuroblastomas arise from the olfactory epithelium superior to the middle turbinate. These neoplasms account for only 15% of all malignant tumors of the paranasal sinuses. Olfactory esthesioneuroblastomas are initially unilateral and can grow into the adjacent sinuses and the contralateral nasal cavity; they can spread to the orbit and the brain. Classification No TNM staging system has been created for these tumors; a clinical grouping system has been developed that has no prognostic value. This system designates the following groups: (1) Group A consists of patients with tumors limited to the nasal cavity; (2) Group B includes patients whose tumors are localized in the nasal cavity and the paranasal sinuses; and (3) patients in Group C have tumors that extend beyond both the nasal cavity and the paranasal sinuses. Metastasis to the neck is seen in approximately 1020% of cases in all three groups. Clinical Findings Histologically, olfactory esthesioneuroblastomas can appear similar to both peripheral neuroblastomas and other sinonasal malignant tumors. Two features often seen on microscopy are rosettes and neurofibrillary processes. Immunocytochemical staining of the specimen, though showing tremendous variability, is an important and often necessary step in making an accurate diagnosis. Histologically, olfactory esthesioneuroblastomas do not appear to stain for keratin and epithelial membrane antigen. The most common positive immunoreactions are with neuron -specific enolase, S-100, microtubule-associated protein, Class III -tubulin isotype, neurofilament, and synaptophysin. Treatment & Prognosis Patients with esthesioneuroblastomas are best treated with combined-modality therapy, even if the tumors are designated as either Kadish Group A or B neoplasms. The 5-year disease-free survival for single-modality therapy for patients in Kadish Groups A and B is 55% compared with 61% for patients in Kadish Group C. The local tumor control rate of combined therapy is 87% versus 51% for radiation alone, and 0% for surgery alone. Surgical resection may involve either local resection or craniofacial resection with radiation doses of 6065 Gy postoperatively. Chao KS, Kaplan C, Simpson JR et al. Esthesioneuroblastoma: the impact of treatment modality. Head Neck. 2001;23:749. (The Kadish staging system in patients with esthesioneuroblastomas did not prognosticate local control or disease-free survival.) [PMID: 11505485] | Malignant Mucosal Melanomas General Considerations Respiratory tract mucosal melanomas occur in the nasal cavity and paranasal sinuses. They are exceedingly rare, with only 0.51.5% of all melanomas occurring in the sinonasal cavity. These neoplasms originate from melanocytes within the submucosa and from the mucosa of the paranasal sinuses. They are located most frequently in the anterior septum, followed by the middle and inferior turbinates. The maxillary sinus is the most common sinus cavity involved. Clinical Findings Epistaxis appears to be the most common symptom, and nasal obstruction is also common. On examination, the mass appears to be fleshy and polypoid. Tumors in the nasal cavity tend to be smaller at the time of diagnosis than tumors that arise within the sinuses. Nodal metastasis occurs in 1020% of cases. Staging No TNM staging system exists for mucosal melanomas. However, a practical staging system has been developed: (1) Stage I designates localized disease, (2) Stage II indicates regional metastasis, and (3) Stage III signifies distant metastasis. The factors that influence clinical outcomes include the clinical stage, a lesion thickness > 5 mm, the presence of vascular invasion, and the development of distant metastasis. Treatment & Prognosis The treatment for malignant mucosal melanomas consists of surgical excision followed by postoperative radiation therapy. As a result of this combined treatment approach, the 5-year disease-specific survival rate for sinonasal mucosal melanomas is approximately 47%. Patel SG, Prasad ML, Escrig M et al. Primary mucosal malignant melanoma of the head and neck. Head Neck. 2002;24:247. (Fifty-nine patients at Memorial Sloan-Kettering Cancer Center, in a span of more than 20 years , were reviewed. The only prognostic factors are the stage at presentation, the tumor thickness, vascular invasion, and distant metastasis.) [PMID: 11891956] | Sinonasal Undifferentiated Carcinomas Sinonasal undifferentiated carcinomas are highly aggressive tumors of the paranasal sinuses and often appear to be histologically similar to olfactory esthesioneuroblastomas. Like inverted papillomas, sinonasal undifferentiated carcinomas appear to arise from schneiderian mucosa. They grow rapidly , with extensive local invasion into the sinuses, the orbit, and the brain (Figure 163). Histologically, they appear to stain for keratin and epithelial membrane antigen and do not appear to have an association to Epstein-Barr virus (EBV), which would distinguish these tumors from undifferentiated nasopharyngeal carcinomas. Surgical resection with postoperative radiation therapy is the mainstay of therapy, although this combined approach results in a very poor prognosis. Cerilli LA, Holst VA, Brandwein MS, Stoler MH, Mills SE. Sinonasal undifferentiated carcinoma: immunohistochemical profile and lack of EBV association. Am J Surg Pathol. 2001;25:156. (Sinonasal undifferentiated carcinoma does not demonstrate EBV staining as do other tumors in the region [eg, nasopharyngeal carcinoma]. EBV staining can be used as a marker to distinguish these tumors from other small blue-cell tumors in the sinonasal cavity.) [PMID: 11176064] | Sharara N, Muller S, Olson J, Grist WJ, Grossniklaus HE. Sinonasal undifferentiated carcinoma with orbital invasion: report of three cases. Ophthal Plast Reconstr Surg. 2001;17:288. (Aggressive tumors that appear as small blue cells on histologic examination require differentiation from other tumors such as esthesioneuroblastoma.) [PMID: 11476180] | |