Matthew Spector
Erin Mckean
Presentation
A 55-year-old man with a 50 pack-year smoking history presents with hoarseness for 3 months. This has been progressive in nature and is associated with mild dysphagia, odynophagia, and weight loss. He denies shortness of breath but does report two-pillow orthopnea. On physical exam, he has inspiratory stridor without stertor or retractions. Otologic, nasal, oral cavity, and oropharyngeal examination is unremarkable. He has a 3-cm left level 2 neck mass that is firm, nontender, and mobile. Flexible fiberoptic laryngoscopy reveals an exophytic lesion of the left aryepiglottic fold extending inferiorly onto the false and true vocal cord. There is fixation of the left vocal cord.
Differential Diagnosis
New-onset hoarseness in a smoker with other concerning symptoms should be considered a cancer until proven otherwise. Breathing or swallowing problems are not uncommon as tumors become more advanced. Tumors of the glottic portion of the larynx often present early, as small tumors significantly affect the voice in this region, while other laryngeal subsites (supraglottic or subglottic) may be quite large before detected clinically.
There are other destructive processes of the upper aerodigestive tract that can mimic cancer, and biopsy is important to confirm diagnosis. Infectious processes (laryngitis or thrush), congenital anomalies (laryngocele), and autoimmune diseases (Wegener’s granulomatosis, sarcoidosis, amyloidosis) can have presentations similar to cancer.
Workup
The patient undergoes further evaluation with a CT scan of his neck and chest to evaluate the extent of local, regional, and distant disease. There is no cartilage invasion of the larynx, and there is a single lymph node in the left neck that is 2 cm (Figure 1). There is no metastatic disease in the chest. The patient is staged as a T3N1M0 (stage 3).
FIGURE 1 • CT scan of a primary squamous cell carcinoma of the supraglottic larynx. The contrast-enhancing lesion involves the left arytenoids, aryepiglottic fold, and false vocal cord.
Approximately 30% to 50% of patients with supraglottic tumors, 6% to 18% of patients with glottic tumors, and 4% to 27% of patients with subglottic tumors will have regional metastasis at their initial presentation. Many centers are also using positron emission tomography (PET) scans, although this is not currently the standard of care to evaluate the primary tumor. All patients should undergo operative direct laryngoscopy, esophagoscopy with biopsies under general anesthesia for treatment planning and to confirm absence of a second primary tumor (up to 10% of patients). These tests and procedures are routinely performed to allow appropriate staging for treatment planning (see below) (Figure 2).
FIGURE 2 • Intraoperative direct laryngoscopic view of cancer: T1 squamous cell carcinoma of the right true vocal cord. (Photo courtesy of Norman Hogikyan, MD.)
The workup of an unknown primary tumor (a patient presents with regional metastasis without evidence of primary tumor location) deserves specific mention. First, a thorough head and neck exam, including fiberoptic nasopharyngoscopy and laryngoscopy, is warranted. A fine needle aspiration biopsy of the neck mass should be performed, either directly or with ultrasound guidance, to confirm the diagnosis. A contrast-enhanced CT of the neck and chest including the skull base should be performed to attempt and identify the primary tumor. If no primary tumor is identified with anatomic (CT) imaging, PET scanning may aid in diagnosis. Some may choose to perform a PET/CT alone if clinical evaluation fails to identify a primary tumor site. The patient should then be taken to the operating room for direct laryngoscopy, esophagoscopy, tonsillectomy (if tonsils are present), and biopsies directed by the previous workup. The most common sites for an unknown primary tumor are the tonsils and the tongue base, followed by the nasopharynx and hypopharynx. The advantages of identification of the primary tumor include directed treatment, thereby sparing the patient treatment-related effects in uninvolved areas, as well as adequate treatment of a small primary tumor before it advances.
Discussion
Head and neck cancer is the fifth most common cancer with over 600,000 cases diagnosed each year. Men are affected five times as often as women. Head and neck cancer can be divided into subsites including the larynx, pharynx (nasopharynx, oropharynx, and hypopharynx), and oral cavity. Risk factors for all head and neck cancers include tobacco and alcohol, which work synergistically to increase risk. Human papilloma virus has also been shown as causative to the oropharynx subsite, and these patients generally have a better prognosis.
Overall survival rates are dependent upon tumor subsite and stage at diagnosis. Patients are staged based on the American Joint Committee on Cancer (AJCC) guidelines using the TNM classification system. Stages I and II represent early local disease without regional or distant metastasis. Stages III and IV represent advanced disease with either a locally aggressive tumor or a metastatic disease (regional or distant) (Table 1).
TABLE 1. AJCC TNM Staging for Larynx Cancer
Diagnosis and Treatment
Timely diagnosis and appropriate workup is important in the proper management of head and neck cancer patients. Referral to a head and neck cancer surgeon is most appropriate, and discussion at a head and neck oncology tumor board allows for decisive treatment. There should be a high suspicion for cancer in patients with a neck mass over 50 years of age and patients with previous smoking and/or alcohol history who have new speech or swallowing complaints.
Treatment
According to the AJCC guidelines, surgery or radiation therapy are acceptable options for early stage (I, II) disease. These tumors are limited in their extent, and there is no evidence of regional or distant metastasis. Multimodality treatment is necessary for advanced stage (III, IV) disease, as these tumors are either locally aggressive or have metastasized. Options may include surgery followed by radiation or chemoradiation. It is important to note that each subsite of head and neck cancer (e.g., oral cavity, oropharynx, nasopharynx, hypopharynx, larynx, salivary glands, nose and paranasal sinuses) has a different staging system, prognosis, and varying treatment recommendations. Surgical approaches are specifically tailored to the tumor, and reconstruction must be considered in preoperative planning. Speech and swallowing outcomes, as well as cosmesis, should be a factor in choosing local, regional, or free tissue reconstruction options. Pretreatment dental and speech pathology consultations should be obtained.
Surveillance
Consistent with National Comprehensive Cancer Network (NCCN) guidelines, patients are followed every 1 to 3 months for the first year, every 2 to 4 months for the second year, and every 4 to 6 months until the fifth year. At this point, the patient is considered to be cured of disease and can follow up yearly or on an as-needed basis. A key factor in oncologic surveillance is to encourage patients to be seen early if they develop new pain (or worsening pain) at the primary site, new otalgia, dysphagia, odynophagia, hoarseness, significant weight loss, hemoptysis, or hematemesis.
Case Conclusion
The patient was staged as a T3N1M0 (stage 3) squamous cell cancer of the supraglottic larynx. Options for chemoradiation versus primary surgery with postoperative radiation were discussed. The patient successfully underwent surgical excision of his tumor with total laryngectomy, bilateral selective neck dissections of levels II to IV, cricopharyngeal myotomy, tracheoesophageal puncture for later speech rehabilitation, and placement of a temporary Dobhoff feeding tube. He was discharged on postoperative day 5 uneventfully and was allowed to swallow soft foods at 3 weeks after surgery. His pathology showed one involved lymph node without extracapsular spread or perineural invasion, and he received adjuvant radiation therapy (Figure 3).
FIGURE 3 • Immediate postoperative views of stoma and incisions (with endotracheal tube in stoma and then removed). This is now the patient’s only airway, with complete removal of the larynx and thus disconnection with the upper airway. A red rubber Robinson catheter is seen in the tracheoesophageal puncture site, maintaining an opening for later voice prosthesis placement. (Photos courtesy of Norman Hogikyan, MD.)
TAKE HOME POINTS
· Early stage (I, II) tumors can be treated with single modality therapy, while advanced stage (III, IV) are treated with multimodality therapy. Adequate diagnosis and staging are key to determining the appropriate therapy.
· Timely referral to a head and neck surgeon is important to begin treatment.
SUGGESTED READINGS
Bailey BJ, Calhoun KH, Derkay CS, et al. Head and Neck Surgery – Otolaryngology. Vol. 2, Section 4. ISBN 978-0781729086.
Cummings CW, Haughey BW, Thomas JR, et al. Cummings Otolaryngology: Head and Neck Surgery. ISBN 978-0323019859.
Edge SB, Byrd DR, Compton CC, et al. AJCC Cancer Staging Manual. 7th ed. 2010. ISBN 978-0-387-88440-0.
Galer CE, Kies MS. Evaluation and management of the unknown primary carcinoma of the head and neck. J Natl Compr Canc Netw. 2008;6(10):1068–1075.
NCCN Guidelines for head and neck cancer care. National Comprehensive Cancer Network Web site. http://www.nccn.org/professionals/physician_gls/PDF/head-andneck.pdf.