Lingual foregut duplication in a middle-aged adult.

Lingual duplication cysts are rare in the pediatric population and exceedingly rare in adults. Imaging is an important part of the evaluation of lingual lesions and is helpful in defining the location and extent for surgical planning. We present a lingual duplication cyst and discuss the imaging findings and radiologic differential diagnosis.

Imaging characteristics of primary laryngeal lymphoma.

BACKGROUND AND PURPOSE: The larynx is a rare site for primary NHL. Fewer than 100 cases have been reported in the literature, with the largest imaging review involving only 4 patients. We describe the findings of laryngeal lymphoma on CT, PET, and MR imaging and identify features that may distinguish laryngeal lymphoma from the far more common laryngeal squamous cell carcinoma. MATERIALS AND METHODS: Multi-institutional retrospective chart review revealed 20 patients with histopathologically proved laryngeal lymphoma. Pretreatment CT, PET, and MR images were reviewed by a head and neck radiologist, focusing on extent of tumor, cervical lymph node involvement, and enhancement patterns. RESULTS: Patients ranged from 30 to 90 years of age with a mean of 63 years at the time of initial diagnosis and a 2:1 female predominance. The average tumor size was 37 +/- 19 mm. In all patients, laryngeal lymphoma involved the supraglottis but also extended into the glottis (65%) and hypopharynx (60%). The subglottis was less frequently involved (35%). Laryngeal cartilage invasion and cervical lymphadenopathy were each seen in 20% of patients. Lymphoma was consistently FDG-avid (100%) and usually enhanced uniformly with iodinated contrast (73%). Necrosis and calcification were not seen in any cases. CONCLUSIONS: Although laryngeal lymphoma is rare, particular imaging features suggest this diagnosis. A large uniformly enhancing supraglottic tumor without central necrosis and without cervical lymphadenopathy is a characteristic finding of lymphoma. Similar to squamous cell carcinoma, lymphoma may extend into the subglottis, pharynx, and laryngeal cartilages.

Characteristics of CD4+CD25+ regulatory T cells in the peripheral circulation of patients with head and neck cancer.

Patients with squamous cell carcinoma of the head and neck (SCCHN) have depressed antitumour immunity. The presence of CD4+CD25+ (Treg) cells in these patients might be, in part, responsible for downregulation of antitumour immune responses. To evaluate the frequency and characteristics of Treg in the peripheral circulation of patients with SCCHN, we used multicolour flow cytometry. Expression of CCR7, CD62L, zeta chain and Annexin V binding to Treg and non-Treg CD4+ lymphocyte populations were evaluated. Treg were confirmed to be Foxp3+ and GITR+. The Treg frequency was significantly elevated in patients with active disease and those with no evidence of disease (NED) following curative therapies. Both Treg and non-Treg CD4+ T cells in patients were significantly enriched in CCR7- and CD62L- cell subsets. Although Treg in patients contained a higher proportion of double negative (CCR7-CD62L-) cells, the majority of Tregs were CCR7-CD62L+. The proportion of Annexin V+CD4+ T cells was higher in patients (P<0.00005) than normal controls (NC), and Treg were significantly more sensitive to apoptosis than non-Treg in patients and NC. Expression of zeta was reduced in all subsets of CD4+ T cells obtained from patients vs NC. The data suggest that Treg in patients with SCCHN largely contain T cells with the 'effector' phenotype, which bind Annexin V and have low zeta expression, consistent with their activation state and a rapid turnover in the peripheral circulation.

Prevalence of SDHB, SDHC, and SDHD germline mutations in clinic patients with head and neck paragangliomas.

BACKGROUND: Paragangliomas are rare and highly heritable tumours of neuroectodermal origin that often develop in the head and neck region. Germline mutations in the mitochondrial complex II genes, SDHB, SDHC, and SDHD, cause hereditary paraganglioma (PGL). METHODS: We assessed the frequency of SDHB, SDHC, and SDHD gene mutations by PCR amplification and sequencing in a set of head and neck paraganglioma patients who were previously managed in two otolaryngology clinics in the USA. RESULTS: Fifty-five subjects were grouped into 10 families and 37 non-familial cases. Five of the non-familial cases had multiple tumours. Germline SDHD mutations were identified in five of 10 (50%) familial and two of 37 ( approximately 5%) non-familial cases. R38X, P81L, H102L, Q109X, and L128fsX134 mutations were identified in the familial cases and P81L was identified in the non-familial cases. Both non-familial cases had multiple tumours. P81L and R38X mutations have previously been reported in other PGL families and P81L was suggested as a founder mutation. Allelic analyses of different chromosomes carrying these mutations did not show common disease haplotypes, strongly suggesting that R38X and P81L are potentially recurrent mutations. Germline SDHB mutations were identified in two of 10 (20%) familial and one of 33 ( approximately 3%) non-familial cases. P131R and M71fsX80 were identified in the familial cases and Q59X was identified in the one non-familial case. The non-familial case had a solitary tumour. No mutations could be identified in the SDHC gene in the remaining four families and 20 sporadic cases. CONCLUSIONS: Mutations in SDHD are the leading cause of head and neck paragangliomas in this clinic patient series. SDHD and SDHB mutations account for 70% of familial cases and approximately 8% of non-familial cases. These results also suggest that the commonness of the SDHD P81L mutation in North America is the result of both a founder effect and recurrent mutations.

How I do it: closure of tracheoesophageal puncture site.

BACKGROUND: Tracheoesophageal voice prosthesis is highly effective in providing speech after total laryngectomy. Although it is a safe method, tracheoesophageal fistulas occasionally need to be closed, usually at the request of the patient, because of leakage through an enlarging fistula. We present our technique for closure of tracheoesophageal fistula. METHODS: An incision is made at the mucocutaneous junction of the stoma from the 9 to the 3-o'clock position. Tracheoesophageal space is dissected down to and beyond the fistula. The tracheoesophageal tract is divided. The esophageal mucosa is closed with inverted sutures. After multiple layer closure of the esophageal fistula, the tracheal mucosa is closed with everted sutures. RESULTS: This technique has been used in nine patients. Eight were successful. The remaining patient had radiation therapy failure. CONCLUSION: This method of closure is simple and effective for those patients who require permanent closure of the tracheoesophageal fistula.

The role of topical antibiotic prophylaxis in patients undergoing contaminated head and neck surgery with flap reconstruction.

OBJECTIVES/HYPOTHESIS: Patients undergoing contaminated head and neck surgery with flap reconstruction have wound infection rates of 20% to 25% with parenteral antibiotic prophylaxis. Studies suggest that perioperative antimicrobial mouthwash reduces oropharyngeal flora and may prevent wound infections. We hypothesized that the addition of topical antibiotics to a parenteral prophylactic regimen would reduce the incidence of wound infection in these high-risk patients. STUDY DESIGN: We performed a randomized, prospective clinical trial. METHODS: Patients received either 1) parenteral piperacillin/tazobactam (3.375 g every 6 hours for 48 h) or 2) parenteral piperacillin/tazobactam plus topical piperacillin/tazobactam administered as a mouthwash immediately before surgery and once a day for 2 days postoperatively, with piperacillin/tazobactam added to the intraoperative irrigation solution. The wounds of all patients were evaluated daily using predefined objective criteria. RESULTS: Sixty-two patients met inclusion criteria and were enrolled in the study. The overall wound infection rate was 8.1% (95% confidence interval [CI], 2.7%-17.8%). Two of 31 patients (6.4%) who received parenteral antibiotics alone developed a wound infection compared with 3 of 31 patients (9.7%) randomly assigned to receive topical plus parenteral antibiotics. This difference was not statistically significant (P = >.05). Infection rate was not associated with flap type (rotational vs. free tissue transfer), mandibular reconstruction, age, gender, tumor site, stage, surgical duration, or blood loss. CONCLUSIONS: These results suggest that piperacillin/tazobactam is a highly effective antibiotic for prevention of wound infection in patients undergoing flap reconstruction following contaminated head and neck surgery. However, the addition of topical piperacillin/tazobactam does not appear to enhance the prophylactic benefit of parenteral antibiotics alone.

Proportion of heritable paraganglioma cases and associated clinical characteristics.

OBJECTIVE/HYPOTHESIS: To determine the heritable proportion of paraganglioma (PGL) and identify clinical features associated with heritable PGL. STUDY DESIGN: Patients diagnosed with head and neck PGLs, identified retrospectively through clinical otolaryngology practices and/or participation in previous PGL research studies, were given a medical and family history questionnaire. METHODS: Questionnaire information was used to classify participants as having "heritable" or "non-heritable" cases of PGL. Classification of the participants identified through otolaryngology clinics was used to estimate the heritable proportion of PGL. Statistical analysis was performed to identify significant differences in the clinical characteristics of the heritable versus non-heritable groups. RESULTS: Among the otolaryngology clinic population, 35% were classified as having heritable PGL. Individuals with heritable PGL were younger on average than those with non-heritable PGL. The majority of non-heritable participants were female, but there was an equal gender ratio among the heritable participants. Individuals diagnosed with a carotid body tumor (CBT) were 5.8 times more likely to be classified as heritable than those diagnosed with PGL at other anatomic locations. CONCLUSIONS: Approximately 35% of individuals who present to an otolaryngologist with a head and neck PGL have inherited a predisposition for this growth. Among individuals diagnosed with head and neck PGL, those diagnosed with CBT are 5.8 times more likely to have an inherited predisposition than those diagnosed with PGL at other anatomic locations.

Chemoradiation for metastatic SCCA: role of comorbidity.

OBJECTIVES: A previous study of 371 patients with extracapsular spread (ECS) of cervical metastases from squamous cell carcinoma (SCCA) of the head and neck revealed a survival advantage for patients treated with adjuvant chemoradiation, compared with those treated with surgery and radiation or surgery alone. While all patients in the study were offered adjuvant chemotherapy, only 35% selected this option. Comorbidity was identified as a reason for declining chemotherapy. Recently, Piccirillo demonstrated that the Modified Medical Comorbidity Index (MMCI) is a valid instrument to classify and quantify severity of comorbidity. We applied this instrument to previously reported patients with ECS to determine 1) how comorbidity affected treatment selection, 2) whether the survival advantage of adjuvant chemoradiation persisted after controlling for comorbidity, and 3) the impact of comorbidity on outcome. STUDY DESIGN: This was a nonrandomized, retrospective study. METHODS: Patients in the initial study underwent resection of the primary tumor and neck dissection. Eligible patients elected to receive chemoradiation, radiation, or no further treatment. Comorbidity scores were assigned according to the MMCI. Data were analyzed according to disease-specific survival and overall survival. RESULTS: The study population consisted of 330 patients. More severe comorbidity was related to higher overall mortality rates after controlling for treatment. Adjuvant chemoradiation resulted in improved disease-specific and overall survival compared with adjuvant radiation after adjusting for severity of comorbidity. CONCLUSIONS: These results substantiate the benefits of adjuvant chemoradiation for patients with SCCA of the head and neck. Furthermore, these results reinforce the importance of comorbidity as a prognostic indicator for this population of patients.

Management of swallowing in supraglottic and extended supraglottic laryngectomy patients.

BACKGROUND: Aspiration of food and liquid following supraglottic and supracricoid laryngectomy has been documented and found to be the most frequent major postoperative complication that extends hospitalization. The advantages as well as disadvantages of discharging a patient with percutaneous endoscopic gastrostomy (PEG) placement and home therapy versus an aggressive in-hospital dysphagia management program remain controversial. The present investigation examines an aggressive in-patient postoperative dysphagia management program following decannulation. METHODS: Twenty-one patients participated in a four-part dysphagia management program following decannulation: patient education, indirect therapy, swallowing evaluation, and nutrition education. RESULTS: Eleven patients achieved functional swallowing goals prior to discharge with no reports of pneumonia or rehospitalization over a 3-month follow-up period. Six patients were discharged with a tracheostomy and duo tube; five of these patients were started on an oral diet the same day of decannulation. Four patients decannulated prior to discharge did not achieve functional swallowing. CONCLUSION: Certain patients can achieve functional swallowing goals prior to discharge and avoid the cost and surgical placement of a PEG. This group required an additional 2 to 3 days of hospitalization; however, the usual and customary charges for aggressive dysphagia management in this group were exceeded by charges for PEG placement and in-home therapy according to pricing guidelines for the hospital where these patients were treated. Specific patient profiles of those who were unsuccessful relate to extent of surgery, ie, supraglottic + base of tongue (SUPRA + BOT) and supraglottic + vocal fold (SUPRA + VF) resection, and non-compliance. Complicated patients often require longer rehabilitation and may benefit from a PEG at the time of surgery.

Selective neck dissection in the management of the clinically node-negative neck.

OBJECTIVE: To evaluate the efficacy of the selective neck dissection (SND) in the management of the clinically node-negative neck. STUDY DESIGN: Case histories were evaluated retrospectively. METHODS: The results of 300 neck dissections performed on 210 patients were studied. RESULTS: The primary sites were oral cavity (91), oropharynx (30), hypopharynx (16), and larynx (73). Seventy-one necks (23%) were node positive on pathological examination. The number of positive nodes varied from 1 to 9 per side. Of necks with positive nodes, 17 (24%) had extracapsular spread. The median follow-up was 41 months. Recurrent disease developed in the dissected neck of 11 patients (4%). Two recurrences developed outside the dissected field. The incidence of regional recurrences was similar in patients in whom nodes were negative on histological examination (3%) when compared with patients with positive nodes without extracapsular spread (4%). In contrast, regional recurrence developed in 18% of necks with extracapsular spread. This observation was statistically significant. Patients having more than two metastatic lymph nodes had a higher incidence of recurrent disease than the patients with carcinoma limited to one or two nodes. Recurrence rate in the pathologically node positive (pN+) necks was comparable to recurrence in those pathologically node negative (pNO) necks in the patients who did not have irradiation. CONCLUSION: SND is effective for controlling neck disease and serves to detect patients who require adjuvant therapy.

Laser ablation of granulation tissue at the tracheoesophageal puncture site.

BACKGROUND: Tracheoesophageal fistula speech may be complicated by granulation tissue around the puncture site. Treatment includes antibiotics, antifungals, chemical or electrocautery, and surgical excision of the granulation tissue. Chemical cautery generally requires repeated treatment sessions. METHODS: We report our technique and results of CO(2) laser ablation of granulation tissue at the tracheoesophageal puncture site performed as an outpatient office procedure. RESULTS: Seven of 9 patients (78%) were cured with a single treatment. The prosthesis was replaced immediately after laser ablation of granulation tissue. Of the remaining 2 patients, 1 had a recurrence a year later, requiring repeated laser. The second patient had a large mass requiring 4 laser ablations. There were no complications. CONCLUSIONS: CO(2) laser has the following advantages compared with other methods of treating stomal granulation tissue: office procedure; 78% cured with single treatment; and the extent of tissue necrosis is predictable.

Squamous cell carcinoma of the external auditory canal: an evaluation of a staging system.

OBJECTIVE: The study was conducted to review a staging system proposed by the University of Pittsburgh for temporal bone cancer and to evaluate survival status according to stage, treatment, and certain prognostic factors. STUDY DESIGN: The study was a retrospective case review. SETTING: The study was conducted at a tertiary care medical center and specialty hospital. PATIENTS: Thirty-two patients with primary squamous cell carcinoma of the external auditory canal were studied. INTERVENTION: All patients underwent surgery of the temporal bone. Radiotherapy was given depending on tumor stage and histopathologic findings. MAIN OUTCOME MEASURES: The 2-year survival rates of patients undergoing surgical resection with or without adjuvant radiotherapy. RESULTS: The 2-year survival rates for primary squamous cell carcinoma of the temporal bone were as follows: T1 lesions 100%, T2 80%, T3 50%, and T4 7%. Survival for T3 tumors was 75% with postoperative radiotherapy, compared with 0% with surgery alone. CONCLUSIONS: The 2-year survival data directly correlated with the staging system. The use of adjuvant radiotherapy increased survival rate in patients with a T3 lesion.

Primary surgery as treatment for early squamous cell carcinoma of the tonsil.

BACKGROUND: The management of tonsil carcinoma has gradually evolved such that the literature is replete with outcome summaries of this disease treated with primary RT and chemotherapy. Recently there have been no reports of patient outcomes with primary surgical therapy. Nonsurgical treatment is warranted when tumors are unresectable or if the patient refuses surgery. Our policy has been to treat operable squamous cell carcinoma (SCCA) of the tonsil with surgery. The decision to use adjuvant therapy is based on the surgical and histologic findings. We herein report our results with this treatment protocol. METHODS: A retrospective review of 162 patients with SCCA of the tonsil was performed. Eighty-four patients were treated with surgery, which was followed by RT and/or chemotherapy if histologic signs of aggressive behavior were identified. Patients were followed 2 to 15 years after treatment. RESULTS: Of the 9 patients with stage I disease, 89% are without evidence of recurrent disease and 91% of patients with stage II tonsil cancers are also disease free. The survival rates for stage III and stage IV cancer patients are 79 and 52%, respectively. CONCLUSION: Our data suggest that patients with early tonsil cancer can be effectively treated with surgery. Surgery allows pathologic staging so that patients with advanced tumors can be treated with adjuvant therapy.

Cancer of the tongue in patients less than forty.

INTRODUCTION: It is the opinion of many surgeons that the biologic potential of cancer that develops in young people is different compared with older patients. Prior reports on small series of patients addressing this issue have inadequate statistical power to resolve the question. METHODS: By use of the techniques of meta-analysis, patients less than 40 years old who had undergone treatment of squamous cell carcinoma (SCC) of the oral tongue were examined. Twenty-eight patients who were encountered in the Department of Otolaryngology, University of Pittsburgh School of Medicine, and 94 patients were identified in the literature for a total of 122 patients <40 years old. A control group of 150 patients, aged 40 years and older treated for SCC of the oral tongue between 1982 and 1994 was identified. RESULTS: Three-year disease-free survival in the group of patients aged less than 40 was 53.3% compared with 3-year disease free survivorship of 55.0% in the older cohort of patients. CONCLUSION: These data strongly suggest that the outcomes of treatment for SCC of the oral tongue in young patients are similar compared with patients older than 40 with similar extent of disease.

Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma.

Hereditary paraganglioma (PGL) is characterized by the development of benign, vascularized tumors in the head and neck. The most common tumor site is the carotid body (CB), a chemoreceptive organ that senses oxygen levels in the blood. Analysis of families carrying the PGL1 gene, described here, revealed germ line mutations in the SDHD gene on chromosome 11q23. SDHD encodes a mitochondrial respiratory chain protein-the small subunit of cytochrome b in succinate-ubiquinone oxidoreductase (cybS). In contrast to expectations based on the inheritance pattern of PGL, the SDHD gene showed no evidence of imprinting. These findings indicate that mitochondria play an important role in the pathogenesis of certain tumors and that cybS plays a role in normal CB physiology.

Relating speech and swallow function to dropout in a longitudinal study of head and neck cancer.

The relation between functional outcome and dropout from a 12-month follow-up period was examined in a longitudinal study whose objective was to define and quantify the functional effects of oral surgical resection and reconstruction on speech and swallowing in patients with head and neck cancer. In a group of 150 patients recruited to a surgical study in the Cancer Control Science Program in Head and Neck Cancer Rehabilitation, dropout from all causes and dropout from specific causes (medical, patient, and administrative specific) were assessed in relation to longitudinal speech and swallow function. In univariate analysis, better speech articulation was associated with decreased risk of dropout from all causes and from medical-specific causes. Better swallow performance was associated with decreased risk of medical-specific dropout. Multivariate analysis revealed the following: (1) only articulation function was associated with dropout from all causes; (2) the association of speech articulation function with medical dropout was diminished after adjusting for advanced age and surgical resection variables; (3) the association of speech articulation function became significant for patient-specific dropout after adjusting for advanced age and surgical resection variables and indicated that better function decreased the risk of this type of dropout; and (4) swallowing function was not related to dropout. Patients treated for oral or oropharyngeal cancer who have poorer speech outcomes are more likely to drop out from a longitudinal study. Basing study results on only patients who complete a longitudinal study will understate the level of dysfunction experienced.