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ImportancePopulation-based genomic screening can facilitate early detection of medullary thyroid carcinoma (MTC) in patients with pathogenic/likely pathogenic (P/LP) RET variants.ObjectiveTo evaluate the clinical treatment and patient outcomes after identification of P/LP RET proto-oncogene variants associated with the risk of MTC via a population genomic screening program.Design, Setting, ParticipantsThis retrospective cross-sectional study was completed between June 1, 2016, and May 31, 2022, for a mean follow-up period of 22.4 months (range, 2-76 months). The study included patients who were identified as having P/LP RET variants through a population genomic screening program at a rural tertiary care center and who underwent thyroidectomy after results disclosure.Main Outcomes and MeasuresThe outcomes of interest were preoperative evaluation and treatment-related outcomes. Measures included imaging and laboratory findings, extent of surgery, pathologic diagnosis, and staging.ResultsSeventy-five patients were identified as having P/LP RET variants exclusively through genomic screening. Twenty of these patients (27%; 11 women [55%] and 9 men [45%]; median age, 48 years [range, 22-73 years]) underwent total thyroidectomy; 13 of these patients (65%) also had a central neck dissection. No patients had clinically apparent disease at the time of surgery. Pathologic findings indicated MTC for 12 patients and papillary thyroid carcinoma in 2. Of patients with MTC, 10 had stage I disease, 1 had stage II disease, 1 had stage III disease, and none had stage IV disease. Based on postoperative surveillance imaging and laboratory results, no patient had evidence of recalcitrant disease.Conclusions and RelevanceIn this cross-sectional study, all malignant neoplasms identified on surgical pathology were clinically occult, with surgical intervention based solely on the identification of the P/LP RET variant via population genomic screening. This finding suggests that genomic screening may provide opportunities for early detection and treatment of MTC, with the potential for improved patient outcomes.

The majority of high-volume parathyroid surgeons use intraoperative parathyroid hormone monitoring (IPM) to guide parathyroidectomy in patients with sporadic primary hyperparathyroidism (SPHPT). This surgical adjunct has helped change the operative approach to parathyroidectomy from the traditional bilateral neck exploration (BNE) with visualization of all parathyroids and excision of enlarged glands based on the surgeon’s subjective judgment of gland size to a minimally invasive parathyroidectomy with removal of only the hypersecreting gland(s), preserving all normally functioning tissue. This less invasive approach to parathyroidectomy, safely achieved with the help of IPM, is not only as successful as BNE but also allows for surgery in an ambulatory setting with less neck dissection, often with fewer glands excised, shorter operative time, and fewer postoperative complications.

Frey syndrome (gustatory sweating) is a common and often times significant consequence of parotid gland surgery. The classic signs and symptoms include facial sweating, flushing, rubor, and sensation of heat over the preauricular region during oral stimulation. These clinical findings may provoke quality of life changes, and thus, are considered significant. Both surgical and nonsurgical treatment modalities have been advanced in the treatment of this disorder. Surgical treatment is aimed at prevention of the development of Frey syndrome and consists of a constellation of techniques which are designed to interpose tissue in the parotid surgical bed to act as a barrier to prevent aberrant parasympathetic fiber ingrowth and innervation to facial skin eccrine sweat glands. These techniques include: limiting the extent of parotid surgery when possible, use of local tissue as advancement or rotational flaps, autologous transfer of tissue, and use of allograft material.

Inflammatory thyroid diseases include those which are mediated through autoimmune disorders such as Hashimoto thyroiditis and Graves disease, as well as inflammatory goiter and subacute thyroiditis. In patients with such disorders, the thyroid gland often becomes markedly enlarged with an associated fibro-vascular connective tissue reaction involving the thyroid capsule and surrounding soft tissues. This results in dense, adherent connective tissue which may compress and obscure critical central neck compartment structures and make capsular dissection challenging. Thyroid surgery in patients with fibro-adherent inflammatory thyroid disease is technically challenging and postoperative complication rates are higher for these procedures. Due to these increased risks for complications, operative procedures in these circumstances should be designed with careful consideration of the anatomical changes associated with the underlying inflammatory pathology. Elements important to successful surgical resection include appropriate patient preparation and surgical planning, together with execution of the operative procedure with adaptation to many of the changes induced by inflammation on both the thyroid gland and the surrounding central neck structures.

Cancer of the parathyroid glands is an exceedingly rare endocrine malignancy representing a very uncommon cause of primary hyperparathyroidism. It is characterized by markedly increased serum calcium and parathyroid hormone (PTH) levels far in excess of those exhibited by benign parathyroid disease. Diagnosis is made by histopathology, which may be problematic before initiating treatment for hyperparathyroidism. Because of its rarity, both staging classification and prognostic factors have been difficult to define and less useful in predicting the course of disease and treatment outcomes. Effective management involves comprehensive surgical resection, which offers the best opportunity for cure. Both radiotherapy and chemotherapy are largely ineffective modalities for treatment. Recurrent disease is generally incurable; however, repeat surgery and medical management of tumor-induced hypercalcemia may provide intermediate-term palliation.

Primary mucosal melanomas (PMMs) of the head and neck are uncommon malignancies that arise mainly in the nasal cavity and paranasal sinuses, followed by the oral cavity. The mainstay of treatment is radical surgical resection followed by adjuvant radiotherapy in selected patients with high-risk features. Multimodality therapy has not been well studied and is not standardized. Adjuvant radiotherapy seems to improve locoregional control but does not improve overall survival (OS). Elective neck dissection is advocated in patients with oral PMM. Systemic therapy should be considered only for patients with metastatic or unresectable locoregional disease. Despite improvements in the field of surgery, radiotherapy, and systemic therapy, patients with PMM still face a very unfavorable prognosis (5-year disease-free survival [DFS]

Thyroid nodules in children are extremely uncommon. Most thyroid nodules, both benign and malignant, present as asymptomatic neck masses. A thyroid nodule in a child is significant because of the risk of malignancy. A review of medical records at our institution demonstrated 71 patients 20 years of age and younger with surgically managed thyroid nodules, of which 45 were benign and 26 were malignant. Our diagnostic workup, including serum thyroid studies, radiologic evaluation, and fine-needle aspiration, is discussed. Because of the possibility of malignancy, we recommend that all solitary thyroid nodules be excised in children unless fine-needle aspiration definitively determines a benign histology. The extent and type of surgical management is controversial and is still subject to much debate. Partial thyroidectomy appears adequate for benign disease, but even though there is no statistical difference in survival, we recommend total thyroidectomy for the management of malignant disease.

Objectives/Hypothesis Pharyngocutaneous fistulae (PCF) are known to occur in nearly one-third of patients after salvage total laryngectomy (STL). PCF has severe impact on duration of admission and costs and quality of life and can even cause severe complications such as bleeding, infection and death. Many patients need further surgical procedures. The implications for functional outcome and survival are less clear. Several studies have shown that using vascularized tissue from outside the radiation field reduces the risk of PCFs following STL. This review and meta-analysis aims to identify the evidence base to support this hypothesis. Data Sources English language literature from 2004 to 2013 Review Methods We searched the English language literature for articles published on the subject from 2004 to 2013. Results Adequate data was available to identify pooled incidence rates from seven articles. The pooled relative risk derived from 591 patients was 0.63 (95% CI: 0.47 to 0.85), indicating that patients who have flap reconstruction/reinforcement reduced their risk of PCF by one-third. Conclusion This pooled analysis suggests that there is a clear advantage in using vascularized tissue from outside the radiation field in the laryngectomy defect. While some studies show a clear reduction in PCF rates, others suggest that the fistulae that occur are smaller and rarely need repair. Laryngoscope, 124:1848-1853, 2014

Neck dissection is an important treatment for metastases from upper aerodigestive carcinoma; an event that markedly reduces survival. Since its inception, the philosophy of the procedure has undergone significant change from one of radicalism to the current conservative approach. Furthermore, nonsurgical modalities have been introduced, and, in many situations, have supplanted neck surgery. The refinements of imaging the neck based on the concept of neck level involvement has encouraged new philosophies to evolve that seem to benefit patient outcomes particularly as this relates to diminished morbidity. The purpose of this review was to highlight the new paradigms for surgical removal of neck metastases using an evidence-based approach.

Prophylactic neck dissection (PND) for papillary thyroid carcinoma (PTC) is controversial. Our aim was to assess current levels of evidence (LE) according to the Oxford Centre for Evidence-based Medicine ( http://www.cebm.net/?O=1025 ) regarding the oncologic benefits of PND. Data were analyzed via MEDLINE keywords: PTC, differentiated thyroid carcinoma, PND, central lymph node metastases, central compartment, recurrence-free survival. There was conflicting evidence regarding the rate of reoperation for recurrence, with some studies showing a lower rate after PND with increased recurrence-free survival and a higher rate of undetectable pre- and post-ablation thyroglobulin levels (LE 4), whereas other studies did not show a difference (LE 4). Only one study (LE 4) showed improved disease-specific survival with PND. PND may improve recurrence-free survival, although this is supported by only a low LE. Current recommendations can only be based on low-level evidence.

BACKGROUND: The purpose of this study was to give an overview on hereditary syndromes associated with head and neck paragangliomas (HNPGs). METHODS: Our methods were the review and discussion of the pertinent literature. RESULTS: About one third of all patients with HNPGs are carriers of germline mutations. Hereditary HNPGs have been described in association with mutations of 10 different genes. Mutations of the genes succinate dehydrogenase subunit D (SDHD), succinate dehydrogenase complex assembly factor 2 gene (SDHAF2), succinate dehydrogenase subunit C (SDHC), and succinate dehydrogenase subunit B (SDHB) are the cause of paraganglioma syndromes (PGLs) 1, 2, 3, and 4. Succinate dehydrogenase subunit A (SDHA), von Hippel-Lindau (VHL), and transmembrane protein 127 (TMEM127) gene mutations also harbor the risk for HNPG development. HNPGs in patients with rearranged during transfection (RET), neurofibromatosis type 1 (NF1), and MYC-associated factor X (MAX) gene mutations have been described very infrequently. CONCLUSION: All patients with HNPGs should be offered a molecular genetic screening. This screening may usually be restricted to mutations of the genes SDHD, SDHB, and SDHC. Certain clinical parameters can help to set up the order in which those genes should be tested. (c) 2013 Wiley Periodicals, Inc. Head Neck 36: 907-916, 2014.

Item does not contain fulltext BACKGROUND: The definitive treatment for head and neck paraganglioma (PG) is surgical excision. Unfortunately, surgery, particularly of vagal paraganglioma (VPG; "glomus vagale") and foramen jugulare ("glomus jugulare") tumors, may be complicated by injuries to the lower cranial nerves, a high price to pay for treatment for a benign tumor. Alternatively these tumors may be followed without treatment, or irradiated. The purpose of this review was to compare the existing evidence concerning the efficacy and safety of surgery, external beam radiotherapy (EBRT), and stereotactic radiosurgery (SRS), for jugular paragangliomas (JPGs) and VPGs. METHODS: Relevant articles were reviewed using strict criteria for systematic searches. Forty-one surgical studies met the criteria which included 1310 patients. Twenty articles including 461 patients treated with EBRT, and 14 radiosurgery studies comprising 261 patients were also evaluated. Results were compared between treatment modalities using analysis of variance (ANOVA) tests. RESULTS: A total of 1084 patients with JPGs and 226 VPGs were treated with different surgical procedures. Long-term control of the disease was achieved in 78.2% and 93.3% of patients, respectively. A total of 715 patients with JPG were treated with radiotherapy: 461 with EBRT and 254 with SRS. Control of the disease with both methods was obtained in 89.1% and 93.7% of the patients, respectively. The treatment outcomes of a JPG treated with surgery or radiotherapy were compared. Tumor control failure, major complication rates, and the number of cranial nerve palsies after treatment were significantly higher in surgical than in radiotherapy series. The results of SRS and EBRT in JPGs were compared and no significant differences were observed in tumor control. Because only 1 article reported on the treatment of 10 VPGs with radiotherapy, no comparisons with surgery could be made. Nevertheless, the vagus nerve was functionally preserved in only 11 of 254 surgically treated patients (4.3%). CONCLUSION: There is evidence that EBRT and SRS offer a similar chance of tumor control with lower risks of morbidity compared with surgery in patients with JPGs. Although the evidence is based on retrospective studies, these results suggest that surgery should be considered only for selected cases, but the decision should be individual for every patient.

Item does not contain fulltext In an era of advanced diagnostics, metastasis to cervical lymph nodes from an occult primary tumor is a rare clinical entity and accounts for approximately 3% of head and neck malignancies. Histologically, two thirds of cases are squamous cell carcinomas (SCCs), with other tissue types less common in the neck. With modern imaging and tissue examinations, a primary tumor initially undetected on physical examination is revealed in >50% of patients and the site of the index primary can be predicted with a high level of probability. In the present review, the range and limitations of diagnostic procedures are summarized and the optimal diagnostic workup is proposed. Initial preferred diagnostic procedures are a fine-needle aspiration biopsy (FNAB) and imaging. This allows directed surgical biopsy (such as tonsillectomy), based on the preliminary findings, and prevents misinterpretation of postsurgical images. When no primary lesion is suggested after imaging and panendoscopy, and for patients without a history of smoking and alcohol abuse, molecular profiling of an FNAB sample for human papillomavirus (HPV) and/or Epstein-Barr virus (EBV) is important.

Secondary and tertiary hyperparathyroidism (HPT) develop in patients with renal failure due to a variety of mechanisms including increased phosphorus and fibroblast growth factor 23 (FGF23), and decreased calcium and 1,25-dihydroxy vitamin D levels. Patients present with various bone disorders, cardiovascular disease, and typical laboratory abnormalities. Medical treatment consists of controlling hyperphosphatemia, vitamin D/analog and calcium administration, and calcimimetic agents. Improved medical therapies have led to a decrease in the use of parathyroidectomy (PTX). The surgical indications include parathyroid hormone (PTH) levels >800 pg/ml associated with hypercalcemia and/or hyperphosphatemia despite medical therapy. Other indications include calciphylaxis, fractures, bone pain or pruritis. Transplant recipients often show decreased PTH, calcium and phosphorus levels, but some will have persistent HPT. Evidence suggests that PTX may cause deterioration in renal graft function in the short-term calling into the question the indications for PTX in these patients. Pre-operative imaging is only occasionally helpful except in re-operative PTX. Operative approaches include subtotal PTX, total PTX with or without autotransplantation, and possible thymectomy. Each approach has its proponents, advantages and disadvantages which are discussed. Intraoperative PTH monitoring has a high positive predictive value of cure but a poor negative predictive value and therefore is of limited utility. Hypocalcemia is the most common complication requiring aggressive calcium administration. Benefits of surgery may include improved survival, bone mineral density and alleviation of symptoms.

Objectives/hypothesis Critical evaluation of a directed exploration protocol used by a single surgeon in the management of surgical parathyroid disease. Study design Retrospective chart review was made of patients surgically managed for hyperparathyroidism at an academic tertiary care center. Methods Three hundred forty-six patients were evaluated for biochemically proven hyperparathyroidism between March 1995 and February 2002. A directed exploration protocol was implemented in appropriately selected patients with primary hyperparathyroidism and in patients with secondary or tertiary hyperparathyroidism requiring repeat operation. The protocol included preoperative technetium-99m sestamibi imaging for hyperfunctional parathyroid localization, targeted neck exploration, rapid intraoperative parathyroid hormone determination, and limited-stay discharge from the ambulatory surgical recovery unit. Data collection was accomplished by entering patient evaluation, management, and outcome information prospectively into collective case report forms. A retrospective analysis of the data was conducted for the purpose of evaluating the effectiveness of the protocol. Results Sustained normocalcemia beyond 6 months postoperatively was achieved in 323 of 327 (99%) patients with primary hyperparathyroidism. Eighty-four percent (84%) of patients with secondary or tertiary hyperparathyroidism achieved normocalcemia or had resolution of symptoms as a measure of therapeutic success. The complication rate for the entire series of patients was 2.8%. Ninety-two percent of positive findings on sestamibi scan correctly predicted the location of an adenoma, whereas a negative finding accurately predicted the absence of an enlarged gland in a "usual" location in 81% of patients. Twenty-six patients (9%) had a false-positive finding on the scan, whereby a solitary adenoma was found contralateral to the side indicated by the scan. Overall, the positive predictive value for sestamibi imaging in the series was 91%. Intraoperative parathyroid hormone determination yielded an overall rate of reduction of 80% from preoperative levels during directed exploration. Sustained normocalcemia was achieved in all patients in whom intraoperative parathyroid hormone determination demonstrated a minimum decline of 50% from preoperative levels following resection of hyperfunctional parathyroid tissue (adenoma[s]). The majority (72%) of patients were managed in an outpatient (ambulatory surgery) setting and were discharged to home within 8 to 12 hours after surgery. Conclusion The directed exploration protocol for surgical management of hyperparathyroidism generated surgical rates of success that were as good as and, in most cases, improved over that of traditional bilateral exploration. This achievement was associated with low morbidity and reduced time and facility utilization, conveying improved cost-effectiveness. This surgical strategy should serve to enhance the capability of the surgeon to safely and efficiently manage the majority of patients with surgical parathyroid disease.

Objectives/Hypothesis: To compare patients with a parathyroid hormone index (iPTH) level less than 100 pg/mL (low baseline) with patients with an index level greater than 100 pg/mL (high baseline) relative to intraoperative iPTH levels (IOPTH), surgical findings, imaging, and outcome. Study Design: Retrospective chart review. Methods: The medical records of 284 patients with primary hyperparathyroidism undergoing parathyroid exploration utilizing IOPTH were reviewed. One hundred fifty-eight patients with a low baseline iPTH level were compared with 144 patients with a high baseline iPTH level. Patients were correlated in regard to IOPTH results, surgical findings, preoperative localization imaging, and surgical outcome. Results: There was no significant difference between the two groups in regard to recalcitrant disease. Twenty percent of the low baseline group of patients had multigland disease, versus 8% of the high baseline patients. The kinetic profile of iPTH degradation differed between the two groups, with the low baseline group demonstrating a significantly lower percentage drop compared to the high baseline group. Conclusions: Patients with preoperative low baseline iPTH levels are over two times more likely to have multigland disease than patients with high baseline levels. Preoperative imaging in this group appears to be less likely to yield a solitary adenoma, even in the absence of multigland disease. IOPTH degradation kinetics is variable between groups, resulting in the possibility of inconsistent correlation with complete resection. These findings suggest that the current IOPTH guidelines, with reference to adequacy of resection, may need to be amended, especially for patients with low baseline iPTH levels. Laryngoscope, 2009

Technological developments have significantly contributed to the rapid evolution of the surgical management of parathyroid disorders. The ability to physiologically determine the intraoperative status of the patient is now possible through the assessment of changing levels of intact parathyroid hormone (PTH) during surgery. In most patients with primary hyperparathyroidism, this method provides biochemical confirmation of hyperfunctional gland removal, and is predictive of a eucalcemic state and surgical cure. Patients with renal-induced disease (excluding tertiary hyperparathyroidism) do not follow the same kinetic decline in PTH and are therefore less likely to benefit from this modality. An emerging population of patients with primary disease and lower baseline levels of intact PTH appear to demonstrate a greater likelihood of manifesting multiple gland disease and do not follow a consistently predictable kinetic degradation profile. Although this category of patients will require further investigation, potentially resulting in modification of the utility of intraoperative determination of PTH levels, it is clear that this modality represents a very powerful technique which has significantly enhanced the surgical treatment of parathyroid disease.

Mesenchymal chondrosarcoma of the head and neck is an uncommon tumor with a potential for exhibiting highly aggressive behavior. When these tumors arise in the head and neck region, they appear to have a predilection for the maxillofacial skeleton; less often, they may involve other soft tissue sites in the head and neck. The diagnosis is challenging and may be assisted by molecular pathologic techniques when only limited tissue is available for analysis. Management is primarily surgical. Although adjuvant radiation appears to convey some benefit by reducing tumor bulk when these lesions have extended beyond bony confines, there is no evidence to suggest that this is associated with improved outcome. Chemotherapy does not appear to be effective in the limited experience documented thus far. Patients with complete local control following resection should be followed closely for development of distant metastasis, which signifies a worse clinical outcome. Future effective therapy may be found in the identification of molecular targets responsive to adjuvant chemotherapy or biologic modifiers.

Selective neck dissection is a modification of the more comprehensive modified radical or radical neck dissection that is designed to remove only those nodal levels considered to be at risk for harboring nodal metastases. The role of selective neck dissection continues to evolve: while initially designed as a staging and diagnostic procedure for patients without clinical evidence of nodal disease, a growing body of literature suggests that selective neck dissection has a therapeutic role in patients with clinical and histologic evidence of nodal metastases. The rationale behind selective neck dissection, its application in the clinically negative but histologically node-positive neck and the extended application of selective neck dissection in patients with clinical evidence of nodal disease are discussed.

In the absence of large-scale randomized trials evaluating dissection versus observation of the involved neck after neoadjuvant chemoradiotherapy, there is a need to collect data that will either support or ultimately refute a role for planned posttreatment neck dissection. A significant percentage of patients with extensive (N2 or N3) neck disease who demonstrate a complete response to chemoradiation therapy may harbor residual occult metastases, and identification of this subset of patients remains a clinical challenge. Because surgical salvage rates are greatly diminished when occult nodal disease becomes clinically manifest, planned posttreatment neck dissection is advocated but may not be necessary in all patients. The role of positron emission tomography chemoradiotherapy (PET-CT) in this scenario remains unproven but holds promise in being able to identify which patients may be harboring residual disease in the neck after chemoradiotherapy. The implementation of as yet unidentified molecular tumor markers in combination with PET-CT may ultimately prove to be effective in identifying patients who will best benefit from posttherapy neck dissection. Correlation of imaging results and pathologic node status will be important in determining the accuracy and, therefore, the value of this imaging modality for predicting the presence or absence of residual disease.

Re-operation for hyperparathyroidism represents a surgical challenge. The opportunity for therapeutic success is reduced while the potential for morbidity increased compared with initial exploration. The implementation of correlative localization studies, using a combination of physiologic and anatomic imaging, enables the surgeon to effectively target and remove hyperfunctional parathyroid glands. The ability to biochemically confirm adequate removal of hyperfunctional tissue intraoperatively using rapid parathyroid hormone assessment provides a measure of security in limiting dissection, reducing morbidity and optimizing the opportunity for achievement of eucalcemia. When evaluating patients with recalcitrant hyperparathyroidism, a regimented, methodologic management protocol should be followed to minimize the potential for misdiagnosis and/or unsuccessful reexploration.

(2003). Stomal Recurrence: Pathophysiology, Treatment and Prevention. Acta Oto-Laryngologica: Vol. 123, No. 3, pp. 421-432.

(2002). Current Management of Cutaneous Malignant Melanoma of the Head and Neck. Acta Oto-Laryngologica: Vol. 122, No. 8, pp. 900-906.

(2002). Management of the Neck in Cancer of the Major Salivary Glands, Thyroid and Parathyroid Glands. Acta Oto-Laryngologica: Vol. 122, No. 6, pp. 673-678.

Primary mucosal melanomas (PMMs) of the head and neck are uncommon malignancies that arise mainly in the nasal cavity and paranasal sinuses, followed by the oral cavity. The mainstay of treatment is radical surgical resection followed by adjuvant radiotherapy in selected patients with high-risk features. Multimodality therapy has not been well studied and is not standardized. Adjuvant radiotherapy seems to improve locoregional control but does not improve overall survival (OS). Elective neck dissection is advocated in patients with oral PMM. Systemic therapy should be considered only for patients with metastatic or unresectable locoregional disease. Despite improvements in the field of surgery, radiotherapy, and systemic therapy, patients with PMM still face a very unfavorable prognosis (5-year disease-free survival [DFS]

Objective: The goal was to identify factors associated with the outcome of salvage therapy for patients with isolated cervical recurrences of squamous cell carcinoma in the previously treated neck (ICR-PTN). Study Design and Settings: A tumor registry search for ICR-PTN patients was performed at 7 participating institutions, and the charts were reviewed. Kaplan-Meier plots for survival and time until re-recurrence were used to evaluate the significance of associated variables. Results: Median survival and time until re-recurrence were both 11 months. Survival was better in patients with the following characteristics: nonsurgical initial neck treatment, negative initial disease resection margins, no history of prior recurrence, ipsilateral location of the ICR-PTN relative to the primary, and use of surgical salvage. Conclusions: By pooling the experience of 7 US tertiary care medical centers, we have identified 5 factors that are associated with outcome of salvage therapy for ICR-PTN. Significance: Consideration of these factors, as well as the reviewed literature, should facilitate patient selection for salvage protocols. (Otolaryngol Head Neck Surg 2000;123:368-76.)

Background The efficacy of extending the application of selective neck dissection to include more-extensive neck disease in patients with squamous carcinoma of the upper aerodigestive tract remains controversial. Methods A review of all patients undergoing selective neck dissection at a single institution during a 5-year period was undertaken. The analysis was conducted on 82 patients who received 94 selective neck dissections as part of initial therapy for management of squamous carcinoma of the upper aerodigestive tract, including: oral cavity, oropharynx, larynx, and hypopharynx. Results Forty-six of the 94 dissected necks were supraomohyoid dissections, and 48 were lateral neck dissections. Sixty-five percent of patients were followed a minimum of 2 years and formed the cohort for final analysis. There were eight regional recurrences, three of which occurred in the contralateral, undissected neck. The regional recurrence rate for all patients undergoing selective neck dissection, with or without radiotherapy, according to pathologic N status was as follows: N0 (1/33), 3%; N1 (1/8), 12.5%; and multiple positive nodes (3/26), 11.5%. A comparison of recurrence rates with respect to extent of neck disease (N0–N1 versus multiple positive nodes) for both types of neck dissection did not demonstrate significant differences; supraomohyoid neck dissection, p < .5; lateral neck dissection, p < .25. Conclusions There exists an expanded role for selective neck dissection in selected patients with primary squamous cell carcinoma of the upper aerodigestive tract and multiple N+ cervical disease. The selection of patients who are candidates for selective lymphadenectomy should be based on pathoanatomic considerations with reference to the primary site of tumor and demonstrated level(s) of metastatic involvement. © 1997 John Wiley & Sons, Inc. Head Neck19: 260–265, 1997.

The purpose of this study was to give an overview on hereditary syndromes associated with head and neck paragangliomas (HNPGs).Our methods were the review and discussion of the pertinent literature.About one third of all patients with HNPGs are carriers of germline mutations. Hereditary HNPGs have been described in association with mutations of 10 different genes. Mutations of the genes succinate dehydrogenase subunit D (SDHD), succinate dehydrogenase complex assembly factor 2 gene (SDHAF2), succinate dehydrogenase subunit C (SDHC), and succinate dehydrogenase subunit B (SDHB) are the cause of paraganglioma syndromes (PGLs) 1, 2, 3, and 4. Succinate dehydrogenase subunit A (SDHA), von Hippel-Lindau (VHL), and transmembrane protein 127 (TMEM127) gene mutations also harbor the risk for HNPG development. HNPGs in patients with rearranged during transfection (RET), neurofibromatosis type 1 (NF1), and MYC-associated factor X (MAX) gene mutations have been described very infrequently.All patients with HNPGs should be offered a molecular genetic screening. This screening may usually be restricted to mutations of the genes SDHD, SDHB, and SDHC. Certain clinical parameters can help to set up the order in which those genes should be tested.

This paper was written by members of the International Head and Neck Scientific Group ( http://www.IHNSG.com ).

Item does not contain fulltext Although uncommon, cancer of an unknown primary (CUP) metastatic to cervical lymph nodes poses a range of dilemmas relating to optimal treatment. The ideal resolution would be a properly designed prospective randomized trial, but it is unlikely that this will ever be conducted in this group of patients. Accordingly, knowledge gained from retrospective studies and experience from treating patients with known head and neck primary tumors form the basis of therapeutic strategies in CUP. This review provides a critical appraisal of various treatment approaches described in the literature. Emerging treatment options for CUP with metastases to cervical lymph nodes are discussed in view of recent innovations in the field of head and neck oncology and suitable therapeutic strategies for particular clinical scenarios are presented. For pN1 or cN1 disease without extracapsular extension (ECE), selective neck dissection or radiotherapy offer high rates of regional control. For more advanced neck disease, intensive combined treatment is required, either a combination of neck dissection and radiotherapy, or initial (chemo)radiotherapy followed by neck dissection if a complete response is not recorded on imaging. Each of these approaches seems to be equally effective. Use of extensive bilateral neck/mucosal irradiation must be weighed against toxicity, availability of close follow-up with elective neck imaging and guided fine-needle aspiration biopsy (FNAB) when appropriate, the human papillomavirus (HPV) status of the tumor, and particularly against the distribution pattern (oropharynx in the majority of cases) and the emergence rate of hidden primary lesions (

Objectives: To categorize the clinical presentation of thyroid carcinoma in the pediatric and adolescent population and to present a philosophy of surgical management based on oncologic principles. Results: Over half these children with thyroid carcinoma (55%) presented with an asymptomatic thyroid mass. Twelve of 26 patients presented with lymphatic metastasis. There were no recurrences in any patient when the neck disease was treated with cervical lymphadenectomy or when the patient received postoperative radioablative therapy. Conclusions: The prognosis is excellent even with more extensive disease in children. Total thyroidectomy along with selective neck dissection for regional metastatic disease is recommended for thyroid carcinoma. This is followed by nuclear scintigraphy with subsequent radioablation for residual or recurrent disease and thyroid suppression and/or replacement. (Arch Otolaryngol Head Neck Surg. 1995;121:1261-1264)

Program Description: Over the past several years new technologies, potentially offering safer or less invasive techniques, have been applied to surgery of the thyroid and parathyroid glands. While these technologies may offer benefits to surgeons and patients, each has an associated learning curve and incremental costs compared to the standard techniques. The purpose of this miniseminar is to examine some of these “advances” (in-office ultrasound, laryngeal nerve monitoring, intra-operative parathyroid hormone measurement, minimally invasive and alternate surgical approaches) and to evaluate the benefits and costs and provide guidance for when these technologies should properly be used. Additionally, guidance for how general otolaryngologists can learn these techniques and how these technologies relate to the concept of “standard-of-care” will be addressed. Audience participation will be used to solicit information about usage of these technologies among the membership and to obtain audience opinion to he...

Item does not contain fulltext Merkel cell carcinomas are uncommon, but aggressive, cutaneous malignancies of neuroendocrine differentiation. To the pathologist, these lesions appear as sheets of undifferentiated tumor cells with little cytoplasm and dense nuclear chromatin. They are members of the group of "small round blue cell tumors," which includes small cell carcinomas of the lung, lymphomas, and neuroblastomas. Analogous to other skin malignancies, Merkel cell carcinomas frequently arise in the head and neck region and are commonly found in the elderly population. Merkel cell carcinomas have a high propensity for regional and distant metastases, and recurrences are frequently seen. Surgical excision is the recommended first-line treatment followed by adjuvant radiation therapy. Because of the high incidence of occult regional metastasis, patients with clinical and radioghaphically negative necks should undergo elective dissection, irradiation, or preferably sentinel lymph node biopsy. (c) 2011 Wiley Periodicals, Inc. Head Neck, 2012. 01 september 2012

Treatment results for malignant skull base lesions may be improved with combined modality therapy. Using a novel drug infusion technique that capitalizes on the pharmacodynamic cisplatin-neutralizing properties of thiosulfate, 14 patients (6 untreated, 8 recurrent) received cisplatin (120 to 200 mg/m2 for 1 to 4 weeks × 2-4 cycles) as part of a multimodality treatment program. Histology included squamous cell carcinoma, 11 patients (8 upper aerodigestive tract, 3 cutaneous); sarcoma, 2 patients (malignant fibrous histiocytoma, synovial cell sarcoma); and salivary gland cancer, 1 patient. The lesions involved the lateral skull base (12 patients) and the anterior (2 patients). Dose intensities for cisplatin were between 33.3 and 200 mg/m2/wk. Major responses occurred in 9/14 patients (64.3%), 3 of whom had complete responses. Drug toxicity, occurring in 11 patients, was mild and there were no significant complications as a result of the infusions. Eleven patients subsequently underwent surgical resections. The mean survival time is 23.3 months. Eight patients are alive without disease, 2 alive with disease, and 4 are dead of disease. The effectiveness of this highly selective supradose cisplatin infusion technique and its low morbidity support continued investigations of its application to patients with cranial base malignancies.

Incidental lesions of the thyroid gland are an increasing problem facing clinicians. While asymptomatic palpable lesions are detected in only 4-7% of the population, currently available imaging modalities are sensitive enough to detect lesions in 20-30% of the population. Guidelines for managing these incidentalomas are limited, largely due to lack of well-powered prospective studies. This review will address the currently available data on thyroid incidentalomas, detected through clinical examination, cross-sectional imaging, ultrasound, and PET scans. We will focus on the modalities of detection and risk of malignancy, further investigation and management options and the deficiencies therein. We propose a pragmatic algorithm when faced with this clinical dilemma under differing circumstances.

Background. Parathyroid carcinoma is a rare entity, comprising fewer than 1% of cases of hyperparathyroidism. The disease generally presents with severe hyperparathyroidism and occasionally with vocal cord paralysis or a firm palpable cervical mass. Methods. The classic histopathologic features of trabecular pattern, mitotic figures, thick fibrous bands, and capsular and vascular invasion are not present in every case but are useful in assessing whether a particular hyperfunctional parathyroid lesion is a carcinoma. Nevertheless, recognition of malignancy at initial operation may be difficult. Management primarily involves complete surgical removal through en bloc resection, which may include adjacent central neck structures. Results. Adjuvant radiation therapy has not been proven to be uniformly effective, but several series show results suggestive of a possible survival advantage. Chemotherapy, genetic, and other biomodifying agents remain experimental. Long-term outcome for this neoplasm remains problematic, and complications from intractable hypercalcemia constitute the foremost cause of death. Reported 5-year survivals have ranged between 70% and 85%. Conclusions. Initial surgical success offers the greatest opportunity for cure. Reoperation for recurrence offers the potential for short- and intermediate-term relief from the sequelae of hypercalcemia, but rarely results in cure. Severely elevated calcium levels should be controlled before initial surgery, and much of the treatment for recurrent or persistent disease, including reoperative surgery, is directed at control of hypercalcemia. © 2010 Wiley Periodicals, Inc. Head Neck, 2010