Consensus Statement and Guidelines on the Management of Paragangliomas of the Head and Neck

Paragangliomas (PGLs) of the head and neck (H&N) are uncommon tumors that either arise spontaneously or as part of an inherited syndrome. Hereditary PGL is an autosomal-dominant tumor predisposition syndrome in which an affected individual has greatly increased risk of developing PGL at any or several sites in the autonomic nervous system. The mode of inheritance for some is affected by maternal imprinting. These tumors are generally very slow growing, often taking a decade to double in size. A few are or can become malignant and can metastasize widely. Because of their site of origin, patients with these tumors may develop cranial nerve deficits that have a significant impact on their quality of life. Patients may present to specialists from widely differing disciplines, and some of these may not appreciate the full implications of their patient's disease. As a result, management can become fragmented or inappropriate, and some aspects of care may even be overlooked. This article is the distillation of consensus opinion derived from current published and unpublished data in this field, with particular reference to the management of temporal bone PGLs. We propose guidelines for the management of both sporadic and hereditary PGLs. A multidisciplinary team approach to the management of this complex disorder is advocated. Progress could be made by adopting these guidelines and by widespread dissemination of standardized information. Collaborative research should be promoted with the aim of harnessing advances in molecular genetics to develop targeted therapies for patients, particularly those with hereditary PGL.     

Paragangliomas Arising in the Head and Neck: A Morphologic Review and Genetic Update

Seventy percent of parasympathetic paragangliomas arise in the head and neck and are nonsecretory. Awareness of the differential diagnosis based on location, overlapping morphology, and immunohistochemical profiles aids in the correct diagnosis, particularly on limited tissue samples. Moreover, 30% to 40% of head and neck paragangliomas are known to be associated with hereditary syndromes, with the succinate dehydrogenase enzyme family comprising the most frequent association. The pathologist’s role is becoming increasing critical for facilitating optimal patient care beyond the initial tissue diagnosis of paraganglioma to include screening and documenting potential hereditary tumors requiring further patient counseling and testing.     

Graft Harvesting for Revascularization in the Head and Neck

The techniques for revascularization in the neurocranium, skull base, and neck continue to evolve at an exciting pace. In this body of literature, however, techniques for harvesting radial artery and saphenous vein grafts are mainly reported using traditional open techniques. Minimally invasive procedures are fast becoming an alternative to open techniques in many fields and have the potential to become the standard of care. The cardiovascular literature is replete with reports of endoscopically harvested vascular grafts. This article reviews both methods, since the current state of the art involves knowledge of open and endoscopic harvesting techniques.     

Sarcoidosis of the Head and Neck

Sarcoidosis is a complex disorder that often times involves the head and neck. Despite the presence of strong clinical evidence, tissue diagnosis and imaging is needed for confirmation of the disease. Although typically managed medically, when found in the sinonasal tract or intracranially, it may necessitate the intervention of a rhinologist-skull base surgeon. This article seeks to provide a comprehensive review of head and neck sarcoidosis, as this fascinating disorder often poses a diagnostic and therapeutic challenge. A brief discussion of surgical treatment for pituitary lesions is also provided. Articles from 1997 to 2013 were selected and reviewed by three researchers utilizing the most recent literature regarding sarcoidosis in the head and neck. PubMed searches were conducted using search terms such as “sarcoidosis”, “neurosarcoid”, and “extra-pulmonary sarcoid”, among many others. A large collection of articles was generated and reviewed by the team of authors, and appropriate information was extracted to compose a thorough and expansive review of the subject. 10–15 % of patients with sarcoidosis have head and neck manifestations. Sinonasal and pituitary sarcoidosis presents a diagnostic challenge owing to its non-specific symptoms. Although systemic steroid therapy is often the first time treatment, endoscopic surgery is commonly used to treat advanced pituitary sarcoidosis refractory to medical management. As tissue diagnosis and imaging is key, a multi-disciplinary team approach is advantageous. Our study collates the available literature on head and neck sarcoidosis to provide a comprehensive review of the subject. This provides helpful information to guide all practitioners involved in the care of these challenging patients, namely pathologists, radiologists, otolaryngologists, and skull base surgeons, in the workup and management of head and neck sarcoidosis.     

Neuroendocrine Neoplasms of the Head and Neck: Some Suggestions for the New WHO Classification of Head and Neck Tumors

As knowledge and understanding in pathology evolve, classifications and nomenclature also change to reflect those advances. The 2005 World Health Organization Classification of Head and Neck Tumours was a significant step towards diagnostic standardization of head and neck neuroendocrine carcinomas; however, in the last 10 years there have been new data supporting the recognition of “large cell neuroendocrine carcinoma” as a distinctive high grade carcinoma in the head and neck, a lesion not included in the 2005 Classification. In addition, the terms “middle ear adenoma” and “carcinoid tumor of middle ear” are still widely used to describe a neoplasm that is neither a pure adenoma nor a carcinoid tumor but a lesion with variable mixed exocrine and endocrine differentiation. Largely using the diagnostic criteria of the WHO classification of neuroendocrine carcinomas of the lung, we propose the terms “neuroendocrine carcinoma, grade 1”; “neuroendocrine carcinoma, grade 2”; “neuroendocrine carcinoma, grade 3, large cell type”; and “neuroendocrine carcinoma, grade 3, small cell type” for the classification of neuroendocrine carcinomas of the head and neck in a future WHO classification. In addition, we also proposed the term “mixed epithelial neuroendocrine tumor” of the middle ear as an alternative for “middle ear adenoma” and “carcinoid tumor of the middle ear”.     

Neck dissection for Head and Neck cancers: state of the art and classification

The purposes of this article are to review the history and evolution of neck dissections, including an update on node levels and their anatomical landmark. A number of classification systems were proposed and subsequently established for neck dissection procedures. The system most often employed was published in 1991 by the American Head and Neck Society and American Academy of Otolaryngology- Head and Neck Surgery and revised in 2002 and 2008. According to this classification, neck dissections are grouped into four broad categories: radical neck dissection (RND), modified radical neck dissection (MRND), selective neck dissection (SND) and extended neck dissection (ERND). The choice between different surgeries depends on type and site of head and neck tumor as well nodal involvement.     

Pulmonary Metastasectomy for Head and Neck Cancers

Background: Distant metastases from carcinomas that arise from the head and neck region are infrequent. The most common site is the lung. To evaluate the results of resection of pulmonary metastases for head and neck cancers, we reviewed our own cases of these metastases.Methods: Between November 1966 and March 1995, 83 patients with pulmonary metastases from head and neck cancers underwent 94 thoracic operations. All patients had obtained or had obtainable locoregional control of their primary head and neck cancers. Kaplan-Meier and Cox regression models were used to analyze the prognostic factors for survival after metastasectomy.Results: Median age was 53 years (range, 17–77). Fifty-nine were male and 24 were female. Forty-one patients had squamous cell cancers, and 36 had glandular tumors that consisted mostly of thyroid and adenoid cystic carcinomas. The median disease-free interval from the time of treatment of the head and neck primary cancers to the development of pulmonary metastases was 27 months. Sixty-eight (82%) patients had complete resection. Overall operative mortality rate was 2%. Overall actuarial survival rate after metastasectomy was 50% at 5 years. Patients with glandular tumors had a 5-year survival rate of 64% compared with 34% for patients with squamous cell cancers. When the patients with glandular tumors were analyzed according to their histology, patients with adenoid cystic carcinomas had an 84% 5-year survival, but none remained disease-free. Patients with thyroid cancers fared similarly whether they were treated medically or surgically. On multivariate analysis, the adverse prognostic factors for patients with squamous cell cancers were incomplete resection, age greater than 50 years, and disease-free interval less than or equal to 2 years.Conclusions: Approximately 30% of patients with pulmonary metastases from squamous cell cancers of the head and neck who underwent complete resection of all their metastases can expect to achieve long-term survival. The role of pulmonary resection for patients with glandular tumors is unclear.Presented in part at the 51st Annual Symposium Meeting of the Society of Surgical Oncology, San Diego, March 26–29, 1998.