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Paragangliomas Arising in the Head and Neck: A Morphologic Review and Genetic Update
Affiliation:1. Department of Pathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 085, Houston, TX 77030, USA;2. Clinical Cancer Genetics Program, The University of Texas MD Anderson Cancer Center, 1400 Hermann Pressler Drive, Unit 444, Houston, TX 77230, USA;1. Department of Cardiovascular Surgery, Beijing Anzhen Hospital, Capital Medical University, Beijing Institute of Heart, Lung and Blood Vessel Diseases, Beijing, China;2. Children''s Hospital and Institutes of Biomedical Sciences, Fudan University, Shanghai, China;1. Servicio de Bioquímica, Hospital de Jerez, Jerez, Cádiz, Spain;2. Servicio de Hematología y Hemoterapia, Hospital Clínico San Carlos, Madrid, Spain;1. Department of Neurosurgery, Emory University School of Medicine, Atlanta, Georgia, USA;2. Department of Surgery, Stony Brook University, Long Island, New York, USA;3. Department of Vascular Surgery, 251 Helenic Airforce Hospital, Athens, Greece;4. Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece;5. Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota, USA;6. Department of Neurosurgery, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, USA;7. Department of Neurosurgery, Virginia Commonwealth University, Richmond, Virginia, USA
Abstract: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.
Keywords:Paraganglioma  SDHB immunohistochemistry  Familial syndromes  Morphology  Head and neck neoplasms
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