title: What happens in vagus: a case of recurrent paraganglioma with malignant transformation and an updated treatment algorithm†.
abstract: Paragangliomas (PGLs) are rare, extra-adrenal tumors, originating from neural crest cells and can occur anywhere from the skull base to the pelvic floor. Although these tumors are often benign, a fraction of malignant cases exist. Few isolated cases of malignant head and neck PGL are reported in the literature. Treatment algorithms rely heavily on retrospective case studies and institutional experience. We report an unusual case of an extensive, hereditary PGL, with invasive characteristics, that was refractory to radiation therapy. An operative approach was selected for recurrent disease in the setting of critical neurovascular structure compromise. Six months postoperatively, the patient was recovering as expected and had no evidence of recurrent disease. We propose a modified treatment algorithm based on an updated literature review that encompasses the spectrum of PGL, from benign and asymptomatic to invasive and malignant disease.
authors: Department of Internal Medicine, Mayo Clinic, Scottsdale, AZ, USA ertz-archambault.natalie@mayo.edu.
authors: Natalie M
authors: NM
authors: Ertz-Archambault
authors: Department of Neurologic Surgery, Mayo Clinic, Rochester, MN, USA Department of Otorhinolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, MN, USA.
authors: Jamie J
authors: JJ
authors: Van Gompel
authors: Department of Otorhinolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, MN, USA.
authors: Brian A
authors: BA
authors: Neff
authors: Department of Otorhinolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, MN, USA Department of Anatomy, Mayo Clinic, Rochester, MN, USA.
authors: Jan L
authors: JL
authors: Kasperbauer
authors: Division of Cardiovascular Diseases, Mayo Clinic, Scottsdale, AZ, USA.
authors: Fadi E
authors: FE
authors: Shamoun