All forms of HNPGLs should be managed by a multidisciplinary team (MDT) of core members from skull base otolaryngology, head and neck surgery, clinical genetics, oncology, endocrinology, vascular surgery, radiology and pathology. Extended members include neurosurgery, endocrine surgery, nuclear medicine, speech and language therapy, audiology and dietetics. The surgical, radiologic and oncologic members may also work in the head and neck or skull base MDT, and in most cases, these MDTs will be responsible for the day-to-day management of cases. The PGL-specific MDT should meet at least every three months. Patients should have a clinical assessment with an otolaryngologist member of the MDT. They should have a contrast-enhanced MRI of the head and neck. In addition, temporal bone PGLs should have a CT scan. They should also have a whole-body MRI or gallium DOTATATE PET CT. Plasma metanephrine levels should be measured and, if positive, require long-term endocrine input. Genetic testing is recommended including offering cascade genetic testing to at-risk family members. There should be shared decision-making between patient and clinician. Management options include active surveillance with serial imaging +/- plasma metanephrines, surgery, or radiotherapy. Only about half of HNPGLs show growth following diagnosis. Growth rate is low, and it can be difficult to identify small changes year to year. Therefore, most patients should undergo a prolonged period of surveillance to determine tumour behaviour, except in cases where early treatment is appropriate, which include tympanic PGLs, jugular PGLs with troublesome conductive hearing loss, pulsatile tinnitus or impending/actual facial weakness, significant brain stem compression, secretory tumours, rapid growth, malignancy, and patient choice. Early complete surgical excision is the treatment of choice for tympanic PGLs. Large tympanic PGLs may involve the jugular bulb and be managed as jugular PGL. The majority of large or growing jugular PGLs should be considered for radiotherapy; ideally intensity-modulated radiotherapy or stereotactic radiotherapy. Surgery for jugular PGLs would be for symptoms that would not be adequately treated with radiotherapy, or as salvage treatment. Preoperative embolisation is recommended. For vagal PGLs, radiotherapy is the mainstay treatment for small, growing tumours in the presence of functioning vagal nerve. It is first line for cases of contralateral vagal palsy. Rapidly growing large PGLs, and those associated with SDHB mutation may be better treated with surgical resection to minimise future metastases. Carotid PGLs are treated conservatively initially, with active treatment considered if it grows, behaves aggressively, is secretory, or if cranial nerve function is lost. Radiotherapy is favoured in older patients with large tumours. Surgery is favoured in young patients with small tumours. Preoperative embolisation should be considered. Treatment options for metastatic disease include peptide therapy with octreotide or MIBG, radiotherapy, or surgery. There should be adequate posttreatment surveillance and rehabilitation and results audited.
British Skull Base Society Clinical Consensus Document on Management of Head and Neck Paragangliomas (HNPGLs)
Reviewed by Richard (Wei Chern) Gan
British Skull Base Society Clinical Concensus Document on Managament of Head and Neck Paragangliomas.
CONTRIBUTOR
Richard (Wei Chern) Gan
Royal Brisbane and Women's Hospital, Australia.
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