Abstract
Treatment of jugulotympanic paragangliomas (JTPGLs) remains challenging with no clear guidelines for management or follow-up. The aim of this retrospective case-note study was to assess long-term results of operatively and conservatively managed JTPGLs between years 1974–2013. A total of 36 patients with JTPGLs were identified. Clinical characteristics and management outcomes of patients were reviewed. Data were extracted on demographics, symptoms, timing of diagnosis, tumor location and size, embolization, and management, including pre- and post-operative imaging, analysis of operative techniques, and follow-up. Pulsatile tinnitus and hearing loss were the most common presenting symptoms. Thirty-four (94 %) patients were treated with primary surgical therapy and two (6 %) with radiotherapy. The surgical approaches included endaural approach for Fisch Class A tumors and a variety of approaches for Fisch Class B-D tumors with an increasing predilection for function-preserving surgery. Eight (24 %) patients received subtotal resection. Five (15 %) patients had a local recurrence within 10 years after primary surgery. Two (6 %) patients suffered a permanent cranial nerve (CN) deficit after primary surgery. We advocate radical surgery when tumor resection is possible without compromising CNs. Function-preserving surgery with at least a 10-year follow-up for Fisch Class B–D tumors should be considered if CNs are in danger.
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References
Baysal BE (2002) Hereditary paraganglioma targets diverse paraganglia. J Med Genet 39:617–622
Martin TP, Irving RM, Maher ER (2007) The genetics of paragangliomas: a review. Clin Otolaryngol 32:7–11
Boedeker CC, Ridder GJ, Schipper J (2005) Paragangliomas of the head and neck: diagnosis and treatment. Fam Cancer 41:55–59
Fliedner SM, Lehnert H, Pacak K (2010) Metastatic paraganglioma. Semin Oncol 37:627–637
DeLellis R, Lloyd R, Heitz P (2004) World Health Organization classification of tumours. Iarc Press, Lyon
Künzel J, Iro H, Hornung J, Koch M, Brase C, Klautke G, Zenk J (2012) Function-preserving therapy for jugulotympanic paragangliomas: a retrospective analysis from 2000 to 2010. Laryngoscope 122:1545–1551
Fisch U (1988) Microsurgery of the skull base. Thieme, Stuttgart
Shin SH, Sivalingam S, De Donato G, Falcioni M, Piazza P, Sanna M (2012) Vertebral artery involvement by tympanojugular paragangliomas: management and outcomes with a proposed addition to the fisch classification. Audiol Neurootol 17:92–104
Charlson ME, Pompei P, Ales KL, MacKenzie CR (1987) A New method of classifying prognostic comorbidity in longitudinal studies: development and validation. J Chronic Dis 40:373–383
House JW, Brackmann DE (1985) Facial nerve grading system. Otolaryngol Head Neck Surg 93:146–147
Ahmad R, Sivalingam S, Konishi M, De Donato G, Sanna M (2013) Oncologic outcomes in surgical management of jugular paraganglioma and factors influencing outcomes. Head Neck 35:527–534
Cheesman AD, Kelly AM (2009) Rehabilitation after treatment for jugular foramen lesions. Skull Base 19:99–108
Willen SN, Einstein DB, Maciunas RJ, Megerian CA (2005) Treatment of glomus jugulare tumors in patients with advanced age: planned limited surgical resection followed by staged gamma knife radiosurgery: a preliminary report. Otol Neurotol 26:1229–1234
Cosetti M, Linstrom C, Alexiades G, Tessema B, Parisier S (2008) Glomus tumors in patients of advanced age: a conservative approach. Laryngoscope 118:270–274
Ivan ME, Sughrue ME, Clark AJ, Kane AJ, Aranda D, Barani IJ, Parsa AT (2011) A meta-analysis of tumor control rates and treatment-related morbidity for patients with glomus jugular tumours. J Neurosurg 114:1299–1305
Forbes JA, Brock AA, Ghiassi M, Thompson RC, Haynes DS, Tsai BS (2012) Jugulotympanic paragangliomas: 75 years of evolution in understanding. Neurosurg Focus 33:E13
Suárez C, Rodrigo JP, Bödeker CC, Llorente JL, Silver CE, Jansen JC, Takes RP, Strojan P, Pellitteri PK, Rinaldo A, Mendenhall WM, Ferlito A (2013) Jugular and vagal paragangliomas: systematic study of management with surgery and radiotherapy. Head Neck 35:1195–1204
Bacciu A, Medina M, Ait Mimoune H, D’Orazio F, Pasanisi E, Peretti G, Sanna M (2015) Lower cranial nerves function after surgical treatment of Fisch Class C and D tympanojugular paragangliomas. Eur Arch Otorhinolaryngol 272:311–319
Arístegui Ruiz MÁ, da Costa Belisario J, Martinez Guirado T, Scola Yurrita B (2015) Head and neck paragangliomas: Experience in 126 patients with 162 tumours. Acta Otorrinolaringol Esp. doi:10.1016/j.otorri.2014.11.002 [Epub ahead of print]
Wanna GB, Sweeney AD, Carlson ML, Latuska RF, Rivas A, Bennett ML, Netterville JL, Haynes DS (2014) Subtotal resection for management of large jugular paragangliomas with functional lower cranial nerves. Otolaryngol Head Neck Surg 151:991–995
Larouere MJ, Zappia JJ, Wilner HI, Graham MD, Lundy LB (1994) Selective embolization of glomus jugulare tumours. Skull Base Surg 4:21–25
Murphy TP, Brackmann DE (1989) Effects of preoperative embolization on glomus jugulare tumours. Laryngoscope 99:1244–1247
Boedeker CC (2011) Paragangliomas and paraganglioma syndromes. Laryngorhinootologie 90:S56–S82
Schipper J, Spetzger U, Tatagiba M, Rosahl S, Neumann HP, Boedeker CC, Maier W (2009) Juxtacondylar approach in temporal paraganglioma surgery: when and why? Skull Base 19:43–47
Lustiq LR, Jackler RK (1996) The variable relationship between the lower cranial nerves and jugular foramen tumors: implications for neural preservation. Am J Otol 17:658–668
Pensak ML, Jackler RK (1997) Removal of jugular foramen tumors: the fallopian bridge technique. Otolaryngol Head Neck Surg 117:586–591
Moore MG, Netterville JL, Mendenhall WM, Isaacson B, Nussenbaum B (2016) Head and neck paragangliomas: an update on evaluation and management. Otolaryngol Head Neck Surg 154:597–605
Jacker RK (2008) Atlas of skull base surgery and neurotology. Thieme, Stuttgart
Inserra MM, Pfister M, Jackler RK (2004) Anatomy involved in the jugular foramen approach for jugulotympanic paraganglioma resection. Neurosurg Focus 17:E6
Doty RL, Heidt JM, MacGillivray MR, Dsouza M, Tracey EH, Mirza N, Bigelow D (2016) Influences of age, tongue region, and chorda tympani nerve sectioning on signal detection measures of lingual taste sensitivity. Physiol Behav 155:202–207
Smee RI, Jayasekara J, Williams JR, Hanna C (2015) Paragangliomas: presentation and management by radiotherapy at the Prince of Wales Hospital. J Med Imaging Radiat Oncol 59:229–235
Spector GJ, Compagno J, Perez CA, Maisel RH, Ogura JH (1975) Glomus jugulare tumors: effects of radiotherapy. Cancer 35:1316–1321
Vanmiert PJ (1964) The treatment of chemodectomas by radiotherapy. Proc R Soc Med 57:946–951
Hu K, Persky MS (2003) The multidisciplinary management of paragangliomas of the head and neck, Part 2. Oncology (Williston Park) 17:1143–1153
Powell S, Peters N, Harmer C (1992) Chemodectoma of the head and neck: results of treatment in 84 patients. Int J Radiat Oncol Biol Phys 22:919–924
Dawes PJ, Fillippou M, Welch AR, Dawes JD (1987) The management of glomus jugulare tumours. Clin Otolaryngol 12:15–24
Lybeert ML, van Andel JG, Eijkenboom WM, de Jong PC, Knegt P (1984) Radiotherapy of paragangliomas. Clin Otolaryngol 9:105–109
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Nicoli, T.K., Sinkkonen, S.T., Anttila, T. et al. Jugulotympanic paragangliomas in southern Finland: a 40-year experience suggests individualized surgical management. Eur Arch Otorhinolaryngol 274, 389–397 (2017). https://doi.org/10.1007/s00405-016-4184-1
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DOI: https://doi.org/10.1007/s00405-016-4184-1