[Head and neck paragangliomas: revision of 89 cases in 73 patients]. / Paragangliomas de cabeza y cuello: revisión de 89 casos en 73 pacientes.

INTRODUCTION: Paragangliomas (PGL) are uncommon neuroectodermal tumours. PGL are usually clinically benign tumours, although metastasis has been reported and invasive growth can occur in adjacent tissues (<10 %). Mutations in SDHB, SDHC, and SDHD, which encode sub-units of mitochondrial complex II (succinate dehydrogenase), play an important role in the pathogenesis of these tumours. MATERIAL AND METHOD: Retrospective review of 73 patients with 89 paragangliomas who had undergone resection of the PGL in our hospital. There were 8 patients who displayed multiple PGL. PGL were distributed as follows: 33 were jugular, 17 tympanic, 26 carotid body tumours, and 13 vagal paragangliomas. All these patients had a follow-up time of at least a year. The surgical approach was evaluated in terms of tumour origin, sequelae, and subsequent evolution, as well as the relapses and their relation with location of the primary tumour. RESULTS: The treatment was surgical, using complementary radiosurgery in just 1 patient. The type A infratemporal fossa approach was used in jugular paragangliomas, the approach was cervical in the carotid and vagal ones and, in the tympanics, a transmeatal or transmastoid approach was performed. In the 73 patients making up our study group, there were 11 recurrences which appeared in jugular paragangliomas (two of them in multiple PGL cases). The post-operative sequelae were mainly cranial nerve paralysis (VII, IX, X, XI, and XII), along with cerebrospinal fluid fistulas in 14 of the jugular PGLs. CONCLUSIONS: With this article we try to reflect our experience in the treatment of this type of tumour. Surgical treatment achieves excellent control of the disease with an acceptable morbidity in young or middle-aged patients. In order to diminish the probabilities of facial nerve paralysis in jugular PGL we must avoid the facial nerve transposition in the infratemporal approach.

Paragangliomas de cabeza y cuello: revisión de 89 casos en 73 pacientes / Head and neck paragangliomas: revision of 89 cases in 73 patients

Introducción: Los paragangliomas son tumores poco frecuentes de origen neuroectodérmico. Se los considera tumores benignos, pero en algunas ocasiones tienen un comportamiento biológico similar a los tumores malignos (< 10 %). Las mutaciones germinales en los genes SDHB, SDHC y SDHD, que codifican las subunidades del mismo nombre en el complejo enzimático mitocondrial de la succinato deshidrogenasa, tienen un papel importante en la patogenia. Material y método: Se trata de un estudio retrospectivo en el que se revisa a 73 pacientes intervenidos en nuestro servicio con un total de 89 paragangliomas, ya que 8 pacientes presentaban paragangliomas múltiples. Los paragangliomas se distribuyeron de la siguiente forma: 33 yugulares, 17 timpánicos, 26 carotídeos y 13 vagales. Todos estos pacientes tuvieron un seguimiento mínimo de 1 año. Se evaluaron las vías de abordaje en función de la localización tumoral, las secuelas acaecidas y su ulterior evolución, así como las recurrencias y su relación con la localización del tumor primario. Resultados: El tratamiento fue quirúrgico, utilizando la radiocirugía como tratamiento complementario en un paciente. En los paragangliomas yugulares se realizó un abordaje infratemporal tipo A, en los carotídeos y vagales el abordaje fue cervical y en los timpánicos, transmeatal o transmastoideo. De los 73 pacientes con paragangliomas intervenidos que componen nuestra población en estudio, hubo 11 recurrencias, que aparecieron en los paragangliomas yugulares, que en 2 casos fueron paragangliomas múltiples. Las secuelas postoperatorias fueron sobre todo la parálisis de nervios craneales (VII, IX, X, XI y XII), junto con las fístulas de líquido cefalorraquídeo en el 14 % de los paragangliomas yugulares. Conclusiones: Con este artículo pretendemos reflejar nuestra experiencia en el tratamiento de este tipo de tumores. El tratamiento quirúrgico consigue un excelente control de la enfermedad con una morbilidad aceptable en pacientes de mediana edad o jóvenes. Para disminuir las probabilidades de parálisis facial en los paragangliomas yugulares, debe evitarse la transposición del facial en el abordaje infratemporal de la fosa yugular

Introduction: Paragangliomas (PGL) are uncommon neuroectodermal tumours. PGL are usually clinically benign tumours, although metastasis has been reported and invasive growth can occur in adjacent tissues (<10 %). Mutations in SDHB, SDHC, and SDHD, which encode sub-units of mitochondrial complex II (succinate dehydrogenase), play an important role in the pathogenesis of these tumours. Material and method: Retrospective review of 73 patients with 89 paragangliomas who had undergone resection of the PGL in our hospital. There were 8 patients who displayed multiple PGL. PGL were distributed as follows: 33 were jugular, 17 tympanic, 26 carotid body tumours, and 13 vagal paragangliomas. All these patients had a follow-up time of at least a year. The surgical approach was evaluated in terms of tumour origin, sequelae, and subsequent evolution, as well as the relapses and their relation with location of the primary tumour. Results: The treatment was surgical, using complementary radiosurgery in just 1 patient. The type A infratemporal fossa approach was used in jugular paragangliomas, the approach was cervical in the carotid and vagal ones and, in the tympanics, a transmeatal or transmastoid approach was performed. In the 73 patients making up our study group, there were 11 recurrences which appeared in jugular paragangliomas (two of them in multiple PGL cases). The post-operative sequelae were mainly cranial nerve paralysis (VII, IX, X, XI, and XII), along with cerebrospinal fluid fistulas in 14 of the jugular PGLs. Conclusions: With this article we try to reflect our experience in the treatment of this type of tumour. Surgical treatment achieves excellent control of the disease with an acceptable morbidity in young or middle-aged patients. In order to diminish the probabilities of facial nerve paralysis in jugular PGL we must avoid the facial nerve transposition in the infratemporal approach