Results of a systematic literature review of treatment modalities for jugulotympanic paraganglioma, stratified per Fisch class.

OBJECTIVE: Key for successful jugulotympanic paraganglioma management is a personalised approach aiming for the best practice for each individual patient. To this end, a systematic review is performed, evaluating the local control and complication rates for the different treatment modalities stratified by the broadly accepted Fisch classification. DESIGN: A systematic literature review according to the PRISMA statement was performed. A detailed overview of individual treatment outcomes per Fisch class is provided. MAIN OUTCOME MEASURES: Local control, cranial nerve damage, complications, function recovery. RESULTS: Eighteen studies were selected, resembling 83 patients treated with radiotherapy and 299 with surgery. Excellent local control was found post-surgery for class A and B tumours, and risk of cranial nerve damage was <1%. For class C1-4 tumours, local control was 80%-95% post-surgery (84% post-radiotherapy), and cranial nerve damage was found in 71%-76% (none post-radiotherapy; P < .05). There was no difference in treatment outcomes between tumours of different C class. For class C1-4De/Di tumours, local control was 38%-86% (98% post-radiotherapy; P < .05) and cranial nerve damage/complication rates were 67%-100% (3% post-radiotherapy; P < .05). C1-4DeDi tumours showed lesser local control and cranial nerve damage rates when compared to C1-4De tumours. CONCLUSIONS: An individual risk is constituted for surgery and radiotherapy, stratified per Fisch class. For class A and B tumours, surgery is a suitable treatment option. For class C and D tumours, radiotherapy results in lower complication rates and similar or better local control rates when compared to the surgical group.

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

Glomus tumors: therapeutic role of selective embolization.

The primary goals of preoperative embolization of glomus tumors are to reduce the blood loss in the surgical field, minimize the risk of operative complications, and prevent recurrence by contributing to complete resection. Obliteration of a vascular channel may provide additional help in symptomatic relief by decreasing the tumor volume. Selective transarterial embolotherapy outcomes of 17 patients, most of whom had inoperable tumors and were unable to have surgery/radiotherapy because of poor general health, were evaluated to investigate the therapeutic contributions, efficacy, and safety of embolization techniques in the treatment of different glomus tumors and to examine the role of the embolotherapy in the treatment algorithm of such lesions. The pattern of vascular supply of these tumors was also documented. Eleven glomus jugulotympanicum, 4 glomus caroticum, and 2 glomus vagale tumors were embolized for palliative or curative purposes between 1992 and 2000. Coils and combination of coils plus polyvinyl alcohol were used for embolization. Relief of symptoms and patient satisfaction were analyzed within 3 months after the intervention by a questionnaire combined with full ear, nose, and throat and neurological examinations. The results indicate that the use of embolotherapy in the treatment of glomus tumors must be basically preoperative but is not curative.