Paraganglioma yugular bilateral: reporte de un caso y revisión de la literatura / Bilateral jugular paraganglioma: case report and review of the literature

Resumen El paraganglioma (PG) es una neoplasia infrecuente originada de las células paraganglionares, embriológicamente derivadas de la cresta neural. Se localizan en la cabeza, base de cráneo, cuello, mediastino, abdomen y pelvis. La mayor parte de los PG muestran un curso clínico benigno, sin embargo, algunos casos pueden mostrar un comportamiento biológico agresivo con invasión local y metástasis a distancia. Un avance significativo en patología molecular ha sido el reconocimiento que el 30%-40% de estas neoplasias presentan alteraciones genéticas. Se han descrito más de 45 genes involucrados, incluyendo mutaciones de la línea germinal succinato deshidrogenasa. Actualmente se recomienda hacer test genético a todos los portadores de PG incluyendo los de presentación esporádica. El PG más frecuente se ubica en la glándula suprarrenal llamado feocromocitoma. El diagnóstico definitivo se realiza con histología, sin embargo, el estudio imagenológico puede entregar una aproximación diagnóstica certera. Debido a la aceptación actual que todos los PG tienen potencial metastásico, el concepto de PG benigno y maligno ha cambiado a uno de estimación de riesgo de metástasis, aunque no existe un esquema único aceptado para tal efecto. El tratamiento considera la cirugía, la radioterapia, la observación y terapias combinadas. Dado el lento crecimiento de este tipo de neoplasia y las potenciales complicaciones de la terapia quirúrgica, la observación es una opción especialmente para pacientes añosos dejando las otras opciones para pacientes más jóvenes. En este trabajo se presenta un caso de paraganglioma yugular bilateral gigante tratado con radioterapia de intensidad modulada incluyendo una revisión bibliográfica pertinente.

Abstract Paraganglioma (PG) is a rare neoplasm derived from paraganglionic cells of the neural crest. They are located in the head, skull base, neck, mediastinum, abdomen and pelvis. Most PGs show a benign clinical course, however, some cases may show aggressive biological behavior with local invasion and distant metastasis. A significant advance in molecular pathology has been the recognition that 30%-40% of these neoplasms present genetic alterations; more than 45 genes have been described, including mutations of the germline succinate dehydrogenase. Currently it is recommended to make genetic test to all patients with PG, including sporadic presentation. The most frequent PG is located in the adrenal gland called pheochromocytoma. The definitive diagnosis is made with histology; however, the imaging study can provide an accurate diagnostic approach. It is now accepted that all PG have a metastatic potential, therefore the concept of benign or malignant has been changed to a metastasis risk stratification approach however no single scheme is been widely used. The treatment considers surgery, radiotherapy, observation and combination therapies. Given the slow growth of this type of neoplasia and the potential complications of surgical therapy, observation is an option especially for elderly patients leaving the other options for younger patients. In this work we present a case of giant bilateral jugular paraganglioma treated with intensity modulated radiation therapy, including a pertinent literature review.

Surgical management of glomus jugulare tumors: a proposal for approach selection based on tumor relationships with the facial nerve.

OBJECT: The goal of this paper is to analyze the extension and relationships of glomus jugulare tumor with the temporal bone and the results of its surgical treatment aiming at preservation of the facial nerve. Based on the tumor extension and its relationships with the facial nerve, new criteria to be used in the selection of different surgical approaches are proposed. METHODS: Between December 1997 and December 2007, 34 patients (22 female and 12 male) with glomus jugulare tumors were treated. Their mean age was 48 years. The mean follow-up was 52.5 months. Clinical findings included hearing loss in 88%, swallowing disturbance in 50%, and facial nerve palsy in 41%. Magnetic resonance imaging demonstrated a mass in the jugular foramen in all cases, a mass in the middle ear in 97%, a cervical mass in 85%, and an intradural mass in 41%. The tumor was supplied by the external carotid artery in all cases, the internal carotid artery in 44%, and the vertebral artery in 32%. Preoperative embolization was performed in 15 cases. The approach was tailored to each patient, and 4 types of approaches were designed. The infralabyrinthine retrofacial approach (Type A) was used in 32.5%; infralabyrinthine pre- and retrofacial approach without occlusion of the external acoustic meatus (Type B) in 20.5%; infralabyrinthine pre- and retrofacial approach with occlusion of the external acoustic meatus (Type C) in 41%; and the infralabyrinthine approach with transposition of the facial nerve and removal of the middle ear structures (Type D) in 6% of the patients. RESULTS: Radical removal was achieved in 91% of the cases and partial removal in 9%. Among 20 patients without preoperative facial nerve dysfunction, the nerve was kept in anatomical position in 19 (95%), and facial nerve function was normal during the immediate postoperative period in 17 (85%). Six patients (17.6%) had a new lower cranial nerve deficit, but recovery of swallowing function was adequate in all cases. Voice disturbance remained in all 6 cases. Cerebrospinal fluid leakage occurred in 6 patients (17.6%), with no need for reoperation in any of them. One patient died in the postoperative period due to pulmonary complications. The global recovery, based on the Karnofsky Performance Scale (KPS), was 100% in 15% of the patients, 90% in 45%, 80% in 33%, and 70% in 6%. CONCLUSIONS: Radical removal of glomus jugulare tumor can be achieved without anterior transposition of the facial nerve. The extension of dissection, however, should be tailored to each case based on tumor blood supply, preoperative symptoms, and tumor extension. The operative field provided by the retrofacial infralabyrinthine approach, or the pre- and retrofacial approaches, with or without closure of the external acoustic meatus, allows a wide exposure of the jugular foramen area. Global functional recovery based on the KPS is acceptable in 94% of the patients.

Jugular foramen tumors: diagnosis and treatment.

OBJECT: Jugular foramen tumors are rare skull base lesions that present diagnostic and complex management problems. The purpose of this study was to evaluate a series of patients with jugular foramen tumors who were surgically treated in the past 16 years, and to analyze the surgical technique, complications, and outcomes. METHODS: The authors retrospectively studied 102 patients with jugular foramen tumors treated between January 1987 and May 2004. All patients underwent surgery with a multidisciplinary method combining neurosurgical and ear, nose, and throat techniques. Preoperative embolization was performed for paragangliomas and other highly vascularized lesions. To avoid postoperative cerebrospinal fluid (CSF) leakage and to improve cosmetic results, the surgical defect was reconstructed with specially developed vascularized flaps (temporalis fascia, cervical fascia, sternocleidomastoid muscle, and temporalis muscle). A saphenous graft bypass was used in two patients with tumor infiltrating the internal carotid artery (ICA). Facial nerve reconstruction was performed with grafts of the great auricular nerve or with 12th/seventh cranial nerve anastomosis. Residual malignant and invasive tumors were irradiated after partial removal. The most common tumor was paraganglioma (58 cases), followed by schwannomas (17 cases) and meningiomas (10 cases). Complete excision was possible in 45 patients (77.5%) with paragangliomas and in all patients with schwannomas. The most frequent and also the most dangerous surgical complication was lower cranial nerve deficit. This deficit occurred in 10 patients (10%), but it was transient in four cases. Postoperative facial and cochlear nerve paralysis occurred in eight patients (8%); spontaneous recovery occurred in three of them. In the remaining five patients the facial nerve was reconstructed using great auricular nerve grafts (three cases), sural nerve graft (one case), and hypoglossal/facial nerve anastomosis (one case). Four patients (4%) experienced postoperative CSF leakage, and four (4.2%) died after surgery. Two of them died of aspiration pneumonia complicated with septicemia. Of the remaining two, one died of pulmonary embolism and the other of cerebral hypoxia caused by a large cervical hematoma that led to tracheal deviation. CONCLUSIONS: Paragangliomas are the most common tumors of the jugular foramen region. Surgical management of jugular foramen tumors is complex and difficult. Radical removal of benign jugular foramen tumors is the treatment of choice, may be curative, and is achieved with low mortality and morbidity rates. Larger lesions can be radically excised in one surgical procedure by using a multidisciplinary approach. Reconstruction of the skull base with vascularized myofascial flaps reduces postoperative CSF leaks. Postoperative lower cranial nerves deficits are the most dangerous complication.

[Glomus tumors of the temporal bone: a report of 6 cases]. / Tumores glômicos do osso temporal. Relato de 6 casos.

The authors review the clinical, radiological and pathological features of 6 cases of glomus tumors of the temporal bone. Out of the 6 patients, 5 were female; age was distributed between 22 and 76 years (mean 48 years). The main clinical features were hypoacusia, tinnitus and otoscopic findings suggestive of the diagnosis. In one case was noted the concomitant presence of a neurinoma of the VIII cranial nerve with a ipsilateral glomus tumor, and in another case there was a concomitancy of carotid body tumor with temporal glomus jugularis tumor. Metastases were not observed in any case. Tumoral lesions were successfully ressected employing microsurgical techniques and a multidisciplinary staff involving neurosurgeons, head and neck surgeons and otolaryngologists. Radioteraphy was not employed, neither pre-operative embolization. Some aspects related to the nosology, embriology, pathophysiology, diagnosis and treatment of this interesting type of neoplasms are discussed.

Tumores glômicos do osso temporal: relato de 6 casos / Glomic tumors of the temporal bone: report of 6 cases

Os autores apresentam os achados clínicos, radiológicos e anátomo-patológico de 6 casos de tumor glômico de osso temporal. Cinco pacientes eram do sexo feminino; a idade variava de 22 a 76 anos (média 48 anos). As manifestaçöes clínicas predominantes foram hipoacusia, zumbido e achados otoscópicos sugestivos do diagnóstico. Säo revistos e discutidos vários aspectos relacionados `a origem e fisiopatogênese dos tumores glômicos, assim como seu diagnóstico e tratamento

[Jugular chemodectoma with Garcin syndrome]. / Quemodectoma yugular con sindrome de Garcin.

The authors present an exceptional case of chemodectoma jugulare with important invasion of the skull base involving all the cranial nerves on one side and conforming Garcin's syndrome. Practically all the possible ways of spreading were followed by the tumor, with involvement of the posterior, middle and anterior fossas of the skull base and also orbit, middle ear and neck. The clinical and histopathological difficulties in the diagnosis, especially when the chemodectoma has only neurological signs, are stressed. The association of otologic symptoms makes the diagnosis easier. The patient had an early onset of the symptoms and a rapid evolution considering this type of tumors. The appearance of goitre in this patient is a sign of endocrine disturbance, which is occasionally associated with these tumors. Apparently this chemodectoma was not a secreting tumor, since the blood pressure values were normal. No signs of multicentricity or metastatic growths were detected. The radiological studies, and especially computerized tomography scanning, gave precise information about the spreading of the tumor. The authors consider that the knowledge of this entity is important in order to arrive at a correct diagnosis.