Cross-Sectional Imaging and Thoracoscopic Resection of Functional Intrathoracic Paraganglioma of the Aortic Body.

Paragangliomas are extra-adrenal tumors that are derived from neuroendocrine chromaffin cells. The rare disease has a variable presentation depending upon its anatomic location and functionality. We describe the case of a 56-year-old female patient who had an incidental mass found on imaging. The patient underwent biochemical testing and a mediastinal biopsy due to the nonspecific imaging findings. The mediastinal mass was resected through hemisternotomy and found to be a functional paraganglioma. This case highlights the importance of radiologists to consider the prospect of paragangliomas in the differential diagnosis of an intra-thoracic mass.

Autonomic control of the cardiovascular system in the cat during hypoxemia.

This study aimed to determine the roles played by the autonomic interoreceptors, the carotid bodies (cbs) and the aortic bodies (abs) in anesthetized, paralyzed, artificially ventilated cats' response to systemic hypoxemia. Four 15min challenges stimulated each of 15 animals: (1) hypoxic hypoxia (10%O2 in N2; HH) in the intact (int) cat where both abs and cbs sent neural traffic to the nucleus tractus solitarius (NTS); (2) carbon monoxide hypoxia (30%O2 in N2 with the addition of CO; COH) in the intact cat where only the abs sent neural traffic to the NTS; (3) HH in the cat after transection of both aortic depressor nerves, resecting the aortic bodies (HHabr), where only the cbs sent neural traffic to the NTS; (4) COH to the abr cat where neither abs nor cbs sent neural traffic to the NTS. Cardiac output (C.O.), contractility (dP/dt(MAX)), systolic/diastolic pressures, aortic blood pressure, total peripheral resistance, pulmonary arterial pressure, and pulmonary vascular resistance (PVR) were measured. When both cbs and abs were active the maximum increases were observed except for PVR which decreased. Some variables showed the cbs to have a greater effect than the abs. The abs proved to be important during some challenges for maintaining blood pressure. The data support the critically important role for the chemoreceptor-sympathetic nervous system connection during hypoxemia for maintaining viable homeostasis, with some differences between the cbs and the abs.

Aortic paraganglioma requiring resection and replacement of the aortic root.

This case report demonstrates the resection of an aortic root paraganglioma incidentally discovered in a 32-year-old male with replacement of the root for an oncologically complete resection.

Cervical paragangliomas: neurovascular surgical risk and therapeutic management.

Paragangliomas of the neck region arise most commonly in the carotid and vagal bodies. The goal of this retrospective study is to evaluate intraoperative vascular and neurological morbidity and to define the therapeutic strategy. During the period 1990-2004, 32 patients with 42 neck paragangliomas were referred to our institution (Head and Neck Service, Otolaryngology Federation, CHU La Timone, Marseilles, France). There were 29 carotid body tumors and 11 vagal body tumors. There were 14 men and 18 women. Forty paragangliomas were surgically excised. Mean age of patients with family history of paragangliomas was 34 years and that of patients without any familial history was 47 years. Only one patient had a malignant paraganglioma. A vascular repair procedure was performed in 10% and always occurred in carotid body tumors including the malignant one. Postoperative hypoglossal nerve deficit was reported in five cases (12.5%). Paralysis of vagus nerve was reported in 11 cases (27.5%), nine of whom were patients with vagal body tumors. Knowledge of number of paragangliomas and their location is of main importance and influences the therapeutic strategy. The goal of this strategy is to avoid major neurovascular morbidity and to optimize treatment of multiple or bilateral tumors. Early management of patients prevents progressive neurological deficit due to an enlarging tumor mass and minimizes neurovascular complications.

A glomus vagale tumour presenting with acute left ventricular failure.

A glomus vagale is a rare lesion of paraganglionic cells in the vagal perineurium. Secretion of hormones by such a tumour is exceedingly uncommon, although associated metabolic processes have been demonstrated. Clinical effects of secretion reported previously have been minimal. We report a case where resistant hypertension was eventually found to be due to a secretory glomus vagale, and was greatly alleviated by removal of the lesion.

Differential increases in P2X receptor levels in rat vagal efferent neurones following a vagal nerve section.

Extracellular ATP can influence cells via activation of P2X purinoceptors, the distribution of which can be altered in the central and peripheral nervous systems following injury or tissue damage. Here we have investigated the effect of a unilateral section of the cervical vagus nerve on the distribution of P2X(1), P2X(2), P2X(3), P2X(4) and P2X(7) receptor subunit immunoreactivity (R-IR) in the dorsal vagal motor nucleus (DVN) and the nucleus ambiguus (NA) in the medulla oblongata. As early as 2 days, and followed up to 14 days, there was a dramatic ipsilateral increase in P2X(1), P2X(2) and P2X(4)R-IR in the cell soma of vagal efferent neurones in the DVN following the nerve section, but not the NA. There were no changes in P2X(3) and P2X(7)R-IR in either nuclei. To test for possible functional consequences of increased P2X receptor levels, whole-cell patch-clamp recordings were made from DVN cells in brainstem slices 4 days following unilateral vagotomy. Application of ATP revealed large cell-to-cell variance in the current amplitude in neurones from both sectioned and control DVN. However, when ATP responses were compared to those elicited by the nicotinic acetylcholine receptor agonist carbachol, the mean ratio of the peak ATP-evoked current to the peak carbachol-evoked current was significantly larger in DVN neurones ipsilateral to the section. Thus the increase in P2XR levels in DVN cells ipsilateral to a nerve section are likely to reflect an increase in expression of functional P2XRs on the cell surface.

[Glomus vagale tumor--diagnosis and treatment]. / Glomus vagale--diagnostyka i leczenie.

Paraganglioma, being tumors arising from the paraganglion cells of the parasympathetic system, were first reported in man in 1935. Within, the head and neck glomus vagale tumors account for less than 5% of all paragangliomas, with approximately 10% being multicentric. Although histologically these tumors have been shown to contain neurosecretory granules, they are rarely functional. Vagal chemodectomas are histologically benign, but are one of the most difficult tumors of the skull base to manage. Authors present a case of 56 years old woman with glomus vagale tumor treated in ENT Dept. in Poznan.

An unusual bilateral cervical paraganglioma: a case report.

Paragangliomas are neoplasms originating from paraganglion tissue derived from mesoderm, the most common location being adjacent to carotid bifurcation. Rarely these tumours can be bilateral. We present here a case of bilateral paraganglioma occurring in a young woman. On one side the tumour was arising from the vagus nerve and on the other, from the carotid body. Clinical, radiological and histopathological features and treatment dilemmas are discussed.

Un caso de paraganglioma del arco aórtico / A case of paraganglioma of the aortic arch

Se presenta el caso de una mujer de 20 años con un paraganglioma secretor de localización infrecuente en el arco aórtico. Su presentación clínica tuvo lugar tras una cesárea, precisando de tratamiento previo a la cirugía para normalizar la tensión arterial. Su tratamiento quirúrgico fue la escisión completa, con buena evolución clínica de la paciente. Se comentan las peculiaridades de dicha presentación anatómica y su frecuencia. (AU)

Metastatic hypervascular lymph nodes in malignant glomus vagale tumor: angiography findings.

Malignant vagal paraganglioma is very uncommon and the diagnosis of malignancy is made on the basis of presence of distant metastasis rather than the histological findings. We report angiography findings of metastatic cervical lymph nodes in a case of malignant vagal paraganglioma.

Definitive radiotherapy in the management of chemodectomas arising in the temporal bone, carotid body, and glomus vagale.

PURPOSE: To evaluate the results of treatment for 71 patients with 80 chemodectomas of the temporal bone, carotid body, or glomus vagale who were treated with radiation therapy (RT) alone (72 tumors in 71 patients) or subtotal resection and RT (8 tumors) at the University of Florida between 1968 and 1998. METHODS AND MATERIALS: Sixty-six lesions were previously untreated, whereas 14 had undergone prior treatment (surgery, 11 lesions; RT, 1 lesion; or both, 2 lesions) and were treated for locally recurrent disease. All three patients who received prior RT had been treated at other institutions. Patients had minimum follow-up times as follows: 2 years, 66 patients (93%); 5 years, 53 patients (75%); 10 years, 37 patients (52%); 15 years, 29 patients (41%); 20 years, 18 patients (25%); 25 years, 12 patients (17%); and 30 years, 4 patients (6%). RESULTS: There were five local recurrences at 2.6 years, 4.6 years, 5.3 years, 8.3 years, and 18.8 years, respectively. Four were in glomus jugulare tumors and one was a carotid body tumor. Two of the four patients with glomus jugulare failures were salvaged, one with stereotactic radiosurgery and one with surgery and postoperative RT at another institution. Two of the five recurrences had been treated previously at other institutions with RT and/or surgery. Treatment for a third recurrence was discontinued, against medical advice, before receiving the prescribed dose. There were, therefore, only 2 failures in 65 previously untreated lesions receiving the prescribed course of RT. The overall crude local control rate for all 80 lesions was 94%, with an ultimate local control rate of 96% after salvage treatment. The incidence of treatment-related complications was low. CONCLUSIONS: Irradiation offers a high probability of tumor control with relatively minimal risks for patients with chemodectomas of the temporal bone and neck. There were no severe treatment complications.

Functional mapping of glomus tumors reveals underlying embryology.

A functional map of cranial nerves was created by the multidimensional scaling (MDS) of adults' symptoms from glomus tumors. Differences between cranial-nerve deficits in eight different subsets of glomus tumors were input to MDS. MDS determines coordinates of points (representing cranial nerves) such that distances between points are related as closely as possible to differences in the observed symptoms. For example, two nerves that are equally likely to be affected by a glomus tumor would be put close together in the calculated map, even though they may be physically distant. The result resembles a stage in the developing nervous system. This correlation provides mathematical confirmation that adults' glomus tumors are related to early embryology. This in turn suggests that functional maps can provide a useful way for computers to depict underlying patterns in medical data.

Management of cervical paragangliomas.

Two cases of vagal body tumours are reported for their surgical consequences of immediate post-operative airway obstruction in one case, and tachycardia hypotension and severe dysphagia requiring cricopharyngeal myotomy in another. An unusual case of carotid body tumour which had the same clinical picture was also reported for comparative surgical treatment. The surgical management of both tumours was reviewed and it was found that 28-43 per cent of carotid body tumour resection would have vascular operation while dissection of vagal body tumour usually did not. The angiographic diagnostic criteria for vagal body tumour were also reviewed and found to be inadequate. Additional angiographic criterion was proposed.