Paragangliomas of the head and neck

BACKGROUND

Extraadrenal paragangliomas of the head and neck are tumors with variable clinical behavior. Because tumor growth as well as surgery can cause disabling loss of function, knowledge of the natural history of paragangliomas is important for the development of treatment strategies.



METHODS

The tumor volume, growth rate, and tumor doubling time of 48 paragangliomas at different anatomic locations in the head and neck region were estimated retrospectively using sequential radiologic imaging.



RESULTS

During a mean follow-up period of 4.2 years, a volume increase of > 20% was observed in 60% of the paragangliomas. In these cases the median growth rate was 1.0 mm/year and the median tumor doubling time was 4.2 years. More growing tumors were observed in intermediate size tumors than in very small or large paragangliomas, suggesting a biphasic growth pattern.



CONCLUSIONS

The majority of head and neck paragangliomas have a very low growth rate. Although management of paragangliomas also is determined by other parameters, preoperative estimation of the tumor doubling time may influence the treatment proposal. A “wait and scan” policy must be considered in all cases. Cancer 2000;88:2811–6. © 2000 American Cancer Society.

Estimation of growth rate in patients with head and neck paragangliomas influences the treatment proposal

Background

Paragangliomas are highly vascular tumors of neural crest origin that involve the walls of blood vessels or specific nerves within the head and neck. They may be multicentric, and they are rarely malignant. Surgery is the preferred treatment, and these tumors frequently extend to the skull base. There has been controversy concerning the role of preoperative angiography and embolization of these tumors and the benefits that these procedures offer in the evaluation and management of paragangliomas.



Methods

Forty-seven patients with 53 paragangliomas were treated from the period of 1990–2000. Initial evaluation usually included CT and/or MRI. All patients underwent bilateral carotid angiography, embolization of the tumor nidus, and cerebral angiography to define the patency of the circle of Willis. Carotid occlusion studies were performed with the patient under neuroleptic anesthesia when indicated. The tumors were excised within 48 hours of embolization.



Results

Carotid body tumors represented the most common paraganglioma, accounting for 28 tumors (53%). All patients underwent angiography and embolization with six patients (13%), demonstrating complications (three of these patients had embolized tumor involving the affected nerves). Cerebral angiography was performed in 28 patients, and 5 of these patients underwent and tolerated carotid occlusion studies. The range of mean blood loss according to tumor type was 450 to 517 mL. Postoperative cranial nerve dysfunction depended on the tumor type resected. Carotid body tumor surgery frequently required sympathetic chain resection (21%), with jugular and vagal paraganglioma removal frequently resulting in lower cranial nerve resection. These patients required various modes of postoperative rehabilitation, especially vocal cord medialization and swallowing therapy.



Conclusions

The combined endovascular and surgical treatment of paragangliomas is acceptably safe and effective for treating these highly vascular neoplasms. Adequate resection may often require sacrifice of one or more cranial nerves, and appropriate rehabilitation is important in the treatment regimen. © 2002 Wiley Periodicals, Inc.

Combined endovascular and surgical treatment of head and neck paragangliomas—A team approach†

BACKGROUND: The baroreflex normally serves to buffer blood pressure against excessive rise or fall. Baroreflex failure occurs when afferent baroreceptive nerves or their central connections become impaired. In baroreflex failure, there is loss of buffering ability, and wide excursions of pressure and heart rate occur. Such excursions may derive from endogenous factors such as stress or drowsiness, which result in quite high and quite low pressures, respectively. They may also derive from exogenous factors such as drugs or environmental influences. METHODS AND RESULTS: Impairment of the baroreflex may produce an unusually broad spectrum of clinical presentations; with acute baroreflex failure, a hypertensive crisis is the most common presentation. Over succeeding days to weeks, or in the absence of an acute event, volatile hypertension with periods of hypotension occurs and may continue for many years, usually with some attenuation of pressor surges and greater prominence of depressor valleys during long-term follow-up. With incomplete loss of baroreflex afferents, a mild syndrome of orthostatic tachycardia or orthostatic intolerance may appear. Finally, if the baroreflex failure occurs without concomitant destruction of the parasympathetic efferent vagal fibers, a resting state may lead to malignant vagotonia with severe bradycardia and hypotension and episodes of sinus arrest. CONCLUSIONS: Although baroreflex failure is not the most common cause of the above conditions, correct differentiation from other cardiovascular disorders is important, because therapy of baroreflex failure requires specific strategies, which may lead to successful control.

/pdf/four-faces-of-baroreflex-failure-hypertensive-crisis-4yi4txy7aq.pdf

Four faces of baroreflex failure: hypertensive crisis, volatile hypertension, orthostatic tachycardia, and malignant vagotonia.

Paragangliomas of the head and neck are highly vascular lesions originating from paraganglionic tissue located at the carotid bifurcation (carotid body tumors), along the vagus nerve (vagal paragangliomas), and in the jugular fossa and tympanic cavity (jugulotympanic paragangliomas). Diagnostic imaging can be considered in two clinical situations: (1) patients who present with clinical symptoms suggestive of a paraganglioma, and (2) individuals from families with hereditary paragangliomas. It is not only necessary to detect and characterize the lesion, but also to study the presence of multiplicity. For these purposes, MR imaging, and especially 3D TOF MRA, is the modality of choice. CT scanning is especially useful to show destruction of the temporal bone. Angiography in combination with embolization will mainly be used prior to surgical resection, but can also be used for diagnostic purposes when the diagnosis is not yet clear. Many parameters play a role in the decision to treat of which multifocality and impairment of cranial nerves are the most important. The primary therapeutic option for paragangliomas is complete excision of tumor with preservation of vital neurovascular structures. Resection however, should be balanced against a more conservative "wait and scan" policy or palliative treatments such as radiotherapy.

Imaging and management of head and neck paragangliomas.

Minimal morbidity occurs with resection of most carotid body tumors (CBT). With larger tumors significant injury to the cranial nerves has been reported. In order to assess the operative sequelae rate, 30 patients with CBT were reviewed. Sixteen patients either presented with bilateral carotid body tumors or had previously undergone a resection of the contralateral carotid body tumors, for a total carotid body tumor count of 46. Sixteen patients demonstrated a familial pattern while 14 were nonfamilial. Within the familial group, 14 of 16 presented with multiple paragangliomas as compared to 6 of 14 in the nonfamilial group. Tumor size ranged from 0.8 to 12 cm. Vascular replacement occurred in 2 of 20 patients with tumors 5.0 cm. Four patients lost cranial nerves with the resection: superior laryngeal nerve (SLN), 4; cranial nerve X, 1; cranial nerve XII, 1. Ten patients developed baroreceptor failure secondary to bilateral loss of carotid sinus function. First-bite pain occurred in 10 of 25 operative patients. Cranial nerve loss can be minimal with resection of carotid body tumors, however, baroreceptor failure and first-bite pain are postoperative sequelae that are often disregarded in the postoperative period

Carotid body tumors: A review of 30 patients with 46 tumors

Transmastoid labyrinthectomy is an effective procedure for the control of episodic vertigo due to unilateral peripheral vestibular dysfunction. However, its application in older patients has been limited, primarily because of concerns that older patients may not compensate well postoperatively. Poor vestibular compensation results in constant disequilibrium, motion intolerance, and visual symptomatology. The purpose of this report is to review our results with transmastoid labyrinthectomy in patients over the age of 65. Complete records were available for 22 patients operated on between July 1984 and June 1994. Patient's records were reviewed for age, preoperative duration of symptoms, coexistent medical conditions, vertigo control, and postoperative disequilibrium. Advanced age need not be a contraindication to transmastoid labyrinthectomy.

Mark E. Reiber

Papers

Carotid body tumors: A review of 30 patients with 46 tumors

Transmastoid labyrinthectomy in older patients

Congenital absence of stapes and facial nerve dehiscence.