Vagal paraganglioma (2 case reports).
N Machado, N Rajan, BH Rao
Department of Surgery, Kasturba Medical College and Hospital, Manipal, Karnataka.
Department of Surgery, Kasturba Medical College and Hospital, Manipal, Karnataka.
Paragangliomas are rare tumours of the head and neck and are a diagnostic challenge. Two cases of paragangliomas arising from the vagus are being reported. In the first case, an angiogram raised the possibility of intravagal tumour and exploration followed by biopsy confirmed the diagnosis. In the second case, it was a diagnostic surprise where exploration was done on a clinical suspicion of parotid swelling and on the table, the swelling was found to arise from the vagus nerve pushing the parotid gland laterally.
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Machado N, Rajan N, Rao B H. Vagal paraganglioma (2 case reports). J Postgrad Med 1991;37:56-8,58A
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Machado N, Rajan N, Rao B H. Vagal paraganglioma (2 case reports). J Postgrad Med [serial online] 1991 [cited 2023 Jun 8 ];37:56-8,58A
Available from: https://www.jpgmonline.com/text.asp?1991/37/1/56/799
Paragangliomas are neoplasms that arise from paraganglionic "bodies" of the autonomic nervous system. These paraganglia are distributed symmetrically and segmentally in the para-axial regions of the trunk and are typically related to arterial vasculature and cranial nerves of the ontogenetic gill arches. On the basis of anatomical distribution, innervation and microscopic structure they are grouped as branchiomeric, intravagal, aortosympathetic and visceral- autonomic paraganglia. The branchiomeric paraganglia include jugulotympanic, intercarotid, subclavian, laryngeal, coronary, aorticopulmonary and pulmonary paraganglia. Paraganglia are microscopic in size and are composed of two types of cells-chief cells, which are granular T cells that contain catecholamines and Schwann-like satellite cells. Paraganglia are classified as chromaffin and non-chromaffin type based on a chromaffin reaction in a cell or tissue. Even though the chromaffin reaction is highly indicative, it is not an absolute proof of the presence of a catecholamine or an indole amine.
A 45-year-old male patient was admitted on 9-3-1988 with a slowly increasing swelling of six months' duration in the right upper region of the neck. It was situated just below the mastoid, deep to the origin of the sternomastoid. The size at the time of admission was 6 x 4 cm. He complained of cough whenever pressure was applied to the swelling. There was no history of hoarseness of voice, nasal regurgitation, syncopeal attacks or associated pain. There was no history of trauma or fever. Examination revealed a firm, pulsatile swelling that was mobile only in the transverse plane. The carotids were palpable anterior to the swelling and were of normal volume. Bruit was heard over the swelling, Examination of oropharynx revealed no medial displaccment of peritonsillar structures and indirect laryngoscopy revealed no vocal cord paralysis. Investigations revealed a normal hcmogram. Urinary vanillyimandelic acid (VMA) was within normal limits. Plain X-ray neck was normal. Carotid angiogram showed a tumour blush posterior to the carotids. The carotids were displaced anteriorly and the carotid fork was not splayed (See [Figure:1]).
The neck was explored by a long oblique incision along the anterior border of sternomastoid extending from the mastoid to just above the clavicle. There was a vascular brownish-red tumour in-between the carotids anterolaterally and the internal jugular vein posteromedially, both of which were compressed. The tumour was arising from the vagus nerve and extending upto the base of skull. 'The tumour was completely excised along with the vagus nerve (See [Figure:2]). Post-operative recovery was uneventful except for hoarseness of voice. Five months later, the voice was covered rcmarkably. Biopsy was reported as Paraganglioma (See [Figure:3]).
The second case was a 40-year-old male patient, presented on 18.1.1989 with a history of painless swelling of one year duration, which had been slowly increasing in size. There was no other significant history. Examination revealed a swelling of 4 x 3 cm in size in the parotid region on right side. It was firm, smooth and nonpulatile. Basic investigations were within normal limits. Suspecting a parotid swelling, the patient was explored using a lazy 'S' incision, which revealed a vascular tumour arising from the vagus nerve. 'The carotid vessels and the parotid gland were pushed anterolaterally. 'The tumour was excised completely along with the part of the nerve. Post -operatively, the patient had hoarseness of voice and nasal regurgitation. Patient was discharged after 2 weeks. Biopsy was reported as Paraganglioma. After 6 months, patient was found to have a near normal voice with no nasal regurgitation.
Paragangliomas are rare tumours. Approximately 10% of thcm arise from the vagus nerve, commonly in association with one of the Vagal ganglia. There are about 80 report cases of Vagal Paragangliomas. Intravagal tumours are however not restricted to this site and may be found at various sites along the nerve and may extend to the level of carotid artery bifurcation. If the tumour lies inferiority, it may displace the carotids anteriorly and often compress the jugular vein as in our case. Intravagal Paragangliomas typically arise from nests of paraganglionic tissue within the perineurium of the Vagal nerve just below the ganglion nodosum of the vagus nerve. Paragangliomas of all types have a propensity for multicentricity. Intravagal Paragangliomas are associated with other neural crest tumours in 13% of cases. A familial incidence of 8% is reported for all Paragangliomas.
Paralysis of vagus nerve resulting in hoarseness of voice may be the first symptom. Inadequate closure of glottis may lead to aspiration of fluid. Sometimes the patient may manifest painless swelling with no other symptoms as in our case. Such tumours if allowed to progress, commonly result in additional symptomatology depending upon the location of Paraganglioma. Tumours arising in or expanding into jugular foramen may be associated with symptoms involving paralysis of 9th and 11th cranial nerves which manifest as dysphagia and shoulder drop. Paralysis and anaesthesia of the palate cause various amount of nasal reflux of fluids and aspiration.
A preoperative diagnosis may be made with some certainty based on a high index of suspicion from the history of painless, pulsatile swelling, characteristically mobile laterally but immobile vertically, reflecting its attachment to the vagus nerve.
If confirmed by a carotid angiogram, it shows a tumour blush. This appears to be larger than their actual size because of pharyngeal plexus, which surrounds Vagal Paraganglioma. The carotids are pushed anteriorly and the fork is not splayed as in carotid body tumour. C. T. scan is useful in establishing the diagnosis but its special value is in its ability to assess local infiltration to the base of skull. Incisional biopsy is unnecessary and contraindicated because of the vascular nature of the lesion and the possibility of uncontrolled haemorrhage. It may also make removal of tumour more difficult because of obliteration of tissue plane.
Surgical removal is the treatment of choice for Vagal Paraganglioma. Surgical removal requires sacrifice of vagus nerve, though in some early cases it can be preserved as the nerve fibres are splayed over the tumour. The tumour may extend posteriorly and if the mastoid tip is involved, transmastoid or transcervical approach is required . If intracranial extension is noted, a posterior fossa craniotomy is required. Radiation therapy is reserved for all patients who are too ill to tolerate surgical management, for lesions, which are incompletely excised, for recurrence and for metastasis. Radiation produces extensive fibrosis and deposition of collagen but has no effect on the chief cells or vasculature and consequently cannot be expected to eradicate the primary tumour. Infiltration into the cranial vault is the usual malignant manifestation of this tumour, though Vagal Paraganglioma with lymph mode metastasis have been reported. Considering distant metastasis as the only criterion, of malignancy, Druck et al had found 19% incidence of malignancy. Regional lymph nodes and lungs are common sites of secondaries. Lifetime follow-up is important for patients with intravagal Paragangliomas and also for family members because of the multicentricity and malignant potential of the tumours as well as the possibility of familial occurrence. Prognosis in general is guarded. Early diagnosis and complete surgical removal offers the best chance of survival. Intracranial extension appears to be a major cause of death.
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