Eur J Cardiothorac Surg 1999;16:569-572
© 1999 Elsevier Science NL
Massive plexiform neurofibroma of the sympathetic trunk
D.W. Olivera,
F.C. Wellsb,
B.G.H. Lambertya,
A. Watersc
a Department of Plastic and Reconstructive Surgery, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2QQ, UK
b Cardiothoracic Surgical Unit, Papworth Hospital NHS Trust, Papworth Everad, Cambridge CB3 8RE, UK
c Department of Neurosurgery, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2QQ, UK
Corresponding author. Tel.: +44-1480-830-541; fax: +44-1480-831-315
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Abstract
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A case report of a massive plexiform neurofibroma of the sympathetic trunk is reported. Symptoms included dysphagia, shortness of breath and paraesthesia of the right hand. The nerve of origin and extent of the tumour were unusual. The tumour mass partially filled the right hemithorax, surrounded the right brachial plexus, extended to the base of skull and invaded the cervical vertebrae threatening to compromise the spinal cord. Surgical resection required combined cardiothoracic, neurosurgical and plastic and reconstructive surgical teams. Early outcome following surgery was excellent. This case illustrates the need for a combined surgical approach for such a lesion and we believe it is the first reported case of plexiform neurofibroma of the sympathetic trunk.
Key Words: Plexiform neurofibroma • Sympathetic trunk
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1. Introduction
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Plexiform neurofibroma of the sympathetic trunk is unusual both in terms of the nerve of origin and the extent of tumour, involving thorax, brachial plexus cervical vertebrae and extending to the base of skull. Surgical resection was attempted with cardiothoracic, neurosurgical and plastic and reconstructive surgical teams.
Nerve sheath tumours are believed to originate from Schwann cells but some may contain large amounts of collagen and are therefore subdivided into schwannoma and neurofibroma. However they exist as a spectrum of tumours ranging from discrete paraneural schwannoma to poorly delineated plexiform neurofibroma. Plexiform neurofibromas differ from non-plexiform neurofibroma in that they infiltrate locally and are poorly circumscribed. Plexiform neurofibroma are associated with neurofibromatosis type I (von Recklinghausen's disease) or may occur in the absence of this syndrome, as in our case. There is an associated low risk of malignant transformation [1].
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2. Case report
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A 55-year-old female presented with a 6 month history of increasing shortness of breath and decreasing exercise tolerance. She also complained of increasing dysphagia and of tingling in the right hand. She had also noticed a swelling on the right side of her neck. Past medical history revealed that 22 years previously she had undergone resection of a retroperitoneal neurofibroma. The patient was not known to have von Recklinghausen's disease as there were no other manifestations of neurofibromatosis such as cutaneous neurofibromas, café au lait pigmentation or axillary freckling. Examination revealed a smooth firm mass above the right clavicle, 6 cm in diameter, extending into the chest. There was paraesthesia in the right hand corresponding to the C8 and T1 nerve roots. The extent of the tumour only became apparent after pre-operative investigations.
Chest X-ray revealed a thoracic mass in the upper right lung field extending behind the sternum and right clavicle displacing the trachea (Fig. 1). Further imaging showed a large encapsulated mass within the chest, lying behind the compressed superior vena cava and brachiocephalic veins. The trachea was compressed and displaced to the left. The mass extended inferiorly to the carina and superiorly through the thoracic inlet. Computerised tomography showed erosion of the C7 vertebral body (Fig. 2) and the magnetic resonance image demonstrated invasion of the spinal canal at this level. The tumour mass was seen extending into the neck towards the base of skull. We did not assess the patient endoscopically pre-operatively as imaging had shown no tracheal or oesophageal involvement.
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Fig. 1. Pre-operative chest X-ray showing tumour mass at the thoracic inlet displacing the trachea to the left.
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Ultrasound guided biopsy was undertaken. This was inconclusive, but suggested neurofibroma and therefore open biopsy was performed. This confirmed the histological diagnosis of plexiform neurofibroma.
Operative resection of the lesion was undertaken due to respiratory compromise, superior vena caval obstruction, the potential for spinal cord involvement, oesophageal compression and upper limb paraesthesia. There was also the need to exclude sarcomatous change. It was felt that resection was feasible but this should be a three-team approach with both a lateral thoracotomy and a median sternotomy. Thoracic surgeons were to resect the intra- thoracic tumour. Neurosurgeons were to resect the cervical component, particularly as pre-operative imaging had shown the risk of dural or nerve involvement by tumour and therefore the potential for intra- or extradural haemorrage in the spinal canal with the possible result of quadraplegia. Plastic and reconstructive surgeons were to assist with nerve dissection and be available to reconstruct any defect of the brachial plexus or soft tissues (which was not the case on this occasion). Therefore this combined approach allowed the tumour to be resected with confidence.
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3. Operative findings
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With the patient intubated in the left lateral position a right sided thoracotomy was performed through the bed of the fourth rib. This exposed the massive intra-thoracic, but extra-pleural tumour mass lobulated by the azygos vein. This incision allowed the tumour to be evaluated safely and under control, particularly for any macroscopic indication of sarcomatous change. The azygos lobe was trapped between the tumour mass and the thoracic wall. From its position in the posterior mediastinum the tumour could not have been arising from a peripheral nerve, all of which were seen to be intact. The tumor was seen to be arising from the sympathetic trunk on the right below the stellate ganglion which was preserved, running over the heads of the ribs in the lateral mediastinum. There appeared to be a segmental arterial supply to the tumour rather than a single feeding vessel. The thoracic tumour was excised after ligation of the azygous vein and dissection from the mediastinal structures which were not directly invaded by tumour. The tumour was dissected from within the chest up to the thoracic inlet after excision of the first rib from within the thorax to open the thoracic inlet. The lateral thoracotomy was then closed over two drains. The patient was then turned supine and a median sternotomy performed and extended with a right sided cervical incision along the anterior border of sternocleido-mastoid to the angle of the mandible.
Dissection was continued down to the innominate vein and subclavian vein. All the great vessels were stretched tightly over the tumour mass and were dissected from the tumour. In order to facilitate exposure the medial end of the right clavicle and the manubrium were excised. Dissection continued laterally and the right brachial plexus was freed from the tumour mass.
In the neck the tumour was found to extend cranially from the base of skull with caudal extension involving the lower cervical vertebrae which were invaded by tumour. It was necessary to amputate the tumour at the level of the vertebral bodies and remove the remaining tumour piece-meal. Tumour was resected from these structures without damage to the spinal canal although the transverse cervical artery and sternocleidomastoid on the right were sacrificed.
After achieving haemostasis the cervical incision and median sternotomy were closed.
The lesion was removed in two main specimens, the thoracic lesion measuring 13x11x9 cm and a cervical specimen 13x10x4 cm.
Macroscopically the specimens consisted of a smooth lobulated mass with a homogenous whirled off-white appearance with areas of haemorrhage. Histologically the tumour comprised loosely arranged spindle cells, with no pleomorphism and few mitosis, the appearance of plexiform neurofibromata.
Twelve months post-operatively the patient is well with no shortness of breath, normal swallowing and improved neurology although in the initial post-operative period she did complain of stiffness in the right shoulder and some minor weakness affecting the small muscles of the right hand. The stiffness in the shoulder has responded to physiotherapy and the weakness in the right hand resolved. Further post-operative investigation has shown normal function in the phrenic nerves, the right having been close to the tumour mass, equal hemi-diaphragms on chest X-ray and normal respiratory function tests. She is asymptomatic from her right sided cervical sympathectomy and does not have Horner's syndrome. Early outcome following surgery was excellent.
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4. Discussion
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We do not believe plexiform neurofibroma arising from the sympathetic trunk has previously been reported. In the head and neck plexiform neurofibroma have previously been reported in the oral cavity [2], in the major salivary glands [3] and the larynx [4]. They have also been reported in children [5]. As in a previous case involving the vagus nerve [6] it may not be until resection is attempted that the nerve of origin can be identified with confidence, however magnetic resonance imaging of tumours of the thoracic inlet has been shown to be beneficial in planning surgery for these tumours [7]. Magnetic resonance imaging should therefore be considered an essential investigation. The involvement of the spinal cord necessitated urgent surgical intervention since external compression of the vertebral bodies can cause their collapse and spinal damage from extra-spinal tumours [8]. In our case it was only by following the course of the tumour during the resection that the origin in the right sympathetic trunk became apparent arising below the stellate ganglion. The tumour mass extended away from the anatomical site of origin whilst histologically the nerve itself is thought to be located within the tumour mass or on the periphery of these lesions and cannot be spared if the lesion is to be resected. Of note is that the sympathetic origin of the nerve cannot be confirmed histologically with such advanced tumours as there are no specific histological features of autonomic fibres. As with other case reports the nerve of origin is an anatomical diagnosis at the time of surgery.
This case illustrates the enormous size that these tumours can reach, and the potential need for a combined surgical approach since plexiform neurofibromas may not be contained within anatomical confines of a single speciality. This case could act as model for the future management of similar complex patients.
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References
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Sakai F., Sone S., Kiyono K., Maruyama Y., Oguchi K., Imai N., et al. Magnetic resonance imaging of neurogenic tumours of the thoracic inlet: determination of the parent nerve. J Thorac Imaging 1996;11:272-278.[Medline]
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Received March 29, 1999;
received in revised form June 29, 1999;
accepted August 24, 1999.
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Abstract
1. Introduction
