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Sudden onset of thoracic pain: neurofibroma with intracystic haemorrhage

Department of Cardiothoracic Surgery, Thoracic Surgery Unit, University Hospital of Siena, Viale Bracci 1, 53100 Siena, Italy

^* Corresponding author. Tel.: +39-0328-742-1444
campioneandrea{at}libero.it

Received December 19, 2003; received in revised form April 5, 2004; accepted April 15, 2004

	Abstract

Top Abstract 1. Introduction 2. Case report 3. Discussion References

 
Intercostal nerve sheath tumors are normally benign and asymptomatic. Nevertheless surgical resection can eliminate the risk of malignant transformation and also achieve complete remission in case of symptoms.

Key Words: Mediastinal tumor; Neurofibroma; Thoracoscopy

	1. Introduction

Top Abstract 1. Introduction 2. Case report 3. Discussion References

 
The nerve sheath tumours in the thorax often occur in either costovertebral sulcus. Schwannoma and neurofibroma are benign peripheral nerve neoplasm, which represent the most common mediastinal neurogenic tumors, and rarely deteriorate into malignancy [1]. According to some reports, patients with malignant schwannomas have very poor prognoses, especially when associated with Von Recklinghausen's disease [2]. On the other hand, surgical resection is generally curative in benign neoplasm and eliminate the risk of malignant transformation.

Intercostals nerve sheath tumors are rare and normally benign tumors. Though thoracoscopic approach can be the first attempt to cure them [3], when intervertebral foramen or adjacent structures seem to be involved, open thoracotomy is mandatory. These neoplasm are most often asymptomatic, but some of them can cause symptoms because of pressure on adjacent nerve with paresthesia or back pain. Here, we present a case with a sudden onset of symptoms probably related to intracystic haemorrhage.

	2. Case report

Top Abstract 1. Introduction 2. Case report 3. Discussion References

 
We present a case of a 37-year-old healthy white man who complained a sudden onset of radicular thoracic pain, involving the right hemithorax from spine to sternum. He was referred to our hospital and he underwent routine clinical assessment. Roentgenograms showed evidence of a paravertebral mass, which was further detected with chest CT scan.

The lesion was diagnosed on imaging as a neurogenic tumor (Fig. 1). Under video-assisted thoracoscopy, we found a spherical smooth-featured neoplasm of the right paravertebral sulcus.

View larger version (152K): [in this window] [in a new window]   Fig. 1 Chest CT scan showing a mass in the paravertebral sulcus suggestive for neurogenic tumor.

A cystic cavity with sign of internal recent haemorrhage was noted. Postoperative course was uneventful and the patient was discharged with complete remission from symptoms. Histopathologic examination confirmed a cystic neoplasm, which was identified as neurofibroma.

	3. Discussion

Top Abstract 1. Introduction 2. Case report 3. Discussion References

 
Schwannoma and neurofibroma represent the most common mediastinal neurogenic tumors. Most of the neurogenic tumors are asymptomatic, although a small percentage of patients experience paresthesia or back pain from compression of adjacent structures or from intraspinal tumor extension.

Some benign mediastinal tumors can present with paravertebral pain. An acute onset of severe back pain and headache is described for spinal subarachnoidal venous hemorrhage from a posterior mediastinal tumor [4].

In intercostal schwannoma, neuralgic symptoms [5] or recurrent chest pain are often present, while a giant intercostal nerve tumor can present with Horner's syndrome [6]. In our case, an asymptomatic cystic neoplasm became symptomatic probably because of internal sheath haemorrhage, which caused rapid compression to nearby structures and intercostal nerve. As a consequence of their benign nature, minimally invasive surgery could be desirable, and conversion to thoracotomy is then encouraged to enhance radical excision when posterior neurogenic tumor seem to involve the spinal canal [7] or other surrounding structures.

Contraindications to VATS include intraspinal extension, spinal artery involvement, tumors more than 6 cm in diameter and middle mediastinal tumor [8]. Some dumbbell neurofibroma can also be resected through a combined thoracoscopic and posterior-spinal approach [9].

Thoracoscopic approach can reach less morbidity, facilitate patient's recovery, avoiding rib retraction and muscle transection thus reducing postoperative pain. Only occasionally, a peripheral neurologic defect may be noted after resection. Operative mortality and morbidity should approach zero (rare complications such as Horner's syndrome and hoarseness are reported). Recurrence in benign lesions is unusual. Among nerve sheath tumors, malignant transformation is rare.

The small percentage of malignant tumors tend to occur in patients with von Recklinghausen's disease, with high rate of local recurrence and distant metastasis (78 and 63%, respectively) with an approximate long-term survival of 50% of cases [10].

doi:10.1016/j.icvts.2004.04.008

	References

Top Abstract 1. Introduction 2. Case report 3. Discussion References

 

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Permission Requests Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Campione, A. Articles by Gotti, G. Search for Related Content PubMed PubMed Citation Articles by Campione, A. Articles by Gotti, G. Related Collections Mediastinum Minimally invasive surgery Chest wall