A. Seguin, MD, S. Mussot, MD, E. Fadel, MD,
A. Chapelier, MD, and P. Dartevelle, MD
Department of Thoracic and Vascular Surgery and Heart-Lung Transplantation, Marie-Lannelongue Hospital, France.
Background. Neurogenic tumors of the thorax can derive from any tissue of neural crest origin and thus from the peripheral, autonomic, or paraganglionic nervous systems. The goal of this retrospective study was to report our 22-year single-center experience with neurogenic tumors of the thorax. Pathologic types, preoperative evaluation, operative procedures, adjuvant treatments, results, and survival will be considered.
Methods. Between 1980-2002, 109 patients including 15 infants and children underwent surgical resection of neurogenic intrathoracic tumors.
Results. Nerve cell tumors were the majority in infants and children (93.3%) and were mostly malignant (43%). Nerve sheath tumors were more frequent in adults (79.8%) and only 6,4% of neurogenic tumors in adults were malignant lesions. 16 tumors (14.7%) were associated with von Recklinghausen’s disease. The tumors were symptomatic in 86,7% of children and 26.7% of adults. Exceptional tumor locations were noted as vagus (5), phrenic (2), or brachial plexus nerve (6), intrabronchial (1) and intrapulmonary (1) and a dumbbell tumor was identified in 10 patients ; they had required specific surgical strategy. Resection was complete for all cases except 2 patients with malignant lesions. Postoperative chemo- and/or radiotherapy was administrated to 4 patients.
The mean follow-up period was 10 years. There was no perioperative death, no late death and no recurrence related to benign tumors. Patients with a malignant schwannoma had a poor prognosis, especially when associated with von Recklinghausen’s disease.
Conclusions. Only complete resection of neurogenic intrathoracic tumors can provide long term survival in malignant disease and complete recovery in benign tumors.
Introduction
Neurogenic tumors of the thorax can derive from any tissue of the neural crest and thus from the peripheral, autonomic, or paraganglionic nervous systems. They can occur anywhere in the thorax, although almost all develop themselves in the mediastinum and account for 12% to 21% of all mediastinal masses [1]. The majority are found in the costovertebral sulcus and are related to the sympathetic chain or the rami of intercostal nerves. They are often in close proximity to the intraspinal canal [2]. They can be multifocal and associated with cutaneous tumors of the same ectodermic origin. They can also derive from peripheral nerves such as the vagus, recurrent laryngeal, phrenic and brachial plexus. Some tumors secrete catecholamines or their degradation products. They are observed in children and adults, with different patterns for each. Rare neurologic tumors, such as granular cell tumors and melanotic schwannomas of both nerve sheath and ganglion cell origin, also may occur in the mediastinum.
Classifications are based on the cell type of origin and the presence of benign versus malignant characteristics (Table 1).
The objective of this retrospective study was to report our 22-year single-center experience with neurogenic tumors of the thorax, considering pathologic types, preoperative evaluation, adjuvant treatments, results, and survival. We have insisted on operative procedures which necessitated specific surgical strategy in particular location as dumbbell tumors or thoracic inlet lesions.
Patients and Methods
Between January 1980 and October 2002, 109 patients underwent surgery for neurogenic intrathoracic tumors at the Department of Thoracic and Vascular Surgery and Heart-Lung Transplantation, Marie-Lannelongue Hospital, France. There were 59 (54%) men and 50 women, with a mean age of 39.3 years (range 0 to 89 years). 15 infants and children (with age less than 15 years) were included.
Preoperative Evaluation and investigation
Functional status was evaluated preoperatively. Physical examination, chest radiography, spirometry were routinely performed. Computed tomography (CT) established the location of the tumor and its possible extension into the spinal canal. If CT clearly showed that the tumor was extra dural and extra spinal, no further radiological investigation was required. If CT was inconclusive, MRI was done, defining accurately the existence and extension of the intraspinal component of the tumor. Thoracic aortography visualising the artery of Adamkiewicz, was performed in particular cases.In smokers and patients older than 40 years, fibroscopic examination was performed preoperatively.
Serum and urinary levels of catecholamines and their degradation products, (metanephrines and vanillymandelic acid) were measured in all children and when symptoms suggest a biologically active tumor. Iodine 131 meta-iodobenzyguanidine scintigraphy was obtained only once.
The patients were postoperatively seen at 6 months and once a year. There were clinical and radiological controls. In cases of malignancy, follow-up was more frequent with specific extension check-up.
Statistical Analysis
All results are expressed as the mean ± standard error. Categorical data were calculated as percentages and compared using the Ç2 test. Survival rates were calculated by life-table analysis. Kaplan-Meier curves were plotted and compared using the log-rank test for univariate analysis. Differences were considered significant when p was less than 0.05.
Results
The mean follow-up period was 10 years (range, 4 months to 22 years).
Table 2 gives the classification of pathologic types of tumors by age group. Nerve cell tumors were the majority in children (93.3%), there were malignant in 6 cases out of 15 (42.3%). Nerve sheath tumors appeared in our patients from 15 years of age and were the most frequent type in adults (79.8%). Malignancy occurred in only 6.4% of cases in this age group.
Table 3 summarize clinical findings and discovery circumstances of tumors regarding the age of the patients. Only 26.6% were symptomatic in adults in contrast with 86.7% in children. Several symptoms or signs were associated in the same patient. Their presence, in particular in young patients was significant for malignancy. In adults, the diagnosis was made by a routine chest radiography obtained for other indications in the majority of cases.
Ten patients (2 children and 8 adults) had a dumbbell tumors identified.
Histological diagnosis by tumor biopsy was obtained in 10 patients before definitive operation.
The lesion was associated with von Recklinghausen’s disease in 16 cases. For 9 of them the neurogenic tumor was the first pathological manifestation of this disease. All of them had cutaneous lesions at physical examination and 7 had family past history of von Recklinghausen’s disease. We noted 3 malignant peripheral nerve sheath tumors (MPNST) (18.7%): 2 neurofibrosarcomas and 1 malignant schwannoma.
Measurement of vanillymandelic acid and homovanillymandelic acid levels were performed for 16 patients. They were elevated in only one patient who had a neuroblastoma.
Metaiodobenzyl guanidine scintigraphy was used in one case of ganglioneuroblastoma.
Only one patient with a malignant lesion was found metastatic at the time of diagnosis.
Treatment and surgical technique
No neoadjuvant treatment was administrated except in one case of ganglioneuroblastoma (chemotherapy).
A posterolateral thoracotomy was performed in the fifth intercostal space in the majority of patients, or in the 4th, 6th, 7th, 8th or 9th space (n=46) to provide better access to the tumor. 9 cervicotomies where also performed; 3 were combined by manubriotomy (1), thoracotomy (1) and sternotomy (1) because of the extension in malignant tumors (Table 4). For 10 thoracic inlet lesions, an anterior transcervical-thoracic approach was performed (Fig 1).
In dumbbell tumors (n=10), different strategies were applied: First vertebral laminectomy (2), contemporaneous laminectomy (1), laminectomy in a second time (1). 5 patients underwent complete tumor resection without laminectomy and the last one needed a shoulder’s disarticulation.
Another shoulder disarticulation was performed for paralysis and severe pain in the right arm related to a malignant lesion of the brachial plexus.
Tumor resection was complete in all cases except 2, due to invasion of adjacent structures.
Postoperative adjuvant treatments included chemotherapy in one patient and radio chemotherapy in 3, depending on the tumor staging and the quality of the resection.
Tumor location was very rare in 16 cases : vagus nerve (5), phrenic nerve (2), brachial plexus nerve (6), intrabronchial (1) treated by sleeve resection, intrapulmonary (1) in which a superior lobectomy was performed and 12th intercostal nerve (1) in which a combined approach thoracic and abdominal by lombotomy was necessary.
Operative Morbidity and Mortality
No in-hospital mortality was observed. Postoperative morbidity was 15.6%. One inferior limb phlebitis and 1 cardiovascular complication (arrhythmia) were observed, in addition to the standard risks of thoracic surgery such as bleeding (n=3), pneumonia (n=4), and chylothorax (n=2). The majority of sequelae were neurological, with 4 cases of Horner’s syndrome, 2 cases of recurrent laryngeal nerve paralysis, 1 case of phrenic nerve paralysis and 1 case of crural nerve paralysis due to nerve sacrifice following tumor resection in all cases. In tumor originating from intercostal nerves, the nerve root was sacrified if necessary but with no significant deficits.
Survivals
Benign neurogenic tumors of the thorax. In this group, overall 5-year and 10-year survival rates were 97.3% and 95.6% respectively (Fig 2). No significant difference was observed according to histologic type: 5-year and 10-year survival rates were 100% for schwannoma; 83.3% for neurofibromas and 100% for ganglioneuroma. No recurrence of benign neurogenic tumor was observed.
Malignant neurogenic tumors of the thorax. Overall 2-year, 5-year and 10-year survival rates were 56.3%, 45% and 45% respectively (Fig 2). But according to histologic type, prognostic is very different : 6 cases of neuroblastomas were recorded (Fig 3). 2-year, 5-year and 10-year survival rate were stable at 60%, 2 patients died in the first year. One of metastasis at 1 month and the second of pneumonia at 11 months. 1 case of ganglioneuroblastoma was recorded and the patient is still alive at 17 months without recurrence. In our study, 6 patients had MPNST (4 malignant schwannomas and 2 neurofibrosarcomas), 3 associated with von Recklinghausen’s disease. 2-year and 5-year survival rates were 44.4% and 22.2% respectively. 1 patient is still alive at 9 month, 4 died of metastatic lesions in the first year and the last one died of a malignant pancreatic lesion at 15 years. For this last patient, histology said neurofibroma with beginning transformation in neurofibrosarcomas, which can explain its prolonged survival.
Association with von Recklinghausen’s disease significantly influenced survival: overall 2-year, 5-year and 10 years were 84.8%, 75.4% and 75.4% respectively in patient with von Recklinghausen’s disease whereas they were 95.1%, 93.8% and 92.1% respectively without (p=0.01) (Fig 4). It could be explain by malignant lesions more frequent in this disease (18.75% versus 10.7%) but this difference was not significant in our study.
By the univariate model none of the following variables did affect survival: sex, ASA, side of lesion nor localisation. Only histologic type and von Recklinghausen’s disease had incidence on survival rate in our study, and as yet said, they could be linked.
Comment
Tumor presentation
As we observed, no clinical nor radiologic criteria can assert benignity or malignancy of such lesions. Histological examination is indispensable to confirm clinical orientation or supposition. All tumors should be considered malignant until proven otherwise. We did not recommend preoperative biopsy because of the potential risk of dissemination and because it does not change the treatment based on complete resection. We presented 10 patients in which diagnostic was made by biopsies but they have been practiced in others centers and secondly refer at our department.
Some authors have published their experience by video-thoracoscopy approach [ref] but we did not agree with this procedure until all carcinological rules are followed.
Incidence and diagnosis
Benign nerve sheath tumors :
The schwannoma is the most common lesion, may be multiple in 10% of patients and mostly asymptomatic (62.6% in our study). Some of the benign tumors may cause symptoms because of pressure on adjacent nerve; thoracic pain, Horner’s syndrome, hoarseness, and upper extremity weakness or pain occasionally observed. Some can cause symptomatic extradural compression of the spinal cord as the result of growth through the intervertebral foramen into the spinal canal. Symptoms in infants and children are observed in 50% to 60% of cases. Women are more affected than men according to Shields [3] but we have no statistically significant difference in our study.
Thoracic neurogenic tumors can be familial and multifocal, especially when associated with von Recklinghausen’s disease. Neurofibromatosis-1 (NF-1) is a common autosomal dominant disorder in which affected individuals may develop benign and malignant tumors. Although it is generally believed that the tumors associated with this syndrome are all neurofibromas, some of the individual tumors may be schwannomas and some mixture of the two types [3]. In our study, among 16 patients with von Recklinghausen’s disease, the lesions were 5 schwannomas, 6 neurofibromas, 2 ganglioneuromas, 2 neurofibrosarcomas and 1 malignant schwannoma.
Solitary neurofibromas have been described in patients of all ages and both sexes, with a higher incidence in the third and fourth decades of life [2]. In our experience, mean patient age with solitary neurofibroma and without von Recklinghausen’s disease or family past history was 33 years. Multiple neurofibromas predominate in children and young adults that exhibit other manifestations of neurofibromatosis [2]. In our study, all patients with multiple neurofibromas had von Recklinghausen’s disease. Neurofibromas are benign neoplasms that are cured by surgical excision but they can become malignant, especially in patients with neurofibromatosis. They develop sarcomas of nerve sheath origin in up to 2% to 13% of instances. The association between malignant peripheral nerve sheath tumor and NF-1 has been extensively documented [4]. 3 patients (18.7%) had a malignant lesion (2 neurofibrosarcomas and one malignant schwannoma) out of 16 associated with von Recklinghausen’s disease. The treatment of patients with neurofibromatosis that develop malignant transformation of their neurogenic tumors is difficult, due to the presence of multiple neoplasms. The 5-year survival rates is lower than 20% [2]. Only one (histologicaly a beginning neurofibrosarcoma) was alive at 5 years in our study. Malignant peripheral nerve sheath tumors have a high risk of local recurrence and metastasis 5 to 10 years after therapy [5].
Surgical resection was incriminated in malignant tumor transformation without any proof [6]. Transformation risk seems to depend on " blastoma tendency" of the disease which is correlated with the skin lesions abundance [7]. Complete investigations are performed in patients presenting cutaneous lesions and suspected of NF-1. Asymptomatic thoracic tumors can be detected by the chest radiography and proposed to surgical treatment in patients with high risk of malignant transformation. Early operation is the best treatment because tumor evolution can not be suspected. About 60% of neurogenic tumors in von Recklinghausen’s disease seems to be definitively cured after surgical resection.
An other schwannoma type, or cellular schwannoma, has been described [8-10]. It has a predilection to occur in the paravertebral areas [11]. Despite its pseudosarcomatous appearance, the tumor is benign, rarely may recur but do not metastasize according to Fletcher [9]. We note only 2 cellular schwannomas in our study without recurrence or metastasis.
Both the schwann cells and melanocytes arise from the neural crest, melanocytic differentiation with the production of pigment may be seen in some schwann cell tumors. As a rule the melanotic tumors of probable ganglion cell origin are highly malignant, in contrast with the benign behaviour of the melanotic schwannomas of nerve sheath origin [11].
Granular cell tumors are uncommon, generally benign lesions that occur in multiple sites throughout the body [2]. Malignancy may occur in 1 to 3.5% of all granular cell tumors [11].
We have not observed melanotic schwannoma or granular cell tumors in our study.
Malignant nerve sheath tumors :
Malignant peripheral nerve sheath tumors (MPNST) are the malignant variants of schwannomas and neurofibromas. MPNST are locally invasive neoplasms that require surgical resection, usually followed by radiotherapy and/or chemotherapy. They can metastasize to the lung and others organs. Five-year survival rates up to 75% have been reported in patients without neurofibromatosis [2]. In our study, 6 patients had MPNST, 3 associated with von Recklinghausen’s disease. Radical intervention was performed in all cases. 1 is still alive at 9 month, 4 died of metastatic lesions in the first year and the last one died of a malignant pancreatic lesion at 15 years.
Benign nerve cells tumors :
Ganglioneuromas are benign tumors that derive from the segmental sympathetic chain paraganglia or from the intrathoracic parasympathetic ganglia such as the aortic body and the vagal body. They can be familial neoplasms and predominate in children older than 3 to 4 years and in young adults [2]. All 15 ganglioneuromas in our study appeared before the age of 30. They are cured by surgical excision. No recurrence was observed in our study. Rarely, they can undergo malignant transformation to malignant tumors of peripheral nerve sheath origin [2]
Malignant nerve cell tumors :
Ganglioneuroblastomas are rare but also more aggressive, presenting generally in early childhood with direct invasion or with metastasis [5]. Only one, observed in a 14 years child, was metastatic at the time of diagnosis. After operation and chemotherapy, the patient is still alive at 2 years but with a local recurrence.
Neuroblastoma is the most frequent malignant neoplasm of children less than two years old. 86% occurs in the first two years of life and are very exceptional in adults [5]. 4 out of 6 cases in our study (66.7%) occurred in the first two years, 1 at 8 and 1 at 18 years, the only adult case. Neuroblastoma usually derive from the adrenal gland medulla but can develop from the ganglial and neural elements of the posterior mediastinum in approximately 16% of patients. Patients are generally symptomatic. As the result of production of catecholamines, diarrhea and abdominal distension, or sweating and flushing, or a mixture of both may be present [3]. It is interesting to note that 2 infants in our study had a neuroblastoma discovered in the evaluation of a diarrhea whereas the catecholamines were not detected. In literature, neuroblastomas are slightly more prevalent in males but we did not observe any sex difference. The 2-year survival rate following chemotherapy and surgery is 10 to 15 percent [5].
Nerve cell tumors are mostly malignant in infants and children, with a male preponderance. In contrast, nerve sheath tumors are equally malignant in children and adults.
Treatment options
Although observation alone is a reasonable course of action for tumors that have been stable for years, the majority of neurogenic tumors should be resected because of uncertain diagnosis, location near or in the spinal canal, or possible malignancy. Complete surgical resection is the gold standard. Benign neurogenic tumors rarely recur and simple enucleation is sufficient; no adjuvant therapy is indicated. The resection of a benign neurogenic tumor has no bearing on the survival of patients.
Approximately 10% of neurogenic mediastinal tumors extend through the neural foramen into the spinal canal, creating a dumbbell or hour-glass shape. Despite the intraspinal component, up to 40% of these tumors are still asymptomatic at the time of diagnosis. Ninety percent of these dumbbell tumors originate from nerve sheath cells and are benign. If a dumbbell tumor is identified, a one stage or followed, two team approach is indicated to remove the intraspinal canal extension by an appropriate hemilaminectomy and to remove the intrathoracic portion of the tumor. We believe that the combined neurosurgical and thoracic approach is necessary to deal with such lesion in a concomitant procedure. We recommend to begin with the thoracic surgical approach by a postero-lateral position because the second time (neurosurgical approach) is not always necessary. Gentile traction and dissection of the tumor can express the intra luminal portion. But if it is necessary, in the same time of the intervention, we re-install the patient for the laminectomy made by a neurosurgical team. It has been suggested that in patients with demonstrated intraspinal canal extension located in the lower one-half of the thorax, the site of origin and course of the artery of Adamkiewicz, be demonstrated by angiography. The knowledge obtained allowed the surgeon to protect this vessel from injury during the removal of the tumor. We have followed this recommendation. In our experience, no complication occurred in such cases. However, actually with the MRI angiography progress, we are able to located this artery without invasive procedure and we recommend now to use conventional angiography in cases where the MRI does not succeed.
Location in the brachial plexus is not frequent and have been controversial in its approach. Considering this tumor as thoracic inlet lesion, we performed, since 1993, anterior transcervical-thoracic approach including the removal of the internal half of the clavicle if necessary. It provides an excellent exposure and safe dissection of the thoracic inlet [12]. This approach with clavicle preservation is routinely used in head neck surgery as Ducic and al described it [13]. Among the 10 patients with thoracic inlet lesions (and 6 were brachial plexus lesions), 7 had a clavicle preservation and 1 patient, 12 years-old, a clavicle reconstruction. In two we were obliged to perform a shoulder’s disarticulation for malignancy.
Benign nerve sheath tumors infrequently may be located in the visceral compartment when the tumor arises from either the phrenic or vagus nerve [14]. From all the data of the literature it may be concluded that the left vagus nerve is involved more commonly than the right vagus nerve. The tumor is slightly more often a neurofibroma than a schwannoma and either, especially in patient with neurofibromatosis, may be of the plexiform variety. Nerve sheath tumors of the phrenic nerves are much less frequent than are those of the vagal nerve and occurred equally in the right or left side and equally in the two sexes. Surgical resection with an attempt to preserve the function of the affected nerve is recommended to prevent further growth and compression on adjacent tissues [14].
Neurogenic sarcoma does occur in either younger or older individuals than those with benign lesions and tend to occur in patients with von Recklinghausen’s disease. These tumors are aggressive, metastasis to distant sites may be observed in a high percentage of cases with poor prognosis [11].
Neurogenic tumors rarely occur within the endotracheobronchial trees as primary tumors. The incidence of this type of tumor is estimated to be between 0.2% and 4% [15,16]. There are been no recurrence reported to date. The treatment for the endotracheobronchial neurofibromas depends on the size and the location of the tumors, and should be conservative, provided the diagnosis is made early, prior to parenchymal destruction [15-17]. We saw one intrabronchial schwannoma which necessitated sleeve resection without postoperatively complication and no late recurrence.
Neuroblastomas and ganglioneuroblastomas need adjuvant treatment, depending on the tumor staging according to Ribet et al [18]. We followed the same oncological protocol.
The association of a thoracic tumor with von Recklinghausen’s disease has a very poor prognosis in cases of malignant schwannoma. This association is equally fatal with or without resection of the thoracic tumor when the patient is symptomatic [6]. Malignant schwannoma is associated with a poor patient survival rate [19]. A local recurrence will occur in 50% of cases and metastasis are frequent [18]. Radiotherapy and chemotherapy are of no effect. In our report, 4 cases of malignant schwannomas were recorded. Survival rate did not exceed 30 months. The last patient is still alive at 9 months from the intervention without recurrence or metastasis. We have confirmed the ineffectiveness of adjuvant treatment in this tumor.
Neurogenic tumors are among the most common types of mediastinal tumors and are usually located in the posterior mediastinal compartment. Most neurogenic tumors are asymptomatic and detected radiographically. Careful preoperative evaluation is necessary to clarify spinal canal or neural foramen involvement to minimize the risk of bleeding and neurologic injury. All tumors should be considered malignant until proven otherwise. The treatment of neurogenic tumors of the thorax is based on their resection. Surgical excision of most of these tumors is sufficient except for the malignant lesions, especially in infancy and childhood, for which the addition of postoperative irradiation and chemotherapy may be beneficial.
Although recurrence is rare and the prognosis is good for benign tumors, the prognosis is poor for malignant neurogenic neoplasms.
References
1. Reeder LB. Neurogenic tumors of the mediastinum. Semin Thorac Cardiovasc Surg 2000;12(4):261-7.
2. Marchevsky AM. Mediastinal tumors of peripheral nervous system origin. Semin Diagn Pathol 1999;16(1):65-78.
3. Shields TW, Reynolds M. Neurogenic tumors of the thorax. Surg Clin N Am 1988;68:645-68
4. King AA, Debaun MR, Riccardi VM, Gutmann DH. Malignant peripheral nerve sheath tumors in neurofibromatosis 1. Am J Med Genet 2000;93(5):388-92.
5. Bousamra M. Neurogenic tumors of the mediastinum. In : Pearson FG and al, 2nd ed. Thoracic surgery. New York : Churchill Livingston, 2002 : 1732-8
6. Le Brigand H. Prognosis of endothoracic neurogenic tumors of Recklinghausen's disease. Ann Chir Thorac Cardiovasc 1977;16(3):214-5.
7. Hertzog P, Toty L, Personne C, Colchen A, Belami J. Neurogenic endothoracic tumours. Sem Hop 1981;57(1-2):50-5.
8. Woodruff JM, Godwin TA, Erlandson RA, Susin M, Martini N. Cellular schwannoma: a variety of schwannoma sometimes mistaken for a malignant tumor. Am J Surg Pathol 1981;5(8):733-44.
9. Fletcher CD, Davies SE, McKee PH. Cellular schwannoma: a distinct pseudosarcomatous entity. Histopathology 1987;11(1):21-35.
10. Lodding P, Kindblom LG, Angervall L, Stenman G. Cellular schwannoma. A clinicopathologic study of 29 cases. Virchows Arch A Pathol Anat Histopathol 1990;416(3):237-48.
11. Shields TW. Benign and malignant neurogenic tumors of the mediastinum in adults. In : Shields TW et al, 5th ed. General Thoracic Surgery. Philadelphia : Lippincott Williams & Wilkins, 2000 : 2313-29.
12. Dartevelle PG, Chapelier AR, Macchiarini P, et al. Anterior transcervical-thoracic approach for radical resection of lung tumors invading the thoracic inlet. J Thorac Cardiovasc Surg 1993;105(6):1025-34.
13. Ducic Y, Crepeau A, Ducic L, Lamothe A, Corsten M. A logical approach to the thoracic inlet: the Dartevelle approach revisited. Head Neck. 1999;21(8):767-71
14. Sugio K, Takashi I, Keiji I, Masahiro T, Teruyoshi I, Keizo S. Neurogenic tumors of the mediastinum originated from the vagus nerve. Eur J Surg Oncol 1995,21(2):214-6
15. Kilani T, Zaimi M, Labbene N, et al. Endobronchial neurogenic tumors. Ann Chir 1992;46(8):742-7.
16. Hsu HS, Wang CY, Li WY, Huang MH. Endotracheobronchial neurofibromas. Ann Thorac Surg 2002;74(5):1704-6.
17. Tsukada H, Osada H, Kojima K, Yamate N. Bronchial wall schwannoma removed by sleeve resection of the right stem bronchus without lung resection. J Cardiovasc Surg 1998;39(4):511-3.
18. Ribet ME, Cardot GR. Neurogenic tumors of the thorax. Ann Thorac Surg 1994;58(4):1091-5.
19. Ingels GW, Campbell DC, Jr., Giampetro AM, Kozub RE, Bentlage CH. Malignant schwannomas of the mediastinum. Report of two cases and review of the literature. Cancer 1971;27(5):1190-201.
TABLES
Table 1. Type of neurogenic intrathoracic tumors.
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Malignant schwannoma Neurofibrosarcoma |
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Malignant Paraganglioma Malignant Pheochromocytoma |
Table 2. Tumors type according to age of patients
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Nerve cell tumors |
Nerve sheath tumors |
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16 - 32 33 &emdash; 48 49 &emdash; 64 65 &emdash; 80 81- 96 Total |
21 35 25 12 1 109 |
1 0 0 0 0 6 |
0 0 0 0 0 1 |
7 0 0 0 0 15 |
8 28 22 11 1 70 |
3 4 2 1 0 11 |
1 2 1 0 0 4 |
1 1 0 0 0 2 |
NB: neuroblastoma GNB: ganglioneuroblastoma GN: ganglioneuroma
Schw: schwannoma NF: neurofibroma Mal. Schw.:malignant schwannoma
NFsarcoma: neurofibrosarcoma
Table 3. Circumstances of discovery according to age of patients
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(n=15) |
(n=94) |
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Associated disease
Thoracic symptoms
Neurologic signs
Abdominal symptoms
Radiologic discovery |
7 1 1 1 1 1(6.6%)
2 (13.3%) |
8 0 1 16 1 2 (2.1%)
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Table 4. Surgical approachs
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Postero-lateral thoracotomy |
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Antero lateral thoracotomy |
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L anterior transclavicular approach |
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Cervicotomy
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1 1 1 |
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Sternotomy
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1 |
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Laminectomy
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2 1 1 |
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Lombotomy |
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Shoulder disarticulation |
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The Figures
Fig 1. Chest radiograph (A) and CT scan (B) showing a thoracic inlet neurofibroma
Fig 3. Chest radiographies of a neuroblastoma
