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Eur J Cardiothorac Surg 2004;26:807-812
© 2004 Elsevier Science NL
Department of Surgery Course of Interventional Medicine (E1), Graduate School of Medicine, Osaka University, Yamada-oka 2-2, Suita City, Osaka 565-0871, Japan
Received 22 April 2004; received in revised form 18 June 2004; accepted 1 July 2004.
* Corresponding author. Present address: Department of General Thoracic Surgery, Toneyama National Hospital, Toneyama 5-1-1, Toyonaka City, Osaka 560-8552, Japan. Tel.: +81-6-6853-2001; fax: +81-6-6850-1750. (E-mail: stakeda{at}toneyama.hosp.go.jp).
| Abstract |
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| 1. Introduction |
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| 2. Materials and methods |
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2.2. Statistical analysis
Comparison for categorical values of two groups was made by
2-test (StatView 5.0 Abacus Concepts, Berkeley, CA). Statistics was admitted for any P value less than 0.05.
| 3. Results |
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Table 1 and Fig. 1 show the classification of pathologic types of tumors and their distribution by age. There were 51 ganglioneuromas (34.9%), 37 schwannomas (25.3%), 30 neurofibromas (20.5%), 18 neuroblstomas (12.3%), 5 gangliobastomas (3.4%), 2 primitive neuroectodermal tumor (PNET), and 3 were other types. Nerve cell tumors such as ganglioneuromas or neuroblastoma comprised the majority seen in pediatric patients, and benign ganglioneuroma also tended to occur in the younger age group, whereas nerve sheath tumors such as schwannoma or neurofibroma appeared less frequently in children than adults (Fig. 1). Fig. 2 shows the malignancy rate by age group. Overall, 30/146 (20.5%) of neurogenic tumors were malignant and those occurred predominantly in the first 5 years of life. The malignancy rate of patients under 5 years, 514-years-old, and over 15-years-old were 80.0, 14.3, and 5.7%, respectively.
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For these patients, surgical excision was definitely diagnostic and therapeutic. Removal was performed via a posterior or axillary thoracotomy, which was chosen with regard to tumor size, location, and extension until video-assisted thoracic surgery (VATS) was introduced. A complete resection was performed for 111/116 (94.9%) benign tumors including 13 VATS resections, and 19/30 malignant tumors (63.3%), including a laminectomy for six cases of the dumbbell-type. In these six cases (three right and three left), intraforaminal portion was resected by the orthopedic surgeon. In a patient with preoperatively undiagnosed pulmonary mass (Fig. 3), the intraparenchymal tumor was found to be a typical schwannoma of Antoni A type with a palisade arrangement. There were no operative deaths and minimal morbidity, including transient or mild Horner syndrome after resection of superiorposterior mediastinal tumors. There were no tumor-related death in 96 patients with benign tumors who were followed-up for more than 5 years.
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Regarding the malignant neurogenic tumors in children, preoperative stage according to the Evans et al. [6] was as follows: stage I, 9; stage II, 5; stage III, 4; stage IV; 5. Seven early staged patients, who received complete resection, survived more than 5 years. Patients who underwent incomplete resection or biopsy showed poor prognosis. Adjuvant chemotherapy was given to patients with advanced stage neuroblastoma and PNET. Recently induction chemotherapy was given to 3 patients with neuroblastoma, followed by surgery. In a case of PNET (Fig. 4), high-dose chemotherapy was done with support of auto bone marrow transplantation, after complete resection of the tumor. However, the tumor recurred in the right thorax 4.5 years after surgery and the patient died 6 months later despite the salvage chemotherapy. Radiation therapy was employed for adult patients with malignant schwannoma after resection.
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| 4. Discussion |
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Neurogenic tumors are classified as nerve cell (ganglion) tumors, such as ganglioneuroma and neuroblastoma, nerve sheath tumors, including schwannoma and neurofibroma, and paraganglionic tumors. In our series of Japanese patients, paraganglionic tumors were rare, as we had only one case of malignant pheochromocytoma. Similar to previous reports from western countries [2,7,8], nerve cell tumors were more common in children than in adults (Fig. 1). Further, the malignancy rate was higher in infants and young children, at 100% under 2 years of age, 71% for patients 24 years of age, and 16% for those 514 years of age, which was quite similar to a European series [2]. In contrast, nerve sheath tumors were more common in adults (78%) than children (3.3%). Schwannoma was the most common neurogenic tumor seen in adults, however, it was not observed in any patients under 15 years of age in our series. Recklinghasuen nerofibromatosis is an uncommon genetic disease that results in the presence of multiple neurogenic tumors. We encountered only two patient with this condition, and one of them had a malignant schwannoma. Ribet and Cardot [2] noted that only 14.1% of their patients with thoracic neurogenic tumors were associated with Recklinghasuen nerofibromatosis, whereas approximately half of adult patients with malignant neurogenic tumors were reported to have this disease [9]. As mentioned previously, we experienced two cases of PNET. Askin and colleagues [10] first described malignant small cells in the thoracopulmonary region during childhood that may be presented as a chest wall or paravertebral mass. Using electromicroscopical analysis, they identified neurosecretary granules and a cell process suggesting neuronal differentiation. This is now categorized as PNETs. Clinically, PNET are seen in older children or adolescents with female predominance. Our patient showed a typical outcome in comparison to previous reports [10].
Symptoms associated with neurogenic tumors are varied and listed in Table 2. Horner's syndrome was found with some tumors, particularly those in the upper mediastinum. There were few respiratory symptoms, such as dyspnea or cough, found related to tumor enlargement, however, gait disturbance (Ataxia), a peculiar symptom, was prominent in patients with neuroblastoma. Since a neuroblastoma produce cathecholamines, detection of urinary secretion of these by-products, including vanillylmadelic acid (VMA) and homovanillylmadelic acid (HVA), is utilized for mass screening for asymptomatic patients [11], and the oncogene c-myc is also being evaluated. We experienced only a single patient with neuroblastoma that also exhibited severe diarrhea caused by vasoactive intestinal peptide.
Preoperative evaluations were done by radiographic imaging alone in general, except for cases of suspected adult malignant lesions, which were examined by needle aspiration biopsy. Conventional chest X-ray was an initial step for diagnosis even this series encompassed over a half decade. CT scan and MRI techniques have greatly enhanced the accuracy of diagnosis for mediastinal and chest wall lesions as well as distinguishing them from cystic lesions. A radiologically identified dumbbell tumor was seen in 8.9% of the patients in the current series. MRI allows precise identification with the involvement of spinal cord by a tumor; thus, it should be performed preoperatively for patients with suspected neurogenic tumors to exclude intraspinal extension. In addition, evaluation of the Adamkiewicz should be performed to avoid spinal cord injury in dumbbell tumors located in the lower posterior mediastinum [12]. A routine check-up of the abdominal cavity has not established in the diagnostic and therapeutic modalities for benign mediastinal tumors. However, the coexistence of retroperitoneal neurogenic tumors, though rare, should be recognized during the management of patients with a mediastinal neurogenic tumor.
Watchful observation of mediastinal and intrathoracic neurogenic tumors is rarely justified, since surgical removal alone leads to definite diagnosis as well as provides therapy. Intrathoracic neurogenic tumors are essentially amenable to primary surgical therapy. We observed one patient over 5-year period, since she had been treated for post-renal transplant chronic rejection. However, it was hard to remove the tumor via a transthoracic or VATS approach at surgery, because of a foramenal extension.
Benign intrathoracic neurogenic tumors are good candidates for VATS resection, which can be achieved safely and effectively with rapid recovery and less pain resulting in shortening hospital stay [13]. In particular, we applied a thoracoscopic approach assisted with a fine manipulation through the supraclavicular fossa [14] for removal of the superior mediastinal mass.
On the other hand, surgery for dumbbell-type tumors is more complex, as it requires cooperation with an orthopedic surgeon. Akwari and associates [15] first reported that 9.8% of their patients with neurogenic tumors had an extension through an intervertebral foramen. We encountered 13 (8.9%) patients with dumbbell-type out of 146 patients with neurogenic tumors, and one third of them had complaints due to spinal compression. Six patients received a one-stage combined posterolateral thoracotomy and concomitant laminectomy [15,16] in the current series. We recently used a posterior approach in two cases with the patient in the prone position, in which a laminectomy and extradural dissection were done by an orthopedic surgeon assisted with VATS dissection via the thorax, as reported by McKenna et al. [17].
Neuroblastoma remains a challenge to the pediatric surgeons in terms of the multimodality therapy. Recently induction therapy followed by surgical resection was done in our series. The evidence that prognosis of mediastinal neuroblastoma was better than other neuroblastoma may support the potential role of the complete resection when possible [18].
In summary, we reviewed our institutional experience with intrathoracic neurogenic tumors, with emphasis on the clinical spectrum of the disease, as well as diagnosis and treatment. The majority of these tumors, whether benign or malignant, were asymptomatic, yet easily diagnosed using current radiologic imaging techniques. Age may be the most important clinical parameter for distinguishing between histological type and rate of malignancy. In the adult, benign neurogenic tumors can be treated by VATS resection, however, operative approach for the dumbbell tumors should be considered with orthopedic surgeons. Malignant neurogenic tumors in children need multimodality therapy, however, complete resection remains a crucial role. Recognition of the clinical spectrum of neurogenic tumors can lead to immediate and proper surgical intervention.
| Acknowledgments |
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| Footnotes |
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1 Present address: Department of Thoracic Surgery, Dokkyo Medical College, Tochigi 321-0293, Japan. ![]()
| References |
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