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Journal of the Egyptian Nat. Cancer Inst., Vol. 17, No.

1, March: 1-8, 2005

Posterior Mediastinal Tumors: Outcome of Surgery
ABDEL RAHMAN M. ABDEL RAHMAN, M.D.; MOHAMED A. SEDERA, M.D.; ISMAIL A. MOURAD, M.D.; SHERIF A. AZIZ, M.D.; TAREK K.H. SABER, M.D. and MOUSTAFA A. ALSAKARY, M.Sc.
The Department of Surgery, National Cancer Institute Cairo University, Cairo.

ABSTRACT
Background and Purpose: The incidence of posterior mediastinal tumors relative to all tumors of the mediastinum is 23% to 30%. The posterior mediastinum is a potential space along each side of the vertebral column and adjacent proximal portion of the ribs. Primary tumors of posterior mediastinum are usually neurogenic. The aim of this study was to evaluate different surgical approaches used for the resection of posterior mediastinal tumors, and to assess morbidity, mortality and patients' survival. Patients and Methods: Between January 2001 and January 2004, 30 pateints with posterior mediastinal tumors were included. CT scan of the chest and CT guided biopsy were done for all patients; whereas MRI was done for suspected intraspinal extension. Posterolateral thoracotomy was the approach used in most of the patients. The Akwari approach was used in most of the patients with Dumbbell tumors. Results: Neurogenic tumors constituted 67% of cases, being neuroblastoma in 60%. The non neurogenic tumors included a heterogenous group of rare tumors (n=10). Dumbbell tumors were found in 10 patients. Neuroblastoma was the commonest tumor to cause intraspinal extension (40%). Wide local excision was done in 13 patients; whereas extended resection was done in the remaining 17 patients. The mean intra-operative blood loss was 800cc and the mean hospital stay was 12 days. The size of the resected tumor ranged from 3X4cm to 30X22cm, 80% of tumors were malignant. Morbidity in relation to the procedures developed in 8 patients (atelectasis, meningitis, paraplegia, Horner syndrome and mild wound sepsis in 4, 1, 1, 1 and 1 of the patients; respectively). One postoperative mortality, due to meningitis was recorded. The overall survival by the end of three years was 87.7% with a mean survival of 30.4 months. The overall disease free survival was 55.9% with a mean disease free survival of 26.2 months. Conclusions: Posterior mediastinal tumors may reach large size before becoming symptomatic. Complete surgical excision (including adjacent invaded organs) mainly by open technique should be the rule for these patients as there is survival benefit. Great care should be taken when dealing with Dumbbell tumors. Key Words: Posterior mediastinal tumor - Surgery.

INTRODUCTION The mediastinum is an anatomic region located in the center of the thorax, a simple and recent division is a three compartment model consisting of the anterior, visceral and paravertebral sulci. Here the posterior mediastinum is the potential space along each side of the vertebral column and adjacent proximal portion of the ribs [1]. Neurogenic tumors are the commonest posterior mediastinal tumors accounting for 19%-39% of all mediastinal tumors and 75% of all posterior mediastinal tumors [2]. These tumors are benign in 70%-80% of patients, of neural crest origin which give rise to peripheral nerves, including the sympathetic ganglia, paraganglia and schwann cells [3]. The remaining 25% of posterior mediastinal tumors are a heterogenous group of rare tumors including lymphoma, teratoma, sarcoma etc. In addition, many lesions arising outside the mediastinum may project into the posterior compartment and mesquarde as posterior mediastinal mass [4]. Approximately, 40% of mediastinal masses are asymptomatic, and are discovered incidentally on routine chest radiography. Symptoms are usually due to compression or direct invasion of surrounding mediastinal structures or due to paraneoplastic syndromes. Symptoms may include chest pain, cough, dyspnea or neurological abnormalities [5]. Asymptomatic patients are more likely to have benign lesions; whereas symptomatic patients are more often malignant [6]. CT scan of the chest is considered the primary diagnostic modality in patients with posterior mediastinal tumors, however; MRI has become the single best examination when intraspinal extension is suspected. MRI has the

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Thoracoscopy may be used for the diagnosis. The standard posterolateral thoracotomy was used in most of our patients. Survival data were calculated at the end of the study. to evaluate the different surgical approaches used for resection. and detect associated spinal cord pathology [7. Serum markers as VMA. Criteria for Eligibility were: • Any age and sex. • Forced expiratory volume in first second (FEV1) not less than 0. neurological examination and full laboratory investigations. Thoraco-abdominal approach through the bed of the 8th rib was used in 2 patients. Prolene mesh was used for chest wall and diaphragmatic reconstruction. The aim of this work was to study the demographic data of the patients. assessment. • Fitness for general anaethesia. Surgery. PATIENTS AND METHODS Between January 2001 and January 2004. Patients were followed up every 3 months. mortality and patients' survival. mostly by open technique is the main line of treatment for posterior mediastinal tumors. Wide local excision of the mass was done for localized disease in nearly half of the patients. Widening of the intervertebral foramen to deliver the intraspinal component was used in patients with small intraspinal part. These tumors require combined one stage resection by a team of thoracic and neurosurgeons to minimize morbidity and mortality of the procedure [12]. Kaplan-Meier procedure was used for survival estimation and Log rank test for comparing survival curves. Statistical package for social science (SPSS) was used for data analysis.2 Posterior Mediastinal Tumors: Outcome of Surgery ability to distinguish the spinal cord proper from other soft tissue masses within the spinal canal. Fifty percent of the patients were symptomatic. by clinical examination and CT of the chest and 6 monthly thereafter. was used for Dumbbell tumors. NSE were done for patients with neuroblastoma or peripheral neuroectodermal tumors (PNET). The tumors were more common in children (63. 15 patients had left . RESULTS The study group included 16 females (53%) and 14 males (47%). asses thecal sac impingement. MRI was requisted whenever needed. All patients with neuroblastoma or PNET received neo-adjuvant chemotherapy. 30 patients with posterior mediastinal tumors underwent surgery at the National Cancer Institute.8L.7: 1. and to assess morbidity. One stage combined anteriorposterior approach starting with laminectomy at one or multiple levels. Patients were classified into 2 groups. In the presence of a nearby structural invasion.3% of patients to rule out intraspinal extension. Postoperatively. vertebral body. All patients were subjected to full history taking. lung or diaphragm was done with subsequent appropriate reconstruction. Their age ranged from 7 months to 60 years with a median of 15 years. Cairo University.8]. respiratory symptoms were the commonest complaint. Right sided lesions were found in 14 patients. pediatric (<18y) and adult (>18y). have a reported incidence of about 10%. HVA. Good post-operative pain control was achieved by either thoracic epidural catheter or via intrapleural analgesia. Vertebral body reconstruction was done by autologous iliac bone graft for benign lesions and methylemethacrylate for malignant tumors with subsequent vertebral fixation by plate and screws.7%) with a ratio of 1. • Patients with only posterior mediastinal tumors diagnosed by CT scan of the chest • Pre operative tissue diagnosis. and 3 adult patients with rare tumors received preoperative radiotherapy before referal. mean and standard deviation described quantitative data and proportions described qualitative data.3%) than adults (36. 60% of symptoms related to intraspinal component [11]. • No paraplegia. Posterior mediastinotomy was used in one patient with an intraspinal tumor. aiming at complete excision [9]. patients were either followed-up or sent for adjuvant treatment according to the pathology. Tumors with spinal canal extension. and can be used for the resection of small tumors [10]. MRI was done in 83. extended resection including en-bloc excision of the mass with ribs. staging.

Abdel Rahman M. and 7 (70%) of 10 patients with spinal canal extension were detected pre-operatively by CT.16).3%) patients. the difference did not reach a statistically significant level (p=0. to the brain in 3. Of the 14 patients with rib invasion only. presence of spinal canal extension and the type of resection (simple versus extended resection). The overall survival by the end of three years was 87. atelectasis was the commonest (n=4). The non neurogenic group included 10 patients with heterogeneous rare tumors. 8). Analysis of the overall free survival showed no statistically significant difference among neurogenic and non-neurogenic groups (p=0. Dumbbell tumors were diagnosed in 10 (33. The overall disease free survival was 55. By the end of the study. respectively. 16 patients received chemotherapy (patients with pediatric neurogenic tumors). Post-operative pathology identified malignancy in 24 (80%) of patients.5%). liomyoma and angiomyolipoma.7%) were alive with disease. 22 patients (73. Eighteen patients (60%) had a tumor size of ≥10x10cm. they were re-explored. 5 (16. pediatric and adult groups (p=0. chondrosarcoma. Hospital stay ranged from 7 to 28 days with a mean of 12 days. vertebrae in 1 and one to the bone marrow. spindle cell sarcoma. according to their age (pediatric versus adult). One patient was lost for follow-up. Analysis of the disease-free survival showed that the group with no intraspinal extension had a significant higher disease free survival (66. . The Akwari approach using 2 separate median dorsal and thoracotomy incisions was used in 5 patients. while 7 patients received radiotherapy. as it detected all patients with intraspinal extensions (n=10). Intra-operative blood loss was in the range of 50cc-2500cc with a mean of 800cc. chordoma. Nine of the 10 patients with dumbbell tumors were malignant. 11 (78. re-operated upon with unsatisfactory results. and one patient suffered from a superficial wound infection. neuroblastoma was the commonest cause of spinal canal extension (4/10). thoracotomy with widening of the inter-vertebral foramen was used in 4 patients. neuroblastoma was the commonest (n=7).91). malignant schwannoma in 2. one with benign schwannoma and one with ganglioneuroma. Vertebral body invasion (n=4) was detected by CT scan.6%) patients.9% (Fig. 9). undifferentiated large cell tumor.05). Horner syndrome in 1. Four of 5 (80%) patients with lung invasion. (p=0. Abdel Rahman. desmoid tumor. MRI was ordered for 25 patients and was found to be more accurate than CT in the detection of intraspinal extension. CT guided biopsy was positive in 27 (90%) patients.6%) were detected by CT. carcinosarcoma. and the posterior approach using dorsal vertical incision was used in a patient with spindle cell sarcoma. Only one case of postoperative mortality was recorded as a result of meningitis. including one with each of the following histological subtypes. Figures. Postoperatively. 2 with stage II.7% (Fig. Although the group with no spinal canal extension showed a better overall survival. 3 sided lesions and one patient had chordoma presenting with bilateral disease. followed by ganglioneuroblastoma (n=5) [of the 12 patients. Types of resection and types of reconstruction were summarized in tables I and II.9%) compared to the group with spinal canal extension (30%). During the follow-up period. and complete tumor resection was done.3%) were alive disease free and two (6.6%) patients developed metastases. 94% versus 75% for the group with spinal canal extension. et al. paraplegia in 1 due to extradural hematoma.86). Three patients with neuroblastoma developed local recurrence. Although the disease-free survival in the group treated by simple resection (70%) was higher than the group treated by extended resection (45. Morbidity in relation to surgery developed in 8 (26. the difference did not reach a significant value (p=0. The tumor size ranged from 3x4cm and 30x22cm.66). PNET in 4.99) and the group treated by simple versus extended resection (p=0. 8 with stage III and 2 with stage IV]. (1-7) demonstrates some of our pateints. pathology (neurogenic versus non neurogenic). meningitis in 1. rhabdomyosarcoma. Two patients with PNET died after 6 month and one year postoperative. giant cell tumor. Neurogenic tumors constituted 67% of cases (n=20). The patients were classified into different groups.

3 3.3 100 Pathology PNET+ GNB + Ben.3 3.4 Table (1): Types of resection in study pateints. 3 1 1 1 1 Type of resection Rib resection Resection of D2 vertebra. Type of resection Simple resection Extended resection: Tumor + segment of 1 rib Tumor + segment of 2 ribs Tumor + partial 2 vertebrectomies Tumor + 3 ribs + partial 2 verteb Tumor + 4 ribs + total 1 Verteb Tumor + 3 ribs + total 2 verteb Tumor + 2 ribs + 2wedges of lung Tumor + lobectomy Tumor + 4 ribs + upper lobectomy Tumor + 3 ribs + wedge lung excision Tumor + 3 ribs + part of diaphragm Total Number 13 17 3 4 1 1 1 1 1 2 1 1 1 30 Posterior Mediastinal Tumors: Outcome of Surgery % 43.6 3.3 56. Resection of D6. Large cell carcinoma PNET Table (2): Types of reconstruction in study patients. . No.3 6.3 3.3 3.3 3. (1): MRI of a spindle cell sarcoma. (2): Post operative reconstruction.schwannoma 3NB+ desmoid tumor Chordoma Spindle cell sarcoma Chondrosarcoma Giant cell tumor PNET Carcinosarcoma+Malig.3 3. Schwannoma Malignant schwannoma Undiff.7 10 13.3 3. Fig.7 vertebrae Type of reconstruction Double layer prolene mesh repair Methyl methacrylate + plates & screws fixation Iliac bone graft + plates & screws fixation Pathology PNET + 2 Neurolastoma Chondrosarcoma Giant cell tumor Spindle cell sarcoma PNET 3rib + 2partial vertebrectomies Plates & screws fixation Diaphragmatic resection Prolene mesh repair Fig.

Abdel Rahman M.2 Cummulative survival 1( 0. 5 Fig. (4): CT of posterior mediastinal neuroblastoma post chemotherapy. Fig.8 0. 1. Fig. (8): The overall survival of all patients. 1 6 12 Months 20 30 36 ( ( ( Fig. . et al. (3): CT of posterior mediastinal neuroblastoma pre chemotherapy.4 0. (5): Operative specimen. Abdel Rahman. Fig. (7): Operative veiw with 3 ribs resected.2 0 0 Fig.6 0. (6): Posterior mediastinal schwannoma.

Similarly. The most common presentation in our patients was respiratory symptoms (50%). Sixty five percent of our patients with dumbbell tumors were symptomatic. Our patients with thoracic neuroblastoma had a 3 years actuarial survival of 100%.6% and a palpable mass in 3. [15] and Naidich et al. Thoracic neuroblastomas usually have a better survival than abdominal. Similarly. The high incidence of recurrence in our patients was due to high incidence of 0 1 6 10 12 14 16 Months 18 20 24 36 Fig. respiratory symptoms reported in 45% of patients. In the present series.9 months. Recent chromosomal studies for neuroblastomas have shown that thoracic lesions are less likely to express N-myc oncogene amplification.6 0. Posterior mediastinal tumors were neurogenic in origin in 67% of our patients. which was higher than that reported in other studies.2 1( Cummulative survival 0.6% in detection of chest wall invasion and 80% in detection of lung invasion. The overall survival of our patients with spinal canal extension was 75% with a mean survival of 25. Most series reported a diagnostic yield of per-cutaneous needle biopsy in the range of 72%-100% [18] more recently this figure is in excess of 90% [19]. Akwari and associates [11] reported that approximately 10% of all posterior mediastinal neurogenic tumors have intraspinal extension. This amplification has been associated with a poor survival [20]. (9): Disease free survival in all pateints. Askin and colleagues [22] reported a median survival of only 8 months in their PNET cases. autonomic ganglia tumors (65%) and neuroblastoma were diagnosed in 12 (60%) patients. This was comparable with our results as 2 of 4 patients with PNET died 6 and 12 months after surgery. These data were comparable to the present results.17]. DISCUSSION Posterior mediastinal tumors are commonly neurogenic in origin.2 0 Posterior Mediastinal Tumors: Outcome of Surgery ( ( ( ( ( ( ( ( ( with 54% of patients diagnosed as neuroblastoma [13. The higher incidence of malignancy in our patients may be explained by the fact that the National Cancer Institute is a referral center for malignancy especially in the pediatric age. 3 patients developed recurrent disease. The 5 years survival rate for thoracic neuroblastoma was reported to be greater than 70% [21]. Benign tumors predominate in adults.14]. In a retrospective analysis of 16 patients with mediastinal dumbbell tumors [24]. while malignant ones are more common in infants and children. neurological in 6. Similarly. neurological symptoms in 13% and a palpable mass in 5%. Saenz and colleagues [13] and Ribet and Cardot [14] reported similar results.6 1. This higher incidence of dumbbell tumors in our patients may be due to higher incidence of malignancy. all of them belong to the pediatric age group. . 80% of cases were malignant. Previous studies recorded that 60% of patients with posterior mediastinal tumors were malignant and malignancy predominates in infants and children. results obtained in the present series showed CT sensitivity of 78. In the present series. but with a high recurrence rate (25%). CT guided biopsy was diagnostic in 90% of patients.3% of patients presented with dumbbell tumors. other studies reported neurogenic tumors in 75% of their patients [16. as 90% of patients with dumbbell tumors were malignant. 33.7% of patients were stage III at the time of diagnosis. On the other hand.4 0. Suzuki et al. there were 14 benign and only 2 malignant lesions and no recurrence was observed during a follow-up period from 2 months up to 28 years. [8] reported that sensitivity of CT scan in the detection of chest wall invasion was in the range of 60%79% and in the detection of lung invasion was 70%-80%. because 66. Grillo and Ojmann [23] reported symptoms in 60% of their patients. long term survival of patients with PNET is infrequent.8 0.3%.

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