Mediastinal Neurogenic Tumors Research Articles

Posterior Mediastinal Schwannoma Invading Into the Spinal Canal: A Rare Case Report and Review of Literature

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 1 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Schwannoma constitutes about 75% of benign neurogenic tumors found in the posterior mediastinum. Only 9.8% of these neurogenic tumors extend into the intervertebral foramina (dumbbell tumors). We are describing a case of a large 11cm right sided posterior mediastinal schwannoma extending into the spinal canal with no significant neurological compromises. CASE PRESENTATION: A 30-year-old ten-day post-partum Caucasian female presented with acute onset of severe right-sided back pain. She had a history of asthma, systemic lupus erythematosus, Antiphospholipid antibody syndrome and pre-eclampsia during her recent pregnancy. She was a life-long non-smoker with no family cancer history. Physical exam revealed no spinal or flank tenderness. She was noted to have mild leukocytosis and anemia. Chest X-ray revealed a large mass in the posterior mediastinum pressing on the neural foramen [Figure 1]. Computerized tomography and magnetic resonance imaging scans of chest confirmed the presence of a well-defined heterogenous 11cm mass extending into the right T3-T4 neural foramen associated with mass effect exerted upon the mid and distal airway. Diffused ground-glass opacities were noted in all pulmonary lobes [figure 2]. Bronchoscopy revealed no mass in the main bronchus. Trans-tracheal biopsy of the right lung mass was performed with histological confirmation of Schwannoma. DISCUSSION: Differential diagnoses for a large posterior mediastinal mass includes lymphoma, neurogenic tumors, or primary malignancy. Flow cytometry was negative for monoclonal expansion of B or T cell lymphocytes. Diffused ground-glass opacities were attributed to pulmonary edema in setting of a recent pregnancy complicated by pre-eclampsia. Flow volume loop did not show any evidence of airway obstruction. Most schwannomas invading into the spinal canal are asymptomatic as per our literature review. Erosions of vertebral pedicles or the enlargement of the intervertebral foramen is the radiographic evidence of tumor extension into the spine. Surgical resection is the treatment of choice for the management of posterior mediastinal schwannoma. Minimal invasive video-assisted thoracoscopic surgery is an alternative treatment option that is reported relatively safe, more reliable and less morbidity compared to open surgery. CONCLUSIONS: Schwannoma involving the posterior mediastinum can present with an impressive radiographic finding. Schwannoma extending into the spinal foramen is a relatively rare phenomenon. Reference #1: Akwari OE, P. W. (1978). Dumbbell neurogenic tumors of the mediastinum. Diagnosis and management. Mayo Clinic Proceedings, 53(6), 353-358. Reference #2: Onakpoya UU, A. B. (2017, Jan-Feb). Large posterior mediastinal schwannoma in a 45-year-old woman. Lung India, 109-111. Reference #3: Negri G, B. A. (2013, March). Unusual Presentation of Mediastinal neurogenic Tumours. Case Report in Surgery, 2013. DISCLOSURE: The following authors have nothing to disclose: Hau Chieng, Biplab Saha, Muhammad Imtiaz No Product/Research Disclosure Information

Assessment of paraspinal neurogenic tumors with diffusion-weighted MR imaging.

To assess paraspinal neurogenic tumors with diffusion-weighted MR imaging. Retrospective analysis was done upon 34 patients with paraspinal neurogenic tumors that underwent diffusion-weighted MR imaging. The ADC values of the mediastinal neurogenic tumors were calculated and correlated with biopsy results. The ADC of benign paraspinal neurogenic tumors (1.5±0.28×10-3mm2/s) was significantly higher (P=0.001) than that of malignant peripheral nerve sheath tumors (0.995±0.198×10-3mm2/s). Selection of 1.15×10-3mm2/s as a cut-off point for differentiating malignant from benign neurogenic tumors revealed an area under the curve of 0.885, an accuracy of 91.1%, a sensitivity of 90.9%, and specificity of 91.3%. There was significant difference (P=0.04) in the ADC of schwannomas (1.55±0.29×10-3mm2/s) from neurofibromas (1.33±0.08×10-3mm2/s). The cut-off ADC value of 1.44×10-3mm2/s was used to differentiate schwannomas and neurofibromas with an area under the curve of 0.86, an accuracy of 82.6%, a sensitivity of 100%, and a specificity of 76.5%. Diffusion-weighted MR imaging is imaging parameter that can be used for differentiation of benign from malignant paraspinal neurogenic tumors.

P2.04-044 Mediastinal Neurogenic Tumors: Histopathological Characteristics and Surgical Treatment in a Single-Institutional Experience: Topic: Esophageal Cancer and Other Malignancies

Intrathoracic neurogenic tumors are uncommon and typically originate from the peripheral nerves, paraganglionic nerves, or the autonomic nervous system. They are commonly found in the mediastinum, especially in the posterior mediastinum and have a variety of clinical and histological features. Mediastinal neurogenic tumors in adults are generally benign lesions. We retrospectively reviewed our institutional experience of mediastinal neurogenic tumors from 2010 to 2015. The patients were evaluated according to age, gender and histological characteristics of the tumor. There were 78% males and 22% females diagnosed with mediastinal neurogenic tumors. Mean age was 48.4±12 years. Distribution according to the histopathological diagnosis was: 56% schwannoma, 22% malignant schwannoma, 22% ganglioneuroma. The operative procedure performed in all cases was tumor extirpation through thoracotomy. In 10% of cases, presence of intraspinal growth was encountered (the so-called “dumbbell tumors”), thus hemilaminectomy was performed. There were no operative deaths and minimal morbidity. Mean postoperative stay was 5 days. In this study, the most common mediastinal neurogenic tumor found was schwannoma. Neurogenic tumors arising in the mediastinum are generally of benign nature and mostly found in males. The treatment of choice for malignant and benign mediastinal neurogenic tumors is complete resection for the purposes of avoiding local invasion, facilitating differential histopathological diagnosis and preventing malignant degeneration. The surgical management of the dumbbell tumors differs from others.

Tumeurs neurogènes du médiastin de l’adulte

Neurogenic mediastinal tumors are tumors develop at the expense of peripheral nerve elements of the mediastinum, the sympathetic nervous tissue and paraganglia. The series that we propose has the distinction of a high frequency of malignant forms and a wealth of clinical symptomatology. This is a retrospective study of 9patients collected over a period of 6years. The epidemiological aspect, clinical, radiological, surgical treatment and his results, histological diagnosis and prognosis were reviewed. Five women and 4men were identified. All patients were symptomatic at diagnosis. Tumor resection was complete in 8cases. In 3patients, there was a malignant tumor. Two of them had received chemo-radiotherapy. The others showed good clinical progress with a mean of 35months. Neurogenic tumors in adults are usually discovered incidentally. When the malignant forms predominate or in the presence of large tumor, the symptoms can be quite marked. In all cases the treatment based on a complete surgical resection remains the only guarantee of a good prognosis.

Diagnosis of Mediastinal Schwannoma With Endoscopic Ultrasound-Guided Fine Needle Aspiration

Introduction: Endoscopic Ultrasound (EUS) is a minimally invasive modality that can be used for the evaluation and diagnosis of most mediastinal lesions. We present here an uncommon use of EUS and fine needle aspiration (FNA) for the diagnosis of a mediastinal Schwannoma. Case: A 66 y/o man with newly diagnosed prostate cancer underwent CT scan of the chest and abdomen for staging which revealed a 2.5 cm mass in the lower mediastinum. Evaluation by linear scanning EUS revealed a 35mm x 29mm, partially cystic heterogeneous mass adjacent to the distal esophagus and FNA was performed. Four passes were made with a 22 gauge needle under doppler guidance. No other lesions were identified and patient tolerated the procedure well. Cytopathology revealed bland spindle cells that stained positive for S-100 but negative for CD117, Actin and Desmin which is consistent with a Schwannoma. Discussion: Schwannomas are the most common neurogenic mediastinal tumors arising from peripheral nerves. They are typically slow-growing, benign and asymptomatic. They are generally found incidentally on imaging. Imaging may reveal calcifications, cysts and hemorrhage. It is important to differentiate Schwannomas from other mediastinal tumors. Resection is the treatment of choice as they could grow over time and also because of low risk of malignant transformation. The esophageal relationship with the surrounding structures makes EUS-FNA an ideal diagnostic tool for mediastinal tumors. The use of doppler allows for avoidance of blood vessels and safe FNA of lesions. EUS-FNA has been shown to be 97% sensitive and 100% specific for malignant mediastinal lesions1; benign lesions can also be accurately diagnosed. Conclusions: To our knowledge, there have been only two previously reported cases utilizing EUS-FNA for the diagnosis of a mediastinal schwannoma2,3. Most mediastinal lesions are easily and safely accessible with EUS-FNA and the modality has high diagnostic accuracy. This approach should be considered before more invasive mediastinoscopy is perfomed for diagnosis.

171P - Surgical Treatment of Mediastinal Neurogenic Tumors and Dumbell Syndrome in Adults: a 12-Year Experience, January 2002 to January 2014

171P - Surgical Treatment of Mediastinal Neurogenic Tumors and Dumbell Syndrome in Adults: a 12-Year Experience, January 2002 to January 2014

EComment. Is video-assisted thoracoscopic surgery resection really superior to the thoracotomic approach in mediastinal neurogenic tumours?

eComment. Is video-assisted thoracoscopic surgery resection really superior to the thoracotomic approach in mediastinal neurogenic tumours?

Mediastinal Hydatid Cyst Mimicking Malignant Mediastinal Neurogenic Tumor

Hydatid disease is caused by Echinococcus granulosus parasite. It is an endemic disease; particularly in many Mediterranean countries. The liver and the lungs are most frequently involved. Bone involvement is reported in 1% - 2% of the cases and about 50% of those are seen in the spine. Herein we report a case of primary spinal extradural hydatid cyst that caused paraplegia due to compression of the dorsal spinal cord and was diagnosed initially as case of lumber prolapsed intervertebral disc. The cyst was only discovered when the patient had progressive paraplegia and the preoperative provisional diagnosis was posterior mediastinal neurogenic tumor causing destruction of the pedicle and lamina of the fifth thoracic vertebra. Fortunately, she regained near full power after surgical treatment of her spinal cyst.

Anterior Trans Cervicothoracic Approach for Complete Resection of Cervicothoracic Mediastinal Neurogenic Tumors

Introduction:Neurogenic mediastinal tumors comprise a wide range of benign and malignant diseases. A group of these tumors, located at thoracic apex, sometimes spread to cervical spaces causing numerous surgical difficulties. In thoracotomy approaches, due to proximity of the tumors to major blood vessels, complete removal of these tumors from cervical spaces is impossible or may cause intraoperative severe bleeding or other dangerous incidents Because of the adjacent major vessels that are not visible.The aim of this study is to report cases of surgical treatment of such tumors using Anterior Trans Cervicothoracic Approach (ATCA). Materials and Methods:All patients with neurogenic tumors and cervicomediastinal (CM) spread who underwent surgey with ATCA technique during 2005-2011 were included in our study. Then they were evaluated in terms of age, sex, clinical symptoms, radiological and pathological findings, technical success rate of the surgery, surgical complications and first-year relapse rate after the surgery. Results:Our study included 10 patients from whom 9 were female and 1 was male (M/F= 1/9) and the mean age was 27 years. The most common symptoms were pain and feeling of a lump. All patients were operated by this technique successfully. The most common pathological finding was neurofibroma (in 5 patients) and surgical complications occurred in 2 patients (20%) (Wound infection in 1 patient and brachial plexus injury in another patient). There was no mortality. Disease relapse was reported in 1 patient ganglioneuroblastoma who underwent surgical resection for the second time. Conclusion: Considering the successful removal of the tumors and favorable exposure of major vessels in cervicomediastinal spaces, this technique is recommended to resect mediastinal tumors with spread to cervical spaces. However, a more definite conclusion requires further studies.

Thorascopic Resection of An Apical Paraspinal Schwannoma Using the da Vinci Surgical System

Posterior mediastinal neurogenic tumors have traditionally been resected via an open posterolateral thoracotomy. Video-assisted thorascopic surgery has emerged as an alternative technique allowing for improved morbidity with decreased blood loss, less postoperative pain, and a shorter recovery period, among others. The da Vinci surgical system, as first described for urologic procedures, has recently been reported for lung lobectomy. This technique provides the advantages of instrumentation with 6 degrees of freedom, stable operating arms, and improved visualization with the three-dimensional high-definition camera. We describe the technique for thorascopic resection of an apical paraspinal schwannoma of the T1 nerve root with the da Vinci surgical system. This technique used a specialized intraoperative neuromonitoring probe for free-running electromyography (EMG) and triggered EMG. We demonstrate successful resection of a posterior paraspinal schwannoma with the da Vinci surgical system while preserving neurologic function. The patient displayed stable intraoperative monitoring of the T1 nerve root and full intrinsic hand strength postoperatively. The technique described in this article introduces robotic system accuracy and precludes the need for an open thoracotomy. In addition, this approach demonstrates the ability of the da Vinci surgical system to safely dissect tumors from their neural attachments and is applicable to other such lesions of similar size and location.

Extramedullary Hematopoiesis at the Posterior Mediastinum in Patient with Hereditary Spherocytosis: A Case Report

Thoracic extramedullary hematopoiesis (EMH) is a rare disease entity that is usually associated with hematologic disorders, such as myelodysplastic or hemolytic disease. Because thoracic EMH is usually encountered as a mass during radiologic examinations, it should be differentiated from posterior mediastinal neurogenic tumors. Here, the authors report a case of EMH associated with hereditary spherocytosis. The patient underwent a complete excision by thoracoscopic surgery to differentiate it from other mediastinal tumors.

Unusual Presentation of Mediastinal Neurogenic Tumours

Mediastinal neurogenic tumours generally arise as single benign lesions and their typical location is the costovertebral sulcus. In about 10% of cases mediastinal neurogenic tumours may extend to the spinal canal; occasionally they may extend to the cervical region and, more rarely, may be multiple or associated with other synchronous mediastinal lesions. The treatment of choice is surgical resection. This report describes three cases of unusual presentation of mediastinal benign schwannomas successfully treated at our Hospital. In the first case multiple simultaneous paravertebral lesions were resected through a posterior approach. In the second case a tumour of the posterior mediastinum extending to the cervical region was excised through a one-stage combined supraclavicular incision followed by left mini-invasive video-assisted thoracoscopic surgical techniques. The third case describes a patient with a posterior neurogenic mediastinal tumour with a synchronous parathyroid adenoma of the anterior mediastinum, which were both successfully resected by video-assisted thoracoscopic surgery.

A Case of Mediastinal Vagal Schwannoma Diagnosed by EUS-FNA

Purpose: Schwannomas, also known as neurilemmomas, are peripheral nerve sheath tumors that arise from any nerve in the body although they most commonly occur in the soft tissues of the head and neck, as well as extremities. Schwannomas originating from the vagus nerve are extremely rare, accounting for only 2% of all mediastinal neurogenic tumors. Typically vagal schwannomas are asymptomatic but at times present with hoarseness, chest pain, or cough. We present a case of a paraesophageal vagal nerve schwannoma that was diagnosed incidentally during workup of a patient with abdominal pain. Case Report: A 36-year-old male presented with right upper quadrant abdominal pain. Initial labs and abdominal ultrasound were non diagnostic. EGD showed mild gastritis and a bulge at the GE junction. Radial EUS revealed a 3.7 cm × 2.5 cm hypoechoic paraesophageal mass best seen from the GE junction. Linear EUS with FNA showed spindle cells mixed in fascicles in a loose stroma. The spindle cells were strongly positive for S-100 protein and negative for CD34, DOG-1, desmin, smooth muscle actin and CD 117c (C-kit). These findings were consistent with a schwannoma. CT scan of abdomen showed no other masses or lymph nodes. On laparoscopy it was found to be stemming from the right vagus nerve. A segment of the vagus nerve with the mass was resected in its entirety. Histopathological evaluation confirmed the diagnosis and showed no invasive component and no mitotic activity. Discussion: Although there have been case reports describing schwannomas it is often not considered in the differential diagnosis of mediastinal masses. They are often misdiagnosed as GISTs or leiomyoma. They lack specific features on Ultrasound, CT and MRI and hence we depend on pathology to make the diagnoses. With FNA capabilities EUS has emerged as an excellent tool to make the diagnosis. Some authors on EUS have identified a marginal halo in schwannomas. They also suggest that its echogenecity is much lower than the normal surrounding muscle layers. The marginal halo corresponds to a lymphoid cuff that is often present in this tumor (1). These findings if present and if validated by more cases may be useful to differentiate schwannomas from GISTs or leiomyomas. Although they are very rare, schwannomas of the vagus nerve should be included in the differential diagnosis of mediastinal masses, together with other neurogenic tumors, lymphomas, cystic lesions and esophageal tumors.

Experience of video-assisted thoracoscopic resection for posterior mediastinal neurogenic tumours: a retrospective analysis of 58 patients

The objective of this study was to review the experience of video-assisted thoracoscopic resection of posterior mediastinal neurogenic tumours and to investigate the technical features and difficulties of this thoracoscopic approach. From May 2001 to June 2011, 58 consecutive patients underwent thoracoscopic resection of posterior mediastinal tumours sequentially in our institution, including 36 males and 22 females. The median age of the patients was 38.7 years. The median tumour size was 4.9 cm. Twenty-four lesions were located at the left side, 33 lesions at the right side and 1 lesion at the bilateral side. All procedures generally required three ports, and intracapsular enucleation was preferred. For bulky tumours, dense adhesion and massive bleeding, conversion to thoracotomy was performed by extending the anterior incision to 6-10 cm. All procedures were successfully performed without death or serious complications occurring. The average operating duration was 127.2 min. The average intraoperative blood loss was 206.4 mL. The average chest tube drainage duration was 2.72 days. The average post-operative stay was 5.19 days. Fifty-three procedures were performed entirely under thoracoscopy to achieve gross total resection. There were five cases (8.6%) of conversion to thoracotomy procedure. Seven patients suffered from post-operative complications, including four Horner syndromes. No local recurrence occurred after an average follow-up of 44.9 months. Video-assisted thoracoscopic resections of the posterior mediastinal tumours were safe and reliable for selected patients with mastered thoracoscopic skills. Intracapsular enucleation was demonstrated to be a safe procedure. For tumours larger than 6 cm and located in the apex, the risk of the operation increased significantly.

Video-assisted thoracic surgery resection for pediatric mediastinal neurogenic tumors

Background/PurposeVideo-assisted thoracoscopic surgery (VATS) resection of mediastinal neurogenic tumors is still controversial in children. The aim of this study was to review the cases of VATS resection of such tumors in children from 3 institutions located in different countries. MethodsThis retrospective study included 17 children treated between July 1995 and February 2011. Medical charts were reviewed for collection of data on age, sex, histologic type of tumor, clinical manifestations, age and weight at surgery, tumor size, duration of thoracic drainage, surgical complications, tumor recurrence, and mortality. ResultsThirteen (76.5%) males and 4 (23.5%) females were studied. Median age was 16 months (range, 10.6-60 months), and median weight was 11.9 kg (range, 9.3-27.4 kg). Ten children had neuroblastoma (58.8%), 4 had ganglioneuroma (23.5%), and 3 had ganglioneuroblastoma (17.7%). The median duration of the operation was 90 minutes (range, 45-180 minutes), with complete thoracoscopic resection in all cases. Two children (11.8%) developed Horner syndrome postoperatively. No deaths were reported, and no recurrence was noted during a median follow-up period of 16 months (range, 8.9-28.6 months). ConclusionsVideo-assisted thoracoscopic surgery resection of mediastinal neurogenic tumors in children produced good results, with no recurrence and minimal postoperative complications. The major advantages of this approach are the avoidance of thoracotomy complications and the enhanced surgical accuracy provided by improved visualization.

EBUS-TBNA in the Workup of a Mediastinal Mass

Guided by endobronchial ultrasound (EBUS), mediastinal lymph nodes can be reached in a safe, minimally invasive manner, allowing fine needle aspiration for cytologic diagnosis with a high sensitivity and specificity. In describing the following clinical case, we demonstrate the use of EBUS-transbronchial needle aspiration (TBNA) in the workup of a paratracheal mass in a young female patient. Immunocytochemical analysis revealed it to be a peripheral nerve sheath tumor. EBUS-TBNA complemented computerized tomography, fluoro-deoxy-glucose positron emission tomography, and magnetic resonance imaging in establishing the diagnosis of this infrequently encountered mediastinal neoplasm. Fluoro-deoxy-glucose positron emission tomography has a potential to discriminate between high-grade sarcoma and benign soft tissue tumors, but it remains unreliable to differentiate benign schwannoma from low-grade sarcomas such as malignant peripheral nerve sheath tumor. When properly prepared, cell blocks obtained from TBNA of a paratracheal mass offer the possibility of cytologic examination and immunocytochemical staining, confirming the diagnosis of mediastinal neurogenic tumors.

Intrathoracic ganglioneuroma surrounding left vertebral and subclavian arteries successfully resected by modified trapdoor thoracotomy

Neurogenic tumors are derived from tissue of the neural crest. Most of the mediastinal neurogenic tumors occur in the posterior compartment. Extension to the cervical area and the surrounding major arteries is rare. We report a patient with a ganglioneuroma arising from the brachial plexus, extending into the cervical region, and surrounding the left vertebral and subclavian arteries. The patient underwent successful resection by modified trapdoor thoracotomy.

Preferred surgical procedure for posterior mediastinal neurogenic tumor.

To compare the outcomes of video-assisted thoracoscopic surgery (VATS) with those of thoracotomy for the removal of posterior mediastinal neurogenic tumors. Twenty-four patients were enrolled in the VATS group and 29 were enrolled in thoracotomy group between 1996 and 2010. When compared with the thoracotomy group, patients in the VATS group had a shorter operation time (90 ± 29 min vs. 136 ± 41 min, P < 0.001), less blood loss (42 ± 12 mL vs. 209 ± 162 mL, P < 0.001), shorter time to extubation and discharge (2.5 ± 0.7 days vs. 3.1 ± 1.0 days, P= 0.027; 3.6 ± 0.6 days vs. 4.1 ± 1.0 days, P= 0.045). VATS is a safe and effective surgical procedure for posterior mediastinal neurogenic tumors with better outcomes than thoracotomy.

MRI Findings of Mediastinal Neurogenic Tumors

The objective of this article is to review the clinical, pathologic, and MRI findings of mediastinal neurogenic tumors according to the three tumor origins: the peripheral nerves, sympathetic ganglia, and paraganglia. MRI findings reflect pathologic features of mediastinal neurogenic tumors. Integrating consideration of age and clinical findings of the patient, lesion location, and imaging findings is important in the diagnosis of mediastinal neurogenic tumors.

Benign Posterior Mediastinal Neurogenic Tumors: Results of a Comparative Study into Video-Assisted Thoracic Surgery and Thoracotomy (13 Years' Experience)

Mediastinal neurogenic tumors originate from the nerve tissues of the thorax and are generally located in the posterior mediastinum. The present study was performed to compare the results of thoracotomy with those of video-assisted thoracic surgery (VATS) for the surgical treatment of posterior mediastinal neurogenic tumors. Twenty patients who underwent surgical resection for posterior neurogenic tumors between January 1996 and January 2009 were examined retrospectively. Thirteen (65%) patients were treated by thoracotomy (group T) and VATS was used in seven (35%) patients (group V). The duration of surgery was shorter in group V (83.5 ± 19 min) than in group T (124.6 ± 16.6 min; P < 0.0001). Chest drains were withdrawn earlier in group V (after 1 day) than in group T (1.6 ± 0.5 days; P = 0.005). The hospital stay was shorter for group V (1 day) compared with group T (3 ± 0.9 days; P < 0.0001) and group V required fewer analgesics than group T (P < 0.0001). VATS is the preferred treatment for posterior neurogenic tumors that show no preoperative signs of malignancy and do not involve the medulla spinalis.