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Ganglioneuroma is a rare, differentiated, and benign neurogenic tumor that could grow into a huge size with minimal or no symptoms at all. Ganglioneuroma is typically found in older children or adults and is commonly detected within the posterior mediastinum (other than retroperitoneal). Here, we present a case of a 3-year-old patient with shortness of breath, and radiological examination showed a giant mediastinal tumor which proved to be a ganglioneuroma after histopathological examination. This study highlights the possibility of ganglioneuroma occurring in younger children and the role of imaging in assessing ganglioneuroma as a posterior mediastinal tumor.

Malignant peripheral nerve sheath tumors are rare sarcomas of the heart. Herein, we report the case of a 24-year-old man who complained of dyspnea, cough, and upper left back pain. He was found to have multiple primary heart tumors obstructing the right superior pulmonary vein in the left atrium, which were diagnosed as malignant peripheral nerve sheath tumors. The patient underwent successful resection of the tumors and immunohistochemistry was utilized for diagnosis.

Objectives:The origin of neurilemmoma is Schwann cells and it is the most frequent subgroup of neurogenic tumors. It is often detected in the 3rd-5th decades. The region is located in the posterior mediastinum. Recurrence rates are very low when surgically resected. In this study, this type of tumor resected in our clinic was analyzed and evaluated with the literature.Materials and Methods:Twenty-five patients diagnosed with Schwannoma resected between January 2008 and January 2018 in Ankara University Faculty of Medicine, Department of Thoracic Surgery were retrospectively reviewed. All preoperative patients were evaluated by thoracic computed tomography. In case of suspicion of invasion into the spinal canal, magnetic resonance imaging was used.Results:Of all the patients included in the study, 14 (56%) were male and 11 (44%) were female. The mean age was 47±12.6 (31-66) years. The main symptom in the patients was back pain. It was determined that the lesions were frequently located in the paravertebral sulcus. 56% of Schwannoma masses were observed in the right and 44% in the left hemithorax. For resection, 18 patients underwent muscle sparing thoracotomy, six patients underwent video-assisted thoracoscopic surgery, and one patient underwent a median sternotomy procedure.Conclusion:The most common type of neurogenic tumors originating from neural cells is neurilemmomas originating from Schwann cells. It is generally located in the posterior mediastinum. They are asymptomatic between the rates of 40% and 60%. In our patients, the rate of symptomatic patients was higher. Recurrence is very low in patients undergoing resection in Schwannoma.

The most common posterior mediastinal masses are neurogenic tumors such as peripheral nerve sheath tumors (PNST). Schwannomas, a subtype of PNST, are most often benign, well encapsulated tumors of neural crest cell origin, and are frequently incidentally found, ranging in size from small asymptomatic mediastinal tumors to large masses. Rarely, large schwannomas are discovered when symptoms develop due to compression or involvement of nearby structures leading to an array of possible sequela which can include, but not limited to, persistent cough, hemoptysis, and dysphagia. Management decisions are based off of tumor size, location, concern for underlying malignant pathology, and potential for complications related to tumor invasion of vital anatomical structures. A majority of the schwannomas undergo surgical resection, though a subset of small, asymptomatic, benign tumors on imaging or pathology may be managed with surveillance. This case report describes a large posterior mediastinal schwannoma adherent to the posterior aortic arch and encasing the left subclavian and vertebral arteries. Surgical resection required vascular resection of a segment of the left subclavian artery and graft reconstruction using polytetrafluoroethylene (PTFE). This report further highlights the importance of preoperative planning with consideration of a multidisciplinary approach in preparation for resection of large, complex posterior mediastinal masses.

Castleman disease is a relatively rare disease, characterized by well-circumscribed benign lymph-node hyperplasia. The disease may develop anywhere in the lymphatic system, but is most commonly reported as unicentric Castleman disease in the mediastinum along the tracheobronchial tree. It is usually asymptomatic and detected on plain chest radiography as an incidental finding. We report an incidentally detected case of Castleman disease in the paravertebral space that was preoperatively diagnosed as a neurogenic tumor and treated by complete surgical resection.

Chordomas are rare, locally aggressive bone malignancies with poor prognoses. However, those with minimal or no bone involvement are more easily resectable because of their well-delineated margins and thus have better prognoses. Such extraosseous chordomas of the spine are localized both intradurally and extradurally. Only a few case reports have focused on extraosseous, extradural spinal chordomas. Radiologically, this type of chordoma has a dumbbell shape; however, dumbbell-shaped spinal tumors are traditionally thought to be neurogenic tumors (i.e., schwannomas or neurofibromas). We herein report a unique case involving a woman with a dumbbell-shaped extraosseous chordoma protruding predominantly into the retropharyngeal space. A 44-year-old woman presented for evaluation of a left submandibular mass. A T2-hyperintense, gadolinium-enhancing mass was found in her cervical spinal canal, protruding through the C2/3 neural foramen into the retropharyngeal space with minimal vertebral involvement. The initial diagnosis was a neurogenic tumor, most likely a schwannoma. After subtotal removal, the pathologic diagnosis was a chordoma. Because chordomas and schwannomas have significantly different prognoses, caution is warranted when a dumbbell-shaped tumor is identified in the spine with minimal or no vertebral deterioration on radiology. This report also provides the first thorough review of extraosseous dumbbell-shaped intraspinal–extraspinal chordomas.

Ependymomas arise from ependymal cells and can grow at any site in the central nervous system (CNS), as well as in some locations outside of the CNS. The latter is rare, contributing to the frequent misdiagnoses of such cases. Herein, we present the case of a 54-year-old man with a history of lower limb weakness and numbness. Magnetic resonance imaging revealed an extradural, heterogeneously enhanced solid lesion with a regular and well-defined border in the posterior mediastinum. A post-resection histopathological examination revealed tumor-forming perivascular pseudo-rosettes that showed immunoreactivity against glial fibrillary acidic protein, epithelial membrane antigen, and vimentin, as well as a high Ki-67 labeling index. Based on pathological features, a diagnosis of anaplastic ependymoma was established.

Background Mucosal Schwann cell hamartomas are rare neurogenic tumors which present most commonly in the distal colon. They are usually discovered as small, single polyps in asymptomatic patients. Case presentation An asymptomatic 64-year-old man was referred to us after a 12 mm subepithelial lesion was discovered incidentally on screening colonoscopy. Follow-up colonoscopy conducted 2 months later revealed that the tumor had disappeared, leaving multiple edematous, submucosal tumor-like elevations presenting as skip lesions throughout the sigmoid colon. Lesions had elongated pits on magnifying endoscopy and were limited to the first layer on endoscopic ultrasound. Biopsies revealed unclearly delineated foci of spindle-shaped cells limited to the lamina propria. On immunohistochemistry, all lesions were positive for S-100 protein and negative for CD34, CD56, and neurofilament protein. The patient was diagnosed with multiple mucosal Schwann cell hamartomas of the sigmoid colon. He remains asymptomatic after 18 months of follow-up, but the lesions have also remained unchanged. Conclusion We report a case of multiple non-polypoid mucosal Schwann cell hamartomas. Endoscopic findings may assist in the differential diagnosis, particularly when presenting as non-polypoid, submucosal tumor-like lesions.

Background: MYC associated factor X (MAX) is a tumor suppressor gene and has been identified as one of the pathogenic genes of hereditary pheochromocytoma (PCC). To date, there have been no reports of ganglioneuroma (GN) with MAX variants. Case Presentation: The proband was a 45-years-old Chinese female with paroxysmal hypertension and palpitations who had undergone adrenalectomy for PCC 14 years ago. Her plasma free normetanephrine and 24-h urinary norepinephrine excretion were significantly increased, and abdominal computed tomography (CT) revealed an irregular mass in the left adrenal region, suggesting a recurrence of PCC. The mass was surgically removed and pathologically diagnosed as PCC with lymph node metastasis. The proband's son suffered from paroxysmal hypertension and palpitations. His plasma free metanephrine levels were normal. CT revealed a mass in the right adrenal. The tumor was surgically removed, and the pathological diagnosis was GN. Genetic testing of peripheral blood DNA revealed that the proband and her son had germline pathogenic MAX variant c.C97T, p.Arg33Ter, while proband's parents did not have MAX variants. Tumor DNA sequencing showed the same MAX variant (c.C97T, p.Arg33Ter) in PCC of the proband and GN of her son, both with retention of heterozygosity. Immunohistochemistry demonstrated loss of MAX protein expression in most tumor cells in PCC of the proband and some Schwannian cells in GN of the proband's son. Conclusion: We report a family with a new clinical phenotype of germline pathogenic variants in MAX who developed both PCC and GN. Germline pathogenic variants in MAX may contribute to the development of GN. Our findings suggest that it is not just paternally inherited MAX variants that can cause tumors.

The neurogenic tumor of frequent occurrence in the presacral area is a schwannoma. Giant presacral schwannoma has a risk for anterior surgical approach because of its massive size and proximity to abundant vascularity of presacral region. We report a single stage posterior approach for total resection of a giant presacral schwannoma. A 40-year-old female patient experienced left buttock pain and tingling sensation at left S1 dermatome. Magnetic resonance imaging showed that the presacral huge mass at S1-3 level with osseous extension and structural remodeling in left sacral ala. The presacral mass was ranging in maximum diameter from 8.0 to 8.6 cm. S2 foramen laminectomy was performed to expose the mass. The tumor capsule and the root were carefully dissected away. The tumor was removed while preserving the capsule by dissecting the plane between the inner wall of the capsule and the tumor. The single stage posterior approach for presacral giant schwannoma is feasible, and it can be a good surgical alternative to prevent pelvic organ or vascular damage and anterior approach related dystocia and infertility.

Background To accurately identify primary retroperitoneal tumors by multi‐slice spiral CT (MSCT) for better treatment. Materials and Methods The common types of 380 cases of primary retroperitoneal tumor, lesion sites, and MSCT features were compared with pathological results. Fisher's or chi‐square test approaches have been applied in this study. Results Multi‐slice computerized tomography multi‐directional reconstruction has a high accuracy for primary retroperitoneal tumors (95.7%). Seventy‐three liposarcoma cases were located in peri‐renal space, accounting for 76.8% (13/95) of the tumors in this region. Meanwhile, 65 cases of neurogenic tumors were located in the paravertebral column, accounting for 90.3% (65/72) of the tumors in this region. MSCT examination could effectively distinguish benign from malignant of primary retroperitoneal tumor (sensitivity = 87.2%, specificity = 82.7%, accuracy rate = 84.5%). Malignant tumors showed more irregularity shape than benign tumor (χ 2 = 20.468, P

Introduction: The aim of this paper is to present a case of rare retroperitoneal tumor. Material and Methods. A 72 year old man presented with six month history of abdominal girth, lumbar pain and occasional episodes of constipation was admitted in our clinic. A computed tomographic abdominal scan revealed a retroperitoneal solid mass. Surgical exploration performed later displayed solid and well delimited mass throughout retroperitoneal space followed by excision of lesion and multiple drainages. Microscopic pathological examination was benign nerve sheath tumor. Conclusion. These tumors are characteristically firm, smooth, and asymptomatic. They range in size from 1-2 mm to over 20 cm. Awareness of this indolent and rare condition is an important prerequisite for early diagnosis and appropriate treatment. Surgical excision can usually be accomplished by enucleating of the tumor without damage to the adjacent nerve.

A chest wall schwannoma arises from peripheral nerve sheath Schwann cells of the intercostal nerves. We describe the presentation and imaging findings of a patient who presented with a chest wall swelling. The imaging findings were highly suspicious for a chest wall schwannoma and the histopathology confirmed the diagnosis following surgical excision. Imaging findings are reviewed in detail.

Background: Malignant tumors arising from peripheral nerves or displaying differentiation along the lines of the various elements of the nerve sheath are referred to Malignant peripheral nerve sheath tumors (MPNST) and also called malignant schwannomas or neurofibrosarcomas. The common sites of involvement are head, neck, extremities and thorax. This case was discussed due to its rare incidence, namely less than 5% of soft tissue malignant tumor. The overall five year survival rate is approximately 50% in patients with resectable tumor. Case: We report a case of MPNST arising from mediastinum, the patient was a male, 41-years-old, with chief complaint are chest pain and mass in the back. Contrast enhanced chest CT Scan showed a solid mass (10.02 × 6.97 × 10.53 cm) in the right side of the posterior mediastinum. On microscopic examination with hematoxylin eosin imunostaining, of the mass on mediastinum showed typical features of MPNST, which were positive for S-100 imunostaining. Based on convensional histopathologic and imunostaining, this case was concluded as Malignant peripheral nerve sheath tumors (MPNST). Conclusion: The treatment of chest wall MPNSTs is multimodality, including wide local excision, adjuvant radiotherapy, and chemotherapy. In our case, we performed direct excision due to the infiltrating mass to the surrounding tissue, so the prognosis for this case was good. This case indicates the role of chemotherapy in treatment of advanced MPNST. Studies shows the superiority of the doxorubicin–ifosfamide regimen. As in this case the patient showed complete respons after surgery and adjuvant chemotherapy.

Background: Due to variations in location and size, laparoscopic surgery for paraaortic or paracaval neurogenic tumors is challenging. We evaluated the surgical outcomes, as well as surgical tips and tricks. Methods: Between 2000 and 2015, 25 procedures were performed in 24 patients. One patient underwent second surgery due to the recurrence of paraganglioma. Data were collected on the tumor diameter, tumor location, perioperative outcomes, pathology, and last known disease status. Regarding the operative procedures, we reviewed the operative charts or videos to identify surgical tips and tricks. Results: The median tumor diameter was 5.0 cm (range, 1.5-10). The tumor location was suprahilar in 10, hilar in 6, and infrahilar in 9. Regarding the approach, a transperitoneal approach was selected in 24 cases and retroperitoneal approach in 1. The median operative time and blood loss were 208 minutes (range, 73-513) and 10 mL (range, 0-1,020), respectively. No patient required blood transfusion or conversion to open surgery. Pathological examination revealed paraganglioma in 12, ganglioneuroma in 7, and schwannoma in 6. At the last follow-up, 23 patients were free of disease, while one patient developed metastatic multiple recurrence of paraganglioma 54 months after the second laparoscopic surgery. A review of the surgical records revealed several tips and tricks, including taping the vena cava/ renal vein (n=2) being helpful for detaching a retrocaval tumor from these great vessels, or rotating the kidney to provide a favorable operative view of tumors behind the renal hilum (n=2). In recent cases, 3D-CT was helpful for preoperative planning. Conclusion: Laparoscopic resection of paraaortic or paracaval neurogenic tumors is feasible in experienced hands. Surgeons should be familiar with detaching maneuvers around great vessels and the mobilization of adjacent organs. Careful preoperative planning is mandatory.

Schwannoma is the most common nerve sheath tumor in the posterior mediastinum, whereas intrathoracic paraspinal malignant peripheral nerve sheath tumor (MPNST) is quite rare. Both benign and malignant nerve sheath tumors may be symptomatic, rendering clinical differentiation of limited utility. On radiographic imaging, erosion of the ribs and vertebral bodies, irregularity in contour, and inhomogeneity in attenuation are not sufficiently reliable for diagnosis of MPNST. Histologically, MPNSTs reveal hypercellularity, nuclear atypia, and mitotic activity. Surgical resection is the main modality of treatment. Postoperative radiation therapy for MPNST has led to a significant reduction in local recurrence. The prognosis is unfavorable. Herein, we present an unusual case of a posterior mediastinal mass in a 50-year-old female with delayed diagnosis of 2 years. After surgical intervention, the histologic finding was MPNST. Postoperative radiation therapy was applied because of incomplete resection. The follow-up chest computed tomography 5 months later revealed a residual soft tissue mass with significant reduction in size over the parathoracic spine area. No neurologic sequelae were identified after surgery.

Various histologic types of neurogenic tumors may originate in the mediastinum and chest wall. It is possible to make accurate diagnosis of these tumors by using the multiplanar capability and high contrast resolution of MR imaging because of these characteristic imaging findings. MR and histologic features of these tumors are illustrated and described in this essay.

Schwannoma of the mediastinum is a rare and typically benign type of tumor. The present study describes the case of a 61-year-old woman who presented with a continuous cough and facial edema. Pre-operative chest radiography, enhanced computed tomography (CT) and three-dimensional CT scans identified a well-circumscribed mass. The large cyst, measuring 6.5×6.1×5.0 cm, was located anterior to the trachea and posterior to the superior vena cava. The mass was observed to be in close proximity to the right pulmonary hilum and the superior vena cava was flattened due to the pressure on the right vagus nerve. Therefore, the encapsulated tumor was completely resected under thoracoscopy and was subsequently diagnosed as a benign schwannoma upon pathological examination. The respiratory tract symptoms and facial edema resolved immediately after the surgery, and no recurrence was observed during the 6-month follow-up period. At the time of writing, the patient remained alive. The present study records the rarely successful resection of a middle mediastinal tumor by video-assisted thoracoscopy.

The susceptibilities of Nagase analbuminemic rats (NAR) and control Sprague‐Dawley rats (SDR) to N‐ethyl‐N‐nitrosourea (ENU) were compared. In Experiment I, the rats were given daily subcutaneous injections of 10 mg/kg of ENU for a week from 4 weeks of age. In Experiment II, mother rats were given a single subcutaneous injection of 60 mg/kg of ENU on day 17 of pregnancy and tumor development in their offspring was examined. In Experiment I, the incidence of neurogenic tumors was slightly, but not significantly, higher in NAR than in control rats. In Experiment II, the incidence of total tumors including neurogenic tumors was significantly higher in NAR (40/43, 93.0%) than in SDR (13/61, 21.3%). NAR showed particularly high susceptibility to induction of nenrogenic tumors (34/43, 79.1%) and renal tumors (15/43, 34.9%). In an attempt to elucidate the underlying mechanisms of the increased susceptibility of NAR to ENU, O6‐ethylguanine, a major premutagenic ethylated DNA adduct, was quantitated in fetal brain DNA of NAR and SDR after a pulse exposure to 60 mg/kg ENU. No significant difference in the initial formation or subsequent repair of O6‐ethylguanine was observed in the two strains, indicating that abnormality at some later stage(s) of chemical carcinogenesis may lead to the increased susceptibility of NAR to induction of neurogenic tumors.

A schwannoma of the larynx is a rare benign tumor that usually presents as a submucosal mass in the pyriform sinus and the aryepiglottic space, and this type of schwannoma constitutes a diagnostic and therapeutic challenge for otolaryngologists. We present here two cases of supraglottic schwannomas that were misdiagnosed as laryngoceles. Both were excised through a lateral thyrotomy approach without a tracheostomy, and the laryngeal function was successfully maintained. We discuss the clinical and imaging findings and the management of this rare neoplasm with focusing on the differential diagnosis of laryngeal schwannoma and laryngocele. We also review the relevant medical literature.

We present the case of an 8-year-old male with an intestinal obstruction and a simultaneous incidental adrenal mass. The patient had a newborn history of intestinal obstruction secondary to ileal atresia. Intestinal length at the time of operation was measured to be 50% of predicted. The patient's present intestinal obstruction presented a challenge to the evaluation and management of a concurrent adrenal mass.

We present three patients with intrathoracic malignant neurogenic tumor. Two lesions showed no sign of invasion into adjacent structures, while the third lesion extended to the intraspinal canal with vertebral involvement. Although all three lesions were completely excised, each patient relapsed within 1 year of the initial treatment. One patient with local recurrence underwent radiation therapy, but the recurrent tumor continued to progress. Chemotherapy was subsequently performed. Two patients with distant metastases also received chemotherapy. Because there is no effective chemotherapeutic regimen for intrathoracic malignant neurogenic tumor, all three patients received high-dose chemotherapy followed by hematopoietic stem cell transplantation. Although the relapsed lesions temporarily regressed after treatment, all three patients showed disease recrudescence and ultimately died of their disease. A comparison of the intrathoracic malignant neurogenic tumors and the benign neurogenic tumors resected at our institution revealed no meaningful differences distinguishing malignant from benign neurogenic tumors prior to surgery.