Decision Making in Retroperitoneal Nerve Sheath and Nerve-Associated Tumors: A Modular Approach.

BACKGROUND: Surgical treatment of retroperitoneal nerve and nerve-associated tumors is challenging, especially in cases with large extent. A single surgical access may have limitations and jeopardize patients. OBJECTIVE: To present a series of patients to illustrate our individually tailored treatment concept and decision pathway. METHODS: Retrospectively, clinical findings and imaging were related to surgical features and outcome. An algorithm for choice of approach was established. RESULTS: From 2012 to 2017, we operated on n = 13 patients with retroperitoneal tumors, of these n = 9 were included (n = 6 female, n = 3 male). Histological findings included n = 2 schwannomas, n = 2 malignant peripheral nerve sheath tumors, n = 1 non-origin sarcoma, n = 1 perineurioma, n = 1 intraneural ganglion cyst, n = 1 lymphoma, and n = 1 paraganglioma. In n = 6 patients, we used a monoportal (retroperitoneal/transperitoneal) approach; in n = 2 patients, a biportal retroperitoneal to inguinal/transperitoneal to dorsal approach; and in n = 1 patient, a triportal transperitoneal to dorsal to gluteal approach. In n = 2 patients, we performed an open biopsy only; in n = 2 patients, a tumor enucleation; in n = 3 patients, a subtotal function-sparing resection; in n = 1 patient, a complete resection; and in n = 1 patient, intraneural decompression. In n = 1 patient, a new motor deficit appeared. n = 4 patients required further radio-oncological treatment. n = 8/9 patients are alive without tumor progress or recurrence. CONCLUSION: Retroperitoneal nerve or nerve-associated tumors encompass multiple entities. Depending on suspected histology and tumor extension, extensile or combined surgical approaches may be necessary. We present our algorithm for assessment and decision-making regarding surgical access ports and pathways.

Surgical treatment of posterior mediastinal neurogenic tumors.

BACKGROUND: Posterior mediastinal neurogenic tumors are among the most frequent mediastinal masses in adults. These tumors may be dumbbell shaped, extending into the spinal canal, exclusively paraspinal or apical tumors extending in the cervical region. In this report, we present our experience in the surgical resection of these tumors and discuss the surgical strategies for such tumors. METHODS: A retrospective analysis was performed of 121 patients who underwent surgery for posterior mediastinal neurogenic tumors at our department during the period 2009 to 2016. Seventy-four tumors were excised via video-assisted thoracic surgery (VATS). Other approaches included thoracotomy, supraclavicular incision, supraclavicular incision plus thoracotomy/VATS, and a posterior approach with laminectomy combined with thoracotomy/VATS. RESULTS: Tumors were resected completely in 119 cases and partially in two. The majority of the tumors were benign nerve sheath tumors. No recurrence developed during postoperative median follow-up period of 31 months. CONCLUSION: Most posterior neurogenic tumors can be resected via VATS. Thoracotomy is the appropriate surgical approach for large tumors. A supraclavicular approach is recommended for tumors extending in the cervical region, and this can be combined with VATS or thoracotomy in case of larger masses. A posterior approach could be used for patients with dumbbell tumors.

Multinodular and vacuolating neuronal tumors in epilepsy: dysplasia or neoplasia?

Multinodular and vacuolating neuronal tumor (MVNT) is a new pattern of neuronal tumour included in the recently revised WHO 2016 classification of tumors of the CNS. There are 15 reports in the literature to date. They are typically associated with late onset epilepsy and a neoplastic vs. malformative biology has been questioned. We present a series of ten cases and compare their pathological and genetic features to better characterized epilepsy-associated malformations including focal cortical dysplasia type II (FCDII) and low-grade epilepsy-associated tumors (LEAT). Clinical and neuroradiology data were reviewed and a broad immunohistochemistry panel was applied to explore neuronal and glial differentiation, interneuronal populations, mTOR pathway activation and neurodegenerative changes. Next generation sequencing was performed for targeted multi-gene analysis to identify mutations common to epilepsy lesions including FCDII and LEAT. All of the surgical cases in this series presented with seizures, and were located in the temporal lobe. There was a lack of any progressive changes on serial pre-operative MRI and a mean age at surgery of 45 years. The vacuolated cells of the lesion expressed mature neuronal markers (neurofilament/SMI32, MAP2, synaptophysin). Prominent labelling of the lesional cells for developmentally regulated proteins (OTX1, TBR1, SOX2, MAP1b, CD34, GFAPδ) and oligodendroglial lineage markers (OLIG2, SMI94) was observed. No mutations were detected in the mTOR pathway genes, BRAF, FGFR1 or MYB. Clinical, pathological and genetic data could indicate that MVNT aligns more with a malformative lesion than a true neoplasm with origin from a progenitor neuro-glial cell type showing aberrant maturation.

Intrathoracic neurogenic tumor with malignant transition-20 years operation experience in a medical center of China.

OBJECTIVE: Malignant peripheral neurogenic tumor is always found in large peripheral nerve of the extremities, however, benign peripheral neurogenic tumors of chest is an uncommon disorder, furthermore, malignant transition is even rare. So far, few cases have been reported. We studied 45 neurogenic thoracic tumors patients with malignant transition. METHODS: We retrospectively reviewed clinical data of 45 malignant neurogenic thoracic tumors from 1992 to 2012, including 11 (24.44%) cases of borderline tumors (group X), 13 cases of (28.88%) low-grade malignant tumor (group L), and 21 cases (46.66%) of malignant tumors (group M). Specifically, we reviewed the clinical characteristics, surgical approach, postoperative outcome, complications and prognosis of these patients. RESULTS: All tumors are located in the thoracic cavity, and arising from sympathetic or spinal nerve or their branches from the lung or posterior mediastinum. The patients from groups X and L had more complete resection than group M. Unfortunately, one patient was dead intra operatively in Group M. Group M had more recurrence than groups X and L. Our results indicates that, the survival rate may be correlated with tumor size, malignant degree (P=0.018), tumor recurrence and incomplete resection (P<0.05). CONCLUSION: The most effective treatment method for these tumors is early identification and resection by minimally invasive surgery during benign stage. In addition, regarding low-grade malignant tumor, non-radical surgery could also be responsible for the low survival rate.

A comparative study of video-assisted thoracoscopic resection versus thoracotomy for neurogenic tumours arising at the thoracic apex.

OBJECTIVES: The surgical outcome of neurogenic tumours arising at the thoracic apex remains largely undefined. In this retrospective study, we compared the efficacy and safety of thoracoscopic surgery and thoracotomy for neurogenic tumours at the thoracic apex in 63 patients who received surgical treatment between 1992 and 2012 at our medical centre. METHODS: Forty-four (69.8%) patients received thoracotomy (Group A) and 19 (30.2%) patients underwent video-assisted thoracoscopic surgery (Group B). Operative time, estimated blood loss (EBL), postoperative length of hospital stay and nervous system complications were recorded. RESULTS: The two groups of patients were comparable in demographic and baseline characteristics except that Group A patients had a significantly larger tumour size (mean, 4.9 ± 1.0 cm) than Group B patients (mean, 4.1 ± 1.2 cm; P = 0.01). The mean operative time was markedly greater for Group A (120.2 ± 41.2 min) than Group B (93.2 ± 34.5 min; P = 0.009). Group A had significantly greater EBL (245.23 ± 197.78 ml) than Group B (117.4 ± 138.2 ml; P < 0.001). Total tumour resection was achieved in all patients and all neurogenic tumours were benign. The mean length of postoperative hospital stay was markedly longer in Group A (7.0 ± 2.1 days) than Group B (4.8 ± 2.0; P < 0.001). Postoperatively, brachial plexus injury was found in 1 patient (2.3%) in Group A and 4 patients (21.1%) in Group B (P = 0.026). CONCLUSIONS: Though thoracoscopic surgery is associated with diminished blood loss, reduced operative time and shortened hospital stay, it has a markedly increased incidence of brachial plexus injury.

Intraoperative esophagoscopy provides accuracy and safety in video-assisted thoracoscopic enucleation of benign esophageal submucosal tumors.

Benign esophageal tumors are rare; complete surgical resection is essential for the management of the submucosal tumors. Larger, symptomatic, or non-diagnostic lesions should be resected for both diagnostic and therapeutic indications. Video-assisted thoracic surgery has become a popular treatment in the field of thoracic surgery; however, thoracoscopic esophageal surgery may lead to an increase in operative complications. The effect and safety of thoracoscopic surgery for esophageal submucosal lesions were evaluated. A retrospective study evaluated patients undergoing thoracoscopic treatment of benign submucosal tumors. Between March 2011 and December 2013, 17 patients underwent thoracoscopic resection of benign submucocal tumors. Intraoperative esophagoscopy was performed for tumor localization by transillumination and confirmation of mucosal integrity after enucleation in every patient. Median patient age was 47 years (range 30-65). The median surgery time was 170 minutes (range 80-429). The median tumor size was 3.8 cm (range 1.3-9). The median hospital stay was 4 days (range 2-12). There were 16 leiomyoma and 1 neurogenic tumor. There was one case of conversion to thoracotomy because of residual tumor after enucleation. Mucosal injuries occurred in three patients, two accidentally and one intentionally; each patient was treated with primary repair and confirmed integrity with flexible esophagoscopy at operating room. The small sized tumor with intraoperative esophagoscopy could be localized. Esophagoscopic assistance was necessary in eight patients to have better idea where to make myotomy. There were no major morbidities such as postoperative leakage or mortality. Esophageal submucosal tumors can be treated safely with thoracoscopic surgery. However, intraoperative esophagoscopy allows accurate tumor localization, direction of esophageal access incision, and decreases complications during VATS enucleation of esophageal submucosal tumors.