Paraspinal Tumors Research Articles

Early-Onset Spinal Deformity in Neurofibromatosis Type 1: Natural History, Treatment, and Imaging Surveillance.

Early-onset scoliosis (EOS) or kyphosis is common in patients with neurofibromatosis (NF) and is characterized by rapid progression of deformity. Traditional growing rods provide good functional and deformity outcomes in patients with NF and EOS; magnetically controlled growing rods (MCGRs) also provide good deformity correction, although high rates of revision have been reported after their use. Among patients with NF type 1 (NF1), morphologic characteristics of the spinal deformity are different in those with paraspinal neurofibromas than in those without paraspinal tumors. Patients with NF1 are at low risk for developing malignant peripheral nerve sheath tumors during childhood (<1%) and their lifetime (8% to 12%), and routine imaging surveillance for malignancy in the absence of symptoms should be clinically directed. Further investigation is needed to standardize screening for EOS in children with NF1 and to develop guidelines for ideal imaging modalities, including their frequency and a timeline.

Incidence of NUT carcinoma in Western Australia from 1989 to 2014: a review of pediatric and adolescent cases from Perth Children\u2019s Hospital

BackgroundNUT carcinoma (NC), previously known as NUT midline carcinoma, is a rare and very aggressive cancer that occurs in both children and adults. NC is largely chemoresistant, with an overall survival of less than 7 months. Because the carcinoma is not restricted to a particular organ, diagnosis is often a challenge. In the absence of a clearly determined incidence for NC, we sought to study the diagnosis of patients in a well-defined population.MethodsWe systematically reviewed records of all patients that presented to the Oncology Department of the Princess Margaret Hospital for Children from 1989 to 2014. This institution in the geographically isolated state of Western Australia has a catchment population of around 2 million. We then identified all high grade undifferentiated sarcomas or carcinomas in the 0–16 year age group.ResultsOver 26 years, we found 14 patients of 16 years or younger with undifferentiated malignant tumors. Of these, five tumors were positive by immunohistochemistry for the NUT/NUTM1 (Nuclear Protein in Testis) protein and/or the translocation t(15;19). Three patients presented with thoracic tumors, one with a para-spinal tumor, and one had an upper airway nasopharyngeal carcinoma. In all five cases, there was an initial response to therapy and then progression. This 26-year survey was conducted in a geographically isolated state with a well-defined population, and we determined an estimated incidence of NC of around 0.41 per million child years (0–16 yrs. of age) at risk. From three patients it was feasible to derive cell lines for further genetic analyses and drug screening.ConclusionsFor the first time, the incidence of NC could be determined in a well-defined geographic area. The calculated rate of NC incidence is consistent with a history of under-recognition for this malignancy. These findings indicate that improved diagnostic detection of NC would enable better management and counselling of patients. Our findings emphasize the heterogeneity of NC, and they highlight the need to develop personalised therapy options, and to consider a diagnosis of NC in undifferentiated malignant tumors.

Establishment of a modified percutaneous CT-guided paraspinal intramuscular VX-2 squamous cell carcinoma dual tumor model in rabbits.

BackgroundThe rabbit VX-2 tumor model is a commonly used transplanted tumor model and is widely used in surgical, radiological, and interventional studies. Most of the known tumor models for each site are single solid tumors. This study aimed to establish an accurate and stable intramuscular dual tumor model guided by computed tomography (CT).MethodsIn this study, we compared three different inoculation methods to select the most appropriate dual tumor model. Six New Zealand White rabbits were used as tumor-carrying rabbits for tumor harvesting. Thirty rabbits were divided into three groups as experimental rabbits. Group A applied the tumor cell suspension method, in which the suspension was injected into the designated location with a syringe under CT guidance. Groups B and C used tumor tissue strips obtained in vivo or under direct in vitro vision. The tumor tissue strips were implanted into the designated locations using a guide needle under CT guidance. The differences in tumorigenic rate, the size difference between bilateral tumors, and metastasis between the three methods were compared.ResultsIt was found that group A obtained a 100% tumor survival rate, but the size of the tumor was more variable, and needle tract implantation metastasis occurred in 5 cases. In group B, tumor tissue strips were taken in vivo for implantation, in which one case failed to survive. Tumor tissue strips in group C were obtained in vitro under direct vision. The tumor tissue strips obtained in vitro by puncture using a biopsy needle in group C had a 100% tumorigenicity rate and stable tumor size. No significant needle tract implantation metastases were found in either group B or C. The variance of tumor size obtained in group A was significantly higher than in groups B and C. The variance of tumor size in group C was the smallest. Group C had high tumorigenicity and a more stable size and morphology of the formed tumors.ConclusionThe results showed that the method of obtaining tumor tissue strips using in vitro direct vision puncture and implanting them into the muscle with CT guidance and guide needles can establish an accurate and stable dual tumor model. This dual tumor model can provide substantial support for relevant preclinical studies.

Lumbar Paraspinal Hemangiopericytoma, An Unusual Location: Surgery with Preoperative Embolization

Background: Lumbar paraspinal hemangiopericytoma is rare. The hemorrhagic nature of the tumor causes problems of hemostasis and makes the resection delicate. We report a rare case of lumbar paraspinal hemangiopericytoma managed with preoperative embolization. Clinical Case: It is about a woman of 31 years, admitted for a large left lumbar paraspinal mass that evolved for 6 years. The clinical exam showed an asymmetry of the left paraspinal muscle. The mass painless was extended from the lumbar region. The neurological exam was normal. MRI showed the left large paraspinal mass tissue. It was extended from lumbar vertebrae, L1 to L4, and measured 100 × 50 × 50 mm. It was an iso-intense signal on T1-weighted with strong enhancement after gadolinium. It was hypervascular and supplied by left intercostal T12, L1, and L2 pedicles. The complete exclusion of the hyper-vascular left paraspinal tumor was obtained after selective embolization of the artery left L1 of the pedicle of the intercostal left L1 and trunks intercostal T12 and L2 left. The total resection of a mass encapsulated was performed. The operative outcome was good. The histology concluded to a hemangiopericytoma. No chemotherapy or radiotherapy was prescribed. After 5 years, the patient was asymptomatic. MRI control confirmed tumor resection with a residue at the level of the left intervertebral foramen L1 - L2. Conclusion: Lumbar paraspinal hemangiopericytoma is an extremely rare tumor. Selective preoperative embolization is recommended before the resection of large tumors to reduce vascular supply. A follow-up extended for these patients is necessary, given the frequency of recurrences.

A Novel Endoscopic Technique for Biopsy and Tissue Diagnosis for a Paraspinal Thoracic Tumor in a Pediatric Patient: A Case Report.

Conventional approaches to the thoracic spine can require extensive tissue dissection, bony disruption, and instability that may warrant the need for instrumentation and fusion. Furthermore, anterior approaches may require the involvement of various surgeons from multiple disciplines to ensure a successful operation and mitigate complications. Currently, available minimally invasive approaches still require bony removal and usually rely heavily on computed tomography (CT)-guided imaging without direct gross visualization. Endoscopic spinal procedures have provided an ultra-minimally invasive alternative to access many areas in and around the spinal column. We present a 12-year-old boy with a right-sided 2.0 × 3.2-cm paravertebral lesion at the level of T5. The patient successfully underwent an endoscopic approach to the lesion with minimal tissue and bony disruption for tissue diagnosis and tumor resection. At initial and 6-month follow-up, the patient remained asymptomatic and without issues. We demonstrate here the feasibility and suggest the safety of a posterior ultra-minimally invasive endoscopic spinal approach to obtain a tissue biopsy of an incidentally found ventrolateral paraspinal tumor in the thoracic region in a pediatric patient. This minimal approach can prove to achieve similar results as other approaches that may otherwise necessitate more extensive or transthoracic procedures.

Spinal Deformities after Childhood Tumors.

Simple SummaryA significant number of children surviving intra- or juxta-spinal tumors develop secondary spinal deformities and disabilities. This retrospective non-comparative study focuses on deformity analysis, age, and skeletal maturity dependent treatment options and results. Patients who developed severe scoliosis, pathological kyphosis, and/or lordosis were either treated conservatively or surgically by using growth-friendly spinal implants in younger children or definite spinal fusion during puberty. Despite severe spinal deformity, some patients were not surgically corrected in order to preserve mobility through trunk motion or malignant tumor progression. Growth-friendly spinal implants and spinal fusions were able to significantly reduce pathological curves. The first method, with bilateral rib-to-pelvis fixation, still allows spinal magnetic resonance imaging or neurosurgical intervention if needed. Severe ad hoc curve correction enhances the risk of neurological deterioration because of prior spinal cord impairment. The data of this study may help to improve the individual patient care.Childhood tumors of the central nervous system (CNS) and other entities affecting the spine are rare. Treatment options vary from surgical biopsy to partial, subtotal, and total resection, to radiation, to chemotherapy. The aim of this study is to investigate spinal deformity and subsequent surgical interventions in this patient cohort. A retrospective review at our institution identified children with CNS tumors, spinal tumors, and juxta-spinal tumors, as well as spinal deformities. Tumor entity, treatment, mobilization, and radiographic images were analyzed relative to the spinal deformity, using curve angles in two planes. Conservative or surgical interventions such as orthotic braces, growth-friendly spinal implants, and spinal fusions were evaluated and analyzed with respect to treatment results. Tumor entities in the 76 patients of this study included CNS tumors (n = 41), neurofibromatosis with spinal or paraspinal tumors (n = 14), bone tumors (n = 12), embryonal tumors (n = 7), and others (n = 2). The initial treatment consisted of surgical biopsy (n = 5), partial, subtotal, or total surgical resection (n = 59), or none (n = 12), followed by chemotherapy, radiotherapy, or both (n = 40). Out of 65 evaluated patients, 25 revealed a moderate or severe scoliotic deformity of 71° (range 21–116°), pathological thoracic kyphosis of 66° (range 50–130°), and lordosis of 61° (range 41–97°). Surgical treatment was performed on 21 patients with implantation of growth-friendly spinal implants (n = 9) as well as twelve dorsal spinal fusions (two with prior halo distraction). Surgical interventions significantly improved spinal deformities without additional neurological impairment. With the increasing number of children surviving rare tumors, attention should be focused on long-term problems such as spinal deformities and consequent disabilities. A significant number of children with CNS tumors, spinal tumors or juxta-spinal tumors required surgical intervention. Early information about spinal deformities and a close follow-up are mandatory for this patient group.

Histopathological Spectrum of Central Nervous System Tumors: an Experience at a Hospital in Nepal.

More than half of Central Nervous System tumors are benign; however, they can cause substantial morbidity. The classification of central nervous system is vital for their varied outcomes and management. The objective of this study is to provide the histopathological spectrum of central nervous system tumors in a central hospital in Nepal. The present study is a retrospective cross-sectional study conducted at Department of Pathology, Kathmandu Model Hospital, Kathmandu, Nepal from January 2010 to December 2017 of 162 cases of clinically diagnosed cases of central nervous system tumors. All patients were classified according to the World Health Organization classification of central nervous system tumors. Nine of these162 patients did not have any tumor. The most common categories of tumors were astrocytic and oligodendroglial tumors (39.2%), meningiomas (21.5%), cranial and para spinal tumors (15%), tumors of sellar region including pituitary adenoma (4.5%), and metastatic tumors (3.2%). Glioblastoma(51.6%) and diffuse astrocytoma (21.6%) were the most common astrocytic and oligodendroglial tumors. The most common site of tumors in the brain was frontal (14.37%) followed by temporal (10.45%) region in the brain and dorsal region in spine. This study gives the current scenario of the epidemiology and clinicohistopathological aspects of different brain tumors as encountered in a tertiary level hospital in Kathmandu.

Stereotactic radiosurgery for the treatment of bulky spine metastases.

Stereotactic radiosurgery (SRS) has shown durable local control for the treatment of metastatic diseasespinal metastases. Multilevel disease or epidural or paraspinal involvement present challenges to achieving local control, and this study aims to analyze treatment outcomes for such lesions. Patients treated at a single institution with SRS to the spine from 2010-2018 were retrospectively reviewed. Inclusion criteria required clinical follow-up with either a pain assessment or imaging study. Bulky spine metastasis was defined as consisting of multilevel disease or epidural or paraspinal tumor involvement. 54 patients treated for 62 lesions met inclusion criteria. 42 treatments included at least two vertebrae, and 21and 31 had paraspinal and epidural involvement, respectively. Treatment regimens had a median 24Gy in 3 fractions to a volume of 37.75 cm3. Median follow-up was 14.36months, with 5 instances (8%) of local failure. Median overall survival was 13.32months. Pain improvement was achieved in 47 treatments (76%), and pain improved with treatment (p < 0.0001). Severe pain (HR = 3.08, p = 0.05), additional bone metastases (HR = 4.82, p = 0.05), and paraspinal involvement (HR = 3.93, p < 0.005) were predictive for worse overall survival. Kaplan-Meier analysis demonstrated that prior chemotherapy (p = 0.03) and additional bone metastases (p = 0.02) were predictive of worse overall survival. Grade < 3 toxicity was observed in 19 cases; no grade ≥ 3 side effects were observed. SRS can effectively treat bulky metastases to the spine, resulting in improvement of pain with minimal toxicity. Severe pain independently predicts for worse overall survival, indicating that treatment prior to worsening of pain is strongly recommended.

SAT-195 The Diagnosis and Management of a Functional Paravertebral Paraganglioma

A 32-year-old male presented with a 4-year history of palpitations, diaphoresis, and orthostatic hypotension. One year prior to presentation, he began experiencing severe, episodic headaches and neck pressure. Neurological work-up included an MRI head, neck, and spine, identifying a left posterior mediastinal lesion adjacent to the T5 vertebral body, approximately 2.4 x 2.0 cm. A community surgical center attempted mass resection via Video -assisted (VATS) approach. Resection was aborted intraoperatively due to a hypertensive crisis following lesion manipulation, with his systolic blood pressure increasing above 325 mmHg.Subsequent evaluation revealed a 24-hour urine norepinephrine of 6117 nmol/d (normal <575) and normetanephrines of 16.6 umol/d (normal <3.4). An MIBG study showed intense tracer avidity in the left paraspinal lesion and minimal uptake in the adrenal glands.A functional paravertebral paraganglioma was diagnosed and the patient was initiated on alpha and beta-blockade in preparation for staged combined treatment.Spinal angiography was performed to define the arterial supply of spinal cord and tumor, which confirmed a hypervascular left thoracic paraspinal tumor. Arterial supply emanated from the left T4 intercostal and left supreme intercostal arteries and a prominent draining vein was present. Trans-arterial particle embolization was performed via both arteries with minimal residual tumor blush present post procedure.Successful VATS excision of the tumor was completed one week after arterial embolization with minimal blood loss. Pathology of the resected tumor revealed an overall intact tumor that stained diffusely positive with synaptophysin and sustentacular cells highlighted by S100 protein with a few foci of coagulative necrosis.Episodes of hypertension (maximum BP 210/80) occurred during embolization and surgical resection with vascular manipulation. These were managed with intraoperative nitroprusside and phentolamine. Peri-procedural plasma metanephrine and cathecholamine levels were collected. Normetanephrine levels (normal <=0.89 nmol/L) were 4.66 before embolization, >6.25 after embolization, 3.70 the day after embolization, 4.10 before VATS, and 0.69 after VATS. Norepinephrine levels (normal 0.8–3.4 nmol/L) were 24.3, 111.7, 61.1, 19.6, and 4 respectively. Genetic testing showed no hereditary predisposition.Discussion:Functional neuroendocrine tumours may be difficult to diagnose due to the wide variety of clinical presentations and must be considered when investigating a vascular mediastinal tumor. In our case, pre-operative embolization did not normalise plasma catecholamine levels, likely from incomplete arterial embolization, but helped reduce intra-operative surgical bleeding risk. Alpha- and beta-blockade should be continued until surgical resection, with dose adjustment as required.

Clinical Experience of Automated SBRT Paraspinal and Other Metastatic Tumor Planning With Constrained Hierarchical Optimization

PurposeWe report on the clinical performance of a fully automated approach to treatment planning based on a Pareto optimal, constrained hierarchical optimization algorithm, named Expedited Constrained Hierarchical Optimization (ECHO). Methods and materialsFrom April 2017 to October 2018, ECHO produced 640 treated plans for 523 patients who underwent stereotactic body radiation therapy (RT) for paraspinal and other metastatic tumors. A total of 182 plans were for 24 Gy in a single fraction, 387 plans were for 27 Gy in 3 fractions, and the remainder were for other prescriptions or fractionations. Of the plans, 84.5% were for paraspinal tumors, with 69, 302, and 170 in the cervical, thoracic, and lumbosacral spine, respectively. For each case, after contouring, a template plan using 9 intensity modulated RT fields based on disease site and tumor location was sent to ECHO through an application program interface plug-in from the treatment planning system. ECHO returned a plan that satisfied all critical structure hard constraints with optimal target volume coverage and the lowest achievable normal tissue doses. Upon ECHO completion, the planner received an e-mail indicating the plan was ready for review. The plan was accepted if all clinical criteria were met. Otherwise, a limited number of parameters could be adjusted for another ECHO run. ResultsThe median planning target volume size was 84.3 cm3 (range, 6.9-633.2). The median time to produce 1 ECHO plan was 63.5 minutes (range, 11-340 minutes) and was largely dependent on the field sizes. Of the cases, 79.7% required 1 run to produce a clinically accepted plan, 13.3% required 1 additional run with minimal parameter adjustments, and 7.0% required ≥2 additional runs with significant parameter modifications. All plans met or bettered the institutional clinical criteria. ConclusionsWe successfully implemented automated stereotactic body RT paraspinal and other metastatic tumors planning. ECHO produced high-quality plans, improved planning efficiency and robustness, and enabled expedited treatment planning at our clinic.

15 Comparison of paraspinal metastasis SBRT plans: Cyberknife® versus VMAT

Introduction.The irradiation of paraspinal tumors requires the use of highly modulated plans due to the restricted dose to the spinal cord. The objective was to compare the performances of new generation linear accelerator (Linac) versus robotic radiation system dedicated for the stereotactic treatment of paraspinal tumors. Cyberknife® plans were compared to volumetric modulated arc therapy (VMAT) plans delivered with a Versa HD®. The impact of the tumor location on the plan quality was considered.

1219P - Maternal immunization against ALK as a weapon to fight neuroblastoma

BackgroundNeuroblastoma (NB) is the most common extracranial solid tumor in infancy. Some germline and somatic mutations associated with NB have been identified as activating mutations of the anaplastic lymphoma kinase (ALK) oncogene. Because of the nature of NB, which can occur in the early post-natal life or even during fetal life, we evaluated the efficacy of a DNA vaccine against ALK in a spontaneous preclinical model of NB, harboring ALKF1174L mutation in association with MYCN amplification (ALKF1174L/MYCN mice) by means of maternal immunization (MI). MethodsMYCN transgenic females were immunized against ALK by DNA vaccination by using a plasmid coding for the extracellular and trans-membrane domains of human ALK (ALK-ECTM) followed by electroporation, and then they were mated with an ALKF1174L-transgenic male. In ALKF1174L/MYCN offspring, the presence of abdominal, cervical and paraspinal tumors has been evaluated and quantified by Magnetic Resonance Imaging. The humoral immune response induced against ALK in the mothers and their offspring, as well as the presence of immune-complexes containing ALK, have been evaluated by ELISA. ALK expression in tumor tissue was assessed by Western blot. ResultsPre-birth immunization against ALK leads to an extended survival time and to a lower tumor growth kinetic in ALKF1174L/MYCN offspring born from ALK-ECTM-vaccinated mothers (ALK-ECTM offspring) as compared to controls born from control empty vector-vaccinated mothers. Maternally derived anti-ALK antibodies were successfully transferred from mothers to newborns. Moreover, anti-ALK IgM were found in the sera of five- and six-week old ALK-ECTM offspring, suggesting the induction of the pups’ own immune response against ALK. This effect could be due to the breast milk-mediated transfer of immune-complexes containing ALK, found in the milk of vaccinated mothers and in their offspring sera. Finally, MI against ALK induces a decrease in ALK expression in ALK-ECTM offspring’ tumor tissue. ConclusionsOverall, these results indicate that MI against ALK induces an active immunization against this oncoantigen in the offspring, impairing tumor development and enhancing survival time in a preclinical model of NB. Legal entity responsible for the studyDepartment of Molecular Biotechnology and Health Sciences. FundingAssociazione Italiana per la ricerca sul Cancro. DisclosureAll authors have declared no conflicts of interest.

OS2.3 Relevance of Neurofibromatosistype 1 and schwannomotosis in extramedullary spine tumors

Abstract BACKGROUND Extramedullary spine tumors represent two-thirds of all primary spine neoplasms. Approximately half of these are peripheral nerve sheath tumors, mainly neurofibromas and schwannomas and neurofibromatosis or schwannomatosis can be suspected. Given the rarity of this condition the clinical genetic and radiological features remains to be better define. The aim of this study was to characterize the clinical, radiologic presentation of patients with widespread spinal disease and to identify gene mutation. MATERIAL AND METHODS We selected patient with a at least: intradural extramedullary, or extradural intraspinal (tumor within the spinal canal), or extradural paraspinal (tumor at the neural foramenor extending outward into adjacent tissues) neoplasms and no other tumors such as meningiomas in the spine at spine MRI. Patients’ DNA were analyzed by Targeted NGS by means a custom gene panel including NF1, NF2, LZTR1, SMARCB1 genes. RESULTS 63 patients were identified31 had few isolated tumors, involving spinal roots (Multiple Neurofibromas Few Spinal Root, MNFSR), 18 had bilateral neurofibromas involving all spinal roots. 14 had a single lesion; 10 cases were familiar and 53 sporadic. Genetic analysis showed NF1 gene mutations (in prevalence splicing or missense) in 49 cases LZTR1 mutations in 3 and in the others 11 no mutation or deletion was detected. Pain was the hallmark symptom in patients with LZTR1 mutations, while all familial cases all had NF1 diagnosis. About 50% of them had few cutaneous manifestations. CONCLUSION In patients with extramedullary spine tumors is important to look for signs of neurofibromatosis or schawannomatosis and if there are present genetic testing should be performed.

Epidemiologic and histologic characteristics of central nervous system lesions: A 20-year experience of a single institution in Lebanon.

e13544 Background: Lesions of the central nervous system (CNS) are a heterogeneous group of tumoral and non-tumoral lesions, benign or malignant, primary or metastatic. The overall incidence rate of all brain tumors is estimated to be 10.82 per 100 000 person-years. There is no data in Lebanon characterizing the CNS lesions. The aim of our study is to report the epidemiologic and histologic characteristics of these lesions in a Lebanese tertiary center. Methods: This is a retrospective study evaluating the characteristics of the CNS lesions diagnosed in 20 years (from January 1998 to June 2017) in the pathology laboratory of Hôtel Dieu de France, Saint Joseph University hospital of Beirut, Lebanon. The data were extracted from the computerized records of the laboratory. Different variables were evaluated, from the samples types and epidemiologic characteristics, to the revealing symptoms and the histologic subtypes. Statistical analysis was performed using SPSS 24.0. Results: 2025 tumoral lesions over 20 years were interpreted. 25.4% of the samples were obtained by biopsy, 65% by surgical resection, 3.7% were cerebral spinal fluid and 5.9% were undetermined. 52.2% of the patients were men and 47.8% were women with a median age of 50 years, from 0 to 89 years. The most frequent revealing symptoms were epilepsy (22.5%), headache (20.6%) and motor impairment (19.9%). 90.7% of the CNS tumors were primary and 9.3% were metastatic. Lung (35.6%) and breast (16.5 %) were the two most frequent primaries of the metastatic lesions. 46.2% of primary CNS tumors were glial, 42.5% non-glial, 3.5% mixed neuroglial and 6.4% mesenchymal. The two most frequent glial tumors were astrocytic (56.4%) and oligodendroglial (17.9%) tumors, while the most frequent non-glial tumors were meningeal tumors (58%) and cranial nerve and paraspinal tumors (16.3%). Conclusions: The histological and epidemiological characteristics of brain tumors in Lebanon are consistent in many features with what is known in the literature, but the Lebanese population differs by a younger age of onset, a lower rate of meningiomas, and a higher rate of gliomas.

Retroperitoneoscopic resection of a T11-L2 right-sided ganglioneuroma

Paraspinal tumors arising from the sympathetic chain include those on the ganglioneuroma–neuroblastoma spectrum. Accurate diagnosis often requires excision owing to the histopathologic heterogeneity of these tumors and the risk of false negative biopsy results. Choice of approach is dictated by location and extirpation is usually amenable to minimally invasive techniques. We present a patient whose paraspinal tumor included the T11-L2 vertebral body levels and was removed using a retroperitoneoscopic approach. This approach is rarely considered in pediatric general surgery and afforded a useful alternative to thoracoscopy or laparoscopy.

Preoperative Planning Using 3-Dimensional Printing for Complex Paraspinal Schwannoma Resection: 2-Dimensional Operative Video.

Schwannomas are benign peripheral nerve sheath tumors that are typically round, smooth, and straightforward to resect. Occasionally they are more complicated to resect because of their size, location, degree of bony erosion, and vascular or neural compression. These complex lesions frequently require multidisciplinary surgical teams in order to decrease surgical morbidity. Historically, surgical planning consisted of imaging review and verbal communication among team members. 3-dimensional (3D) printing offers a new method for preoperative planning and is becoming more popular in Neurosurgery. This video demonstrates the use of 3D printing for complex paraspinal tumors in the cervical, thoracic, and sacral regions. The Institutional Review Board Office for Human Research Protection does not require approval for single participant case studies, or a case series with multiple participants. The subjects involved in this study were not identifiable and the general surgical consent form included permission for intraoperative photos.

Surgical treatment of posterior mediastinal neurogenic tumors.

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. 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. 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. 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.

The implications of intradural extension in paraspinal malignant peripheral nerve sheath tumors: effects on central nervous system metastases and overall survival.

OBJECTIVEIn this study, the authors sought to compare tumors with intradural extension to those remaining in the epidural or paraspinal space with the hypothesis that intradural extension may be a mechanism for seeding of the CSF with malignant cells, thereby resulting in higher rates of CNS metastases and shorter overall survival.METHODSThe authors searched the medical record for cases of malignant peripheral nerve sheath tumors (MPNSTs) identified from 1994 to 2017. The charts of the identified patients were then reviewed for tumor location to identify patients with paraspinal malignancy. All patients included in the study had tumor specimens that were reviewed in the surgical pathology department. Paraspinal tumors with intradural extension were identified in the lumbar, sacral, and spinal accessory nerves, and attempts were made to match this cohort to another cohort of patients who had paraspinal tumors of the cranial nerves and lumbar and sacral spinal regions without intradural extension. Further information was collected on all patients with and without intradural extension, including date of diagnosis by pathology specimen review; nerve or nerves of tumor origin; presence, location, and diagnostic date of any CNS metastases; and either the date of death or date of last follow-up.RESULTSThe authors identified 6 of 179 (3.4%) patients who had intradural tumor extension and compared these patients with 12 patients who harbored paraspinal tumors that did not have intradural extension. All tumors were diagnosed as high-grade MPNSTs according to the surgical pathology findings. Four of 6 (66.7%) patients with intradural extension had documented CNS metastases. The presence of CNS metastases was significantly higher in the intradural group than in the paraspinal group (intradural, 66.7% vs paraspinal, 0%; p < 0.01). Time from diagnosis until death was 11.2 months in the intradural group and approximately 72 months in the paraspinal, extradural cohort.CONCLUSIONSIn patients with intradural extension of paraspinal MPNSTs, significantly higher rates of CNS metastases are seen with a reduced interval of time from diagnosis to metastatic lesion detection. Intradural tumor extension is also a poor prognostic factor for survival, with these patients showing a reduced mean time from diagnosis to death.

Management of Dumbbell and Paraspinal Tumors of the Thoracic Spine Using a Single-stage Posterolateral Approach: Case Series.

This study investigated the surgical results of a single-stage posterolateral approach with arc incision, unilateral laminectomy, and costotransversectomy for the management of dumbbell tumors and paraspinal tumors of the thoracic spine. From January 2010 to March 2017, 14 patients with dumbbell tumors or paraspinal tumors of the thoracic spine who underwent resection with single-stage posterolateral approach were followed up and analyzed retrospectively. The operations were performed using a single-stage posterolateral approach with arc incision, unilateral laminectomy, and costotransversectomy without any instrumentation. We reviewed the scores of clinical symptoms and imaging results, including postoperative MRI and reconstructed 3D-CT images. Gross total removal was achieved in 13 patients, and subtotal removal was achieved in 1 case. Histopathology revealed schwannoma in 9 patients, angiolipoma in 1 patient, and paraganglioma and mixed hemangioma in 2 patients each. No significant operative or postoperative complications occurred in any patient. The 14 patients were followed up for 14-68 months (mean 39.4 months). At the final follow-up, no obvious spinal deformity or tumor recurrence was found in any patient except one with paraganglioma. Single-stage posterolateral approach is a good alternative surgical method for removing dumbbell tumors and paraspinal tumors of the thoracic spine without necessitating a subsequent anterior operation.

Ganglioneuroma of the retropharyngeal space in a patient with glottic cancer

We describe the case of a 71-year-old man with a ganglioneuroma of the retropharyngeal space. The patient presented with a submucosal bulge of the left oropharyngeal wall during follow-up examination...