Lumbar lipomyelomeningocele and sacrococcygeal teratoma in siblings: support for an alternative theory of spinal teratoma formation

2010 ◽  
Vol 5 (6) ◽  
pp. 626-629 ◽  
Author(s):  
Seth F. Oliveria ◽  
Eric M. Thompson ◽  
Nathan R. Selden
Keyword(s):  
Spinal Cord ◽  
Partial Resection ◽  
Sacrococcygeal Teratoma ◽  
Alternative Theory ◽  
Type Iii ◽  
First Child ◽  
Teratoma Formation

Sacrococcygeal teratomas may arise in association with regional developmental errors affecting the caudal embryonic segments and may originate within lumbosacral lipomas. It is therefore possible that sacrococcygeal teratomas and lumbosacral lipomas represent related disorders of embryogenesis. Accordingly, the authors report the cases of 2 siblings. The first child (female) was born with a mature Altman Type III sacrococcygeal teratoma that was resected when she was a neonate. Subsequently, a younger brother was found soon after birth to have an L-4–level lipomyelomeningocele and underwent partial resection and spinal cord untethering at 4 months of age. Although familial forms of each of these conditions have been reported, this is, to the authors' knowledge, the first reported occurrence of lipomyelomeningocele and sacrococcygeal teratoma in siblings. They propose that an inherited regional tendency to developmental error affecting the caudal embryonic segments was shared by these siblings and resulted in spinal teratoma formation in one of them.

scholarly journals HGG-12. A CASE OF PEDIATRIC SPINAL HIGH-GRADE GLIOMA WITH NTRK1 GENE FUSION

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii346-iii346
Author(s):  
Tamaki Morisako ◽  
Daisuke Umebayashi ◽  
Kazuaki Kamata ◽  
Hiroyuki Yamamoto ◽  
Takumi Yamanaka ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Mr Imaging ◽  
Tumor Progression ◽  
Gene Fusion ◽  
High Grade Glioma ◽  
Gene Panel ◽  
Partial Resection ◽  
Low Grade ◽  
High Grade ◽  
Surgical Specimen

Abstract INTRODUCTION Tumors arising from the spinal cord are uncommon, especially high-grade tumors in pediatric patients. We report a case of high-grade glioma in the spinal cord harboring NTRK1 gene fusion, who received effective entrectinib therapy. CASE REPORT: A 5-year-old boy presented right hemiparesis and MR imaging revealed an intramedullary enhancing mass at the vertebral body level between C3 and Th1. He underwent microsurgical partial resection and the histological diagnosis was low-grade astrocytoma. After the first-line chemotherapy with vincristine and carboplatin, his right hemiparesis deteriorated and recurrent MR imaging showed growth of the tumor. He underwent microsurgical partial resection again and the histological examination was high-grade glioma with endothelial proliferation and necrosis. The chemoradiotherapy with temozolomide and focal irradiation of 50.4 Gy were given, and his neurological symptom slightly improved. One month later, he presented respiratory disturbance and required assisted ventilation with tracheostomy. MR imaging showed tumor progression invading upward to medulla oblongata. NTRK1 gene fusion was detected in the previous surgical specimen by a gene panel testing, and he received entrectinib, a potent inhibitor of tropomyosin receptor kinase (TRK). Since then, no tumor progression has been demonstrated for several months by MRI and he has been stable neurologically. CONCLUSION High-grade spinal cord tumors are rare and effective treatment strategies have not been addressed. Although the frequency of the gene fusion is very low in pediatric gliomas, identification of the driver gene aberration like in this case by a gene panel can provide potential targeted therapies for selected patients.

A case of neonatal neuroblastoma mimicking Altman type III sacrococcygeal teratoma

2005 ◽  
Vol 40 (3) ◽  
pp. 578-580 ◽  
Author(s):  
Keiichiro Tanaka ◽  
Masaki Kanai ◽  
Jyoji Yosizawa ◽  
Yoji Yamazaki
Keyword(s):  
Sacrococcygeal Teratoma ◽  
Type Iii

LONG-TERM OUTCOME OF TOTAL AND NEAR-TOTAL RESECTION OF SPINAL CORD LIPOMAS AND RADICAL RECONSTRUCTION OF THE NEURAL PLACODE

Neurosurgery ◽  
2009 ◽  
Vol 65 (3) ◽  
pp. 511-529 ◽  
Author(s):  
Dachling Pang ◽  
John Zovickian ◽  
Angelica Oviedo
Keyword(s):  
Magnetic Resonance Imaging ◽  
Spinal Cord ◽  
Magnetic Resonance ◽  
Partial Resection ◽  
Resonance Imaging ◽  
Surgical Morbidity ◽  
Total Resection ◽  
Sagittal Diameter ◽  
Radical Reconstruction

Abstract OBJECTIVE Partial resection of complex spinal cord lipomas is associated with a high rate of symptomatic recurrence caused by retethering, presumably promoted by a tight content-container relationship between the spinal cord and the dural sac, and incomplete detachment of the terminal neural placode from residual lipoma. Since 1991, we have performed more than 250 total/near-total resections of complex lipomas with radical reconstruction of the neural placodes. Sixteen years of follow-up have proven the long-term benefits of this technique. Part I of this series introduces our technique of total resection and reports the immediate surgical results. Part II will analyze the long-term outcomes of both total and partial resection and identify the factors affecting outcome. METHODS From 1991 to 2006, 238 patients (age range, 2 months–72 years) with dorsal, transitional, and chaotic lipomas underwent total or near-total lipoma resection and radical placode reconstruction. Eighty-four percent of the patients were children younger than 18 years and 16% were adults. The technique consisted of wide bony exposure, complete unhinging of the lateral adhesions of the lipoma-placode assembly from the inner dura, untethering of the terminal conus, radical resection of the fat off the neural plate along a white fibrous plane at the cord-lipoma interface, meticulous pia-to-pia neurulation of the supple neural placode with microsutures, and expansile duraplasty with a bovine pericardial graft. Elaborate electrophysiological monitoring was used. RESULTS Three postoperative observations concern us. The first is that of the 238 patients, 138 (58%) had no residual fat on postoperative magnetic resonance imaging; 81 patients (36%) had less than 20 mm3 of residual fat, the majority of which were small bits enclosed by neurulation; and 19 patients (8%), mainly of the chaotic lipoma group, had more than 20 mm3 of fat. There are no significant differences in the amount of residual fat among lipoma types, but redo lipomas are more likely than virgin (previously unoperated on) lipomas to have residual fat by a factor of 2 (P = 0.0214). The second concern is that the state of the reconstructed placode is objectively measured by the cord-sac ratio, obtained by dividing the sagittal diameter of the reconstructed neural tube by the sagittal diameter of the thecal sac. A total of 162 patients (68%) had cord-sac ratios less than 30% (low), 61 (25.6%) had ratios between 30% and 50% (medium), and only 15 (6.3%) had high ratios of more than 50%. Seventy-four percent of patients with virgin lipomas had low cord-sac ratios compared with 56.3% in the redo lipoma patients. The overall distribution of cord-sac ratio is significantly different between redo and virgin lipomas (P = 0.00376) but not among lipoma types. Finally, the incidence of combined neurological and urological complications was 4.2%. The combined cerebrospinal fluid leak and wound infection/dehiscence incidence was 2.5%. Both sets of surgical morbidity compared favorably with the published rates reported for partial resection. CONCLUSION Total/near-total resection of spinal cord lipomas and complete reconstruction of the neural placode can be achieved with low surgical morbidity and a high yield of agreeable postoperative cord-sac relationship. Some large rambling transitional lipomas and most chaotic lipomas are the most difficult lesions to resect and tend to have less favorable results on postresection magnetic resonance imaging.

scholarly journals Sacrococcygeal teratoma with intradural extension: case report

2015 ◽  
Vol 15 (4) ◽  
pp. 380-383 ◽  
Author(s):  
Shima Shahjouei ◽  
Sara Hanaei ◽  
Farideh Nejat ◽  
Maryam Monajemzadeh ◽  
Mostafa El Khashab
Keyword(s):  
Case Report ◽  
Alpha Fetoprotein ◽  
Sacrococcygeal Teratoma ◽  
Rare Entity ◽  
Imaging Findings ◽  
Healthy Neonate ◽  
Serum Alpha Fetoprotein ◽  
Teratoma Formation ◽  
High Level

Intradural sacrococcygeal teratoma (SCT) is a rare entity that has been reported in only a few cases previously. The authors present the case of a 2-week-old, otherwise healthy neonate with a mass in the buttock. The imaging findings and the high level of serum alpha-fetoprotein were highly suggestive of SCT. On operation the authors found intradural extension of the teratoma. The lesion was managed successfully without any remaining sequelae. The authors briefly review the currently proposed etiology regarding teratoma formation and the intradural extension of SCT.

Unusual intradural spinal metastasis of a cranial chordoma

1971 ◽  
Vol 34 (4) ◽  
pp. 560-562 ◽  
Author(s):  
Daniel R. Stough ◽  
Joe T. Hartzog ◽  
Robert G. Fisher
Keyword(s):  
Spinal Cord ◽  
Unusual Case ◽  
Spinal Metastasis ◽  
Partial Resection ◽  
Content Type ◽  
Irradiation Therapy ◽  
Fine Print

✓ In this unusual case, multiple intradural spinal cord implants of chordoma were removed 5 years after partial resection of an intracranial chordoma. The “seeding” had occurred in spite of combined surgical and irradiation therapy.

scholarly journals Clinical and Radiological Characterization of an Infant with Caudal Regression Syndrome Type III

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Kavinda Dayasiri ◽  
V. Thadchanamoorthy ◽  
Kaushika Thudugala ◽  
Aruni Ranaweera ◽  
N. Parthipan
Keyword(s):  
Spinal Cord ◽  
Lumbar Vertebra ◽  
Syndrome Type ◽  
Caudal Regression Syndrome ◽  
Type Iii ◽  
Caudal Regression ◽  
Long Term Prognosis ◽  
Genetic And Environmental Factors

Caudal regression syndrome is a rare disorder of developmental failure of lumbosacral vertebra and corresponding spinal cord during notochord formation. The severity varies from absent coccyx to complete absence of lumbosacral vertebra and caudal spinal cord. Both genetic and environmental factors are believed to play roles in aetiopathogenesis of caudal regression. The authors report a two-month-old child born to a diabetic mother, in whom the diagnosis of caudal regression syndrome type III was confirmed based on clinical and radiological characteristics. The child was managed by the multidisciplinary team to continue supportive care and screen and monitor for long-term complications. The long-term prognosis for mobility was less favourable given the presence of bilateral hip dysplasia and involvement of lumbar vertebra in addition to sacral agenesis.

Visualization of Poliovirus Type III in Paraffin Sections of Monkey Spinal Cord by Indirect Immuno-fluorescence

Nature ◽  
1963 ◽  
Vol 200 (4905) ◽  
pp. 497-498 ◽  
Author(s):  
E. KOVÁCS ◽  
R. K. BARATAWIDJAJA ◽  
N. A. LABZOFFSKY
Keyword(s):  
Spinal Cord ◽  
Paraffin Sections ◽  
Poliovirus Type ◽  
Type Iii

scholarly journals Long-Term Outcome of Total and Near-Total Resection of Spinal Cord Lipomas and Radical Reconstruction of the Neural Placode, Part II

Neurosurgery ◽  
2010 ◽  
Vol 66 (2) ◽  
pp. 253-273 ◽  
Author(s):  
Dachling Pang ◽  
John Zovickian ◽  
Angelica Oviedo
Keyword(s):  
Spinal Cord ◽  
Free Probability ◽  
Partial Resection ◽  
The Other ◽  
Nonsurgical Treatment ◽  
Predictor Variables ◽  
Cerebrospinal Fluid Leakage ◽  
Total Resection ◽  
Patient Profile

Abstract OBJECTIVE To show the long-term benefits of total and near-total resection of complex spinal cord lipomas and reconstruction of the neural placode. METHODS We analyzed 238 patients with dorsal, transitional, and chaotic lipomas who had total resection as described in part I for overall progression-free survival probability (PFS, Kaplan-Meier analysis) over 16 years. We also analyzed subgroup proportional recurrence hazard (Cox analysis) of 6 outcome predictors of sex, lipoma type, age, preoperative symptoms, previous surgery, and postoperative cord-sac ratio. These results were compared with an age-matched, lesion-matched series of 116 patients followed for 11 years after partial lipoma resection and with the Parisian series of nonsurgical treatment. RESULTS The immediate effects of surgery were similar between total and partial resection: both achieved greater than 95% symptom stabilization or improvement rate. The neuro-urologic complication rates for the groups were also similar, 4.2% and 5.2% for total and partial resection, respectively. The combined cerebrospinal fluid leakage and wound complication rate of total resection was much lower at 2.5% than the 6.9% for partial resection, but both were better than published rates. The overall PFS for total resection was 82.8% at 16 years, comparing much more favorably with 34.6% for partial resection at 10.5 years (P < .0001). Culling only the asymptomatic patients with virgin (previously unoperated) lipomas to match the patient profile of the Parisian series, the PFS for prophylactic total resection for this subgroup increased to 98.4% at 16 years, versus 67% at 9 years for no surgery and 43.3% at 10.5 years for our own partial resection series, with a remarkable statistical difference between total and partial resection (P = .00001). Subgroup analyses showed that sex and lipoma type did not affect outcome. For the other predictor variables, while univariate analyses showed that young age, absence of symptom, and virgin lipomas correlated with better statistical PFS than older age, symptoms, and redo lipomas, these effects vanished with multivariate analyses. Cord-sac ratio stood alone as the only influential outcome predictor in multivariate analysis, with a 96.6% PFS for a low ratio of <30% and an 80.6% progression-free probability for a high ratio of >50%, and a 3-fold increase in recurrence hazard for high ratios (P = .0009). This suggested that all the individual effects of the other predictor variables could be reduced to whether a low cord-sac ratio could be achieved with total lipoma resection and placode reconstruction. Cord-sac ratio was the obvious factor that differentiated the outcomes between total and partial resection, the latter associated with a >90% chance of having a high cord-sac ratio. CONCLUSION Total and near-total resection of lipomas and complete reconstruction of the neural placode produced a much better long-term progression-free probability than partial resection and nonsurgical treatment. The perioperative complications for total resection were low and compared favorably with published results. A low postoperative cord-sac ratio and well-executed placode neurulation were strongly correlated with good outcome. The ideal preoperative patient profile with early disease stabilization and the best recurrence-free probability is an asymptomatic child less than 2 years without previous lipoma surgery. There are strong indications that partial resection in many cases produces worse scarring on the neural placode and worse prognosis than no surgery.

scholarly journals The BCM rule allows a spinal cord model to learn rhythmic movements

2021 ◽  
Author(s):  
Matthias Kohler ◽  
Philipp Stratmann ◽  
Florian Roehrbein ◽  
Alois Knoll ◽  
Alin Albu-Schaeffer ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Visual Cortex ◽  
Learning Rule ◽  
Biomechanical Properties ◽  
Pattern Generator ◽  
Alternative Theory ◽  
Rhythmic Movements ◽  
Animal Locomotion ◽  
Network Properties ◽  
Genetically Determined

Animal locomotion is hypothesized to be controlled by a central pattern generator in the spinal cord. Experiments and models show that rhythm generating neurons and genetically determined network properties could sustain oscillatory output activity suitable for locomotion. However, current CPG models do not explain how a spinal cord circuitry, which has the same basic genetic plan across species, can adapt to control the different biomechanical properties and locomotion patterns existing in these species. Here we demonstrate that rhythmic and alternating movements in pendulum models can be learned by a monolayer spinal cord circuitry model using the BCM learning rule, which has been previously proposed to explain learning in the visual cortex. These results provide an alternative theory to CPG models, because rhythm generating neurons and genetically defined connectivity are not required in our model.

scholarly journals Features and risk factors for recurrence of intradural spinal tumors

2019 ◽  
Vol 18 (1) ◽  
pp. 21-29
Author(s):  
V. A. Byvaltsev ◽  
I. A. Stepanov
Keyword(s):  
Risk Factors ◽  
Spinal Cord ◽  
Neurological Deficit ◽  
Malignant Tumors ◽  
Significant Risk ◽  
Partial Resection ◽  
Spinal Tumors ◽  
Benign Tumors ◽  
Spinal Cord Tumors ◽  
Risk Factors For Recurrence

Spinal cord tumors include a variety of nosological units and are classified according to their localization and histological type. The search for literature sources in the Pubmed, EMBASE and eLibrary databases demonstrated the absence of studies devoted to study of the features and risk factors for the recurrence of intradural spinal tumors. the purpose of this study was to reveal features and risk factors of recurrence of intradural spinal tumors after microneurosurgical resection. material and methods. The study included medical records of 196 patients with intradural extramedullary and intramedullary spinal tumors. The extent of microneurosurgical tumor resection, clinical efficacy of surgery, and risk factors for recurrence of intradural spinal cord tumors were been analyzed. results. Improvement in neurologic deficit after surgery was noted in 116 (59.1 %) cases, neurologic status remained the same in 47 (24.0 %) patients, and worsening of neurological deficit was observed in 33 (16.8 %) cases. Total microneurosurgical resection of intradural spinal tumors was performed in 140 (71.4 %) patients, subtotal resection in 22 (11.2 %) patients, partial resection in 25 (12.7 %) patients and spinal cord decompression or biopsy and/or its roots were performed in 9 (4.6 %) of patients. The likelihood of recurrence-free survival of patients with benign intradural spinal cord tumors was significantly higher than that of patients with malignant tumors (p<0.001). Benign tumors (χ2=34.7, p<0.05), thoracic and lumbosacral tumors (χ2=10.3, p<0.05), low degree of neurological deficit (χ2=31.5, p<0.05), absence of syringomyelia/syringobulbia signs (χ2=13,2, p<0,05), as well as extramedullary tumors (χ2=12,6, p<0.05) allowed us to perform total degree microneurosurgical resection. Malignant tumors (χ2=34.8, p<0.05), cervical and thoracic tumors (χ2=8,4, p<0,05), high degree of neurological deficit (χ2=12,9, p<0.05), partial resection, biopsy or decompression of neural structures (χ2=9.7, p<0.05) and intramedullary tumors statistically significantly increased the risk of their recurrence. conclusion. Histological pattern, tumor localization, preoperative clinical and neurological deficit according to the McCormick classification and the extent of surgery are significant risk factors for recurrence of intradural spinal tumors.

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