The neurofibromatosis (NF) are a set of genetic disorders which cause tumors to grow along various types of nerves and, in addition, can affect the development of non-nervous tissues such as bones and skin. NF causes tumors to grow anywhere on or in the body. It also leads to developmental abnormalities. For example, individuals with NF have a higher incidence of learning disabilities. Neurofibromatosis(NF) has been classified into two distinct types: NF-I and NF-II. neurofibromatosis 1(NF-I), also known as von Recklinghausen NF or Peripheral NF, occurring in 1:4,000 births, is characterized by multiple cafe-au-lait spots and neurofibromas on or under the skin. Enlargement and deformation of bones and curvature of the spine (scoliosis) may also occur. Occasionally, tumors may develop in the brain, on cranial nerves, or on the spinal cord. About 50% of people with NF also have learning disabilities. Neurofibromatosis 2(NF-II), also known as Bilateral Acoustic NF(BAN), is much rarer occurring in 1:50,000 births. NF-II is characterized by multiple tumors on the cranial and spinal nerves, and by other lesions of the brain and spinal cord. Tumors affecting both of the auditory nerves are the hallmark. Hearing loss beginning in the teens or early twenties is generally the first symptom. We reported a 10-year-old female patient with NF-I, she has pain and edema in left leg, no symptoms of NF.
Objective : The efficacy of preoperative embolization for hypervascular metastatic spine disease (MSD) such as renal cell and thyroid cancers has been reported. However, the debate on the efficacy of preoperative embolization for non-hypervascular MSD still remains unsettled. The purpose of this study is to determine whether preoperative embolization for non-hypervascular MSD decreases perioperative blood loss. Methods : A total of 79 patients (36 cases of preoperative embolization and 43 cases of non-embolization) who underwent surgery for metastatic spine lesions were included. Representative hypervascular tumors such as renal cell and thyroid cancers were excluded. Intraoperative and perioperative estimated blood losses (EBL), total number of transfusion and calibrated EBL were recorded in the embolization and non-embolization groups. The differences in EBL were also compared along with the type of surgery. In addition, the incidence of Adamkiewicz artery and complications of embolization were assessed. Results : The average age of 50 males and 29 females was $57.6{\pm}13.5$ years. Lung (30), hepatocellular (14), gastrointestinal (nine) and others (26) were the primary cancers. The demographic data was not significantly different between the embolization and the non-embolization groups. There were no significant differences in intraoperative EBL, perioperative EBL, total transfusion and calibrated EBL between two groups. However, intraoperative EBL and total transfusion in patients with preoperative embolization were significantly lower than in non-embolization in the corpectomy group (1645.5 vs. 892.6 mL, p=0.017 for intraoperative EBL and 6.1 vs. 3.9, p=0.018 for number of transfusion). In addition, the presence of Adamkiewicz artery at the index level was noted in two patients. Disruption of this major feeder artery resulted in significant changes in intraoperative neuromonitoring. Conclusion : Preoperative embolization for non-hypervascular MSD did not reduce perioperative blood loss. However, the embolization significantly reduced intraoperative bleeding and total transfusion in corpectomy group. Moreover, the procedure provided insights into the anatomy of tumor and spinal cord vasculature.
The Journal of the Korean bone and joint tumor society
/
v.10
no.2
/
pp.79-87
/
2004
Purpose: The recent development of MR has made to possible radiological diagnosis in various soft tssue tumors. But multifarious components within soft tissue tumors and their periodic change have made to difficult even differentiation of malignant from benign soft tissue tumors solely on the MR. So authors retry to differentiate malignant from benign soft tissue tumors with clinical and MR finding complex. Materials and methods: We were analysed 82 pathologically confirmed soft tissue solid tumors (37 cases as malignancy including intermediate tumors and 45 cases as benign including inflammatory masses) which are correlated with clinical findings such as age, size, and location, MR findings such as tumor border, texture on T2 and contrast-T1 images, and enhancement area retrospectively. Many typical lipoma and cysts including of ganglion and abscess are rejected in the benign soft tissue tumor group because not difficult to diagnose on MR. Results: Malignant soft tissue tumors were more frequent in 21~40 and 61~80 years old of the age, above 3.0 cm of the size, trunk-pelvis-lower extremities of the location, and MR findings with irregular border and above 50% of the enhancement area than those of benign soft tissue tumors. Conclusion: The clinical finding that divided to two locations as trunk-pelvis-lower extremities and upper extremities-shoulder-spine was statistically significant to differentiate malignant from benign soft tissue solid tumors. However, the others would provide some useful informations to differentiate them never specific.
Ji-Won Jeon;Kyu-Won Kang;Woo-Keyoung Kim;Sook Yang;Byung-Jae Kang
Journal of Veterinary Science
/
v.25
no.1
/
pp.2.1-2.14
/
2024
Background: Sufficient surgical resection is necessary for effective tumor control, but is usually limited for vertebral tumors, especially in the cervical spine in small animal neurosurgery. Objective: To evaluate the primary stability and safety of customized three-dimensional (3D)-printed implants for cervical spine reconstruction after total vertebrectomy. Methods: Customized guides and implants were designed based on computed tomography (CT) imaging of five beagle cadavers and were 3D-printed. They were used to reconstruct C5 after total vertebrectomy. Postoperative CT images were obtained to evaluate the safety and accuracy of screw positioning. After harvesting 10 vertebral specimens (C3-C7) from intact (group A) and implanted spines (group B), implant stability was analyzed using a 4-point bending test comparing with groups A and C (reconstituted with plate and pins/polymethylmethacrylate after testing in Group A). Results: All customized implants were applied without gross neurovascular damage. In addition, 90% of the screws were in a safe area, with 7.5% in grade 1 (< 1.3 mm) and 2.5% in grade 2 (> 1.3 mm). The mean entry point and angular deviations were 0.81 ± 0.43 mm and 6.50 ± 5.11°, respectively. Groups B and C significantly decreased the range of motion (ROM) in C3-C7 compared with intact spines (p = 0.033, and 0.018). Both groups reduced overall ROM and neutral zone in C4-C6, but only group B showed significance (p = 0.005, and 0.027). Conclusion: Customized 3D-printed implants could safely and accurately replace a cervical vertebra in dog cadavers while providing primary stability.
Ropper, Alexander E.;Huang, Kevin T.;Ho, Allen L.;Wong, Judith M.;Nalbach, Stephen V.;Chi, John H.
Neurospine
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v.15
no.4
/
pp.338-347
/
2018
Objective: Patients with extradural spine tumors are at an increased risk for intraoperative cerebrospinal fluid (CSF) leaks and postoperative wound dehiscence due to radiotherapy and other comorbidities related to systemic cancer treatment. In this case series, we discuss our experience with the management of intraoperative durotomies and wound closure strategies for this complex surgical patient population. Methods: We reviewed our recent single-center experience with spine surgery for primarily extradural tumors, with attention to intraoperative durotomy occurrence and postoperative wound-related complications. Results: A total of 105 patients underwent tumor resection and spinal reconstruction with instrumented fusion for a multitude of pathologies. Twelve of the 105 patients (11.4%) reviewed had intraoperative durotomies. Of these, 3 underwent reoperation for a delayed complication, including 1 epidural hematoma, 1 retained drain, and 1 wound infection. Of the 93 uncomplicated index operations, there were a total of 9 reoperations: 2 for epidural hematoma, 3 for wound infection, 2 for wound dehiscence, and 2 for recurrent primary disease. One patient was readmitted for a delayed spinal fluid leak. The average length of stay for patients with and without intraoperative durotomy was 7.3 and 5.9 days, respectively, with a nonsignificant trend for an increased length of stay in the durotomy cases (p=0.098). Conclusion: Surgery for extradural tumor resections can be complicated by CSF leaks due to the proximity of the tumor to the dura. When encountered, a variety of strategies may be employed to minimize subsequent morbidity.
Of 34 evaluated patients with primary spinal cord tumors, 32 were irradiated at our institution between 1969 and 1983. The results are reported of 32 patients, 16 with ependymoma and 16 with astrocytoma, who were treated with post-operative external beam radiotherapy following biopsy or subtotal resection Twenty-nine patients received $45\~55Gy$ megavoltage beam irradiation in $5\~6$ weeks and the remaining three patients received less than 40Gy. Spinal cord was in the irradiated field for six patients who received more than 50Gy. The minimum follow-up was five years. Five and ten year acturaial survival rates for entire group of patients were $73\%(22/30)\;and\;50\%(8/16)$, including three patients who were salvaged by surgery after radiation failures. Corresponding five and ten year relapse free survival rates were $60\%(18/30)\;and\;32\%(6/19)$, respectively. Of the 29 patients who recived more than 45Gy, relapse free survival at five years was $63\%(17/27)$. Treatment failed in 13 patients and all of those failures were in the irradiated portal. Patients with ependymomas have significantly better relapse free survival than those with astrocytomas, $80\%\;vs.\;40\%$ (p<0.05). There was significant difference in survival between patients with tumors involving the cervical spine and those with tumors in the other loactions, $45\%$ vs. $89\%(p<0.05)$. There was no significant differnece in survival between patients with cauda equina tumors and those with tumors at spinal cord, $100\%\;vs.\;68\%(p>0.05)$. No radiotherapy related neurological deficit was noted with a maximum 20 year follow-up. This study confirms that external beam radiotherapy is a safe and effective treatment modality for primary spinal cord tumors.
Tumors of the central nervous system are common in the pediatric population and constitute the second most prevalent tumor type in children. Within this group, spinal cord tumors are relatively rare and account for 1 to 10% of all pediatric central nervous system tumors. We describe a very rare case of an intradural extramedullary spinal cord tumor with a subcutaneous mass and discuss its clinical presentation, pathogenesis, and treatment. A male infant was delivered normally, with uneventful development. At 16 days post-delivery, his family took him to a pediatrician because of a mass on his upper back. Magnetic resonance imaging of the thoracic spine revealed a well-demarcated soft-tissue mass with central cystic change or necrosis at the subcutaneous layer of the posterior back (T2-7 level). Another mass was found with a fat component at the spinal canal of the T1-3 level, which was intradural extramedullary space. After six weeks, the spinal cord tumor and subcutaneous mass were grossly total resected; pathologic findings indicated an atypical myxoid spindle cell neoplasm, possibly nerve sheath in type. The final diagnosis of the mass was an atypical myxoid spindle cell neoplasm. The postoperative course was uneventful, and the patient was discharged after nine days without any neurological deficit. We report a rare case of an intradural extramedullary spinal tumor with subcutaneous mass in a neonate. It is necessary to monitor the patient's status by examining consecutive radiologic images, and the symptoms and neurological changes should be observed strictly during long-term follow-up.
Background Leptomeningeal metastasis (LM) is an uncommon, but devastating complication of advanced cancer and has no standard treatment. Herein, we analyzed the clinical characteristics and outcomes of patients with solid tumors who were diagnosed with LM. Methods Between January 2007 and December 2017, we retrospectively analyzed the medical records of patients with solid tumors who were diagnosed with LM. Results A total of 58 patients were enrolled in this study. The median age of patients was 51 years (range, 27-72 years), and 62.1% had a poor Eastern Cooperative Oncology Group (ECOG) performance status (PS) (>2). The common types of primary tumor were breast cancer (39.7%), gastric cancer (25.9%), and non-small cell lung cancer (20.7%). Forty-two patients (72.4%) were diagnosed with LM by MRI of the brain and/or spine and cerebrospinal fluid (CSF) analysis, 14 were diagnosed by CSF analysis alone, and 2 were diagnosed by MRI alone. Treatments for LM were performed in 53 patients (91.4%), and best supportive care was provided for 5 patients (8.6%). Intrathecal chemotherapy, radiotherapy, and systemic chemotherapy were administered in 43 (74.1%), 17 (29.3%), and 24 (41.4%) patients, respectively. The median overall survival of the entire cohort was 2.4 months (95% confidence interval, 1.0-3.7). In the analysis of prognostic factors for survival, a good ECOG PS (${\leq}2$), administration of systemic chemotherapy after LM diagnosis, and a prior history of brain radiation were associated with prolonged survival. Conclusion Although the prognosis of LM in patients with solid tumors is poor, systemic chemotherapy might improve survival in selected patients with a good PS.
Chondrosarcomas are malignant cartilage-forming tumors of bone which exhibit resistance to both chemotherapy and radiation treatment. miRNAs have been well demonstrated to regulate gene expression and play essential roles in a variety of biological processes, including proliferation, differentiation, migration, cell cycling and apoptosis. In this study, we obtained evidence that miR-100 acts as a tumor suppressor in human chondrosarcomas. Interestingly, cisplatin resistant chondrosarcoma cells exhibit decreased expression of miR-100 compared with parental cells. In addition, we identified mTOR as a direct target of miR-100. Overexpression of miR-100 complementary pairs to the 3' untranslated region (UTR) of mTOR, resulted in sensitization of cisplatin resistant cells to cisplatin. Moreover, recovery of the mTOR pathway by overexpression of S6K desensitized the chondrosarcoma cells to cisplatin, suggesting the miR-100-mediated sensitization to cisplatin dependent on inhibition of mTOR. In summary, the present studies highlight miR-100 as a tumor suppressor in chondrosarcoma contributing to anti-chemoresistance. Overexpression of miR-100 might be exploited as a therapeutic strategy along with cisplatin-based combined chemotherapy for the treatment of clinical chondrosarcoma patients.
Kim, Kyung-Je;Kang, Ho-Seong;Kim, Yon-Il;Shin, Byung-Joon
The Journal of the Korean bone and joint tumor society
/
v.7
no.4
/
pp.133-138
/
2001
Purpose : To analyze the clinical behaviors and survivorship of metastatic bone tumors. Materials and Methods : One hundred and ninty-eight metastatic bone tumors had been diagnosed from January 1982 to December 1998. Age and sex distribution, primary cancer types. metastatic sites, duration from diagnosed of primary tumors to bony metastases and survivorship were analysed. Results : Mean age was 57(24~86) years old. Lung(32.3%) and breast(16.2%) cancers were two most common primary foci. The spines was the most common site of metastases especially lumbar region(38%). Survivorship analysis was done in one hundred and fifteen patients who had been followed up. The mean survival period was 15.3 months. The survivorship of hepatoma(7.1 Mons), lung(8.72 Mons) and renal cell(4.8 Mons)cancers was relatively shorter and breast cancer(54.1 Mons) longest. Conclusion : The mean age of metastatic bone tumors of this study was older than the past reports. The axial skeletons especially spine was predominant metastatic site. The survivorship of metastatic bone tumor decreased sharply as time goes by, so early diagnosis is clue for longer survival after bony metastases.
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