Diagnosis and treatment of spinal mucus nipple-shaped ventricular membrane tumor

Mucus nipple-type ventricular membrane tumor (myxopapilic ependymomas, MPE) is a rare subtype of ventricular membrane tumor, which originates from the central nervous system of the ventricular membrane cells, good hair in young people, good hair is the lumbar spine of the spinal conical cone and horsetail nerve areaTumor properties belong to WHO grade I, clinically rare, preoperative lypt, most prognosis is good; The First Affiliated Hospital of Anhui Medical University admitted 9 patients with spinal cord MPE from January 2012 to June 2018, and summarized the clinical experience as follows1Object and method1.1 Clinical data
4 cases ofmen and 5 cases of women; age 18 to 52 years old, average age 32.3 years oldClinical manifestations: mainly for lower back pain 9 cases, double lower limb weakness 4 cases, stool dysfunction 1 case, 1 case of sexual function reduction; Preoperative spinal cord McCormick functional classification: I level 4 cases, ii class 4 cases, class III 1 cases1.2 imaging dataall patients advanced to the full spinal cord MRI flat sweep plus enhanced scanning, 8 cases for single lesions, 1 cases for multipleThe main body is located in the lumbar vertebrae and below the vertebral tube, the affected spinal section 1 to 9 (average 3.8)MRI shows that the tumor is equal T1, long T2 signal, tumor signal is not uniform, after strengthening the performance of uniform or uneven reinforcement (Figure 1), most of the performance of sausage-like, a few forms of leaf-like1.3 surgical treatmentall cases of routine transdermal ization of the positive into the path of tumor removal surgery, the normal movement and movement induced electrocution and real-time lower limb, myophysiological monitoring, supplemented by the surgical B super-judge tumor has no residue and surgical removal degreeAccording to the surgical conditions to decide whether to retain the vertebral plate, combined with postoperative review OF MRI results to determine whether the patient postoperative radiotherapy4 patients went to vertebral plate decompression surgery, 1 patient walked semi-vertebral plate into the road surgery, 2 patients line vertebral plate reset, 2 patients line spinal fixation 1.4 follow-up establish patient follow-up cards, through outpatient and telephone follow-up, follow-up includes patient clinical performance, spinal cord McCormick functional classification, review of spinal cord MRI 2 Results (Figure 1) a total of 9 patients in this group received 10 post-middle-middle-path spinal tumor microsurgery, of which 1 patient tired of 9 back vertebral sections, divided into 2 operations, respectively, to remove the upper thoracic vertebrae (T3 to 7) and lumbar vertebrae (L4 to 5) section lesions Eight patients received a total excision of the tumor, and one patient received only a complete excision after the tumor was closely attached to the horsetail nerve In 8 patients, symptoms of lower back pain were alleviated to varying degrees, 3 cases of lower limb muscle strength were improved, 1 case of lower limb muscle strength decreased (III), and the muscle strength was restored to V.(-) level after functional rehabilitation training, with no deaths Postoperative pathology was confirmed to be mucus nipple-type ventricular membrane tumor (WHOI grade), immune groupation showed positive expression of GFAP, Vimentin and S-100, CK all expressed negative, Ki-67 proliferation index of about 1% Figure 1 is the middle semi vertebral plate into the road to remove the lumbar MPE 1A to 1C preoperative MRI tumor is an equal T1 signal, long T2 signal, uneven reinforcement; 1D ultrasound detection tumor upper and lower pole; 1E fully exposed tumor is a sausage-like; 1F surgery completely excision tumor; 1G pathological picture (Sumuin-I-Red staining x 100 times) follow-up 3 to 60 months, preoperative 3 cases of spinal cord McCormick functional grade II patients improved to I level, 1 case of Class III patients improved to II after surgery, the rest of the patients did not change; 3 Discussion 3.1 Epidemiology ventricular membrane tumor is the most common intraspinal tumor, accounting for about 15% of the vertebral tube tumor, accounting for 60% of the myelin tumor MPE was first reported in 1932 as a variant of the ventricular membrane tumor, which is classified as WHOI according to the WHO central nervous system tumor classification The annual incidence rate of MPE is 0.06 to 0.08 per 100,000, which is usually characterized by histological inertia, slow growth and a longer course of disease Although confined to the central nervous system, metastasis or spread with cerebrospinal fluid MPE is good in adults aged 20 to 50, mostly in men, about 66%, the vast majority of MPE occurs in the spinal cord conical horsetail area, occasionally can occur in other spinal cord sections, the essence of the brain, subcutaneous tissues, peritoneals and broad ligaments and other areas, and even spread to the liver and lungs Studies have confirmed a higher incidence of myelin external levels in PATIENTs under 20 years of age CimINO and other studies of 11 children's MPE patients clinical characteristics, compared with the same medical center of 38 adult MPE patients, the incidence of myelin MPE significantly increased (P 0001) 3.2 clinical performance MPE occurred at different sites, its clinical performance is not the same The vast majority of tumors that occur in the conical horsetail area are characterized by pain in the lower back, radiation pain in the lower extremities, sphincter dysfunction, numbness of the lower limbs can occur when the tumor volume is larger, male patients can be characterized by sexual dysfunction, other parts such as intracranial MPE can be expressed as tinnitus, facial numbness, eye movement disorders, etc 3.3 diagnostic MRI enhanced scan is its preferred examination method, in T1WI performance is equivalent or slightly lower signal, T2WI performance is uneven slightly higher signal, enhanced after the performance of significant reinforcement, a small number of tumors can be accompanied by cystic or invasive vertebrae Because MPE is rare in clinical practice, MRI results are often misdiagnosed before surgery, and need to be identified with neurosaroma, astrocyma, hemangioma and metastatic tumor The final diagnosis of MPE should be combined with postoperative pathological results, MPE's giant examination is generally leaf-like or salamious lumps, thin envelope, red meat, soft, easy to bleed mirror screening to the nipple and mucus into basic characteristics (Figure 1G), nipples are covered with single layer or multi-layer edhotosis cells, cubic or short shuttle-shaped, nipple structure center of the blood vessels and connective tissue obvious mucus change, in addition to the nipple structure, there may be epithelial area, tubular structure, fissure-like structure or micro-sac structure, occasionally visible false chrysanthemum structure Tumor cell nuclei is like no or rare, often visible bleeding, thrombosis formation The reason for the appearance of mucus nipple-like is not clear; the microstructure evidence suggests that mucus-like substances represent the sedimentation of the substrate material around the tumor cells, which are caused by abnormal co-location of the normal end silk mid-tube membrane cells and collagen, and that the mucus-like matrix that makes up the tumor may pose a great challenge to the tumor's total cutting 3.4 surgical treatment surgery is the preferred method for treating MPE, the tumor has a complete envelope, the operation is good, but the growth of huge tumors and horsetail nerves and other structures adhere tightly, overemphasis on the full-cut may cause muscle-tail nerve damage and sphincter function damage, sensory loss and other symptoms, the study shows that the application of electromyography testing to enhance the overall rate of MPE microsurgery, reduced complications this group of cases under neuroelectrophysiological monitoring with intraoperative ultrasound monitoring to achieve good results, the summary experience is as follows: (1) vertebral plate removal should fully expose the upper and lower poles of the tumor, to avoid blind lycation and pull the normal spinal cord, ultrasonic positioning in surgery can determine the scope of vertebral plate excision at both ends of the tumor After exposing the spinal cord with a sharp blade to cut the back side of the spinal cord, according to the tumor midline growth or partial growth choice of the middle line or side cut, in principle, as far as possible along the rear positive cut, so as not to damage the side of the back beam, causing deep sensory disorders (2) separation, display technology: along the correct tumor-spinal cord interface separation and removal of tumors, separation interface only pull the tumor, do not pull the spinal cord, avoid the spinal cord surface of the large veins, intermittently cut the spinal cord to remove the tumor, too much cut off the back of the spinal cord reflux veins will aggravate postoperative sensory dysfunction or increase new sensory disorders If the tumor has a coating, choose to micro-strippe along the tumor envelope separation, clean up the wild blood liquid to Linger's liquid flushing-based, less use of attractor, to avoid direct contact with the spinal cord tissue, for the local envelope loss, tumor horsetail nerve adhesion serious people can not be over-pursuit of total cutting, should be under the guidance of electrophysiological monitoring, sharp separation along the direction of the nerve (3) to remove the hemorrhage technique: the tumor texture is soft, no obvious boundary, should choose the tumor internal attraction excision, under the microscope carefully identify normal spinal cord tissue General hemorrhage as far as possible without an electric coagulant, gelatin sponge gently attached to it If it is necessary, choose a low-power flush with Linger's liquid to prevent thermal conduction damage Signs of the total excision of the tumor during surgery are: the spinal cord subsidence of the bulging, the cavity of the spinal cord at both ends of the tumor is connected to the tumor bed and there is a clear cerebrospinal fluid outflow, and the tumor bed is smooth, white or light yellow An ultrasound can ensure that the cyst has been drained and that the substantial tumor has been removed (4) attach importance to postoperative spinal stability: MPE most tired and multi-sectional sections, surgery for full exposure often requires full vertebral plate cutting, some cases of tumor removal spinal itema, often need to remove the vertebral plate, but in the early cases of this group, the removal of vertebral plate patients in the follow-up process found different degrees of spinal instability, in the medium term also tried 1 semi-vertebral plate excision, but to take open the window or tumor large-scale spinal cord injury Now the rapid development of neurospinal surgery, for the first phase of spinal fixation to provide the possibility, the group of 2 patients in the tumor after full cutting at the same time to do multi-section spinal fixation, effectively ensure spinal stability 3.5 radiotherapy and chemotherapy whether regular radiotherapy after MPE surgery is still controversial, some scholars believe that postoperative auxiliary radiotherapy can reduce the progress of tumors, another part of the scholars believe that: for patients with tumor slitence or sub-total cut, postoperative radiotherapy has no significant difference in the prognosis effect, and radiotherapy still has the risk of causing radioactive spinal cord inflammation, spinal cord adhesianduation Therefore, radiotherapy is more than applied to postoperative tumor residue and relapse spread of patients, for the tumor full-cut cases do not do conventional radiotherapy This group of 1 patients in surgery found that the tumor and horsetail nerve adhesion, full cutting difficulties, part of the residual postoperative radiation treatment, the remaining 8 patients completely removed the tumor, so no radiotherapy chemotherapy is more widely used in tumors can not be fully cut remains and radiation insensitive cases, there are cases reported to find that for multiple recurrences and the occurrence of cerebrospinal fluid spread MPE, the treatment of pyrozine may achieve good results 3.6 prognosis analysis MPE patients overall 10 years survival rate of more than 90%, KRAETZIG and other reported 1 group of MPE cases suggest that full-cut patients can obtain 100% 10 yearsurvival rate, distant transfer is mostly located in the thoracic spine (38.9%) and tibia (33.3%), partially transferable to the cranial (11.1%) For most relapsed and sowable cases, surgery is still the preferred treatment option, and radiotherapy is recommended after surgery But there are studies that show that for most OF the MPE that occurs in the distance, such as no clinical symptoms, you can choose close clinical observation and MRI follow-up, because most of the transferred MPE has no clinical progress, for clinical progress, it is still recommended to have surgery and postoperative radiotherapy, this group of 1 patients postoperative review MRI found a small lesions in the vertebral tube, no clinical symptoms, follow-up has not progression in short, for MPE patients, should be in the premise of ensuring safety to pursue the pursuit of full-cut, for patients who are not fully cut, it is recommended postoperative radiotherapy The application of neuroelectrophysiological monitoring and intraoperative B-super-technology can improve the safety of surgery and the rate of tumor total cutting, and can achieve good results in general