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1.
Melanotic neuroectodermal tumor of infancy (MNTI) is a rare but well-documented lesion of neuroectodermal derivation. Maturation of the neural elements has been reported only occasionally. We report a case of MNTI of the maxilla showing maturation of neural elements to ganglionic cells. © Wiley-Liss, Inc.  相似文献   

2.
A wide variety of lesions occur in maxilla. Non specificity of clinical and radiological features makes diagnosis of these lesions a difficult task. We report six interesting cases of maxillary swelling among a total number of 37 such lesions of maxilla. These six cases are as follows two cases of central giant cell granuloma, two cases of fibrous dysplasia, one case of pigmented melanotic neuroectodermal tumor and one case of solitary myofibroma.  相似文献   

3.
婴儿色素性神经外胚瘤临床病理观察   总被引:1,自引:0,他引:1  
目的:探讨色素性神经外胚瘤的病理形态、免疫组化及诊断和鉴别要点。对5例色素性神经外胚瘤病例进行临床病理、免疫组化及电镜观察。结果:色素性神经外胚瘤好发于1岁以内的婴儿,3例肿瘤发生在上颌骨,2例发生在附皋。镜下由两种细胞构成,即上皮样瘤细胞和小圆形似成神经细胞样瘤细胞。免疫组化示上皮样瘤细胞CK、HMB45阳性,小圆形瘤细胞NSE阳性,超微结构显示肿瘤细胞内分别可见前黑色素小体、黑色素小体和神经内分泌颗粒。结论:色素性神经外胚瘤是一种少见的原始性神经外胚层肿瘤,生物学行为属于潜在恶性或低度恶性肿瘤。  相似文献   

4.
目的:初步探讨骨原始神经外胚层肿瘤的临床特征及治疗原则。方法:回顾性分析10例骨原始神经外胚层肿瘤的治疗及生存情况。10例中1例行左肩关节解脱术,1例行右髋关节解脱术,2例行左大腿中段截肢术,6例行骨肿瘤切除加灭活回植加内固定术。10例患者术后均行6周期IFO+VP-16常规化疗。结果:经手术及术后化疗,除3例生存10、14和15个月死亡外,余7例已分别生存9、12、14、16、18、22及25个月。结论:根治性手术治疗加术后早期化疗是治疗骨原始神经外胚层肿瘤的有效手段。  相似文献   

5.
目的 探讨进一步提高嗅沟脑膜瘤治疗效果的有效方法.方法 应用显微神经外科技术治疗嗅沟脑膜瘤67例,其中首次发病57例,复发8例,二次复发2例.术中采用改良Derome入路12例,双侧额底入路28例,改良翼点入路21例,单侧额底入路6例.肿瘤切除包括附着硬膜、肿瘤基底增生破坏的骨质以及受累的副鼻窦黏膜,最后对缺损颅底做修补重建.结果 按照脑膜瘤切除的Simpson分级标准,肿瘤获Simpson Ⅰ类切除59例,Ⅱ类切除7例,Ⅳ类切除1例.术后并发脑脊液漏和颅内感染仅1例,经处理治愈.术后1个月内死亡2例(2.9%),死因分别为心衰和下丘脑损伤.47例(72.3%)患者随访1~10年,平均64个月.2例高龄患者死亡,其中1例死于肺炎,1例死因不详.45例生存患者中,复发3例,为Ⅱ类或Ⅳ类切除者,Ⅰ类切除者均无复发.除3例患者仍存在视力下降和2例患者一侧肢体轻瘫外,其余患者恢复良好,已恢复工作或能生活自理.结论 全切除肿瘤(Simpson Ⅰ类)应是嗅沟脑膜瘤的手术目的,特别是新发的病例.选择适当的手术入路是全切除肿瘤的根本保证.采用适当的带血管蒂材料修补、重建前颅底是手术治疗嗅沟脑膜瘤至关重要和必须做到的.
Abstract:
Objective To explore an effective method for further improving the surgical results of treatment of olfactory groove meningiomas.Methods Sixty seven cases of olfactory groove meningiomas were treated by microneurosurgery, among which fifty seven were de novo cases, eight were recurrent tumors and the other two re-recurrent cases.Modified Derome approach was used in 12 cases, bilateral subfrontal approach in 28 cases, modified pterional approach in 21 cases and unilateral subfrontal approach in six cases.Tumors were resected microsurgically with radical removal of invaded dura, bone, and paranasal sinus mucosa.Reconstruction was performed in patients with skull base defect.Results Simpson grade Ⅰ removal was accomplished in 59 cases, grade Ⅱ in seven cases and grade Ⅳ in one case.Among 57 patients with de novo tumor, Simpson Ⅰ resection was accomplished in 54 cases.Postoperative rhinorrhea and intracranial infection occurred in one case and was cured after temporal lumbar CSF drainage and antibiotic therapy.Two patients ( 2.9% ) died within one month after operation, i.e.one aged patient of heart failure and the other of severe hypothalamus complication.Forty seven patients(72.3% ) were followed up from one to ten years with an average of five years and four months.With the exception of two cases died,among the alive 45 patients, there were only three patients with tumor recurrence, which had undergone Simpson Ⅱ or Ⅳ tumor resection.No recurrence was found in cases with Simpson Ⅰ tumor removal.Previous blurred vision was not improved in three patients, hemiparalysis in two patients, and the other patients recovered well, resuming previous jobs or being able to take care themselves.Conclusions Total tumor removal ( Simpson Ⅰ ) should be the surgical goal for treatment of olfactory groove meningiomas,especially for de novo cases.An appropriate approach is fundamental in the effort to remove an OGM totally.Appropriate anterior skull base reconstruction with vascularized material is important and mandatory.  相似文献   

6.
目的 探讨儿童大脑神经外胚叶肿瘤的临床特点及治疗方法。方法 对我院1999年1月至2003年1月收治的4例小儿大脑神经外胚叶肿瘤的临床资料进行回顾性分析。结果 4例病人1例位于额叶,2例位于顶叶,1例位于颞叶。OT显示为高或等密度影,MRI为短TI长T2信号,增强后均匀强化,肿瘤有明显界线。4例均在显微镜下全切,病理诊断:原始神经外胚叶肿瘤。术后均行放疗。术后8个月~18个月复发。结论 小儿大脑原始神经外胚叶肿瘤为高度恶性的肿瘤,预后差手术加放疗能延长生存时间。  相似文献   

7.
To present four rare cases of peripheral primitive neuroectodermal tumors of different sites of head and neck region. Four cases of different age (range 8–40 years) and sex (three female, one male) with rare primitive neuroectodermal tumor of sinonasal region and neck are presented. Treatment options, biological behavior and prognostic outcome are discussed herewith. Two patients succumbed to the disease within four to six months of treatment; other two patients are still under follow-up depicting the aggressive nature of the tumor. Primitive neuroectodermal tumor belongs to the class of malignant round cell tumor. Immunohistochemistry plays a pivotal role in differentiating this tumor entity. Chemoradiation was tried, but local and systemic spread occurs early and holds poor prognosis. This case series is an attempt to describe the aggressive behavior of this rare tumor with high mortality.  相似文献   

8.
人工特制假体在下肢骨肿瘤保肢治疗中的应用   总被引:1,自引:2,他引:1  
目的探讨应用人工特制假体在下肢骨肿瘤保肢治疗中的方式和疗效。方法35例下肢骨肿瘤患者,23例行定制带股骨(或胫骨)铰链式膝关节,8例行加长柄人工股骨头置换,4例行加长柄全髋置换。结果治疗后随访9~47个月,平均22个月,生存的30例按Enneking肢体肌肉骨骼肿瘤外科治疗重建术后功能评估标准进行评估。4~5分26例,3分3例,1分1例。结论采用合理的肿瘤边缘完整切除手术,选择特制人工假体并正确安装,辅以综合治疗,能有效提高下肢骨肿瘤保肢率及最大限度保留肢体的功能。  相似文献   

9.
Juvenile granulosa cell tumor (JGCT) in children accounted for 12% of all ovarian tumors treated in the Institut Gustave-Roussy (IGR) Pediatric Department from 1967 to 1985. The median age of the 15 girls was 8 years 7 months (range, 22 months to 15 years 7 months). Precocious pseudopuberty was present in six of the seven girls under 8 years. Of the other seven girls, one developed virilization symptoms. Surgery was the first treatment in each case. According to the Wollner classification, there were six stage I, one stage II, six stage III (including four ruptured tumors), and one stage IV JGCT cases. One patient was not available for staging. An adjuvant treatment (five chemotherapy and one radiotherapy combined with chemotherapy) was administered to six patients. Eleven girls are alive and free of disease, with a median follow-up of 6 years (range, 2 to 18 years). Four girls relapsed 6 to 17 months after surgery and died. Two of these relapses occurred in bone. The prognosis for JGCT in children is favorable for the lower stages when treated with surgery, but the best treatment for extensive and recurrent disease has yet to be determined.  相似文献   

10.
Three cases of primary malignant lymphoma of the maxilla are reported. The primary intraosseous origin of these tumors was demonstrated by x-ray examination and surgical exploration. The initial interpretation as odontogenic infection led to a delay in starting therapy of 9 months in one case. Biopsies of two cases were initially interpreted as sarcoma because of a dense reactive fibrosis between the tumor cells. Subsequently, hemimaxillectomy was performed in one case. Histologically and ultrastructurally the tumor cells showed marked nuclear abnormalities with cleavage, folding, and lobulation. Immunohistochemical studies of two cases showed a monoclonal immunoglobulin expression, IgG-K; T-lymphocyte-associated antigens were not detected on the tumor cells. The findings indicate the existence of a primary B-cell malignant lymphoma of bone with multilobated nuclei. The lymphoid nature may be masked by a dense proliferation of connective tissue. The relation of these tumors to the classifications for malignant lymphoma of lymph node is discussed.  相似文献   

11.
P G Harper  J Pringle  R L Souhami 《Cancer》1981,48(10):2282-2287
Two cases of primitive neuroectodermal primary peripheral nerve tumors (neuroepitheliomas) are presented. They both demonstrated the rosette pattern described by Homer Wright indicating their neuroectodermal origin, and were unlike Schwann cell tumors. In one case, intensive chemotherapy with local radiotherapy had resulted in a disease-free interval of 30 months at the time of this report. These cases support the existence of neuroepithelioma as a distinct tumor type.  相似文献   

12.
Objective To investigate the clinical features of several cases of malignancy with multiple bone lesions as the first manifestation. Methods Forty-nine cases of malignancy with multiple bone lesions as the first manifestation were retrospectively analyzed from May 2018 to July 2019. All patients complained of "pain at the site of bone lesion" upon admission. Baseline patient information, such as age, gender, location of bone lesions, etiology, diagnosis method, time of onset was collected. Results The median age of the patients was 56 years old, of which 83.7% (41/49) were aged ≥50 years. The median time of onset was 2 months. Among the cases, 40 were confirmed as solid tumor bone metastasis, whereas the remaining 9 cases as hematological system tumor. Lung cancer and multiple myeloma were the main tumor types, accounting for 40.8% (20/49) and 16.3% (8/49), respectively. Other common causes were seven cases of cancer affecting the digestive system (three cases of liver cancer, three cases of gastric cancer, and one case of esophageal cancer), as well as seven cases of unknown primary cancer. Half of the solid tumors had only multiple bone metastases but no other distant metastasis. Conclusion Multiple bone lesions accompanied by pain may be the first clinical manifestation in various malignant tumors. The common tumor types were lung cancer, multiple myeloma, and digestive system tumor. It is more common in people aged 50 years and older. Multiple bone lesions might be the only metastasis site of some solid tumors, and its mechanism needs further investigation.  相似文献   

13.
The melanotic neuroectodermal tumor of infancy is an uncommon neoplasm typically of early childhood which has a predilection for the head and neck region, particularly the maxilla. Except for one previous example in the literature, this tumor has consistently behaved in a benign fashion. This study documents the clinical course and pathologic findings of a tumor which began in the maxilla of a 4-month-old boy, followed by a local recurrence, metastasis to a cervical lymph node and finally, widespread dissemination and death at 18 months, 24 months and 38 months, respectively. The tumor was initially composed of nests consisting of melanin-containing cells and small dark cells. An elevated vanillylmandelic acid level was recorded during the course of the disease. At autopsy, the tumor in lymph nodes, liver, bone and soft tissues had a monotonous pattern of small dark cells similar to a conventional neuroblastoma. Previous ultrastructural studies indicate that the melanotic neuroectodermal tumor of infancy is composed of melanocytes and neuroblast-like cells. Our case provided the unique opportunity to examine in sequence the ultrastructural and in vitro characteristics of a recurring and eventually metastasizing melanotic neuroectodermal tumor. Although the neuroblast-like cells were initially difficult to identify by electron microscopy, a melanin-producing cell line and a separate nonpigmented cell line were successfully isolated from various tumor explants. Various stages of melanosome development were identified in the pigmented cells from the local recurrences and in vitro. Dibutyryl cAMP accentuated the formation of pigment and dendritic development in the melanocytes and dendrites only in the small nonpigmented cells. Electron dense granules were observed in the cultured smaller cells and also in the lymph node and soft tissue metastases. Tyrosine hydroxylase activity was demonstrated in the neuroblast-like cells. In the final biopsy and autopsy material, only the neuroblast-like cells remained and the tumor resembled a conventional neuroblastoma.  相似文献   

14.
Extraskeletal osteosarcoma. A clinicopathologic review of 26 cases   总被引:10,自引:0,他引:10  
The clinical records and histopathologic features in 26 cases of extraskeletal osteosarcoma (ESOS) diagnosed at M.D. Anderson Cancer Center (Houston) between 1950 and 1987 were reviewed. Presentation was usually that of an enlarging soft tissue mass. The thigh (11 cases), upper extremity/shoulder girdle (three cases), and retroperitoneum (three cases) were the most common anatomic sites. Tumor size ranged from 2.5 to 30 cm. The predominant histologic pattern was osteoblastic in four cases, chondroblastic in two, fibroblastic or pleomorphic malignant fibrous histiocytoma (MFH)-like in four, giant cell type MFH-like in one, and small cell in one. Various mixtures of these patterns were seen in the remaining 14 tumors. The telangiectatic pattern was not seen as the predominant component in any primary tumor but was observed as a minor component. Thirteen tumors recurred locally and 16 metastasized; five patients had distant metastases at presentation. The lungs, bone, and soft tissue were the most frequent metastatic sites. Sixteen patients died of disease at 2 to 54 months, one patient died of unrelated causes at 61 months, seven patients were alive with no evidence of disease (NED) at 30 to 122 months, and two patients were alive with disease at 28 and 54 months, respectively. Tumor size (less than 5 cm versus greater than or equal to 5 cm) was the main prognostic factor; all patients alive with NED for whom accurate tumor measurements were available (six of seven) had neoplasms measuring less than 5 cm that were amenable to complete surgical excision. Histologic pattern and other clinicopathologic features did not significantly affect outcome.  相似文献   

15.
K H Perzin  N Pushparaj 《Cancer》1984,54(9):1860-1869
Twelve cases of meningiomas involving the nasal cavity and paranasal sinuses are reported. Meningiomas only rarely involve the upper respiratory tract; these 12 cases were found among the 566,000 surgical pathology cases that have been studied in this laboratory. In this series, five meningiomas probably arose in the cranial cavity and secondarily extended into the nasal cavity and paranasal sinuses. Because radiographs demonstrated hyperostosis or bone destruction, three other tumors could have involved the cranial cavity, but this was never proven. In two other cases, the meningiomas appeared to involve only extracranial tissues and thus probably were primary extracranial lesions. (The data available in two other cases were insufficient to assess the possibility of intracranial involvement). These patients clinically had nonspecific signs and symptoms similar to those produced by other types of neoplasms growing in the upper respiratory tract. The tumor involved the sphenoid area in seven cases, the nasal cavity in six, the maxilla in five, the ethmoid sinus in four, and the frontal sinus in one. The diagnosis was first established when biopsy material was examined, although in several cases the presence of meningioma was first suggested on radiographic examination. Treatment depended upon the size and location of the tumor. In some cases, surgical resection appeared to control the lesion. These tumors did not appear to respond well to radiotherapy. The histologic differential diagnosis is discussed.  相似文献   

16.
目的报道临床较为少见的骨血管内皮恶性肿瘤并讨论疾病的性质、诊断标准、治疗与预后。方法回顾我院1996-2001年5年间曾收治的4例骨血管内皮恶性肿瘤,报告其临床,影像学、病理学特点、治疗及随访结果,并作相应讨论。4例骨血管内皮恶性肿瘤患者均为男性,年龄14-59岁,平均38岁,发病部位分别位于股骨上段及股骨颈、颅骨顶部、拇指远节指骨、髂骨翼。其中骨上皮样血管肉瘤、骨血管肉瘤、骨上皮样血管内皮瘤、骨血管内皮瘤各1例。结果4例骨血管内皮恶性肿瘤1例行肿瘤刮除术,3例行肿瘤广泛切除术,4例患者均未行放疗、化疗或放弃放化疗建议。1例术后2年死于肿瘤远处转移;1例术后3年复发,再次手术后已无瘤生存1年8个月;1例术后4年复发,再次手术后已无瘤生存1年4个月;1例已元瘤生存5年3个月。结论骨血管内皮肿瘤的影像学无特异性表现,易与其它骨的良恶性肿瘤相混淆。骨(上皮样)血管肉瘤具有比骨(上皮样)血管内皮瘤更为恶性的生物学行为,预后更差,两者鉴别诊断的关键是病理学依据,骨(上皮样)血管肉瘤必须于早期采取包括肿瘤广泛切除、辅助放疗及化疗在内的综合治疗。  相似文献   

17.
Primitive neuroectodermal tumors/medulloblastoma (PNET/MB) are the most common posterior fossa tumors in childhood. Despite surgery and radiation therapy, 40% to 50% of children with PNET/MB will have recurrent disease. Various chemotherapeutic agents are transiently effective in recurrent PNET/MB, but long-lasting responses are rarely attainable. To increase the rate and duration of response in children with recurrent PNET/MB, the authors treated seven patients (ages 2-18 years; median, 10 years) with lomustine (CCNU) (100 mg/m2), cisplatin (CPDD) (90 mg/m2) and vincristine (VCR) (1.5 mg/m2; maximum, 2 mg) in a 6-week cycle for a maximum of eight cycles. Six of six evaluable patients responded to chemotherapy. Four patients had a complete response; three with complete disappearance of tumor by imaging studies; and one with eradication of extraneural disease for a median of 24 months from relapse (13-29 months). Overall disease-free survival was 18.5 months. All six patients have subsequently died of recurrent tumor. Major toxicities consisted of reversible bone marrow suppression (six of six), high frequency hearing loss (six of six) and decreased renal function (three of six). All patients required dosage modification for toxicity. A regimen of CCNU, VCR, and CPDD is effective therapy in children with relapsed PNET/MB and can produce relatively long-term disease control with good quality of life. Further investigation into the efficacy of this combination as adjuvant chemotherapy in newly diagnosed high-risk PNET/MB is now being performed.  相似文献   

18.
Chordoid meningioma is an uncommon variant of meningioma, which histologically bears a great resemblance to chordoma and often follows an aggressive clinical course. We examine clinicopathologic features of 11 cases of this rare tumor to further elucidate its behavior. Thirteen specimens of chordoid meningioma belonging to 11 patients were obtained at a single institution from 1995 to 2009. Correlations of histologic parameters, immunohistochemical study, and clinical features were assessed. This series included six men and five women with a mean age of 60.8 years at first surgery. Aside from one patient (case 5) who died of disease immediately after the first operation, the mean postoperative follow-up period for the other 10 patients was 41.4 months. Two patients each had a local tumor recurrence. The mean time to recurrence was 10.4 years. No systemic manifestations of Castleman syndrome, such as iron-refractory hypochromic/microcytic anemia and dysgammaglobulinemia, were found. Six tumors (46%) were classified as benign (grade I) and seven tumors (54%) atypical (grade II), if based solely on histologic grading irrespective of chordoid or clear cell components in our cases. Lymphoplasmacytic infiltrate was moderate in one tumor (7%), mild in eight tumors (62%), and absent in four tumors (31%). The inflammatory cells were predominantly T cells (CD3+), with only scarce B cells (CD20+). There was a wide range of MIB-1 labeling indices (0.3–25.8%, mean 7.5%), which increased following tumor recurrence. Our study demonstrates that chordoid meningiomas are not always associated with Castleman’s Syndrome, and that this histologic category can be seen in the elderly as opposed to only in younger age groups.  相似文献   

19.
目的 研究计算机辅助膝关节周围肿瘤的精确切除与重建的新方法,评价计算机辅助技术在膝关节周围骨肿瘤治疗中的价值.方法 2007年1月至2010年7月共收治6例膝关节周围肿瘤患者.其中男4例,女2例.年龄19~40岁.股骨远端骨肉瘤3例,胫骨近端骨肉瘤1例,胫骨近端软骨肉瘤2例.Enneking分期II A期2例,II B期4例.所有患者术前均采用薄层CT和MRI扫描获取骨关节和肿瘤部位的二维图像数据,计算机三维重建建立骨关节和肿瘤浸润区域解剖模型,借助逆向工程和CAD软件三维测量确定肿瘤切除范围,CAD设计个性化辅助手术模板及个性化的骨修复体,计算机辅助模拟膝关节周围肿瘤切除和重建过程.术中按照计算机辅助模拟的手术方案,完整切除肿瘤组织,其中4例患者采用"外形匹配的异体骨+定制个性化膝关节假体"、2例患者采用"外形匹配的异体骨+钢板"重建骨肿瘤切除后遗留骨缺损.随访期间采用骨与软组织肿瘤学会(Musculoskeletal Tumor Society,MSTS)保肢评分系统对随访患者进行功能评价.结果 所有患者随访10-53个月,平均30.5个月.随访期间未发现膝关节假体、钢板的断裂和松动,2例患者出现异体骨吸收,1例患者异体骨感染、行局部清创术治疗,无肿瘤复发和转移.末次随访MSTS评分为18~27分,平均23.4分,其中优4例,良2例.结论 借助于计算机辅助技术,可以实现膝关节周围肿瘤的精确切除和功能重建;异体骨复合金属假体或内固定物重建骨缺损具有良好的临床疗效和应用价值.  相似文献   

20.
OBJECTIVE To discuss the method, safety and effectiveness of the treatment for central-type carcinoma of the lungs by CT guided percutaneous implants of ^125I radioisotopes in tissues.METHODS Twenty-two cases of central-type lung cancer located by plain and/or enhanced CT scans were retrospectively analyzed, among which 18 cases were verified pathologically using a CT guided percutaneous puncture and biopsy before treatment. The CT guided treatment was conducted immediately after the pathological results were obtained. The number of the radioisotopes used was seven to 16. The intensities of radioactivity for ^125I radioisotopes were 22, 26, 30 and 33MBq per particle.The total intensity of radioactivity was 181 to 355 MBq. The puncture point was only one. The frequency for adjustment of needling direction was two to five times. The punctures were conducted through the anterior chest wall in eight cases, through the lateral chest wall in eight cases and thruogh the posterior chest wall in six cases. Six of the 22 cases were given intravenous chemotherapy.RESULTS Of the 22 cases, 20 were followed-up for more than one month, 16 over two months, ten over three months and three cases for six months. In the 20 cases which were followed-up for one month, the diameters of the tumors were reduced by 50.% or more in 18 cases, showed no change in two cases with none showing enlargement. In the 16 cases followed-up for more than two months, the diameters of tumors were reduced by 50% or more in 15 cases, one case showed no change and none enlarged. In the ten cases with three months of follow-up, the tumor diameters were diminished by 50% or more in eight cases, showed no change in one case and enlarged in one case. In the three cases followed-up for six months, the diameters of tumors were reduced by 50% or more in all the cases. Of the 22 cases, atelectasis occurred in 12 before therapy. The lungs expanded again in nine cases in the follow-up after treatment. Symptoms after therapy included metastases of the mediastinal lymph nodes in four cases, newly-occurring thoracic osseous metastases in one case and hepatic metastases in one case.Complications included pneumothorax in 11 cases and exfoliation of particles in one case.CONCLUSION The size of cancer can be markedly reduced after therapy by CT guided percutaneous implant of 1251 radioisotopes in tissues. It is an accurate and safe procedure and is effective for local metastasis.  相似文献   

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