Epstein-Barr virus associated central nervous system leiomyosarcoma occurring after renal transplantation: case report and review of the literature]

Central nervous system leiomyosarcomas are extremely rare, however, they became more frequent among immunodeficient patients, either in a patients infected with human immunodeficiency virus (HIV), or after organ transplantation. The data of the literature indicate that the infection by Epstein-Barr virus (EBV) plays a causal role in the development of these tumours but its precise role in the oncogenesis remains unresolved. We report a new case of EBV associated leiomyosarcoma of the left cavernous sinus occurring after renal transplantation. The epidemiological, clinical, pathological and therapeutic characteristics of these tumours are discussed.     

Primary melanoma of the central nervous system: report of a case and review of the literature

Primary melanoma of the meninges, a rare CNS tumor, is presented. Criteria for diagnosing a primary CNS melanoma are elucidated. Literature is reviewed in this context. The histogenesis of tumor, problem of occult primary melanoma and the role of CT scan and CSF cytology in early diagnosis have also been highlighted.     

Primary central nervous system T-cell lymphoma in aids patients: case report and literature review

According to the published data, most primary central nervous system lymphomas (PCNSLs) are B-cell lymphomas; primary T-cell lymphomas are rare. In a search of the MEDLINE database, we found only 6 cases of primary T-cell PCNSL. Here, we present the case of a 43-year-old man with AIDS, not on highly active antiretroviral therapy, who presented with focal neurologic symptoms and was found on magnetic resonance imaging to have multiple brain lesions. A biopsy showed T-cell lymphoma, and the patient was subsequently treated with whole-brain radiation, to marked clinical response. Reported cases from the literature of primary T-cell PCNSL in AIDS patients are summarized in this review.     

Ionizing radiation and the risk of brain and central nervous system tumors: a systematic review

Although exposure to moderate-to-high doses of ionizing radiation is the only established environmental risk factor for brain and CNS tumors, it is not clear whether this relationship differs across tumor subtypes, by sex or age at exposure, or at the low-to-moderate range of exposure. This systematic review summarizes the epidemiologic evidence on the association between ionizing radiation exposure and risk of brain/CNS tumors. Articles included in this review estimated radiation exposure doses to the brain and reported excess relative risk (ERR) estimates for brain/CNS tumors. Eight cohorts were eligible for inclusion in the analysis. Average age at exposure ranged from 8 months to 26 years. Mean dose to the brain ranged from 0.07 to 10 Gy. Elevated risks for brain/CNS tumors were consistently observed in relation to ionizing radiation exposure, but the strength of this association varied across cohorts. Generally, ionizing radiation was more strongly associated with risk for meningioma compared with glioma. The positive association between ionizing radiation exposure and risk for glioma was stronger for younger vs older ages at exposure. We did not observe an effect modification on the risk for meningioma by sex, age at exposure, time since exposure, or attained age. The etiologic role of ionizing radiation in the development of brain/CNS tumors needs to be clarified further through additional studies that quantify the association between ionizing radiation and risk for brain/CNS tumors at low-to-moderate doses, examine risks across tumor subtypes, and account for potential effect modifiers.     

原发中枢神经系统霍奇金淋巴瘤一例并文献复习

目的 探讨原发中枢神经系统霍奇金淋巴瘤(CNS-HL)的临床特点、诊断、治疗和预后.方法 回顾性分析1例原发CNS-HL患者的临床病理资料,并复习相关文献.结果 该例原发CNS-HL患者以头晕、头痛、恶心、呕吐、视物模糊、肢体无力等起病,影像学提示颅内多发占位,颅外无明确病灶.诊断依据中枢神经系统症状、影像学表现、明确的形态学及免疫组织化学结果.结论 原发CNS-HL罕见,发病率低,病灶局限在颅内,全脑放疗及放化疗联合治疗效果较好.     

中枢神经系统浸润多发性骨髓瘤一例并文献复习

目的 探讨中枢神经系统(CNS)浸润的多发性骨髓瘤(MM)的临床特征、诊断及效果.方法 报道1例CNS浸润的MM,并对其发病机制、临床特点、诊断、治疗等进行文献复习.结果 CNS浸润的MM发病率低,MM一旦出现CNS浸润其临床特点无明显特异性,诊断有赖于病理活组织检查,无标准治疗方案.结论 发生CNS浸润的MM发病率低,预后不良.     

Intravascular lymphomatosis (neoplastic angioendotheliosis) of the central nervous system: case report and literature review

Summary A 63-year-old woman developed gradual slowness, recurrent multifocal deficits, severe constitutional symptoms and hypopituitarism which progressed to death over 2.5 months. Elevation in lactico dehydrogenases was the main biological abnormality. Necropsy showed an intravascular malignant proliferation which proved to be a lymphoma of B-lineage. In order to better define diagnostic criteria, we reviewed previously reported cases of intravascular lymphomatosis of the central nervous system. A strategy for establishing the diagnosis is proposed.     

Smooth muscle tumors of the small bowel: a case report and review of the literature

We report a rare case of peritonitis caused by perforation of smooth muscle tumor of the jejunum. In spite of having all the reported presenting symptoms of these tumors, the diagnosis was not made preoperatively. Although histopathologically diagnosis was that of a benign tumor, the subsequent course was rapid and malignant. A complete review of the literature is presented.     

Central nervous system Hodgkin lymphoma: case report and review of the literature

Central nervous system (CNS) involvement by Hodgkin lymphoma (HL) is extremely rare, accounting for 0.5% or less of HL cases. In contrast, CNS involvement can occur in 5–30% of patients with non-Hodgkin lymphoma. CNS HL can present at any point in the course of HL, most commonly during relapsing disease, and has been described in both immunocompromised and immunocompetent patients. We describe a case of HL affecting the CNS and bone marrow on initial presentation in a 79-year-old immunocompetent female with a prior history of squamous cell carcinoma of the larynx and adenocarcinoma of the lung. Following the case report, a review of the literature on CNS HL is presented.     

侵犯中枢神经系统及眼球的套细胞淋巴瘤一例并文献复习

目的:分析侵犯中枢神经系统（CNS）及眼球的套细胞淋巴瘤患者的临床特点和治疗经验。方法:回顾性分析2018年4月潍坊医学院附属医院收治的1例侵犯CNS及眼球的套细胞淋巴瘤患者的临床资料，并复习相关文献。结果:患者第1个疗程化疗后出现CNS侵犯，腰椎穿刺治疗后病情好转。改为R2方案化疗，化疗过程中出现眼球侵犯，改为R2-HyperCVAD/A方案化疗，但病情出现进展，直至完全失明。给予伊布替尼治疗，CNS及眼球侵犯症状均控制好转。结论:套细胞淋巴瘤侵犯CNS及眼球的病例少见，治疗手段有限，伊布替尼治疗效果良好，可进一步研究。     

Pediatric central nervous system germ cell tumors: a review

Central nervous system (CNS) germ cell tumors (GCTs) represent approximately 3% of primary pediatric brain tumors and encompass a wide pathologic spectrum. CNS GCTs are most commonly located in the pineal and suprasellar regions of the brain and can be divided into major groups including germinomas and nongerminomatous GCTs (NGGCTs), with teratomas often considered a separate category. The clinical presentation varies by location and size, and it frequently includes endocrine abnormalities, visual changes, and signs of increased intracranial pressure. Neuroimaging studies cannot differentiate GCTs from other tumors, and therefore, the diagnosis usually requires histologic confirmation. The rare exceptions are the cases where characteristic elevations of tumor markers, including alpha-fetoprotein and/or beta-human chorionic gonadotropin are documented in the serum and/or cerebrospinal fluid. In these cases, the imaging findings along with the tumor marker elevation may be diagnostic in themselves without the need for tissue confirmation. Treatment and prognosis differ greatly between groups. Germinomas have a superior prognosis than NGGCTs. Five-year overall survival rates >90% were reported initially with the use of craniospinal irradiation. More recently, the use of chemotherapy in addition to radiation therapy has afforded the ability to decrease the dose and volume of radiation therapy without affecting survival rates. NGGCTs are less radiosensitive than germinomas, but the use of adjuvant chemotherapy has improved survival rates in this group as well. The standard management for CNS GCTs remains controversial. Treatment regimens aimed to improve progression-free and overall survival times are ongoing.     

Primary malignant lymphoma of the central nervous system--a case report and review of literature

One case of primary malignant lymphoma of the central nervous system is reported. The patient, a nine year old boy having headache, vomiting, seizures in the right limbs and unconsciousness, was admitted into our hospital and died the next day. A tumor at the base of the left frontal lobe was found on autopsy. There was no evidence of tumor elsewhere. A pathological diagnosis of T-cell malignant lymphoma was established. In this paper, the view that the T-cell lymphoma can arise from the central nervous system is proposed for the first time. Its clinical features, pathomorphology and histogenesis are discussed. Yet, how follicles are formed in the tumor tissue and its significance await further study.     

Mesenchymal tumors associated with hypoglycemia: case report and review of the literature.

Hypoglycemia secondary to malignant tumors is rare. Mesenchymal tumors of nonpancreatic origin are the most common tumors associated with the hypoglycemia syndrome, and the clinical features of 115 reported cases are reviewed. The major anatomic distributions of the tumors are thoracic (30%) abdominal (65%), and uncommon locations (less than 5%). Approximately 50% of the tumors were resectable (59 patients), and in 60% the surgical procedure was curative. In the remaining 40% local recurrence predominated related to site of tumor and presence of contiguous organ invasion. The application of multimodality adjuvant therapy for hypoglycemia associated mesenchymal tumors should be based on an understanding of the natural history of the tumor.     

Obesity as the initial manifestation of central nervous system relapse of acute lymphoblastic leukemia: case report and literature review

A 6-year-old boy with acute lymphoblastic leukemia in remission experienced hyperphagia, obesity, and emotional disorders. Cytomorphologic examination of cerebral spinal fluid (CSF) and cranial MRI did not help in differentiating between central nervous system leukemia (CNSL) and other CNS diseases including tuberculosis in this boy. Flow cytometric CSF analysis on repeated lumber puncture detected lymphoblasts, while microscopic CSF examination did not definitively show relapse disease. The diagnosis of CNSL was thus made and confirmed by the response to leukemia treatment. Obesity can be the first manifestation of CNSL and the diagnosis can be challenging. A combination of CSF cytomorphology, CSF flow cytometry, and cranial MRI can be useful in the diagnosis of the disease. Two mechanisms of CNSL-related obesity are discussed based on the literature review.     

32例原发性中枢神经系统恶性淋巴瘤临床分析及文献复习

背景与目的:原发性中枢神经系统淋巴瘤(primarycentralnervoussystemlymphoma,PCNSL)发病率上升且预后很差。本研究目的是探讨免疫正常的中国人PCNSL的临床特征,评价大剂量甲氨蝶呤(HD-MTX)治疗PCNSL的疗效。方法:回顾性分析经病理证实的32例(中位年龄50岁)PCNSL患者的临床资料和治疗效果。2001年11月以前采用以CHOP方案为主、单用或联合全脑放疗的治疗方法,2001年12月以后采用以HD-MTX为主、单用或联合全脑放疗的治疗方法。结果:32例PCNSL患者中25例(78.1%)45岁以上;24例(75%)主要表现为颅内高压;25例(78.1%)单发病灶;32例患者均未见脑脊液细胞学阳性表现;28例(87.5%)为B细胞淋巴瘤,其中19例为弥漫性大B细胞淋巴瘤。32例中位随访期13.5个月(1～84个月),Kaplan-Meier分析总中位生存期26个月,2年生存率45.7%;HD-MTX联合放疗组患者完全缓解率61.1%,中位生存期在26个月以上,2年生存率65.1%,疗效明显优于非HD-MTX联合放疗组;log-rank检验显示乳酸脱氢酶正常、状态评分在0～1的患者生存期较长。结论:PCNSL多发于中老年人,颅内高压为主要表现,B细胞亚型占绝对优势。HD-MTX联合全脑放射治疗PCNSL有效和可行。     

Primary central nervous system extranodal NK/T-cell lymphoma,nasal type: case report and review of the literature

Primary central nervous system (CNS) extranodal NK/T-cell lymphoma, nasal type (NKTCL), is an extremely rare tumor. To the best of our knowledge, only four cases have been described previously. Here, we report a case of primary CNS NKTCL in a 25-year-old immunocompetent Chinese male. The patient presented with worsening dizziness, headaches, and vomiting for approximately 2 weeks. Magnetic resonance imaging demonstrated three masses with solid components entirely in the parenchyma of the right hemisphere, and no sinonasal/nasopharyngeal lesions were found. The patient underwent a partial resection of the right temporal mass. Histological examination revealed that intermediate-sized, pleomorphic lymphocytes were arranged in an angiocentric distribution with large geographic necroses. The tumor cells expressed CD3ε, CD56, TIA-1, granzyme B, and Epstein−Barr virus-encoded RNAs. A rearrangement study showed T-cell receptor γ-chain gene rearrangement with monoclonal appearance. Postoperative chemotherapy and radiotherapy were also given, but the lymphoma failed to respond to therapy and the patient died 18 months later. Our observation and the four others found in the literature indicate that primary CNS NKTCL occurs predominantly in adult males. This is the youngest patient with primary CNS NKTCL reported.     

Successful multimodal therapy of invasive pulmonary and central nervous system aspergillosis in a neutropenic surgical patient: case report and review of the literature

Invasive aspergillosis predominantly occurs in patients with impaired host defence and is often resistant to different therapeutically strategies. However, mortality significantly increases if the central nervous system is affected. In this report, we describe a case of successful treatment of invasive aspergillosis with cerebral involvement. The treatment consists of a medication of voriconazole and lipid-associated amphotericin B as well as a stereotactic neurosurgical procedure to drain an intracranial abscess.     

Primary central nervous system marginal zone B-cell lymphoma of the Basal Ganglia mimicking low-grade glioma: a case report and review of the literature

Primary central nervous system (CNS) marginal zone B-cell lymphoma (MZBL) is very rare and shows an indolent disease course with potential of being cured. It seems to originate from meningothelial cells, and the most common site of occurrence is the dura of the cerebral convexity. Primary CNS MZBL is often misdiagnosed as meningioma because of its similar tumor locations and appearances on magnetic resonance imaging (MRI). Surgery, radiation therapy, chemotherapy, and combinations of these are considered treatment modalities depending on the case. Herein, we describe an 18-year-old man who presented with acute onset of right-sided central facial nerve palsy, right-sided hemiparesis with motor power grade 4+, dizziness, and dysarthria. After an MRI scan of the brain, wherein he was first diagnosed with high-grade glioma, a biopsy sample showed that he had primary CNS MZBL arising in the left basal ganglia. He was treated with radiation therapy, which resulted in complete remission for 1 year and 10 months up to the date of this case report. It is important to diagnose primary CNS MZBL correctly because it is curable without unnecessary invasive treatment in cases of localized disease.