原发性脑膜Rosai-Dorfman 病的临床诊断

目的 探讨颅内Rosai-Dorfman disease(RDD)的临床特点、病理诊断和鉴别诊断.方法 对1 例颅内RDD进行组织形态学和免疫组织化学分析,并复习相关文献.结果 病变以硬膜为基础,与脑实质分界清楚,可见较多组织细胞,淋巴细胞和浆细胞浸润伴纤维化,其中巨大组织细胞胞浆内可见多少不等的淋巴细胞,免疫组化显示,这些组织细胞S-100 蛋白和CD68 标记阳性,散在分布.结论 颅内RDD非常罕见,病理改变为在炎性细胞背景中散在胞浆含多个淋巴细胞的巨大组织细胞,以S-100 阳性,CD68 阳性,CD1a 阴性为特征.     

儿童脑内多灶性朗格汉斯组织细胞增生症一例并文献复习

目的 提高对儿童脑内朗格汉斯组织细胞增生症的临床特征、诊断及治疗的认识.方法 报告1例4岁患儿脑内多灶性朗格汉斯组织细胞增生症的诊断及治疗过程,并复习相关文献.结果 患儿以颅内压增高为首发症状,影像学检查示颅内多灶性病变,合并蛛网膜囊肿1处.经过一系列实验室检查以进行鉴别诊断后,行开颅手术切除右额叶责任病灶,术后病理证实为颅内朗格汉斯组织细胞增生症.术后患儿颅高压症状缓解,未切除病灶行化疗.6个月随访证实切除病灶部位无复发,未手术部位病灶无进展.结论 有占位效应的幕上脑内多灶性朗格汉斯组织细胞增生症报道较少.对于占位效应明显的病灶,应首选切除,并辅以术后化疗,可望控制病变进展.     

颅内浆细胞肉芽肿(附五例报告及文献复习)

目的 探讨中枢神经系统浆细胞肉芽肿的诊断及治疗。方法 对我院1997年1月至2002年3月收治的5例颅内浆细胞肉芽肿的临床资料进行回顾性分析。结果 5例病人，2例位于左顶叶，余3例分别位于右颞叶、右额叶和枕骨大孔区，头CT显示为高密度，MRI为长或等T1、长T2信号，注射造影剂后明显增强。术前诊断为脑转移瘤、脑囊虫病、脑脓肿和脑膜瘤，术后经病理和免疫组化染色发现肿物主要由3种成熟的细胞组成，即浆细胞、淋巴细胞和组织细胞，以浆细胞为主，浆细胞胞浆内κ、γ轻链均呈阳性表达。手术切除后给予激素治疗，预后良好。结论 颅内浆细胞肉芽肿少见，易引起误诊，免疫组化检查有助于鉴别诊断，手术切除和激素治疗效果较好。     

颅内静脉窦血栓形成的临床与CT DSA

目的 :探讨颅内静脉窦血栓形成的临床特点与影像学改变。方法 :分析 18例颅内静脉窦血栓形成患者的临床资料 ,CT及 DSA改变。结果 :患者男 3例 ,女 15例 ,其中产后发病 11例 ,贫血 2例 ,化脓性中耳炎 1例 ,口服避孕药 1例。上矢状窦血栓形成 13例 ,横窦血栓形成 2例 ,海绵窦血栓形成 2例 ,上矢状窦合并其它静脉窦血栓形成 1例。 18例中痊愈 10例 ,好转 5例 ,死亡 3例。结论 :颅内静脉窦血栓形成早期诊断困难 ,应借助于脑 CT特别是 DSA,及早诊断及恰当治疗可降低病死率     

一例误诊为脑膜瘤的颅内原发罗道病报告并文献复习

正罗道病(Rosai-Dorfman disease,RDD)是一种病因不明、少见的、非肿瘤性的良性组织细胞增生性疾病,又称为窦组织细胞增生伴巨淋巴结病。最常见受累部位为淋巴结,颅内原发性RDD较为罕见。本文复习一例术前诊断为脑膜瘤的颅内原发RDD,对其临床表现、影像学资料、组织病理形态及治疗等资料进行分析,并复习相关文献,报道如下。患者男,44岁,主因查体发现颅内病变1 w入院。既往     

磁共振静脉血管造影对颅内静脉窦血栓形成的诊断价值及正常人静脉窦变异情况研究

目的评价MRV与MRI诊断颅内静脉窦血栓形成的一致性,观察正常人MRV表现及其静脉窦变异情况。方法共29例颅内静脉窦血栓形成患者行MRI和MRV检查,计算二者诊断一致性;同时观察11例正常对照者颅内静脉窦在血流和连接方式等方面的变异情况。结果 MRV与MRI诊断颅内静脉窦血栓形成的一致率：3.0T为92.59%（25/27）、1.5T为88.24%（15/17）。正常对照者静脉窦变异主要集中在横窦,11例中双侧横窦显影粗细大致相同5例、右侧横窦较粗5例、左侧横窦较粗1例。结论MRV与MRI诊断颅内静脉窦血栓形成具有较高的一致性,但对于先天性变异无法区分,需结合MRI综合分析。正常人静脉窦变异主要集中在横窦,传统认为右侧横窦可较左侧横窦粗,但根据目前数据也可出现右侧横窦比左侧横窦细的情况。     

颅内血管外皮细胞瘤的临床特点

目的探讨颅内血管外皮细胞瘤的临床特点。方法回顾性分析26例颅内血管外皮细胞瘤患者的临床资料。结果患者肿瘤位于上矢状窦、枕窦旁10例,大脑半球凸面9例,小脑半球(小脑幕)3例,镰幕交界区2例,鞍区1例,左侧脑室1例。临床表现为头痛、头晕、视力受损、肢体乏力、癫痫发作、听力下降。26例患者行头颅CT,25例表现为颅内占位性病变,1例表现为左侧额顶血肿;23例行头颅MRI检查,其中20例表现为均匀强化;10例病灶位于横窦或矢状窦的患者行头颅MRV检查,上矢状窦及横窦均有不同程度的狭窄。术后病理均为血管外皮细胞瘤:免疫组化显示Vim均为阳性,EMA均为阴性。26例均行手术治疗,其中全切者20例,次全切除5例,大部切除1例;术后常规50Gy剂量的放射治疗;随访2~4年,平均33.2个月,无肿瘤复发。结论颅内血管外皮细胞瘤的临床表现与肿瘤位置相关,肿瘤多起源于硬膜。病灶在CT表现为等或高密度灶,强化MRI显示均匀强化,可见血管流空信号。手术治疗为首选治疗方法,术后辅助放疗有助于延缓肿瘤复发。     

筛窦颅内沟通型巨大皮样囊肿1例并文献复习

目的 探讨筛窦颅内沟通型巨大皮样囊肿的临床特点与外科治疗。方法 回顾分析1例筛窦颅内巨大皮样囊肿患者的临床资料,并结合相关文献进行复习。结果 患者男,58岁,因“左侧肢体乏力4年,进行性加重2年”入院。入院诊断为筛窦颅内沟通巨大肿瘤,考虑畸胎瘤可能,全麻下行右侧扩大翼点入路肿瘤切除术,病理示皮样囊肿,术后无并发症,随访1年,病变无复发。结论 筛窦颅内巨大皮样囊肿比较少见,临床上注意与畸胎瘤及表皮样囊肿的鉴别,治疗首选外科手术,术中、术后采取措施预防无菌性脑膜炎,因病变侵犯颅底,要多学科协作,注意颅底修复重建预防脑脊液漏。     

一期单侧开颅夹闭颅内双侧5枚动脉瘤1例报告及文献复习

目的探讨颅内多发动脉瘤的治疗方法和原则;提高对颅内多发动脉瘤的治疗水平。方法回顾性分析1例颅内双侧5枚动脉瘤患者一期单侧开颅夹闭手术治疗经过及术后恢复情况;并结合相关文献分析颅内多发动脉瘤的诊断与治疗。结果行经翼点入路一期单侧开颅夹闭了5枚颅内动脉瘤(右侧大脑中动脉2枚,前交通动脉1枚,左侧大脑中动脉2枚),手术顺利。术后患者无神经功能缺损。结论颅内多发动脉瘤占动脉瘤总发病率约10%~15%,一期开颅手术治疗可以一次解除动脉瘤破裂的风险,且可以避免多次手术对患者生理及心理造成的创伤。     

Rosai-Dorfman病

<正>颅内Rosai-Dorfman病常见于成人,呈孤立性或多灶性包块生长于硬脑膜,眶内或鼻、副鼻窦包块可扩展至鞍内和颅内。其影像学表现类似脑膜瘤,手术全切除或糖皮质激素治疗预后良好。组织形态学观察,低倍镜下可见病变呈结节状、淡染(图1a);高倍镜下可见浆细胞,胞质淡染、核仁明显的组织细胞,以及淋巴细胞相互混杂浸润(图1b);伸入运动,以及组织细胞胞质内可见完好的淋巴细胞和浆细胞,是其典型组织学特征(图1c),约70%的病例可见,有时不易见。组织细胞CD68和S-100     

Primary intracerebral Rosai-Dorfman disease

Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease (RDD), is an idiopathic histiocytic disorder of lymph nodes and extranodal sites. Central nervous system (CNS) manifestations, particularly in the absence of nodal disease, are rare. Intracranial RDD clinically and radiologically resembles meningioma, and histologic examination is essential for a definitive diagnosis. We report four patients with RDD primary to the CNS without evidence of other sites of involvement, review the literature, and discuss the clinical manifestations, pathology, treatment and outcome.     

Central nervous system involvement in Rosai-Dorfman disease: Report of a case with a review of the literature

A 55-year-old woman had Rosai-Dorfman disease (RDD) forming multiple masses of abnormal histiocytes, three in the cranial cavity and one in the left orbit that was proptotic. The masses were removed and found to consist of abnormal histiocytes that were immunoreactive to cluster designation 68 (CD68) (KP-1), alpha-1-antitrypsin and S-100 protein and showed emperipolesis. A review of 28 cases of RDD, including this instance, revealed a ratio of 5:2 between males and females and a mean age of 32 years. Intracranial involvement was much more frequent than that of the spinal cord. Histologically, central nervous system (CNS) RDD must be distinguished from meningioma, Langerhans cell histiocytosis, and plasma cell granuloma. Surgical extirpation appears to be the treatment of choice for this idiopathic histiocytic proliferative disorder of the CNS in comparison with radiotherapy and steroid treatment, which have also been tried.     

A case of rapidly progressive Rosai-Dorfman disease restricted to the central nervous system

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is an idiopathic condition typically associated with cervical lymphadenopathy, fever and hypergammaglobulinaemia. Extranodal involvement has been reported in diverse sites such as the skin, upper respiratory tract, orbit and the central nervous system. We document a case of intracranial RDD in a 40-year-old woman with rapid evolution over a period of three months. Clinically, the patient suffered from headache. The MRT showed a left parietal tumour with dural attachment. Histologically, the lesion consisted of pale-staining histiocytes with emperipolesis, neutrophilic granulocytes and scattered lymphocytes. Focally, the granulocytes dominated the histological picture. By immunohistochemical analysis, the characteristic histiocytes were positive for S100 protein, CD68 and FXIIIa, but negative for CD1a. No Birbeck-granula were detectable in electron microscopic analysis. Granulocytes showed a positive Anti-HHV6b immunoreaction. The tumour was diagnosed asA'an intracranial manifestation of RDD primary to the CNS with an unusual preponderance of neutrophilic granulocytes and with only scattered lymphocytes. The postoperative clinical staging showed no other manifestations of the disease. On postoperative MRI the lesion had been completely resected. No further therapy has been applied and the patient has had an unremarkable clinical course for the last ten months.     

Isolated intracranial Rosai-Dorfman disease mimicking meningioma

Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease (RDD), is an idiopathic histiocytic proliferation affecting lymph nodes. It is typically characterized by painless cervical lymphadenopathy, fever and weight loss. Although extranodal involvement has been reported in diverse sites, intracranial presentation, particularly in the absence of nodal disease is uncommon. To the best of our knowledge, 48 patients with intracranial masses have been reported previously. A 31-year-old man was admitted to our clinic with a 4-month history of progressive headache. His medical history was unremarkable except for occasional fever. There were not any neurological deficit and weight loss. No lymphadenopathy (particularly bilateral cervical) and extranodal involvement in diverse sites were revealed by physical and radiological examinations. Routine hematological and biochemical studies were normal except for mild leukocytosis and elevated erythrocyte sedimentation rate. The patient underwent magnetic resonance imaging (MRI) testing that revealed an enhancing mass in the left temporal lobe. Preoperative diagnosis was meningioma. The patient underwent a left frontotemporal craniotomy with complete resection of the mass. Histopathology was compatible with RDD. Extranodal RDD is rarely found intracranially. Prognosis is benign especially in the absence of nodal disease. It is clinically and radiologically difficult to distinguish from meningioma, and histological examination is essential for a definitive diagnosis.     

Rosai-Dorfman disease mimicking chronic subdural hematoma

Rosai-Dorfman disease (RDD), otherwise known as sinus histiocytosis with massive lymphadenopathy, is an uncommon, benign systemic histio-proliferative disease characterised by massive lymphadenopathy particularly in the head and neck region, and is often associated with extra-nodal involvement. We report a case in which RDD was mistaken for a chronic subdural hematoma and surgery was attempted.     

A unique case of isolated thoracic spinal Rosai-Dorfman disease related to IgG4

Rosai-Dorfman disease (RDD) is a rare benign histiocytosis usually characterized by massive cervical lymphadenopathy and systemic manifestations. Extranodal, especially spinal involvement, is extremely rare. Our case was deemed worthy of presentation because it was the first reported isolated case of spinal RDD related to IgG4 and mimicked meningioma clinically and radiologically. A case with an intradural extramedullary mass causing neurological compression findings in the thoracic spinal region and radiologically mimicking meningioma is presented. In the histomorphological examination of the resection material, polymorphonuclear leukocytes in the dura, histiocytes showing emperipolesis, an increase in collagenized fibrous connective tissue, and intense lymphoplasmacytic cell infiltration accompanied by obliterative phlebitis were observed. Immunohistochemically, the histiocytic cells were found to be S-100 protein, CD68, and CD163 positive and CD1a and langerin negative, and more than half of the plasma cells were immunoglobulin-G4 (IgG4) positive. Although rare, RDD or IgG4-related meningeal disease should be considered in the differential diagnosis of dural-based spinal masses that radiologically suggest meningioma. The pathologist should be aware that these two histopathological entities may coexist. To our knowledge, this is the first case of “isolated spinal RDD related to IgG4” reported in the literature.     

Rosai-Dorfman disease of the central nervous system.

Rosai-Dorfman disease (RDD) is an idiopathic, non-neoplastic, lymphoproliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. When RDD involves the central nervous system the lesion simulates a meningioma. Histological and immunohistochemical confirmation is essential for a definitive diagnosis. In this paper, ten cases of RDD confined to the central nervous system are reported. Another case with orbital RDD was excluded. Nine cases involved the cranial cavity alone; in one, the cervical extradural region was also involved. Treatment consisted of surgical excision or biopsy. Histology and immunohistochemistry revealed a mixed cell population of predominantly mature histiocytes with evidence of emperipolesis and strong positivity for S100 protein in all cases. No recurrence was observed during follow up ranging from three months to eight years.     

Multiple involvement of the central nervous system in Rosai-Dorfman disease

Rosai-Dorfman disease is a rare, benign, idiopathic histio-proliferative disorder. Only 5% of cases involve the central nervous system. We describe a 10-year-old girl with pain in her lower limbs and back. Spinal magnetic resonance imaging revealed an intradural extramedullary lesion at T₉-T₁₀. We decided on surgical treatment. An anatomic/pathologic examination revealed histiocytic-like cells and extensive fibrosis. Immunohistochemistry revealed positivity for CD68 protein and negativity for CD1a protein. Craniospinal magnetic resonance imaging demonstrated an extra-axial lesion in the right frontal region, a small nodule in the left middle cerebellar peduncle, and another small lesion in the right ventral pons. We performed a complete removal of the frontal lesion. The histologic examination produced results compatible with Rosai-Dorfman disease. Most lesions in intracranial Rosai-Dorfman disease mimic meningioma. The definitive diagnosis relies on pathologic and immunohistochemical characteristics. Surgical removal is generally regarded as the treatment of choice. Disease progression after surgical resection is uncommon. Surgical treatment is not recommended until clear disease progression is detected, or focal disease causes neurologic compression. This disease must be included in the differential diagnosis of lesions that mimic meningioma.     

Primary isolated intracranial Rosai–Dorfman disease: Report of a rare case and review of the literature

Background

Intracranial involvement is an uncommon manifestation of Rosai–Dorfman disease (RDD) and had been rarely reported. In this study, we explore clinical characteristics, imageology manifestations and pathological features of primary intracranial RDD so as to improve the understanding for this disease.