伴IgG4阳性浆细胞数量增多的原发性颅内Rosai-Dorfman病1例报道并文献复习

Rosai-Dorfman病(RDD)为一种罕见的组织细胞增生性疾病, 又被称为窦组织细胞增生伴巨大淋巴细胞病, 由Azoury和Reed于1966年首先报道[1], 1969年病理学家Foucar和Rosai对其做了详细研究并正式命名为RDD[2]。RDD是一种良性淋巴组织增生性疾病, 可发生在各年龄段人群中, 但多见于青少年和中年人, 最常见受累部位是颈部淋巴结。原发于中枢神经系统且不累及身体其他部位的病例较罕见, 且大多为颅内孤立性病灶, 多数为脑实质外肿块, 基底位于硬脑膜, 少有位于脑实质内和脑室内者[3]。因其临床表现无特异性, 影像学表现与脑膜瘤相似, 故多被误诊为脑膜瘤后手术切除, 最终经病理学检查确诊。该病变组织细胞S-100蛋白和CD68表达阳性为可靠诊断依据, 部分病例伴有IgG4阳性浆细胞增多。笔者现对中日友好医院神经外科收治的1例术前诊断为脑膜瘤、术后病理证实为伴IgG4阳性浆细胞数量增多的颅内RDD患者的诊治经过进行报道, 以IgG4阳性浆细胞提高临床工作者对该病的认识。     

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     

中枢神经系统Rosai-Dorfman病的诊断

目的:探讨中枢神经系统窦组织细胞增生症的诊断及治疗。方法:对我院收治的2例颅内窦组织细胞增生症病例的临床资料进行回顾性分析。结果:2例患者病灶分别位于左侧大脑镰旁和左侧中颅窝。术前CT,MRI诊断为脑膜瘤。手术全切肿块。术后病理和免疫组化发现病变组织由大量淋巴细胞、浆细胞及S100和CD68标记阳性的组织细胞构成,并呈“明暗”相间的组织学特征,部分组织细胞内有吞噬淋巴细胞现象。结论:原发颅内窦组织细胞增生症少见,易被误诊,免疫组化检查有助于鉴别诊断。手术是治疗本病的主要手段。     

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     

原发脑功能区罗道病1例报道并文献复习

罗道病(Rosai Dorfman disease, RDD)又称窦组织细胞增生伴巨淋巴结病(sinus histiocytosis with massive lymphadenopathy, SHML), 它是一种罕见的良性组织细胞增生性疾病, 颅内原发RDD更加罕见。笔者现报道1例术前初步诊断为脑膜瘤的原发脑功能区RDD患者的临床资料和诊治过程, 以期为临床工作者提供参考。     

颅内外沟通性Rosai-Dorfman病一例并文献复习

Rosai-Dorfman病(Rosai-Dorfman disease,RDD)是一种良性自限性罕见病,以窦组织细胞增生伴淋巴结肿大为特征,病因不明,累及中枢神经系统的RDD十分罕见[1-2].本文报告1例颅内外沟通性RDD,并进行文献复习.     

颅内血吸虫病肉芽肿一例

患者 男,33岁.因"右颞部胀痛2周,加重3 d"急诊人院.查体:嗜睡,双侧瞳孔直径2 mm,对光灵敏,肢体疼痛刺激定位,刺痛后可对答,病理征(-).头颅CT平扫(图1):右侧颞顶叶大片指状低密度水肿区,右侧脑室明显受压移位,中线结构左移.拟"右颞叶占位性病变",予病变切除及去骨瓣减压术.术中见病变组织色黄,质硬,与周围组织分解不清,无明显血供.病理诊断:右颢寄生虫性肉芽肿性炎症.免疫组化:胶质细胞S-100(+),GFAP(+);淋巴细胞LCA(+),Ki67(+);组织细胞CD68(+),CD1α(-).     

鞍区淋巴瘤误诊为脑膜瘤1例及文献复习

目的 总结鞍区淋巴瘤的临床诊断与治疗原则.方法 回顾性分析1例鞍区淋巴瘤病人的临床资料.术前诊断为鞍区和顶部脑膜瘤,择期行右侧眉弓锁孔入路肿瘤切除术.结果 肿瘤全切除,病理检查提示:淋巴细胞共同抗原阳性,CD79-αCD20 、S-100蛋白、泛素羟基端水解酶-1、非特异性酯酶及胶质纤维酸性蛋白均为阴性.病理诊断为非霍奇金淋巴瘤.病人放弃放、化疗,术后6个月死亡.结论 鞍区淋巴瘤极其少见,原发性脑内恶性淋巴瘤常发生于幕上大脑半球,术前误诊率高、预后差,多种方法综合治疗可提高病人生存期     

颅内外非沟通性Rosai-Dorfman病1例

Rosai-Dorfman病(RDD)是一种良性自限性疾病, 其主要特征是淋巴结肿大和组织细胞吞噬大量淋巴细胞。本文报道1例颅内外非沟通性RDD, 患者因"发现左顶部头皮肿物4个月, 右下肢麻木半个月余"于2020年5月入住十堰市太和医院。患者行头颅磁共振成像(MRI)平扫+增强检查示:左顶骨病灶呈轻度不均匀强化, 其内外侧软组织呈明显强化, 局部内外侧软组织明显不规则增厚, 以外侧为著, 大小约3.2 cm×4.7 cm, 邻近脑实质受压。行左顶部肿物及颅内肿物切除术后, 病理结果显示梭形细胞增生伴炎性反应, 免疫组织化学染色结果支持RDD诊断。患者术后7个月神经功能基本恢复正常, 头颅MRI检查也未见病变复发, 治疗效果满意。     

特发性肥厚性硬脑膜炎临床病理分析

目的探讨特发性肥厚性硬脑膜炎(idiopathic hypertrophic pachymeningitis,IHP)的病理诊断要点。方法回顾性分析3例IHP病人的临床和影像学资料,显微镜下观察其组织学形态。男性2例,女性1例,临床表现为头痛、腰背部疼痛伴双下肢麻木、乏力,均行手术切除。结果影像学显示颅内或椎管内硬膜弥漫增厚或呈结节状。组织学:病变内纤维组织显著增生、胶原化,大量淋巴细胞、浆细胞浸润,淋巴滤泡形成,伴有多少不等的中性粒细胞聚集。免疫表型:增生的纤维组织SMA、MSA阳性;浆细胞CD38、CD138、EMA和Ig G阳性,但Ig G4阴性;CD45RO阳性T淋巴细胞明显多于CD20阳性B淋巴细胞。结论 IHP少见,其正确诊断需结合临床和影像学特征以及病理形态综合分析。     

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.

Intracerebral multifocal Rosai–Dorfman disease

We report a rare occurrence of intracranial Rosai–Dorfman disease (RDD), in a young patient with multiple bilateral intracerebral lesions, and discuss her management. RDD is an histiocytic proliferative disorder, which seldom presents with intracranial pathology. Intraparenchymal RDD without concurrent systemic features is rare. This 31-year old woman presented with two focal seizures, but no peripheral lymphadenopathy, and no significant systemic abnormalities. Preoperative imaging showed intrinsic space-occupying lesions in the left frontal lobe, and right parietal and right inferior frontal white matter. She underwent excision of the peripherally located left frontal lesion, and aside from a five-day course of postoperative dexamethasone, received no further active treatment. Histopathological analysis revealed the characteristic features of RDD. Postoperative imaging, taken at 10 weeks and 12 months, demonstrated resolution of all three lesions. This patient had a rare multifocal, bilateral, intracerebral RDD, illustrating that although a histological diagnosis is necessary, further treatment may not be required.     

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.     

Systemic Rosai–Dorfman disease with central nervous system involvement

Rosai–Dorfman disease (RDD) is a rare idiopathic and lymphoproliferative disorder. Central nervous system (CNS) involvement is infrequent and typically manifests as an isolated lesion. In this article, we describe an unusual case of RDD with multiple lesions in the CNS, upper respiratory tract and lymph nodes. A literature review revealed 45 cases (including the one described herein) of systemic RDD with CNS involvement documented to date. Among these cases, 29 (64.4%) presented with intracranial lesions, 10 (22.2%) with spinal lesions and 6 (13.3%) with both. While the condition of most patients was stable, only four died from RDD or for other reasons. Prognosis is good for most patients, even those with extensive lesions. Although there are various treatments for RDD, surgery is preferred. Given the rarity of RDD, multicenter international collaborations are advocated in order to study disease pathogenesis and develop effective treatment strategies.     

Intracranial Rosai–Dorfman disease in a child mimicking bilateral giant petroclival meningiomas: a case report and review of literature

Objectives and importance Rosai–Dorfman disease (RDD) is a rare but distinctive entity of unknown etiology; isolated intracranial RDD is uncommon. Of 37 reported intracranial RDD cases, only three were reported in children.Clinical presentation We report an unusual case of a 15-year-old boy presenting with 4 months history of raised intracranial pressure with visual deterioration. Computed tomography and magnetic resonance imaging revealed bilateral petroclival enhancing lesions with cavernous sinus extension mimicking meningioma. However, histological examination was diagnostic of RDD.Intervention The patient underwent extended right-sided middle fossa approach and near-total tumor removal from petroclival region and cavernous sinus on both sides in two stages 6 weeks apart.Conclusion Ours is the first case of pediatric isolated intracranial RDD presenting with giant bilateral petroclival masses successfully managed with bilateral extended middle fossa approach in two stages. An optimal treatment for RDD is not established, but complete surgical resection alone seems effective.     

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.     

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.     

Isolated central nervous system Rosai–Dorfman disease and breast cancer: an unusual presentation

The association between Rosai–Dorfman Disease (RDD) and cancer was reported for the first time in 1984. However, there are still a small number of reports of this association. We describe a 60-year-old woman who presented with a focal onset motor seizure followed by tonic-clonic generalization and persistent headache. Magnetic Resonance Imaging disclosed an irregular hyperintense lesion in T2 and vasogenic edema in the left parietal region. Immunohistochemical analysis of a biopsy fragment was positive for protein S-100 and CD68 and negative for CD1a, compatible with the diagnosis of RDD. She previously had breast cancer six years earlier and had used tamoxifen for two years and anostrozol for three years after diagnosis of cancer. RDD has been already associated with different cancers, such as Hodgkin’s lymphoma, non-Hodgkin’s lymphoma, follicular lymphoma, melanoma, adenocarcinoma and small cell lung cancer. As far as we know, this is the first report of an association between breast cancer and RDD.     

Isolated intracranial Rosai–Dorfman disease in a child,a case report and review of the literature

Background

Rosai–Dorfman disease (RDD), otherwise known as sinus histiocytosis with massive lymphadenopathy (SHML), usually affects young adults and commonly presents with massive painless cervical lymphadenopathy. Extranodal disease is present in a third of patients, and it is recognised that this can involve the central nervous system. Intracranial RDD is rare in adults and fewer than 10 paediatric cases have been reported.

Case

A 10-year-old boy with isolated intracranial RDD presents with a painless forehead mass. The management is discussed and the literature reviewed.

Conclusion

This case of isolated intracranial RDD highlights the importance of considering RDD in the differential of paediatric intracranial mass lesions and outlines the diagnostic and treatment challenges faced when managing this rare condition.     

Intracranial presentation of teratocarcinosarcoma

Teratocarcinosarcoma (TCS), an aggressive and extremely rare neoplasm, usually presents as a nasal or paranasal mass. TCS can have intracranial or dural extension from a nasal mass. We found only two instances in the literature that described this lesion as primarily manifesting as an intracranial mass while arising from a primary nasal focus. We describe a patient who had a predominantly frontal-lobe TCS without any symptoms of a primary nasal mass. The gross surgical appearance was a glistening, slimy, firm white mucoid lesion, in contrast to the friable, necrotic and tan-colored lesion usually described. We report a patient with a rare intracranial TCS, and focus on the differential diagnoses of intracranial tumors. Neuropathologists and neurosurgeons should be aware of this mode of presentation for an accurate diagnosis and a well-targeted therapeutic approach.