颅骨浆细胞瘤的诊断和治疗

正浆细胞瘤(plasmacytomas)是浆细胞异常增生为特征的恶性肿瘤,WHO中枢神经统肿瘤分类将其归属于淋巴瘤和造血系统肿瘤。颅内浆细胞瘤非常少见,误诊率较高,多误诊为脊索瘤、脑膜瘤、嗜酸性肉芽肿等其他肿瘤。本文总结我院神经外科2010年1月至2015年11月临床确诊为颅骨浆细胞骨髓瘤3例,并复习相关文献如下,旨在提高病的诊断和治疗水平。一、对象与方法1.临床资料:男2例,女1例,年龄46~67岁,平均58岁;     

孤立性浆细胞瘤导致脊髓压迫症1例报告

孤立性浆细胞瘤引起的脊髓压迫症非常少见,临床上易误诊,现将我院诊治的1例患者资料报告如下. 1 病例 男,33岁.因"双侧下肢进行性麻木、无力2个月"于2005年7月2日入院.患者2个月前出现双侧足底麻木,渐向足背及踝关节以上发展,1个月后双下肢麻木伴乏力,行走易跌倒;曾在当地医院按Guillain-Barre综合征(GBS)予地塞米松治疗20余天,病情无改善.查体:神志清楚,脑神经未见异常,双上肢肌力正常,双下肢肌力Ⅴ- 级,肌张力正常,双侧踝关节以下痛觉减退,双下肢痛觉较上肢敏感,四肢腱反射正常,病理征(-).     

颅内孤立性浆细胞瘤：附四例报告

本文报道罕见的颅内孤立性浆细胞瘤4例,经脑血管造影或CT打描,均误诊为脑膜瘤。手术证实4例分别来自蝶嵴区,中颅窝,岩锥的硬脑膜及顶枕部大脑镰;病理镜检及免疫组织化学检查为浆细胞瘤。     

颅底孤立性浆细胞瘤的临床诊治进展

孤立性浆细胞瘤是由单克隆浆细胞异常增殖引起的恶性肿瘤,在浆细胞瘤中占不足10％,可分为骨性孤立性浆细胞瘤和髓外浆细胞瘤。其中,骨性孤立性浆细胞瘤为原发于骨的单发的浆细胞瘤,多见于椎骨、肋骨、颅骨、骨盆等中轴骨,约75％将进展为多发性骨髓瘤,预后变差。发生于颅底的孤立性浆细胞瘤十分罕见,较多发性骨髓瘤及多数颅底恶性肿瘤预后稍好,但较颅顶孤立性浆细胞瘤预后较差,     

脊柱孤立性浆细胞瘤伴淀粉样变性

目的报告1例脊柱孤立性浆细胞瘤伴淀粉样变性患者的诊断与治疗过程,结合文献探讨其组织病理学特征。方法与结果女性患者,46岁,临床主要表现为双下肢无力,行走不稳伴脚趾麻木感。头部MRI显示,T_(5~7)水平脊髓后方硬脊膜外和T_(6~7)水平附件内不规则占位性病变,增强扫描呈不均匀明显强化,边界清晰,硬脊膜受压向前移位。术中可见T_(5~7)棘突和椎板以及部分T5、T7棘突和椎板被侵蚀,骨皮质呈"虫蚀"样改变;病变位于椎管内硬脊膜外,血供丰富,呈疏松骨样结构,硬脊膜完整。组织学形态观察,肿瘤细胞以密集排列的小细胞为主,可见灶性浆细胞样细胞分化,其间可见片状粉染物质沉积,并多见人工裂纹和散在分布的多核巨细胞。免疫组织化学染色,肿瘤细胞胞质CD138、CD38和波形蛋白弥漫性阳性;白细胞共同抗原散在阳性;免疫球蛋白κ轻链和λ轻链、CD99、S-100蛋白、广谱细胞角蛋白、上皮膜抗原、黑色素瘤相关抗原HMB45和CD34阴性;Ki-67抗原标记指数约1.25%。特殊染色,粉染物质刚果红染色呈棕红色。原位杂交显示免疫球蛋白κ轻链DNA含量高于λ轻链。最终病理诊断为(脊柱)孤立性浆细胞瘤伴淀粉样变性。术后辅以药物化疗。随访18个月肿瘤无复发和转移。结论脊柱孤立性浆细胞瘤伴淀粉样变性临床罕见,影像学检查对诊断有一定提示意义,明确诊断仍依靠特异性组织形态学、免疫表型、特殊染色和原位杂交检测。     

孤立性颅骨骨髓瘤误诊颅骨肿瘤一例

孤立性颅骨骨髓瘤误诊颅骨肿瘤一例鲁道远李知森患者男，１５岁。因额部肿块３个月就诊。体检：左额部见４ｃｍ×３ｃｍ×２ｃｍ的肿物，硬、不活动、无压痛。血象：ＷＢＣ５．８×１０９／Ｌ，血沉２５ｍｍ／ｈ。硷性磷酸酶１９．７金氏单位。颅骨片：示额骨有３ｃｍ×３...     

立体定向切除侧脑室室管膜下孤立性浆细胞瘤1例

患者,女性,2 5岁,间断性头痛2年余,加重伴恶心、呕吐半月余入院就诊。查体:神志清,精神一般,语言流利,神经系统未见明显阳性体征。腰穿:压力2 4 0mmH2 O ,脑脊液常规及生化检查正常。全身骨骼拍片未见异常。髂后上嵴骨穿,骨髓检查细胞分类正常。尿本周氏蛋白试验阴性。头颅CT提示:左侧侧脑室后角外侧可见一类圆形大小约1.8cm×2 .3cm高密度影,CT值5 6hHz ,病灶均匀强化,病灶周围可见不同程度的低密度水肿影,术前考虑脑室管膜瘤。全麻后,在立体定向指导下开颅,术中见骨质无异常,脑膜正常,脑组织张力稍高,显微外科切除肿瘤,术中可见一类圆…     

颅骨骨巨细胞瘤合并脑出血1例

骨巨细胞瘤(Giant cell tumor of bone,GCT)是最常见的骨肿瘤之一,罕见于颅骨,我科2011年1月收治颅骨骨巨细胞瘤合并脑出血一例。1病例男,43岁。因剧烈头痛1天入院。既往有头痛病史,病程     

颅骨巨细胞瘤3例报告并文献分析

目的探讨颅骨巨细胞瘤的诊断要点、治疗及预后。方法回顾性分析2003-09~2010-09我院神经外科手术治疗3例患者临床、影像和病理资料以及术后随访情况。结果本组3例病人,1例术后放疗再手术,1例术后放疗,1例放疗后手术。平均随访37月,1例复发,1例恶变,1例无复发。结论颅骨巨细胞瘤是少见的低度恶性肿瘤,根据生长特点、影像特征不难做出诊断,术前放疗可减少术中出血、局部复发,且预后与手术切除程度有关。     

Kahler's disease presenting with a solitary cranial vault plasmacytoma

INTRODUCTION: Solitary plasmocytoma is rarely located in the cranial vault. Usually observed, in elderly patients, occurrence during adolescence is extremely rare. We report the case of a 16-year-old patient presenting a cranial vault solitary plasmocytoma which revealed Kahler disease. CASE REPORT: A 16-year-old patient consulted for a frontal tumefaction becoming painful with diplopia. The cranio-encephalic radiography and the MRI showed a tumoral process involving cranial vault; biopsy revealed a solitary plasmocytoma. Search for multiple myeloma was negative. Six months later, a disease of Kahler was declared. CONCLUSION: Clinical and biological follow-up of cranial plasmocytoma is necessary, because progression to multiple myeloma is possible as in our observation. Imaging and particularly MRI are needed to specify the axial seat of the tumoral process and its relationship with the nervous and vascular structures.     

Peripheral neuropathy and solitary plasmacytoma.

Three patients with peripheral neuropathy and a solitary plasmacytoma are presented, and the literature is reviewed. It is suggested that middle-aged men with an obscure progressive sensorimotor neuropathy, a raised CSF protein, and otherwise negative investigations should have a full skeletal survey since irradiation of a plasmacytoma may lead to a considerable improvement in the associated neurological disability.     

Impaired vision associated with a solitary intracranial plasmacytoma

Solitary intracranial plasmacytomas (SICPs) are extremely uncommon tumors in the central nervous system, and are often misdiagnosed pre-operatively. We report a patient with SICP, describe the neuroradiological and neurosurgical features and the clinical management of this patient, and review the pertinent literature.     

A case of solitary Blastomyces dermatitidis meningitis

We present a case of a 35 year-old male with Blastomyces dermatitidis meningitis as the primary presentation of blastomycosis infection, without evidence of involvement outside the CNS at time of discharge. We focus on the magnetic resonance imaging findings, with histopathologic correlation.     

Neuropathy with contractures evoking the stiff-man syndrome. Latent solitary plasmacytoma

A further case of apparently primary sensorimotor neuropathy is reported. The patient, a 31-year-old man, developed the syndrome after a 4-year history of a solitary vertebral plasmocytoma revealed by post mortem examination. Circulating monoclonal immunoglobulin and specific anomalies on nerve biopsy were absent. The condition became progressively worse, with initial and predominant autonomic nervous system anomalies. After 3 years, contractures of the limbs and paravertebral muscles, with painful paroxysmal attacks suggestive of the stiff-man syndrome appeared. Pathology confirmed the severity of the root lesions with diffusion to the cranial nerves. Reflex tests could not be conducted but the semiologic characteristics and the efficacity of baclofen were distinctive of continuous muscle fiber activities reported in some peripheral neuropathies. This case was comparable to some reported ones of interneuron rigidity.     

A case of solitary cyst of the kidney

Summary A case of solitary cyst of the kidney is reported. The presence of a chronic vascular nephritis with scar tissue and constriction of the tubules, places this case in the group which develops according to the theory of Virchow.     

A case of Crow-Fukase syndrome with extramedullary plasmacytoma: marked clinical deterioration following a biopsy to plasmacytoma]

A 66-year-old man developed paresthesia of the distal parts of the bilateral lower limbs a week after his upper respiratory infection, followed by the weakness with the legs and paresthesia with the lip area, tongue and finger tips. Those symptoms gradually became worse to the point that he was unable to walk 10 days later. Although skin pigmentation, edema, and lymph node swelling were not found, we made a diagnosis of Crow-Fukase syndrome (CFS) because of clinical features of polyneuropathy, IgG-lambda type M proteinemia, endocrinological abnormality, elevated plasma level of vascular endothelial growth factor (VEGF) and extramedullary plasmacytoma in his abdomen. Following intravenous immunoglobulin therapy (IVIg), he showed marked improvement. However, his neurologic symptoms deteriorated acutely just after open biopsy together with the elevation of VEGF level, and a few days later he was in the state of flaccid quadriparesis. We tried IVIg therapy again and his neurologic symptoms were markedly improved. We speculated that an elevated VEGF, released from plasma cells induced by the bioprocedure, might have caused an increase in microvascular permeability and affected the blood-nerve-barrier, thereby his neurologic symptoms deteriorated. It is thought that this case may support the hypothesis that a significant role is played by VEGF in the pathomechanism of the development of CFS. Additionally we experienced that IVIg was very effective to the neurologic symptoms, and we think that IVIg will be able to be one of the future therapy of the CFS. To our knowledge, there has been no report of CFS which manifested acute deterioration of his neurologic symptoms just after open biopsy with acute onset with Guillain-Barré syndrome like symptoms.     

Solitary plasmacytoma: a case report

We report a case of solitary vertebral plasmacytoma that was peculiar in mode of onset and neurological course. The clinical and radiological evidence pointed to a generic diagnosis of epidural osteolytic process while electroimmunophoresis of blood narrowed the diagnosis to spinal plasmacytoma, confirmed at operation.

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Sommario Gli autori riportano un caso di plasmocitoma solitario vertebrale che ha presentato particolari modalità di esordio e di decorso clinico-neurologico. La integrazione tra studio clinico, radiologico e immunologico ha consentito di giungere all'antto operatorio con un sospetto diagnostico ben motivato di plasmocitoma a fronte di una diagnosi generica di processo osteolitico epidurale di natura da determinare.

     

A case of cranial fasciitis induced by trauma]

We present a 20-month-old boy with a rapid growing occipital mass after head trauma, that was pathologically diagnosed as cranial fasciitis. Cranial fasciitis, first described as a specific entity by Lauer and Enzinger in 1980, is a benign fibroblastic lesion occurring in children that resembles nodular fasciitis pathologically. Nodular fasciitis also shows benign proliferation of fibroblasts and myofibroblasts in the subcutaneous tissues. This disease was firstly reported in 1955. This lesion is arising in the upper and lower extremities and trunk in adult commonly. The rapid growth, abundant cellularity, and mitotic activity cause these lesions to be misdiagnosed as sarcomas frequently. However, these lesions recur rarely, do not develop metastases, and are readily cured only by surgical excision. Cranial fasciitis is similar to nodular fasciitis in clinical cause and pathology. Therefore cranial fasciitis should be considered a variant of nodular fasciitis, although it differs from noduler fasciitis in its frequent site and age. Cranial fasciitis occurs in skull bone and in childhood. The cause of them are still unknown. However, we speculate on the relationship between head trauma and cranial fasciitis making reference to the fact that 5-15% of nodular fasciitis were caused by trauma. So we investigated this relationship in cranial fasciitis, then 8 cases of 40 cases(20%), that we could trace in the world literatures, had histories of previous head trauma. From this result, head trauma is one of a cause of cranial fasciitis probably.