颅内血管外皮细胞瘤的诊断与治疗

目的探讨颅内血管外皮细胞瘤的诊断及治疗。方法2004年3月～2009年12月,手术切除颅内血管外皮瘤9例,结合文献,对其临床资料进行回顾性总结。结果9例血管外皮细胞瘤中6例误诊;全部患者均行手术治疗,其中全切除7例,次全切除2例。5例患者获随访3—36个月,平均随访18个月,其中2例复发,1例发生远处转移。结论颅内血管外皮细胞瘤预后不良,手术切除是其最佳治疗手段,术后辅以放疗可明显改善预后。     

鼻腔鼻窦血管外皮细胞瘤5例临床分析

目的：提高对鼻腔鼻窦血管外皮细胞瘤的认识和诊治水平。方法：回顾性分析5例鼻腔鼻窦血管外皮细胞瘤患者的临床资料及病理资料。结果：5例CT检查结果提示血管性肿瘤,病变多呈均一高密度块影,并有明显强化的特点。病理检查提示肿瘤富含毛细血管,呈树枝状,管腔内壁为正常内皮细胞,管腔周围有弥漫分布的圆形、卵圆形或梭形外皮细胞排列,可见核分裂像。所有病例均行手术切除并经病理检查证实。肿瘤预后与病理分级密切相关;高度恶性肿瘤的复发率明显高于中间性和低度恶性肿瘤。该组误诊率为80%。结论：血管外皮细胞瘤是一类潜在恶性肿瘤,影像学检查有助于病变的定性定位及手术方案的制定,但缺乏特征性表现,最终诊断需依靠病理学检查。完整手术切除仍是目前最佳的治疗方法。其临床行为与病理分级密切相关,临床医师要重视组织病理学特征的描述。所有病例均需密切随访。     

鼻及鼻咽部血管外皮细胞瘤的临床特点及回顾性分析CSCD

目的通通过分析鼻及鼻咽部血管外皮细胞瘤的临床及病理特征,探讨最佳的诊断及治疗方法。方法2014年3月~2022年4月河南科技大学第一附属医院收治的10例鼻及鼻咽部血管外皮细胞瘤的临床资料进行回顾性分析,结合文献对其临床特征、病理特点、治疗及预后进行分析。结果10例患者中,8例发生于鼻腔鼻窦,2例发生于鼻咽部,均在我科行手术治疗,术后随访时间1~9年,9例未见复发及远处转移,1例因心衰死亡。结论鼻及鼻咽部血管外皮细胞瘤临床症状缺乏特异性,确诊应联合应用组织学特征及免疫组化表型的检测;手术治疗为治疗的有效手段,术后可局部复发,但罕见转移。     

颅底B细胞性非霍奇金淋巴瘤的影像学特征分析（附2例报告）

目的：探讨颅底B细胞性非霍奇金淋巴瘤的影像学特点。方法：报道2例颅底B细胞性非霍奇金淋巴瘤,并结合文献对其影像学特征进行总结分析。结果：颅底B细胞性非霍奇金淋巴瘤的影像学表现为T2加权等或低信号,MRI肿瘤强化不明显,颅内侵犯软组织的宽度远大于颅底骨质的破坏范围并沿硬脑膜浸润。结论：颅底B细胞性非霍奇金淋巴瘤具有特征性的影像学表现,对该疾病的诊断和鉴别诊断有一定意义。     

罕见咽后隙浆细胞瘤死亡1例

目的 通过报告1例发生于咽后隙髓外浆细胞瘤的临床病理特征、免疫组化、影像学特点及鉴别诊断,以提高对本病的认识.方法 对咽后隙髓外浆细胞瘤进行组织病理学观察、免疫组化检测及影像学检测,结合文献对其临床表现、病理特点、影像学特征及治疗进行讨论.结果 本例病理学诊断为浆细胞瘤.磁共振示其大小为16 cm×8 cm.患者因经济拮据放弃治疗随访1个月后,死于呼吸困难.结论 极为罕见的咽后间隙巨大髓外浆细胞瘤可能以渐进吞咽困难和上呼吸道阻塞为主要表现,及时诊断和采取适宜方式治疗有利于获得良好预后.     

鼻腔球周皮细胞瘤1例

球周皮细胞瘤（glomangiopericy tomar）即球血管外皮细胞瘤,是一种少见的中间型或低度恶性软组织肿瘤,其同义词有鼻窦血管外皮瘤、鼻窦型血管外皮瘤、鼻窦血管外皮瘤样肿瘤等,现将我科收治的1例鼻腔球周皮细胞瘤报道如下。1临床资料患者女,66岁,因＂左侧鼻腔新生物＂入院。患者入院前半年无明显诱因出现左侧鼻塞,鼻腔偶有疼痛,     

外耳道深部良性纤维组织细胞瘤侵犯颅内1例并文献复习

目的探讨深部良性纤维组织细胞瘤的病理、临床表现、影像学特点及鉴别诊断。方法报道1例外耳道深部良性纤维组织细胞瘤的临床资料,并对相关文献进行复习。结果本例患者病史8年,肿块局部侵犯乳突、翼内外肌、颅内等,但无远处转移。镜检可见梭形的成纤维细胞成漩涡状排列及其间多核巨细胞、炎性细胞浸润,免疫组化检查示：CD68（＋）,Vim（＋）,S-1 0 0（＋）,CD 1 a（-）,Ki-6 7（-）。上述特点符合深部良性纤维组织细胞瘤诊断。结论深部良性纤维组织细胞瘤是一种临床少见、诊断复杂、预后好的良性肿瘤,手术完整切除能明显降低其复发率。     

益气解毒方干预鼻咽癌细胞裸鼠移植瘤肿瘤微环境细胞免疫耐受现象的初步研究

目的通过观察益气解毒方对鼻咽癌细胞裸鼠移植瘤的干预效应,探讨调节性T细胞诱导的鼻咽癌肿瘤微环境免疫耐受性特征及其对干预药物的反应性。方法人鼻咽癌细胞株CNE2细胞建立裸鼠移植瘤模型,然后随机分为模型组、益气解毒方治疗组、顺铂阳性对照组、益气解毒方与顺铂联合治疗组,每组8只动物,分别给予相应处理药物,系统观察各组裸鼠移植瘤生长情况及体积变化。观察期满后处死动物,取瘤体称重,分别计算抑瘤率,并切取瘤体组织标本,观察比较各组移植瘤的病理组织学变化,免疫组化法检测各组标本CD4、CD8、Foxp3表达活性,比较其组间差异。结果各组检测结果比较显示,益气解毒方处理后,移植瘤组织中的CD4、FoxP3表达活性受到明显抑制,CD8表达则明显提高,尤以联合治疗组表现明显,与模型组比较,组间差异具有显著性统计学意义。结论益气解毒方能有效改善鼻咽癌裸鼠移植瘤肿瘤微环境的免疫耐受现象而抑制肿瘤生长速度;联合应用化学药物后,该一效应尤为明显。     

腮腺血管外皮细胞瘤1例及文献复习

血管外皮细胞瘤(hemangiopericytoma)是一种来源于毛细血管外皮的较为罕见肿瘤。其特征为梭形瘤细胞,外围有网状纤维分布在单层内皮细胞的血管腔周围,好发于深层组织,如下肢腹膜后和纵隔等处,发生于腮腺者较少见,不为耳鼻咽喉-头     

颅内三叉神经鞘瘤的CT及MRI诊断

目的分析颅内三叉神经鞘瘤的CT和MRI表现。方法搜集36例经手术和病理证实的颅内三叉神经鞘瘤的CT和MRI资料,探讨颅内三叉神经鞘瘤分型、诊断要点及有关鉴别诊断。结果颅内三叉神经鞘瘤分三型：颅后窝型（8例）、颅中窝型（4例）及骑跨型（24例）。病灶信号混杂,易囊变（12例）,增强后可见不均匀强化;CT能看到岩尖骨质吸收或破坏（17例）。结论CT和MRI的联合应用对三叉神经鞘瘤的定位、定性诊断具有较高的准确性,对临床治疗及手术方式的选择具有指导作用。     

Magnetic resonance imaging of olfactory neuroblastoma

Olfactory neuroblastoma is an uncommon intranasal tumor originating from olfactory neuroepithelium. Despite the development of electron microscopy and immunohistochemical testing, the pathological diagnosis of this tumor is still difficult because of the wide range of histological features. Magnetic resonance imaging (MR) of this tumor and the pattern of contrast enhancement have not been well described. The purpose of this report was to analyze the MR characteristics of olfactory neuroblastomas. The MR signal, pattern of contrast enhancement, and correlation with high-resolution computed tomography (CT) imaging were examined. Seventeen patients with olfactory neuroblastoma were treated at Hokkaido University Hospital and a related hospital during the past 25 years. MR images taken in 12 patients and CT images taken in 9 patients with histologically confirmed olfactory neuroblastoma were retrospectively reviewed. Compared with brain gray matter, 11 tumors were hypointense on T1-weighted images, 9 homogeneously and 2 heterogenously. Eight tumors were hyperintense on T2-weighted images, 3 homogeneously and 5 heterogeneously, although their appearance was less intense than that of sinusitis. Gadolinium enhancement was moderate in one case and marked in 10 of the 11 cases, 9 homogeneously and 2 heterogeneously. Nine of the 11 tumors showed smooth regular shaped margins; 2 of these tumors exhibited irregular infiltrating margins on gadolinium-enhanced images, compared to the pre-contrast T1-weighted images. Eight of the 11 tumors had clearly demarcated margins, while 3 of the 11 tumors did not exhibit gadolinium enhancement. Six of the 12 cases (50%) exhibited intracranial cysts on the gadolinium-enhanced images. T2-weighted or gadolinium-enhanced images successfully distinguished sinusitis from tumors in 4 cases whereas the CT images failed. Gadolinium enhancement, particularly in the tangential plane, demonstrated intracranial extension not apparent on the CT images in one case. In most cases, olfactory neuroblastomas are hypointense on T1-weighted images, hyperintense on T2-weighted images, and show marked homogeneous enhancement with well-demarcated regular margins upon gadolinium enhancement. Although the definite diagnosis is based on histopathology findings and MR features are nonspecific, they may suggest an imaging diagnosis of olfactory neuroblastoma when seen in the superior nasal cavity. MR is superior to CT both in delineating the extent of the tumor and in making an imaging diagnosis.     

Extra- and intracranial dumbbell-shaped hemangiopericytoma

Hemangiopericytomas are malignant tumors arising from pericytic cells and account for less than 1% of all vascular neoplasms. We report a rare case of an extra- and intracranial dumbbell-shaped hemangiopericytoma originating from the soft tissue of the neck and penetrating the skull base with invasion into the posterior cranial fossa. The 59-year-old female patient presented with a large pulsating neck mass and reported weakness, abnormal fatigue and headache. MRI revealed an inhomogeneously enhancing tumor and cerebral angiography showed intensive vascularization. Preoperative embolization was performed in order to decrease the operative blood loss. The tumor was operated via a far lateral approach through an osteoclastic suboccipital craniotomy. Total resection of both the intra- and extracranial part of the neoplasm (grade I by Simpson) could be achieved. The histopathological analysis revealed a mesenchymal, hypervascular tumor with the classic staghorn vascular pattern. In this article, we discuss the clinical presentation and multidisciplinary management of hemangiopericytoma and describe the radiological and pathological features of this tumor entity.     

An immunohistochemical study of sinonasal hemangiopericytoma

We present herein the imaging and pathological features of a 28-year-old male with a sinonasal hemangiopericytoma-like tumor occupying the left nasal meatus. At the initial visit, a nasal polyp was suspected, but, as the patient was bleeding readily, an angiomatoid lesion was also regarded as a possible diagnosis.Based on a thorough histopathological analysis, a sinonasal hemangiopericytoma-like tumor was diagnosed. Hematoxylin and eosin staining also showed a mild degree of nuclear pleomorphism and a slight increase in mitotic activity, and immunohistochemical studies using anti-CD34, MIB-1, and Vimentin antibodies were useful for distinguishing the hemangiopericytoma-like tumor from true hemangiopericytoma and a solitary fibrous tumor.     

Solitary fibrous tumors of the head and neck: a clinicopathologic and radiologic review

OBJECTIVE: To describe the clinicopathologic and radiologic features of solitary fibrous tumors of the head and neck. DESIGN: Retrospective analysis. SETTING: Tertiary referral center that performs head and neck surgical oncology. PATIENTS: Twelve patients with solitary fibrous tumors of the head and neck identified from the pathology and soft tissue tumor databases at Memorial Sloan-Kettering Cancer Center, New York, NY, from 1990 to 2004. All cases were reviewed by 3 experienced pathologists, 1 of whom is an experienced soft tissue tumor pathologist. The diagnosis was confirmed by microscopic features on hematoxylin-eosin staining and by positive staining for CD34 and Bcl2 on immunohistochemical analysis. Tumors were scored for mitotic activity, cellularity, nuclear pleomorphism, necrosis, and the presence of a malignant component. Details on patient characteristics, tumor characteristics, previous treatment and surgery, adjuvant treatment, and outcome were recorded from clinical records. RESULTS: Solitary fibrous tumors occurred in patients over a wide age range (27-78 years; median age, 52 years). Seven patients (58%) were women, and 5 (42%) were men. Most tumors presented as a slow-growing painless mass with a duration ranging from 2 months to 5 years. The tumors ranged from 1 x 1 cm to 6 x 5 cm. Patients presented with a subcutaneous mass of the scalp or face in 4 cases, intraoral mass in 4, sinonasal mass in 3, and paraspinal mass in 1. Computed tomographic and/or magnetic resonance imaging scans of 7 of the 12 patients showed well-circumscribed tumors that enhanced strongly with contrast. Treatment for all of the patients was surgical resection. Pathologic findings showed that 9 tumors were benign and 3 were malignant. Three patients had a positive surgical resection margin. All patients were alive at a median follow-up of 8 months (range, 1-76 months). Local recurrence occurred in 1 patient who had positive surgical margins 3 years after the initial surgery. CONCLUSIONS: Solitary fibrous tumors of the head and neck region are rare and most commonly benign. The diagnosis depends on microscopic and immunohistochemical features, although imaging may help. Patients with these tumors can be safely treated with local excision, but tumors with positive margins require close follow-up over several years owing to the potential for late local recurrence.     

Sinonasal glomangiopericytoma--case report

Haemangiopericytoma is a rare vascular tumor originated from perivascular cells. A sinonasal-type of haemangiopericytoma is called glomangiopericytoma. This type of hemangiopericytoma is usually of low malignant potential. The most common symptoms are nasal breathing disturbances and epistaxis. The clinical diagnosis may be difficult. The radiological imaging techniques (CT) can provide adequate information with regard to extension of the tumor, although final histological confirmation is essential. Surgery is the treatment of choice. We present a case of 45-year-old male patient complaining of epistaxis and left side nasal obstruction. Radiological examination (CT) showed the tumor localized in the left nasal cavity extending to the left maxillary sinus and ethmoidal cells. The tumor was removed via lateral rhinotomy with complete amelioration of symptoms. The tumor was diagnosed as glomangiopericytoma by histologic and immunohistochemical findings. During the 16-months follow-up no recurrence or epistaxis was observed.     

颞骨炎性肌纤维母细胞瘤1例报告并文献复习

目的：探讨颞骨炎性肌纤维母细胞瘤的临床表现，术中所见，以及组织病理学特征。方法：结合文献复习，报告1例颞骨炎性肌纤维母细胞瘤患者的临床资料并行光镜检查和免疫组织化学染色分析。结果：肿瘤呈侵袭性生长，有明显的骨质吸收、破坏。病理组织学检查主要由梭形细胞和大量炎症细胞构成。免疫组织化学显示波形蛋白、平滑肌特异性肌动蛋白以及CD68均为阳性，S-100、CK均为阴性。结论：颞骨炎性肌纤维母细胞瘤是一种少见的呈侵袭性生长的肿瘤，容易局部复发。治疗以手术为主，辅以激素治疗。     

头颈部血管平滑肌瘤

目的 提高对头颈部血管平滑肌瘤（angioleiomyoma,ALM）的认识。方法　收集并分析20例头颈部ALM的临床、影像、病理及术后随访资料。结果　女性多见,发病年龄平均52.5岁。无痛性肿块13例、鼻塞或（和）反复鼻出血4例、体检意外发现病变3例。B超示呈瘤体内有较丰富散在点状或条状血流信号;MRI示T1WI呈低-等信号,T2WI呈内部为非均质高信号、边缘为环形低信号,增强后明显强化。HE染色示血管壁平滑肌呈束状排列,血管壁增厚,管腔多呈裂隙状。免疫酶标检测示calponin、desmin和SMA阳性表达;部分病例孕激素、雌激素受体阳性表达。所有病例行肿块切除后无复发、恶变及转移。结论　头颈部ALM行B超和MRI检查有助于诊断,组织学检查和免疫酶标检测可明确诊断,性激素可呈阳性表达,手术治疗预后良好。     

3例累及邻近多部位的儿童鼻腔鼻窦胚胎型横纹肌肉瘤临床分析

目的探讨儿童横纹肌肉瘤（rhabdomyosarcoma,RMS）的临床特点、影像学检查及临床病理学特征，以期减少漏诊误诊，提高临床诊断和鉴别诊断水平。方法回顾性分析3例不典型学龄前期儿童RMS的详细临床资料并复习相关文献。结果2例以眼部症状为主诉收入院，1例罕见以反复鼻腔出血并睡觉打鼾为主诉收入院，相关影像学检查显示肿瘤多已广泛侵犯破坏周围结构，行相关手术病理确诊，均为胚胎性RMS。免疫组化显示3例患者Myogenin均为阳性，Ki 67（LIt分别约为90%、80%、70%），其中2例VIM阳性，2例DES（部分或者局灶性）阳性，2例CD99阳性。后转入肿瘤科规律治疗。结论儿童RMS早期临床表现不典型呈现多样化，具有恶性程度高、侵袭性强、进展快等特点，需要根据临床特征结合影像学检查及免疫组化结果确诊。