CLINICOPATHOLOGICAL REVIEW: ESTHESIONEUROBLASTOMA
CLINICOPATHOLOGICAL REVIEW: ESTHESIONEUROBLASTOMA
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临床病理学回顾：感受神经母细胞瘤
DOI：
10.1227/01.neu.0000338948.47709.79
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发表时间：
2009
期刊：
影响因子：
4.8
通讯作者：
V. Prabhu
中科院分区：
文献类型：
--
作者：
T. Bragg;Joseph Scianna;A. Kassam;B. Emami;H. G. Brown;L. Hacein;Joseph I. Clark;K. Muzaffar;N. Boulis;V. Prabhu
布拉格和穆扎法尔博士：一位62岁右利手的白人男性，因右鼻肿块和进行性鼻畸形导致鼻充血和嗅觉丧失恶化6个月。他否认头痛、视力改变、鼻出血或溢液。他注意到他的右鼻孔丰满，右鼻唇沟闭塞，右眼明显流泪。患者无病史或当前用药，儿童时接受过扁桃体切除术。他报告偶尔使用酒精和戒烟4至5个月前提交。他的家族史对于一个患有皮肤癌的兄弟和一个患有白血病的妹妹来说意义重大。在神经系统检查中，患者除嗅觉丧失外无颅神经缺损。前鼻孔镜检查显示鼻中隔明显向左偏移，一个巨大的、肉质的、坚硬的、无压痛的、无搏动的肿块完全阻塞了右鼻腔。肿块的大小阻止了纤维喉镜进入右鼻腔;然而，通过左鼻腔的鼻咽喉镜检查显示肿块延伸到鼻咽。耳镜检查正常，未发现颈部淋巴结病。对右鼻病变进行门诊光纤活检。病变有明显的血管，随后的出血成功地控制鼻腔填塞。颅面区域的磁共振成像（MRI）显示右鼻腔有一个8.4 × 4.4 × 2.5 cm的肿块，导致右侧蝶窦、额窦和上颌窦阻塞（图1A）。与阻塞窦内的分泌物不同，造影剂注射后肿块明显增大（图1A，箭头）。头颅计算机断层扫描（CT）显示右侧上颌窦内侧壁后部破坏（图1B，短箭头），而蝶窦的骨间隔保持完整（图1B，长箭头）。肿块向上延伸至右侧筛房（图1C，箭头），筛房间隔破坏，右侧纸样板中部骨质溶解（图1D，箭头）。对比增强的冠状位T1加权MRI扫描显示肿瘤通过右侧纸样板延伸，抬高但未侵犯眶周（图1，D和E，箭头）。还观察到通过筛状板向下额叶的颅内延伸，测量为9 7 6 mm（图1F，箭头）。希安娜医生：单侧鼻塞、鼻出血、流泪、嗅觉丧失和面部疼痛是颅面部良性和恶性病变的常见症状。最初的体征和症状往往是微妙的，延迟诊断数月至数年（1，8，51）。直到出现继发性症状，如鼻衄、面部疼痛、面部畸形或颅神经损伤（尤其是眼肌）时，才考虑是否有Malig综合征。头痛、复视、眼痛和眼球突出提示肿瘤侵入颅内、眶内和海绵窦（1，10，49，51）。以这种方式出现的良性疾病包括鼻中隔偏曲、鼻甲肥大、过敏性鼻炎、急性或慢性鼻窦炎、腺样体肥大和鼻息肉病。内翻性乳头状瘤和青少年鼻血管纤维瘤也是良性病变，但具有恶性潜能。恶性病变包括嗅神经母细胞瘤、鳞状细胞癌和腺癌。需要考虑其他恶性肿瘤，包括转移性肿瘤、横纹肌肉瘤、脉络膜炎、粘膜黑色素瘤、神经内分泌癌。
Drs. Bragg and Muzaffar: A 62yearold righthanded Caucasian man presented with a right nasal mass and progressive nasal deformity resulting in worsening nasal congestion and anosmia for 6 months. He denied head ache, visual changes, epistaxis, or rhinorrhea. He noted fullness of his right nostril, obliteration of the right nasolabial fold, and significant tearing from the right eye. He had no medical history or current medication use and had undergone tonsillectomy as a child. He re ported occasional use of alcohol and had quit smokeless tobacco 4 to 5 months before presentation. His family history was significant for a brother with skin cancer and a sister with leukemia. On neurological examination, the patient had no cranial nerve deficits aside from anosmia. An anterior rhinoscopy revealed significant deviation of the nasal septum to the left by a large, fleshy, firm, nontender, nonpulsatile mass that completely obliterated the right nasal cavity. The size of the mass precluded passage of a flexible fiberoptic laryngoscope into the right nasal cavity; however, naso phar yn goscopy through the left nasal cavity showed the mass extending into the nasopharynx. An otoscopic examination was normal, and no cervical lymphadenopathy was detected. An outpatient fiberoptic biopsy of the right nasal lesion was performed. The lesion had significant vascularity, and subsequent bleeding was controlled successfully with nasal packing. Magnetic resonance imaging (MRI) of the craniofacial region demonstrated an 8.4 4.4 2.5-cm mass in the right nasal cavity, causing obstruction of the right sphenoid, frontal, and maxillary sinuses (Fig. 1A). The mass en hanced markedly after contrast administration, unlike the trapped secretions in the obstructed sinuses (Fig. 1A, arrows). Cranial computed tomographic (CT) scanning showed destruction of the posterior aspect of the medial wall of the right maxillary sinus (Fig. 1B, short arrow), whereas the bony septa of the sphenoid sinus remained intact (Fig. 1B, long arrow). The mass extended superiorly to the right ethmoid cells (Fig. 1C, arrow) with destruction of the ethmoid cell septa and osteolysis of the midportion of the right lamina papyracea (Fig. 1D, arrow). Contrastenhanced coronal T1-weighted MRI scans showed tumor extension through the right lamina papyracea, elevating but not invading through the periorbita (Fig. 1, D and E, arrows). Intracranial extension to the inferior frontal lobes through the cribriform plate, measuring 9 7 6 mm, was noted as well (Fig. 1F, arrow). Dr. Scianna: Unilateral nasal obstruction, epistaxis, tearing, anosmia, and facial pain are common presenting symptoms of both benign and malignant craniofacial lesions. Initial signs and symptoms are often subtle, delaying diagnosis for months to several years (1, 8, 51). Malig nancy is not considered until secondary symptoms, such as epistaxis, facial pain, facial de formity, or cranial nerve impairment (especially in ocular muscles), occur. Headaches, diplopia, ocular pain, and exophthalmos suggest tumor invasion into the intracranial or intraorbital compartments and cavernous sinus (1, 10, 49, 51). Benign conditions that can present in this manner include a deviated nasal septum, turbinate hypertrophy, allergic rhinitis, acute or chronic sinusitis, adenoid hypertrophy, and nasal polyposis. Inverted papilloma and juvenile nasal angiofibroma are also benign lesions but with malignant potential. Malignant lesions include esthesioneuroblastoma, squamous cell carcinoma, and adenocarcinoma. Other malignancies need to be considered, including metastatic tumors, rhabdomyo sar coma, chordoma, mucosal melanoma, neuroendocrine carCASE PROBLEMS IN NEUROSURGERY