鼻部胚胎性横纹肌肉瘤误诊鼻前庭囊肿1例分析

横纹肌肉瘤是儿童和青少年软组织肉瘤中最常见的类型,其中以胚胎性横纹肌肉瘤(embryonal dlabdomyosarcoma,ERMs)为最常见亚型,占横纹肌肉瘤的50%～60%[1].我科曾收治1例患儿,误诊为鼻前庭囊肿,现报告如下.     

中耳胚胎性横纹肌肉瘤1例

患儿,女,4岁.发现左耳道肿物,耳道流脓数天,于1998年4月5日就诊于我院门诊.患儿以往无耳流脓史.检查：左耳道内段有肉芽状肿物,少量脓性分泌物,鼓膜窥不清.     

鼻部胚胎型横纹肌肉瘤13例临床分析

目的:提高临床对鼻部胚胎型横纹肌肉瘤(ERMS)的诊疗水平.方法:回顾性分析13例鼻部ERMS患者的病史资料并复习文献.结果:鼻部ERMS男性多发.Ⅰ期1例,Ⅱ期4例,Ⅲ期7例,Ⅳ期1例.单纯手术治疗2例,手术加放、化疗9例,手术加放疗1例,放疗加化疗1例.2年生存率为69.2%(9/13),3年生存率为30.8%(5/13).结论:鼻部ERMS生长较为隐蔽,对可疑患者应及时做病理活检及免疫组织化学确诊.早期诊断、综合治疗将有利于其预后.手术联合放、化疗是治疗鼻部ERMS的有效手段.     

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。     

非上皮源性鼻咽部恶性肿瘤13例分析

非上皮源性鼻咽部恶性肿瘤远比上皮源性(鼻咽癌)少见，据统计我国两者之比为1：99～211，在欧美国家其比例则为1：3～9。非上皮源性鼻咽部恶性肿瘤中最常见的为恶性淋巴瘤，其他有纤维肉瘤、血管内皮肉瘤、恶性黑色素瘤等。国内报道较少，常英展在1997年报告鼻咽部血管内皮肉瘤1例，崔楫安、史书垣在1998年报告鼻咽部神经纤维肉瘤1例。为提高临床对本病的认识，现将我院耳鼻咽喉科自1990～2000年间收治的13例报道如下。     

Embryonal rhabdomyosarcoma of nasopharynx

A rare case of Embryonal Rhabdomyosarcoma in a 5 year male child involving Nasopharynx with intracranial. secondaries is reported. The child presented with difficulty in breathing and swallowing with change of the voice and bleeding from nose and mouth off and on. Histopathology of the excised mass proved the diagnosis of RMS & CT scan showed secondaries in the left frontal & temporal to be of brain. In spite of the surgery and chemotherapy, the disease proved to be fatal within 3 months of diagnosis.     

Botryoid rhabdomyosarcoma of the nasopharynx

A case report of a Stage III botryoid rhabdomyosarcoma of the nasopharynx associated with a six-and-a-half-year survival is presented. Treatment consisted of surgery, radiotherapy (6,000 rads TCT) to the nasopharynx and maxillary sinuses bilaterally, and six cycles of polychemotherapy with Vincristine, Adriamycin, Cyclophosphamide and DTIC, without major loss of function or cosmetic deformity. The histology of the lesion is discussed with reference to recent classification and prognosis. The authors suggest that the histological type and prognosis of rhabdomyosarcoma of the nasopharynx in children may be better correlated in future studies.     

Embryonal rhabdomyosarcoma of adult nasopharynx

Adult rhabdomyosarcomas in the head and neck are extremely rare and carry a poor prognosis. They should be considered as a distinct clinical entity. The authors report a case of embryonal rhabdomyosarcoma in an adult nasopharynx treated with a combined modality treatment of chemotherapy and radiotherapy as per the European International Society of Paediatric Oncology (SIOP) MMT 89 study, group D. The patient responded to treatment and 10 years later, he is still alive with no signs of metastatic disease.     

婴儿中耳胚胎型横纹肌肉瘤1例并文献复习

目的 提高耳鼻喉科临床医师对婴儿中耳横纹肌肉瘤的认识.方法 对1例婴儿中耳胚胎型横纹肌肉瘤进行临床资料分析并复习相关文献.结果 患儿女,10月龄,患儿家长发现患儿左侧耳道肿物,伴耳道少量出血.CT及MRI提示左侧外耳道、鼓室及乳突内占位性病变,咽旁间隙、颈部、双侧腋窝淋巴结转移.通过活检手术后病理及免疫组化检查确诊为横...     

鼻咽恶性淋巴瘤43例误诊分析

目的 分析鼻咽恶性淋巴瘤的误诊原因，探讨减少误诊的措施。方法对我院1990至2002年间住院治疗的120例鼻咽恶性淋巴瘤病人进行回顾性分析。结果120例鼻咽恶性淋巴瘤中有43例误诊，误诊率高达35.8％。结论鼻咽恶性淋巴瘤易发生临床误诊和病理误诊，全面掌握鼻咽恶性淋巴瘤的临床表现特点、重视鼻咽的检查和活检、注意影像学检查、结合实验室检查及免疫组化等是减少鼻咽恶性淋巴瘤误诊的有效措施。     

鼻腔鼻窦胚胎型横纹肌肉瘤磁共振表现及临床价值

目的 探讨鼻腔鼻窦胚胎型横纹肌肉瘤(embryonal rhabdomyosarcoma,ERMS)的磁共振(MRI)表现和临床价值.方法 回顾性分析5例经组织病理学证实的鼻腔鼻窦ERMS的临床、病理和MRI资料,重点分析肿瘤发生的年龄、临床症状、发生部位和MRI特点,以及MRI在诊断和治疗过程中的价值.结果 ERMS患者主要临床症状包括:鼻塞4例次,眼球突出4例次,视力下降2例次,涕中带血1例次,嗅觉下降1例次,眼球活动受限1例次.本组5例中,有4例病变主体位于筛窦,1例位于鼻中隔,均累及眼眶、前颅窝底脑膜及多个鼻窦.依据国际横纹肌肉瘤研究组(Intergroup Rhabdomyosarcoma Studies,IRS)分期标准,Ⅱ期1例,Ⅲ期4例.病变与脑灰质比较,T1W1为均匀等信号2例;以等信号为主,散在小片状高信号3例.T2W1以稍高信号为主,混杂片状低信号2例;以稍高信号为主,见散在小点状、片状更高信号3例.5例ERMS增强扫描均为明显不均匀强化,内见散在片状无强化区,其中2例见线环样、葡萄样强化.4例肿瘤内有出血.全部5例均有不同程度的骨质推压改变和骨质破坏,骨质破坏区均见残存骨质.结论 鼻窦ERMS的MRI表现具有一定的特征性,MRI能准确显示肿瘤的侵犯范围,对肿瘤的定性诊断、临床分期和治疗效果的评价均有一定的指导意义.     

Embryonal rhabdomyosarcoma of the nasopharynx in a 3-year old girl

A case of rhabdomyosarcoma embryonal of the nasopharynx in a 3-year-old girl has been described. Clinical manifestations and initial diagnostic difficulties resulting in delay of proper diagnosis were discussed. Division of RMS, etiology, differentiation of the most often applied clinical sign and modern therapeutic opportunities depending on the location, histopathological type, the disease advancement level and the patient's age were described. Late outcome and the initially positive results of the employed treatment were emphasized in the presented case. The authors would like to underline the necessity to apply all imaging methods available (CT scan and MRI), in order to determine the exact site and extensiveness of malignancy which is crucial for evaluation of appropriate treatment and therapy.     

Primary embryonal rhabdomyosarcoma of the anterior neck and thyroid

Primary embryonal rhabdomyosarcoma of anterior neck involving the thyroid is extremely rare. This report is only the second of its kind that describes this form of nonorbital nonparameningeal rhabdomyosarcoma in a 7‐year‐old boy and adds to a new, seldom‐reported variant of rhabdomyosarcoma in the head‐neck region. The child presented with a huge anterior neck swelling that clinically resembled a thyroid mass. Computed tomography scan showed a heterogeneous mass in the anterior neck replacing the entire right lobe of thyroid. Fine‐needle aspiration cytology was nondiagnostic. Right hemithyroidectomy with selective neck dissection suggested embryonal rhabdomyosarcoma by histopathology; the diagnosis was confirmed by positive reactions to desmin and myogenin. The child was subsequently treated with chemotherapy. Repeat chemotherapy with radiotherapy was required when recurrences were detected in the mediastinum and cervical lymph nodes at 13‐month follow‐up. Laryngoscope, 123:2072–2076, 2013     

鼻内镜下鼻窦胚胎型横纹肌肉瘤手术1例并文献回顾

<正>横纹肌肉瘤(rhabdomyosarcoma,RMS)是具有高度恶性的肿瘤,占肉瘤的15%~20%,多发生于下肢肌肉,发生于鼻腔、鼻窦的罕见[1]。2012年10月我科收治成人鼻窦胚胎型横纹肌肉瘤1例,现报告如下。1病例资料患者,男,61岁,因右视力模糊、畏光、流泪伴鼻塞、头痛1个月,加重伴复视1周入院。患者入院前1个月无明显诱因出现右眼视力模糊、畏光、流泪,