Eosinophilic granuloma

嗜酸性肉芽肿
  • 文章类型: Case Reports
    脊髓嗜酸性肉芽肿(EG)是罕见的肿瘤,主要报道在儿科年龄组。它们占原发性骨肿瘤的<1%,颈椎受累并不常见。
    一名20岁男性出现颈部疼痛4个月。六年前,他接受了6个周期的长春碱治疗腋窝淋巴结活检证实的组织细胞增生症;这导致了不完全缓解。目前的磁共振/计算机断层扫描(CT)成像显示,具有寰枢椎不稳定性的溶解性C2身体病变。当CT引导活检提示EG时,他接受了明确的手术和辅助放疗。
    颈椎EG在成人中很少见。CT引导下活检应明确诊断,并应进行明确的手术和辅助放疗。
    UNASSIGNED: Spinal eosinophilic granulomas (EG) are rare tumors, mostly reported in the pediatric age group. They constitute <1% of primary bone neoplasms, and cervical spine involvement is uncommon.
    UNASSIGNED: A 20-year-old male presented with neck pain for a 4-month duration. Six years previously, he had received six cycles of vinblastine for biopsy-proven histiocytosis of an axillary lymph node; this resulted in incomplete remission. Present magnetic resonance/computed tomography (CT) imaging revealed a lytic C2 body lesion with atlantoaxial instability. When the CT-guided biopsy was suggestive of EG, he was managed with definitive surgery and adjuvant radiotherapy.
    UNASSIGNED: Cervical spine EG is rare in adults. CT-guided biopsy should confirm the diagnosis and should be followed by definitive surgery and adjuvant radiotherapy.
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  • 文章类型: Case Reports
    嗜酸性肉芽肿(EG),朗格汉斯细胞组织细胞增生症(LCH)的亚型,单骨形式,是一种罕见的疾病,其特征是孤立的骨病变。这种情况伴有硬膜外血肿(EDH)更为罕见。这种情况是独特的,因为它是第一个涉及癫痫发作后延迟EDH的病例。我们描述了EG伴随EDH的显着例子,并考虑了这种共病的罕见性。一名32个月大的男孩在头部轻伤后发展出快速增长的头骨。在活检的手术准备过程中,病人经历了一次抽搐。癫痫发作后的成像显示肿块附近有EDH。切除肿块,确认为EG,但利润率为正。患者在全身骨骼评估后接受化疗,根据组织细胞增生症协会建立的LCHIII方案。EG是一种罕见的肿瘤,通常表现为颅骨上的无痛生长,随着时间的推移逐渐扩大。EG和EDH之间的相关性非常罕见,只有几个记录在案的案例。此案例研究强调了在颅骨肿块扩大的鉴别诊断中考虑EG的重要性,即使与EDH相关联。及时的诊断和治疗可以预防严重的并发症并改善患者的预后。
    Eosinophilic granuloma (EG), a subtype of Langerhans cell histiocytosis (LCH), the monostotic form, is a rare condition characterized by a solitary bone lesion. It is even more unusual for this condition to be accompanied by an epidural hematoma (EDH). This case is unique in that it is the first to involve delayed EDH following a seizure. We describe a remarkable example of EG accompanied by an EDH and consider the rarity of this comorbidity. A 32-month-old boy developed a rapidly growing skull mass following a minor head injury. During surgical preparation for a biopsy, the patient experienced a single convulsion. Imaging following the seizure revealed an EDH in the vicinity of the mass. The mass was excised and confirmed to be an EG, but with positive margins. The patient underwent chemotherapy after systemic skeletal evaluation, in accordance with the LCH III protocol established by the Histiocytosis Society. EG is a rare neoplasm that typically presents as a painless growth on the skull that gradually enlarges over time. The correlation between EG and EDH is exceedingly uncommon, with only a few documented cases. This case study underscores the significance of considering EG in the differential diagnosis of an expanding cranium mass, even when associated with EDH. Prompt diagnosis and treatment can prevent serious complications and improve patient outcomes.
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  • 文章类型: Case Reports
    背景:嗜酸性肉芽肿(EG)是一种影响骨组织细胞的增殖性疾病。在疾病的早期阶段没有特定的临床体征或影像学表现,容易忽视和误诊。因为这种疾病的稀有性,目前没有标准化的治疗原则。很少有关于此类事件影响儿童轴心的报道。我们发现一例儿童的EG导致寰枢关节脱位和轴向骨破坏。
    方法:在没有明确解释的情况下出现咽部不适超过六个月后,一个6岁的男孩被送到我们医院。经过仔细评估,病理提示轴性EG的可能性很大。最终,我们决定用后路椎弓根螺钉固定和局部类固醇注射治疗这个男孩。
    结论:上颈椎的EGs在儿童中并不常见,它们非常容易被忽视或误诊。后路椎弓根螺钉固定和局部类固醇注射是影响寰枢关节的轴向EGs患者的有效治疗方法。
    BACKGROUND: Eosinophilic granuloma (EG) is a proliferative condition that affects the cells of bone tissue. There are no specific clinical signs or imaging manifestations in the early stages of the disease, making it simple to overlook and misdiagnose. Because of the disease\'s rarity, there is presently no standardized treatment principle. There are few accounts of such occurrences affecting the axis among children. We discovered a case of a child whose EG resulted in atlantoaxial joint dislocation and destruction of the axial bone.
    METHODS: After having pharyngeal discomfort for more than six months without a clear explanation, a 6-year-old boy was brought to our hospital. Following a careful evaluation, the pathology indicated a strong likelihood of an axial EG. Ultimately, we decided to treat the boy with posterior pedicle screw fixation and local steroid injections.
    CONCLUSIONS: EGs of the upper cervical spine are quite uncommon in children, and they are exceedingly easy to overlook or misdiagnose. Posterior pedicle screw fixation and local steroid injections are effective treatments for patients with axial EGs affecting the atlantoaxial junction.
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  • 文章类型: Case Reports
    背景:嗜酸性肉芽肿(EG)-朗格汉斯细胞组织细胞增生症的最常见形式-很少发生,仅肋骨和锁骨受累的表现极为罕见。EG症状通常包括疼痛,肿胀,和软组织肿块。骨EG的临床诊断复杂,鉴别诊断包括尤因肉瘤,结核病,多发性骨髓瘤,淋巴瘤原发性骨恶性肿瘤,和其他溶骨性病变。
    方法:患者是一名11岁女性,在就诊前2天发现右锁骨和胸骨交界处皮下肿块,无明显诱因。最初,我们考虑皮下囊肿或炎性肿块。彩色超声和计算机断层扫描检查显示骨髓炎。最后,患者在病理组织活检后被诊断为EG,经手术和抗感染治疗后,患儿康复。
    方法:患者在专科医院行手术切除肿瘤,经病理检查确诊为EG。
    方法:患者到专科医院手术切除肿块,并接受抗感染治疗。
    结果:患者在手术切除和抗生素治疗后康复。
    结论:在本报告中,我们强调EG在儿童中的临床表现并不具有特异性.此外,检查年龄,历史,存在的症状,这些部位的数量对于做出正确的诊断至关重要,组织学检查是必要的,以确认诊断。
    BACKGROUND: Eosinophilic granuloma (EG) - the most common form of Langerhans cell histiocytosis - occurs rarely, and manifestations with only rib and clavicle involvement are extremely rare. EG symptoms often include pain, swelling, and soft tissue mass. The clinical diagnosis of bone EG is complex, and the differential diagnosis includes Ewing sarcoma, tuberculosis, multiple myeloma, lymphoma, primary bone malignancy, and other osteolytic lesions.
    METHODS: The patient was an 11-year-old female who found a subcutaneous mass at the junction of the right clavicle and sternum 2 days before presenting at the clinic without apparent triggers. Initially, we considered a subcutaneous cyst or inflammatory mass. Color ultrasound and computed tomography examination revealed osteomyelitis. Finally, the patient was diagnosed with EG after a pathological tissue biopsy, and the child recovered after surgery and anti-infective treatment.
    METHODS: The patient underwent surgery to remove the tumor at a specialist hospital and was diagnosed with EG by pathological examination.
    METHODS: The patient went to a specialist hospital for surgery to remove the mass and underwent anti-infective treatment.
    RESULTS: The patient recovered after surgical resection and antibiotic treatment.
    CONCLUSIONS: In this report, we emphasize that the clinical presentation of EG in children is not specific. Furthermore, examining age, history, presence of symptoms, and the number of sites is essential to make a correct diagnosis, and a histological examination is necessary to confirm the diagnosis.
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  • 文章类型: Journal Article
    背景嗜酸性肉芽肿(EG)属于朗格汉斯细胞组织细胞增生症(LCH)家族,被认为是一种良性疾病,通常见于15岁以下儿童。这里,作者描述了EG的异常定位和临床表现。
    方法:作者报告了一个9岁的女孩,其EG表现为斜坡的溶骨性病变。经蝶窦切除和组织学确认后,开始辅助化疗。表现出的体征和症状是体重减轻,偶发的鬼脸,和温和的类似弹道的运动。经过32个月的随访,患者的初始症状完全缓解,肿瘤无进一步生长.
    结论:尽管这些病变很少见,当遇到斜坡的溶骨性病变时,应将EG视为鉴别诊断。
    BACKGROUND: Eosinophilic granuloma (EG) belongs to the family of Langerhans cell histiocytosis (LCH) and is considered to be a benign disease typically found in children younger than 15 years of age. Here, the authors describe an EG of unusual localization and clinical presentation.
    METHODS: The authors report a 9-year-old girl with an EG presenting as an osteolytic lesion of the clivus. After transsphenoidal resection and histological confirmation, adjuvant chemotherapy was initiated. Presenting signs and symptoms were weight loss, episodic grimacing, and moderate ballism-like movements. After a follow-up-period of 32 months, the patient presented with a total resolution of initial symptoms and no further tumor growth.
    CONCLUSIONS: Although these lesions are rare, one should consider EG as a differential diagnosis when confronted with osteolytic lesions of the clivus.
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  • 文章类型: Case Reports
    朗格汉斯细胞组织细胞增生症(LCH),早期被认为是组织细胞增生症X,是一种罕见的血液病,涉及婴幼儿。LCH是由通常的抗原呈递细胞的无限制刺激和增殖引起的,朗格汉斯细胞(LC)和疾病表现出涉及多个部位的广泛的临床和放射学特征。由于发病率相对较低,有关LCH流行病学的数据有限,每年每百万人口大约有2-5例。LCH有男性倾向,10-20%的病例涉及颌骨,只有1%的病例影响上颌骨,伪装成牙周或根尖周病理学。我们报告了一例48岁女性LCH累及后上颌骨。这是一个与年龄相对应的独特表现,性别,位置和严重性。牙科临床医生应该意识到这一点,并将其视为与未解决的牙周病理学有关的鉴别诊断的一部分,因为它可以在临床和影像学上进行模仿。
    Langerhans cell histiocytosis (LCH), earlier recognised as histiocytosis X, is a rare haematological illness involving infants and young children. LCH is caused by unrestrained stimulation and proliferation of usual antigen presenting cells, Langerhans cells (LCs) and the disease demonstrates extensive clinical and radiographic features involving multiple sites. Since the incidence is relatively low limited data is available regarding the epidemiology of LCH, with approximation of 2-5 cases per million populations per year. LCH has male predilection with jaws involved in 10-20% cases and only 1% of the cases affecting maxilla, masquerading as periodontal or periapical pathology. We report a case of 48-year-old female with LCH involving posterior maxilla. This is a unique presentation corresponding to age, gender, location and severity. Dental clinicians should be aware of this and consider it to be a part of their differential diagnosis pertaining to unresolved periodontal pathology as it mimics clinically and radiographically.
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  • 文章类型: Systematic Review
    背景:嗜酸性肉芽肿(EG)是朗格汉斯细胞组织细胞增生症的最常见形式,表现为颅骨的单个溶骨性病变。其诊断基于典型的临床和放射学特征。虽然手术切除一直是EG的标准治疗方法,越来越多的证据支持警惕等待,因为单局部颅骨病变似乎经常经历自发缓解。然而,对这一假设的组织病理学确认仍然非常有限。
    方法:方法。这里,我们报告了一例具有典型临床和放射学特征的EG,由于干预情况,以延迟的方式切除。此外,我们对EG保守管理的文献进行了系统回顾。
    结果:在我们的案例中,组织学检查显示正在进行的骨再生,没有疾病的痕迹。通过我们的文献综述,我们发现47例颅骨EG在观察等待下进行了治疗。43例(91%)不需要积极干预。由于症状的持续/进展或家庭要求,四名患者(9%)接受了手术或化疗。除我们以外的其他三份报告均记录了组织病理学检查后手术切除的EG的自发性疾病缓解。
    结论:我们的报告提供了进一步的证据,表明观察等待可能是治疗单头盖骨EG的合理选择。
    Eosinophilic granuloma (EG) is the most common form of Langerhans cell histiocytosis, presenting as a single osteolytic lesion of the calvarium. Its diagnosis is based on typical clinical and radiological features. While surgical resection has been the standard treatment for EG, growing evidence favors watchful waiting, as unifocal calvarial lesions appear to frequently undergo spontaneous remission. However, histopathological confirmations of this hypothesis are still very limited.
    Methods. Here, we report a case of EG with typical clinical and radiological features which, due to intervening circumstances, was resected in a delayed fashion. Moreover, we perform a systematic review of the literature on conservative management of EG.
    In our case, histological examination showed ongoing bone regeneration with no traces of the disease. Through our literature review, we found 47 cases of calvarial EG managed with watchful waiting. No active intervention was required in 43 cases (91%). Four patients (9%) received surgery or chemotherapy due to the persistence/progression of symptoms or family request. Three reports other than ours documented spontaneous disease remission in surgically resected EG upon histopathological examination.
    Our report provides further evidence that watchful waiting can be a reasonable option in the management of single calvarial EG.
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  • 文章类型: Case Reports
    朗格汉斯细胞组织细胞增生症(LCH)是一种罕见的增殖性疾病,原因不明。其临床表现各不相同,可涉及多种器官。诊断LCH,射线照片,组织病理学和免疫组织化学结果是必不可少的。明确诊断的金标准是免疫组织化学结果中CD1a/CD207和S100阳性。不同的治疗计划可用于患有LCH的患者。据我们所知,100万儿童的LCH发病率约为8.9例,100万成人的LCH发病率约为1-2例。我们的案例表明,早期诊断这种罕见疾病对于防止任何进一步传播的重要性。该病例报告是关于一名35岁的男性患者患有尿崩症,主要主诉为牙齿活动。根据他的X光片,发现溶骨性病变。做了活检,由于组织病理学和免疫组织化学结果,它被诊断为成人LCH病例。本文表明,虽然LCH的发病率在成年人中很少见,处理患有尿崩症和溶骨性骨病变的患者,原因不明,必须考虑LCH。由于LCH的最初表现大多是口头的,早期预后良好,如果进展,它可以变成致命的,牙科医生意识到这种疾病是很重要的,其临床表现,和病人管理。如果有人怀疑,X光检查,活检,组织病理学,必须进行免疫组织化学检查。
    Langerhans cell histiocytosis (LCH) is an uncommon proliferative disease with an unknown cause. Its clinical manifestations vary and can involve a variety of organs. To diagnose LCH, radiographs, histopathological and immunohistochemical findings are essential. The gold standard for a definite diagnosis is positive CD1a/CD207 and S100 in the immunohistochemical results. Different treatment plans are available for patients struggling with LCH. To our knowledge, the LCH incidence rate is about 8.9 in one million children and 1-2 cases in one million adults. Our case shows the importance of early diagnosis of this rare condition for the prevention of any further spreading. This case report is about a 35-year-old male patient struggling with diabetes insipidus with a chief complaint of tooth mobility. Based on his X-ray radiographs, osteolytic lesions were found. A biopsy was performed, and due to histopathological and immunohistochemical findings, it was diagnosed as a case of LCH in adults. This paper shows that although the incidence rate of LCH is rare in adults, dealing with a patient struggling with diabetes insipidus and osteolytic bone lesions with an unknown cause, LCH must be taken into consideration. Due to the fact that LCH\'s first manifestations are mostly first oral, its good prognosis in the early stages, and if it progresses, it can turn fatal, it is important that dentists are aware of this disease, its clinical manifestations, and patient management. In case of suspicion, X-ray examination, biopsy, histopathological, and immunohistochemical exams must be performed.
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  • 文章类型: Case Reports
    许多侵袭性非牙髓放射状病变显示出与牙髓起源的根尖周病变非常相似的临床和影像学特征。由于牙髓和非牙髓病变的治疗方法明显不同,准确的诊断势在必行。因此,本研究旨在介绍一个临床病例,该病例涉及模拟牙髓起源的根尖周病变的朗格汉斯细胞组织细胞增生症(LCH)病变的诊断和治疗。由于牙齿36区域的轻微疼痛,一名51岁的男性患者被转诊到私人牙科诊所。尽管没有发现假体或牙髓失败的迹象,影像学检查显示,射线可透图像与牙齿的根尖周区相关的边界定义不清。然后进行根尖切除术和骨刮除,鉴于临床和实验室特征,明确诊断为单发嗜酸性肉芽肿.手术治疗足以缓解症状,未观察到复发。鉴于目前的情况,牙医应该意识到LCH病变,因为它们可能模仿牙髓根尖周病,导致误诊和治疗并发症。此外,在许多情况下,牙槽骨病变可能是LCH的第一个或唯一标志。
    Many aggressive non-endodontic radiolucent lesions show very similar clinical and radiographical features to periapical lesions of endodontic origin. Since the treatments of endodontic and non-endodontic lesions differ markedly, a precise diagnosis is imperative. Thus, the present study aimed at presenting a clinical case on the diagnosis and management of a Langerhans cell histiocytosis (LCH) lesion mimicking a periapical lesion of endodontic origin. A 51-year-old male patient was referred to a private dental office due to slight pain from the region of tooth 36. Although no sign of prosthetic or endodontic failure was noted, radiographical examination revealed a radiolucent image with poorly defined borders associated with the periapical region of the tooth. Apicoectomy and bone curettage were then performed and, given the clinical and laboratory features, the definitive diagnosis of solitary eosinophilic granuloma was made. The surgical treatment was sufficient for the remission of the symptoms, and recurrence was not observed. Given the current case, dentists should be aware of LCH lesions as they may mimic endodontic periapical pathoses, leading to misdiagnosis and therapeutic complications. Moreover, alveolar bone lesions may be the first or only sign of LCH in many cases.
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  • 文章类型: Case Reports
    背景:孤立性嗜酸性肉芽肿(EG)发生在颅底区,它在以前的三个出版物中都有描述。作者报告了首例前斜突(ACP)的EG,仅限于ACP,视力下降。
    方法:一名38岁女性表现为左眼视力下降5个月。她的视力是3/10,其他神经系统检查完好无损,没有其他骨性或软组织损伤。使用左侧开颅手术和经硬膜临床切除术切除病变,对内和外视神经进行减压。病变是EG的特征,2年后未发现复发。
    结论:EG可局限于ACP并损害视力。影像学检查是敏感的,但不是特异性的,手术减压是诊断和治疗的导向。在类似情况下,可能需要密切观察甚至辅助治疗。
    BACKGROUND: Solitary eosinophilic granuloma (EG) occurs anecdotally in the skull base region, and it has been described in only three previous publications. The authors report the first case of EG of the anterior clinoid process (ACP), which was confined to the ACP and presented with decreased vision.
    METHODS: A 38-year-old woman presented with decreased vision of the left eye of 5 months\' duration. Her visual acuity was 3/10, other neurological examinations were intact, and there were no other osseous or soft tissue lesions. The lesion was excised using a left-sided craniotomy and transdural clinoidectomy, decompressing the optic nerve both intra- and extradurally. The lesion was characteristic for EG, and no recurrence was detected after 2 years.
    CONCLUSIONS: EG can be confined to the ACP and impair vision. Imaging studies are sensitive but not specific, and surgical decompression is both diagnostic and treatment oriented. Close observation and even adjuvant therapy may be indicated in similar cases.
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