granular cell tumors

颗粒细胞瘤
  • 文章类型: Case Reports
    颗粒细胞瘤(GCTs)是组织病理学病因不确定的肿瘤,因此没有普遍接受的治疗策略。GCTs的特征在于丰富的嗜酸性颗粒。由于它们主要位于皮肤和皮下组织中,胃GCT非常罕见。本研究记录了一名52岁男子在普陀区人民医院胃肠病门诊就诊的病例(舟山,中国)由于上腹部丰满。内窥镜超声检查显示胃体粘膜下层有一个清晰的低回声结节。使用内镜粘膜下剥离术将病变完全切除,患者术后完全康复。免疫组织化学显示S100和CD68阳性,肿瘤细胞周围有CD34表达。在直到2024年5月的电话随访中,患者的饱腹感和不适感得到缓解。CD34表达模式的特征可能为胃GCT的病理诊断提供新的依据。对于小于2cm的胃GCTs,内镜切除是一种可行的选择。
    Granular cell tumors (GCTs) are neoplasms of uncertain histopathological etiology and therefore there are no universally accepted treatment strategies. GCTs are characterized by abundant eosinophilic granules. Since they are predominantly located in the skin and subcutaneous tissues, gastric GCTs are exceedingly rare. The present study documents the case of a 52-year-old man who visited the Gastroenterology Clinic of the People\'s Hospital of Putuo District (Zhoushan, China) due to upper abdominal fullness. Endoscopic ultrasonography revealed a well-defined hypoechoic nodule in the submucosal layer of the stomach body. The lesion was completely excised using endoscopic submucosal dissection and the patient made a full postoperative recovery. Immunohistochemistry showed positivity for S100 and CD68, with CD34 expression surrounding the tumor cells. At telephone follow-up until May 2024, the patient\'s fullness and discomfort were noted to be relieved. The characteristics of the CD34 expression pattern may serve as a novel basis for the pathological diagnosis of gastric GCTs. Endoscopic resection is a feasible option for gastric GCTs smaller than 2 cm.
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  • 文章类型: Case Reports
    颗粒细胞瘤是一种罕见的来源于雪旺氏细胞的软组织肿瘤,以其浸润性为特征,在组织学上具有细嗜酸性粒细胞的多边形细胞的非包裹巢和薄片。赫林,我们报告了一个10岁的沙特女性,她出现了多个无症状的皮肤病变,最大的位于右肩和左脚。术前检查显示没有肝转移,患者成功接受了完整的手术切除。组织病理学显示,大的无细胞增殖不清,具有明显的嗜酸性粒细胞性颗粒细胞质和轻度上皮增生,与颗粒细胞肿瘤一致。颗粒细胞瘤是一种罕见的实体,仅占所有软组织肿瘤的0.5%。它们具有特征性的组织学特征,并且可以同时表现出恶性和特征。由于这种疾病的罕见,需要进一步研究,以增进我们的理解并在未来的实践中提高认识。
    Granular cell tumors are rare soft tissue neoplasms derived from Schwann cells and are characterized by their infiltrative, non-encapsulated nests and sheets of polygonal cells with fine eosinophilic cytoplasmic granules on histology. Herin, we report a case of a 10-year-old Saudi female who presented to the hospital with multiple asymptomatic skin lesions, the largest located on the right shoulder and left foot. Preoperative workup revealed the absence of liver metastasis, and the patient underwent complete surgical excision successfully. Histopathology revealed ill-defined proliferation of large bland cells with prominent eosinophilic granular cytoplasm and mild epithelial hyperplasia consistent with granular cell tumors. Granular cell tumors are a rare entity that represent only 0.5% of all soft tissue tumors. They have characteristic histological features and can present with both malignant and being features. Due to the rarity of this disease, further research is needed to enhance our understanding and improve recognition in future practice.
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  • 文章类型: Case Reports
    颗粒细胞肿瘤(GCT)是罕见的神经起源的软组织肿瘤。它们已经在多个解剖部位被报道。然而,全世界仅报告了14例由腹壁引起的病例.虽然它们可以通过多种方式在临床上表现出来,他们经常作为一个小的,具有良性特征的缓慢生长结节。他们可以,然而,是恶性的,在极少数情况下,据报道它们会转移。这里,我们介绍了一例罕见的腹壁GCT,这是通过局部切除来管理的。本文的目的是报告患者的临床病史,介绍,和手术管理,以及回顾当前的文献,以强调这种罕见实体的存在以及可能发生这种情况的可能性,并应将其视为临床实践中的鉴别诊断。
    Granular cell tumours (GCTs) are rare soft tissue tumours of neural origin. They have been reported in multiple anatomical sites. However, only 14 cases worldwide have been reported arising from the abdominal wall. While they can clinically manifest in a variety of ways, often they present as a small, slow-growing nodule with benign features. They can, however, be malignant, and in rare cases, they have been reported to metastasise. Here, we present a case of a rare abdominal wall GCT, which was managed with local excision. The purpose of this paper is to report the patient\'s clinical history, presentation, and surgical management, as well as to review the current literature to highlight the existence of this rare entity and the possibility that this may occur and should be considered a differential diagnosis in clinical practice.
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  • 文章类型: Case Reports
    颗粒细胞瘤是由雪旺氏细胞发展而来的罕见肿瘤,是周围神经系统神经元周围的神经胶质细胞,在髓鞘形成过程中起作用。在人类中,颗粒细胞肿瘤很少与中枢神经系统相关。在这份报告中,我们分析了一名颗粒细胞瘤患者,并回顾了目前的文献。
    Granular cell tumors are rare tumors that develop from Schwann cells, which are glial cells surrounding neurons of the peripheral nervous system, which serve in the process of myelination. Granular cell tumors are rarely associated with the central nervous system in humans. In this report, we analyze a patient with granular cell tumor and review the current literature.
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  • 文章类型: Journal Article
    颗粒细胞瘤(GCT)占所有软组织肉瘤(STS)的0.5%,当转移时,他们表现出攻击性行为并决定有限的生存。转移性GCT相对耐药;然而,越来越多的证据表明,在本组织学研究中使用帕唑帕尼和其他靶向治疗有益处.本文就帕唑帕尼及其他靶向治疗在GCTs治疗中的作用作一综述,以及GCT中描述的病理学和分子生物学的一些见解。在我们的搜索中找到的256篇文章中,10篇病例报告文章符合纳入标准。帕唑帕尼是最常用的全身疗法。帕唑帕尼治疗的中位报告时间为7个月。十分之八的患者(80%)经历了帕唑帕尼的疾病控制,而十分之四(40%)的患者实现了客观的RECIST反应。分子研究表明,帕唑帕尼在GCT中的抗肿瘤作用可能是由于ATP6AP1/2基因的功能丧失,从而通过几种分子途径增强信号传导,如SFKs,STAT5a/b,和PDGFR-β。其他报道的恶性GCTs靶向治疗包括帕唑帕尼联合克唑替尼,显示一名患者的疾病控制了四个月,和PI3K抑制剂,在另一名患者中实现了9个月的疾病控制。达沙替尼和甲地孕酮在另外两个不同的患者中无效。帕唑帕尼已被证明在晚期GCTs中具有活性,可被视为优选的治疗选择。
    Granular cell tumors (GCT) represent 0.5% of all soft tissue sarcomas (STS), and when metastatic, they exhibit aggressive behavior and determine limited survival. Metastatic GCTs are relatively chemo-resistant; however, there is growing evidence of the benefit of using pazopanib and other targeted therapies in this histology. This is a review of the role of pazopanib and other targeted therapies in the treatment of GCTs, along with some insights on pathology and molecular biology described in GCTs. From 256 articles found in our search, 10 case-report articles met the inclusion criteria. Pazopanib was the most employed systemic therapy. The median reported time on therapy with pazopanib was seven months. Eight out of ten patients (80%) experienced disease control with pazopanib, while four out of ten (40%) patients achieved an objective RECIST response. Molecular studies suggested that antitumoral effects of pazopanib in GCT might be due to a loss-of-function of ATP6AP1/2 genes which consequently enhance signaling through several molecular pathways, such as SFKs, STAT5a/b, and PDGFR-β. Other reported targeted therapies for malignant GCTs included pazopanib in combination with crizotinib, which showed disease control for four months in one patient, and a PI3K inhibitor which achieved disease control for nine months in another patient. Dasatinib and megestrol were ineffective in two other different patients. Pazopanib has been demonstrated to be active in advanced GCTs and may be considered as a preferable treatment option.
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  • 文章类型: Case Reports
    我们描述了一名20岁女性患者中罕见的经典霍奇金淋巴瘤(HL)病例。经过数月的胸部创伤治疗后,她出现了胸壁肿胀。肿胀最初被视为感染的血肿,病人被转诊接受手术疏散。在手术过程中,发现肿胀是一个延伸到纵隔的肿块。做了活检,后来诊断为颗粒细胞瘤(GCT)。在与多学科外科团队讨论后,决定通过电视辅助胸腔镜手术(VATS)进行手术切除。心胸外科,肿瘤学,病理学,和放射学。在手术过程中,进行了冰冻切片活检,没有淋巴瘤或颗粒细胞瘤的特征。手术后进行中线胸骨切开术,以控制意外受伤的主要血管的出血。出血得到控制,对肿块进行解剖并送去组织病理学检查。组织病理学显示结节状经典HL特征,患者被转诊接受化疗。在我们的案例中,患者最初被诊断为GCT,但是完全切除和充分的活检,该肿块被发现是经典的HL。可能的交叉细胞性受到质疑,这两个发现之间可能的相关性促使我们报告这个病例.
    We describe a rare case of classical Hodgkin lymphoma (HL) in a 20-year-old female patient. She presented to our hospital with chest wall swelling after months of post-chest trauma management. The swelling was initially treated as an infected hematoma, and the patient was referred for surgical evacuation. During the surgery, the swelling was found to be a mass that extended to the mediastinum. A biopsy was taken, which later resulted in the diagnosis of a granular cell tumor (GCT). A decision on surgical resection by video-assisted thoracoscopic surgery (VATS) was taken after discussion with the multidisciplinary team of surgery, cardiothoracic surgery, oncology, pathology, and radiology. During the surgery, a frozen section biopsy was taken, which showed no features of lymphoma or granular cell tumors. The surgery was followed by a midline sternotomy to control the bleeding from an accidentally injured major vessel. The bleeding was controlled, and the mass was dissected and sent for histopathological examination. The histopathology showed nodular classical HL features, and the patient was referred for chemotherapy. In our case, the patient was initially diagnosed with GCT, but with complete resection and an adequate biopsy, the mass was found to be a classical HL. Possible cross-cellularity is questioned, and the possible correlation between the two findings encouraged us to report this case.
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  • 文章类型: Journal Article
    未经评估:大多数报告垂体后叶肿瘤(PPTs)的研究都是小病例系列或单个病例。
    UNASSIGNED:确定了2010年1月至2021年12月在三级中心进行PPT组织学诊断的患者。我们报告了临床症状,内分泌评估,放射学和病理学特征,和PPTs的手术结果。
    未经批准:共有51名患者(23名男性,本研究包括51.3±10.3岁)的PPT。主要症状是视觉缺陷,头痛,和垂体功能减退,而尿崩症并不常见(9.8%)。典型的放射学特征是在T1对比成像上均匀增强(84.3%)规则形状的肿块,而没有囊性改变。钙化,或海绵窦侵入。我们在38/51例患者中实现了总切除(74.5%)。病理上,所有肿瘤均显示甲状腺转录因子1免疫反应性。在29例鞍上PPTs患者中,经颅组有2/15例,内镜组有0/14例。梭形细胞嗜酸细胞瘤(SCO)患者更有可能接受手术治疗(25%vs0%,P=0.018),具有较高的Ki-67指数(16.7%vs0%>5%P=0.050),与垂体细胞瘤或颗粒细胞瘤患者相比,2年无复发生存率较低(67.5%vs90.9%)。
    UNASSIGNED:对于鞍区和鞍上肿块具有均匀增强的规则病变的患者,应考虑PPTs的鉴别诊断。SCOs具有较高的增殖活性和复发风险。
    UNASSIGNED: Most studies reporting posterior pituitary tumors (PPTs) are small case series or single cases.
    UNASSIGNED: Patients with a histological diagnosis of PPT from January 2010 to December 2021 in a tertiary center were identified. We reported clinical symptoms, endocrine assessments, radiological and pathological features, and surgical outcomes of PPTs.
    UNASSIGNED: A total of 51 patients (23 males, 51.3 ± 10.3 years old) with PPT were included in this study. Major symptoms were visual defects, headache, and hypopituitarism, while diabetes insipidus was uncommon (9.8%). The typical radiological feature was homogeneous enhancement (84.3%) of a regular-shaped mass on T1 contrast imaging without cystic change, calcification, or cavernous sinus invasion. We achieved gross total resection in 38/51 patients (74.5%). Pathologically, all tumors showed thyroid transcription factor 1 immunoreactivity. Among 29 patients with suprasellar PPTs, postoperative hemorrhage due to tumor residue was encountered in 2/15 cases in the transcranial group and 0/14 in the endoscopy group. Patients with spindle cell oncocytoma (SCO) were more likely to be surgically treated (25% vs 0%, P = 0.018), harbor a higher Ki-67 index (16.7% vs 0% > 5% P = 0.050), and present a lower 2-year recurrence-free survival rate (67.5% vs 90.9%) compared with patients with pituicytoma or granular cell tumor.
    UNASSIGNED: PPTs should be considered in the differential diagnosis of patients with sellar and suprasellar masses with a regular lesion with homogeneous enhancement. SCOs had high proliferation activity and risk of recurrence.
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  • 文章类型: Case Reports
    颗粒细胞瘤是具有多个定位的罕见肿瘤。在45-65%的病例中,最常见的定位在颈面部区域。该肿瘤的特征取决于其临床和组织病理学发现。已经报道了一些描述更不寻常定位的案例,例如在腋窝或胃肠道中。在这篇文章中,我们描述了一个原始的定位:颞下颌关节的区域。此病例报告描述了圣约瑟夫医院颌面和口腔外科的临床病例。通常描述的这种解剖位置的肿瘤病变通常是骨或滑膜病变。这里,临床检查与上述任何描述都不一致,MRI也证实这种肿瘤特征与这种病变不一致。
    Granular cell tumors are rare tumors with multiple localizations. The most common localizations are in the cervicofacial region in 45-65% of cases. The characterization of this tumor depends on its clinical and histopathological findings. A few cases have been reported describing more unusual localizations, such as in the juxta-axilla or in the gastrointestinal tract. In this article, we describe an original localization: the region of the temporomandibular joint. This case-report describes a clinical case that was managed in the Department of Maxillofacial and Oral Surgery of the Saint-Joseph Hospital. The tumoral lesions usually described for this anatomical location are usually bony or synovial lesions. Here, the clinical examination didn\'t correspond to any of those descriptions and the MRI also confirmed that this tumor characteristics weren\'t compatible with this kind of lesion.
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  • 文章类型: Case Reports
    颗粒细胞肿瘤(GCT)是罕见的软组织肿瘤,其特征是肿瘤细胞的细胞质颗粒外观。恶性GCT占GCT的不到2%,主要见于下肢皮下软组织,尤其是大腿.本报告介绍了一例右侧多裂肌恶性颗粒细胞瘤。一名69岁的妇女出现在外科医生那里,腰椎和髋关节有3个月的轻度疼痛史,没有特别的历史。CT和MRI显示软组织肿瘤,最大直径为7.5cm。质量中有零散的未增强的低信号阴影。对原发肿瘤进行了广泛切除,这被解释为恶性GCT。在快速增长的肌内肿瘤的鉴别诊断中应考虑GCT。我们调查了恶性颗粒细胞瘤的CT和MRI表现。
    Granular cell tumors (GCTs) are uncommon soft tissue tumors characterized by cytoplasmic granular appearance of the neoplastic cells. Malignant GCTs comprise less than 2% of GCTs and are mostly found in the subcutaneous soft tissues of the lower extremities, especially the thighs. This report presents a case of malignant granular cell tumor in the right multifidus muscle. A 69-year-old woman presented to the surgeon with a 3 month history of light pain in the lumbar area and hip joint, with no particular history. CT and MRI revealed a soft tissue tumor with a maximum diameter of 7.5 cm. There is patchy unenhanced hypointense shadow in the mass. Widely excision was performed for the primary tumor, which was interpreted as an malignant GCTs. GCTs should be considered in the differential diagnosis in a rapidly growing intramuscular tumors. We investigated the CT and MRI findings of malignant granular cell tumor.
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  • 文章类型: Case Reports
    背景:颗粒细胞瘤(GCT)通常是良性的,主要发生在躯干和头部。阴道肿瘤很少见,特别是在儿童中。
    方法:一名9岁女孩因阴道不规则出血入院1年。出血在前1个月恶化。B超和计算机断层扫描显示阴道有圆形肿块。我们通过宫腔镜探查和开放技术切除肿瘤进行了阴道镜检查。切除肿瘤的病理证实为阴道壁GCT。在接下来的16个月中没有发现复发。
    结论:据我们所知,我们的青春期前女孩是文献中最年轻的GCT患者。组织病理学评估和具有清晰边缘的完整肿瘤切除是良性或恶性GCTs的主要治疗方法。
    BACKGROUND: Granular cell tumors (GCTs) are usually benign and occur primarily in the trunk and head. Vaginal tumors are rare, particularly in children.
    METHODS: A 9-year-old girl was admitted with irregular vaginal bleeding for 1 year. The bleeding had worsened in the previous 1 month. B-ultrasound and computed tomography showed a round mass in the vagina. We performed vaginoscopy using a hysteroscopic exploration and tumor resection via an open technique. The pathology of the resected tumor confirmed a vaginal wall GCT. No recurrence was noted in the following 16 months.
    CONCLUSIONS: To our knowledge, our prepubescent girl is the youngest patient with GCT in the literature. Histopathological evaluation and complete tumor resection with clear margins is the primary treatment for benign or malignant GCTs.
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