Granular cell tumor

颗粒细胞肿瘤
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    文章类型: Journal Article
    Testicular tumors are rarely reported in rabbits. In this case study, a 4-year-old Holland lop rabbit, previously diagnosed with unilateral cryptorchidism, was presented because of enlargement of the descended testis. The rabbit was clinically normal. Following unilateral orchiectomy and scrotal ablation, histopathological analysis revealed 2 distinct types of testicular tumor in the descended testis: a granular cell tumor and a seminoma. To the best of the author\'s knowledge, this is the first documented report of simultaneous testicular tumors in the testis of a rabbit with unilateral cryptorchidism.
    Tumeur à cellules granulaires et séminome simultanés dans le testicule descendu d’un lapin cryptorchideLes tumeurs testiculaires sont rarement rapportées chez le lapin. Dans cette étude de cas, un lapin Holland Lop de 4 ans, précédemment diagnostiqué avec une cryptorchidie unilatérale, a été présenté en raison d’une hypertrophie du testicule descendu. Le lapin était cliniquement normal. Après orchidectomie unilatérale et ablation scrotale, l’analyse histopathologique a révélé 2 types distincts de tumeur testiculaire dans le testicule descendu : une tumeur à cellules granuleuses et un séminome. À la connaissance de l’auteur, il s’agit du premier rapport documenté de tumeurs testiculaires simultanées dans le testicule d’un lapin atteint de cryptorchidie unilatérale.(Traduit par Dr Serge Messier).
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  • 文章类型: Journal Article
    食管细胞瘤是罕见的。对一名48岁女性进行的食管胃十二指肠镜检查显示食管病变升高,存在长段Barrett食管。内窥镜超声检查显示15毫米均匀的低回声肿瘤从固有层粘膜延伸到粘膜下层。活检组织的病理检查显示,组织细胞样细胞的片状簇具有丰富的嗜酸性粒细胞性细胞质。免疫组织化学检查显示S-100(+)和CD68(+),因此提示诊断为颗粒细胞瘤。采用内镜黏膜下剥离术切除肿瘤。病理上,背景粘膜是Barrett粘膜。这是首例报道的长段Barrett食管中的食管颗粒细胞瘤。
    Esophageal cell tumors are rare. Esophagogastroduodenoscopy performed on a 48-year-old woman revealed an elevated esophageal lesion and the presence of long-segment Barrett\'s esophagus. Endoscopic ultrasonography showed a 15 mm homogeneous hypoechoic tumor extending from the lamina propria mucosa to the submucosa. Pathological examination of the biopsy tissue revealed a sheet-like cluster of histiocytoid cells with an abundant eosinophilic granular cytoplasm. Immunohistochemical examination revealed S-100 (+) and CD68 (+), thus suggesting the diagnosis of a granular cell tumor. The tumor was resected by endoscopic submucosal dissection. Pathologically, the background mucosa was Barrett\'s mucosa. This is the first reported case of an esophageal granular cell tumor in long-segment Barrett\'s esophagus.
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  • 文章类型: Journal Article
    背景:颗粒细胞瘤(GCT)是一种罕见的口腔良性病变,其发病机制尚不清楚。由于其罕见发生且与其他口腔病变相似,在最初的临床诊断中,它们经常被遗忘。因此,了解其普遍性,临床和病理特征对于准确诊断和适当管理至关重要。
    方法:对6个巴西和阿根廷口腔诊断中心诊断为GCT的所有病例进行HE染色重新评估,临床,人口统计学,收集和评估组织病理学数据。
    结果:该系列包括45名女性(73.8%)和16名男性(26.2%),男女比例为2.8:1,平均年龄为35.3±16.9岁(范围:7-77岁)。大多数病例发生在舌头上(n=49;81.6%),临床上表现为无症状的丘疹或结节(n=50;89.3%),具有正常变色(n=25;45.5%)或淡黄色(n=11;20.0%)着色,大小范围为0.2至3.0cm(平均±SD:1.40±0.75cm)。形态学上,大多数肿瘤的特征是有限的增殖(n=52;88.1%)细胞通常圆形到多边形,嗜酸性粒细胞,细颗粒状细胞质。在所有病变中均发现了Milian的卵形体(n=61;100%)。骨骼肌横纹肌(n=44;72.1%)和神经纤维(n=42;68.9%)的捕获是常见的发现。23例(39.0%)出现假性上皮瘤增生(PEH)。在仅27.7%的病例(n=13)中,临床和组织病理学诊断之间存在一致性。结果信息来自16例患者(26.2%),临床随访4-36个月(平均13.3个月),没有发生局部复发。
    结论:GCT的临床和组织病理学特征与先前研究中描述的一致。总的来说,这些病变对成年女性的舌头外侧区域有好感。在口腔黄色或正常色斑丘疹和结节的鉴别诊断中,必须考虑GCT。组织病理学评估对于明确诊断至关重要,预后良好。
    BACKGROUND: Granular Cell Tumor (GCT) is an uncommon benign lesion in the oral cavity whose pathogenesis remains poorly understood. Due to their infrequent occurrence and similarity to other oral lesions, they are often forgotten during the initial clinical diagnosis. Therefore, understanding its prevalence, clinical and pathological characteristics is crucial for an accurate diagnosis and adequate management.
    METHODS: All cases diagnosed as GCTs in six Brazilian and Argentinian oral diagnostic centers were re-evaluated by HE staining, and clinical, demographic, and histopathological data were collected and evaluated.
    RESULTS: The series comprised 45 female (73.8%) and 16 male (26.2%), with a 2.8:1 female-to-male ratio and a mean age of 35.3 ± 16.9 years (range: 7-77 years). Most cases occurred on the tongue (n = 49; 81.6%) and presented clinically as asymptomatic papules or nodules (n = 50; 89.3%) with a normochromic (n = 25; 45.5%) or yellowish (n = 11; 20.0%) coloration and sizes ranging from 0.2 to 3.0 cm (mean ± SD: 1.40 ± 0.75 cm). Morphologically, most tumors were characterized by a poorly delimited proliferation (n = 52; 88.1%) of cells typically rounded to polygonal containing abundant, eosinophilic, finely granular cytoplasm. Pustulo-ovoid bodies of Milian were identified in all lesions (n = 61; 100%). Entrapment of skeletal striated muscle (n = 44; 72.1%) and nerve fibers (n = 42; 68.9%) were common findings. Pseudoepitheliomatous hyperplasia (PEH) was observed in 23 cases (39.0%). In only 27.7% of cases (n = 13) there was agreement between the clinical and histopathological diagnosis. Outcome information was available from 16 patients (26.2%), with clinical follow-up ranging from 4 to 36 months (mean 13.3 months), and none developed local recurrence.
    CONCLUSIONS: The clinical and histopathological features of GCTs were consistent with those described in previous studies. In general, these lesions have a predilection for the lateral region of the tongue in adult women. It is essential to consider GCTs in the differential diagnosis of yellow or normochromic papules and nodules in the oral cavity. Histopathological evaluation is essential for the definitive diagnosis and the prognosis is excellent.
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  • 文章类型: Case Reports
    皮肤颗粒细胞瘤(GCT)是罕见的肿瘤,通常表现出良性的临床行为,并且可能是雪旺氏细胞起源。由于浸润性生长模式,GCTs的一些组织学和免疫组织化学变异可能会带来挑战。神经周浸润,和Melan-A的表达在这个案例报告中,我们介绍了一名27岁的男性,他在几年前曾被诊断为背部典型的GCT。来自近端手掌的当前活检显示细胞学上相似的肿瘤,具有广泛的神经周围扩散和Melan-A的显着阳性。虽然不常见,这些特征与GCT的组织学表现一致.目前对GCT的组织发生的看法,临床关联,与黑色素瘤的鉴别诊断,并讨论了恶性GCTs的组织学标准。一组免疫组织化学染色,包括抑制素-α和黑色素瘤中优先表达的抗原(PRAME),建议用于罕见的Melan-A阳性GCTs。
    Cutaneous granular cell tumors (GCTs) are rare tumors that typically exhibit benign clinical behavior and are likely of Schwann cell origin. Some histologic and immunohistochemical variants of GCTs may present challenges due to infiltrative growth patterns, perineural invasion, and expression of Melan-A. In this case report, we present a 27-year-old male who had previously been diagnosed with a typical GCT on the back a few years ago. The current biopsy from the proximal palm demonstrated a cytologically similar tumor with extensive perineural spread and notable positivity for Melan-A. Although uncommon, these features are consistent with the histological appearances of GCTs. The current views on the histogenesis of GCTs, clinical associations, differential diagnosis with melanoma, and histological criteria for malignant GCTs are discussed. A panel of immunohistochemical stains, including Inhibin-α and preferentially expressed antigen in melanoma (PRAME), is proposed for use in rare instances of Melan-A-positive GCTs.
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  • 文章类型: Case Reports
    Here, we describe the first case of a granular cell tumor (GCT) derived from the brachial nerve. Eleven-year-old neutered female Chihuahua presented to the hospital with a bulge from the left neck to the axilla. The dog had a spherical subcutaneous mass on the cervical subcutis, and cytology hinted at adenocarcinoma or neuroendocrine tumor. However, the origin of the tumor remains unknown. During resection of the mass, bleeding was difficult to control owing to the high blood flow, and tumor removal was extremely difficult. The caudal aspect of the mass was attached to the brachial nerve and had to be removed, along with parts of the nerve fibers. The patient\'s postoperative course was fair, but it developed paralysis of the left thoracic limb. Pathology revealed that the mass was positive for S100 and vimentin, and GCT was diagnosed. Non-oral GCTs are extremely rare. The clinical diagnosis of GCT is difficult and is often confirmed histopathologically by excision. Although most cases of GCT are benign, they must be recognized as hemorrhagic, indistinct masses that mimic malignancy. Excision carries the risk of hemorrhage and damage to the surrounding tissues to secure margins.
    Descrevemos aqui o primeiro caso de um tumor de células granulares (TCG) derivado do nervo braquial. Uma chihuahua castrada de 11 anos de idade deu entrada no hospital com uma protuberância do pescoço esquerdo até a axila. A cadela apresentava uma massa subcutânea esférica no subcutâneo cervical, e a citologia indicava adenocarcinoma ou tumor neuroendócrino. Entretanto, a origem do tumor permanece desconhecida. Durante a ressecção da massa, foi difícil controlar o sangramento devido ao alto fluxo sanguíneo, e a remoção do tumor foi difícil. O aspecto caudal da massa estava ligado ao nervo braquial e teve de ser removido, juntamente com partes das fibras nervosas. A evolução pós-operatória da paciente foi regular, mas ele desenvolveu paralisia do membro torácico esquerdo. O exame anatomopatológico revelou que a massa era positiva para S100 e vimentina, e o TCG foi diagnosticado. Os TCGs não orais são extremamente raros. O diagnóstico clínico do TCG é difícil e geralmente é confirmado histopatologicamente por excisão. Embora a maioria dos casos de TCG seja benigna, eles devem ser reconhecidos como massas hemorrágicas e indistintas que simulam malignidade. A excisão acarreta o risco de hemorragia e danos aos tecidos circundantes para garantir as margens.
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  • 文章类型: Journal Article
    背景:梭形细胞嗜酸细胞瘤(SCO)和颗粒细胞瘤(GCT)是罕见的原发性垂体肿瘤;这些病变的最佳治疗模式尚不清楚,大部分尚未探索。因此,利用国家登记册,我们分析了流行病学,管理模式,以及SCOs和GCTs的手术结果。
    方法:国家癌症数据库(NCDB;2003-2017年)和监测,流行病学,和最终结果计划(SEER;2004-2018年)被查询为垂体SCOs或GCTs患者.发病率,手术切除的范围,亚完全切除病变的术后放疗使用率构成了主要结局.还通过事件发生时间Kaplan-Meier曲线分析了全因死亡率。
    结果:SCO和GCT的年发病率分别为0.017和0.023/1,000,000。它们占NCDB登记的良性垂体瘤的0.1%。在研究期间,NCDB中共发现112,241例良性垂体肿瘤,其中SCOs83例(0.07%),GCTs59例(0.05%)。诊断时的中位年龄为55岁,44%是女性,出现时的中位最大肿瘤直径为2.1cm。54%的患者实现了总切除。10例患者(7%)进行了术后放疗。比较GCT和SCO患者,前者在诊断时更可能更年轻(48.0vs.分别为59.0;p<0.01)和女性(59%vs.34%,p=0.01)。GCT和SCO在诊断时的大小方面没有差异(中位最大直径:1.9cm与2.2cm,分别为;p=0.59)或总切除率(62%与49%,p=0.32)。在将SCO和GCT与垂体腺瘤进行年龄匹配后,性别,和肿瘤大小,前者不太可能接受总切除(53%vs.72%;p=0.03)。与垂体腺瘤患者相比,SCO和GCT患者的总生存期较短(p<0.01),30天死亡率较高(3.1%vs0.0%;p=0.013)。
    结论:SCO和GCT是罕见的垂体瘤,他们的管理带来了特殊的挑战。总切除通常是不可能的,和辅助放疗可以在次全切除后使用。
    BACKGROUND: Spindle cell oncocytomas (SCO) and granular cell tumors (GCT) are rare primary pituitary neoplasms; the optimal treatment paradigms for these lesions are unknown and largely unexplored. Thus, using national registries, we analyze the epidemiology, management patterns, and surgical outcomes of SCOs and GCTs.
    METHODS: The National Cancer Database (NCDB; years 2003-2017) and the Surveillance, Epidemiology, and End Results Program (SEER; years 2004-2018) were queried for patients with pituitary SCOs or GCTs. Incidence, extent of surgical resection, and rate of postoperative radiation use for subtotally resected lesions comprised the primary outcomes of interest. All-cause mortality was also analyzed via time-to-event Kaplan-Meier curves.
    RESULTS: SCOs and GCTs have an annual incidence of 0.017 and 0.023 per 1,000,000, respectively. They comprise 0.1% of the benign pituitary tumors registered in NCDB. A total of 112,241 benign pituitary tumors were identified in NCDB during the study period, of which 83 (0.07%) were SCOs and 59 (0.05%) were GCTs. Median age at diagnosis was 55 years, 44% were females, and median maximal tumor diameter at presentation was 2.1 cm. Gross total resection was achieved in 54% patients. Ten patients (7%) had postoperative radiation. Comparing patients with GCTs versus SCOs, the former were more likely to be younger at diagnosis (48.0 vs. 59.0, respectively; p < 0.01) and female (59% vs. 34%, p = 0.01). GCTs and SCOs did not differ in terms of size at diagnoses (median maximal diameter: 1.9 cm vs. 2.2 cm, respectively; p = 0.59) or gross total resection rates (62% vs. 49%, p = 0.32). After matching SCOs and GCTs with pituitary adenomas on age, sex, and tumor size, the former were less likely to undergo gross total resection (53% vs. 72%; p = 0.03). Patients with SCOs and GCTs had a shorter overall survival when compared to patients with pituitary adenomas (p < 0.01) and a higher rate of thirty-day mortality (3.1% vs 0.0%; p = 0.013).
    CONCLUSIONS: SCOs and GCTs are rare pituitary tumors, and their management entails particular challenges. Gross total resection is often not possible, and adjuvant radiation might be employed following subtotal resection.
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  • 文章类型: Journal Article
    颗粒细胞瘤(GCT)是一种罕见的肿瘤,由神经周雪旺细胞引起,可出现在身体的任何地方,在乳腺中特别罕见。成像通常显示不规则,无钙化肿块,在乳房X线照相术上有高密度,在US上有强烈的后阴影,模仿恶性肿瘤。良性GCT可以是局部侵袭性的并且侵入皮肤或胸壁。核心活检对于诊断是必要的。具有显著的细胞质嗜酸性颗粒的多角形至纺锤形细胞在免疫组织化学上显示S-100和CD68染色,缺乏细胞角蛋白,雌激素,或孕酮表达。绝大多数GCT是良性的,尽管局部渗透,肿瘤通过广泛的局部切除治愈。
    Granular cell tumor (GCT) is an uncommon neoplasm arising from perineural Schwann cells that can arise anywhere in the body and is particularly rare in the breast. Imaging typically shows an irregular, noncalcified mass with high density on mammography and intense posterior shadowing on US that mimics malignancy. Benign GCTs can be locally aggressive and invade the skin or chest wall. Core biopsy is necessary for diagnosis. Polygonal- to spindle-shaped cells with prominent cytoplasmic eosinophilic granules show S-100 and CD68 staining on immunohistochemistry and lack cytokeratin, estrogen, or progesterone expression. The vast majority of GCTs are benign, albeit locally infiltrative, tumors cured by wide local excision.
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  • 文章类型: Case Reports
    颗粒细胞瘤发生在所有年龄和许多解剖部位。在颅面区域,它们通常出现在软组织中,不是骨头。我们介绍了一名12岁女孩的蝶骨和中央颅底原发性骨内颗粒细胞肿瘤。
    一名12岁女性,患有镰状细胞病和Jeavons综合征,出现癫痫发作。影像学检查和部分切除术显示,涉及蝶骨体的扩张性良性颗粒细胞瘤(GCT),翼状突起,和中央颅底。经过36个月的随访,该疾病保持稳定。
    GCT主要累及骨性蝶骨/颅底,以前没有在儿童中报道过。虽然大部分是良性的,有些是侵略性的,恶性转化在1-2%。手术是治疗的主要手段,但是在颅底,这可能受到相邻关键结构的限制。决策是由解剖范围指导的,组织学,和临床行为。
    UNASSIGNED: Granular cell tumors occur in all ages and many anatomic sites. In the craniofacial region, they typically arise in soft tissue, not bone. We present a primary intra-osseous granular cell tumor of the sphenoid and central skull base arising in a 12- year- old girl.
    UNASSIGNED: A 12-year-old female with sickle cell disease and Jeavons syndrome presented with seizures. Imaging and partial resection revealed an expansile benign granular cell tumor (GCT) involving the sphenoid body, pterygoid process, and central skull base. The disease has remained stable after 36-month follow up.
    UNASSIGNED: GCT primarily involving the osseous sphenoid/skull base has not been previously reported in a child. Although mostly benign, some are aggressive, with malignant transformation in 1-2%. Surgery is the mainstay of treatment, but in the skull base this may be limited by adjacent critical structures. Decision-making is guided by anatomic extent, histology, and clinical behavior.
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  • 文章类型: Journal Article
    颗粒细胞瘤,这被认为是一种雪旺氏细胞表型,是一种非常罕见的肿瘤,属于软组织肿瘤。它可以被归类为良性的,非典型或恶性,根据特定的组织学标准,大多数情况下表现出懒惰的行为。它的生物学和临床课程知之甚少,其最佳管理尚未确定,鉴于罕见的案件。在这里,我们描述了在30年的惰性期后发展的中上背部皮肤中的非典型颗粒细胞肿瘤。尽管手术完全切除,病人经历了复发,局部和肺部,经过积极的临床过程。转移性疾病的管理数据极其匮乏,完全由病例报告组成。因此,我们根据软组织肉瘤指南对患者进行全身治疗,导致疾病进展,致命的结果。总之,复发性和/或转移性颗粒细胞瘤是一种罕见的疾病,可能危及生命,对不同疗法的反应是未知的。非典型和恶性颗粒细胞瘤的生物学行为与良性颗粒细胞瘤的生物学行为有很大不同,引起软组织肉瘤,其诊断应提醒临床医生。应该探讨辅助化疗和放疗在这种情况下的作用,以限制疾病复发。
    Granular cell tumor, which is thought to recapitulate a Schwann cell phenotype, is a very rare neoplasm that belongs to soft tissue tumors. It can be classified as benign, atypical or malignant, based on specific histological criteria, with the majority of cases exhibiting an indolent behavior. Its biology and clinical course are poorly understood and its optimal management is yet to be defined, given the rarity of cases. Here we describe an atypical granular cell tumor in the upper middle back skin that evolved after a thirty-year indolent period. Despite complete surgical removal, the patient experienced a recurrence, both local and in the lungs, following an aggressive clinical course. Data on management of metastatic disease are extremely scarce, comprised exclusively of case reports. Therefore, we administered to the patient systemic therapy according to soft tissue sarcoma guidelines, which led to disease progression, with fatal outcome. In conclusion, recurrent and/or metastatic granular cell tumor is a rare disease that can be life-threatening, for which response to different therapies is unknown. The biologic behavior of atypical and malignant granular cell tumor is quite different from its benign counterpart, evoking soft tissue sarcomas, and its diagnosis should alert clinicians. The role of adjuvant chemotherapy and radiation therapy in this setting should be explored, to limit disease recurrence.
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  • 文章类型: Journal Article
    涉及胃肠道(GIT)的小儿颗粒细胞瘤(GCT)很少见,迄今报道的病例报告/系列报道有限。
    小儿GITGCT的多中心回顾性研究。
    共有10例病例纳入研究,中位年龄为13.5岁(范围:7-18岁),主要为女性患者(60%)。在一半的患者中,没有明显的病史,其余5人患有克罗恩病(10%),嗜酸性粒细胞性食管炎(EoE)(10%),克罗恩病和EoE(10%),生长激素缺乏(10%),和先天性表皮发育不全(10%)。GCT的中位尺寸为1.3cm(范围:1-1.6cm),更常见于食道(70%),其次是胃(20%)和直肠(10%)。大多数病例表现为圆形/多边形肿瘤细胞,颗粒细胞质丰富,这些病例都没有核非典型,有丝分裂活性增加,或者肿瘤细胞坏死.除临床随访外,我们的病例均未接受GCT的特异性治疗,并且没有患者有局部复发或转移性疾病的证据。
    我们展示了GITGCT的多中心体验,所有病例均为良性病程.有趣的是,4例食管GCT病例(包括2例EoE患者)显示下层粘膜中富含嗜酸性粒细胞的食管炎。
    UNASSIGNED: Pediatric granular cell tumors (GCT) involving the gastrointestinal tract (GIT) are rare with limited case report/series reported to date.
    UNASSIGNED: Multicenter retrospective study of pediatric GIT GCT.
    UNASSIGNED: A total of 10 cases were included in the study with a median age of 13.5 years (range: 7-18 years) and were predominantly female patients (60%). In half of the patients no significant medical history was present with the remaining 5 having Crohn disease (10%), eosinophilic esophagitis (EoE) (10%), Crohn disease and EoE (10%), growth hormone deficiency (10%), and aplasia cutis congenita (10%). The GCT median size was 1.3 cm (range: 1-1.6 cm) and were more commonly located in the esophagus (70%) followed by the stomach (20%) and rectum (10%). Most of the cases showed round/polygonal tumor cells with abundant granular cytoplasm, and none of the cases had nuclear atypia, increased mitotic activity, or tumor cell necrosis. None of our cases received specific therapy for GCT other than clinical follow-up, and none of the patients had evidence of local recurrence or metastatic disease.
    UNASSIGNED: We present our multicenter experience with GIT GCT, all cases had a benign course. Interestingly, 4 of the esophageal GCT cases (including 2 patients with EoE) showed an eosinophil-rich esophagitis in the underlying mucosa.
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