Mesenchymal neoplasm

  • 文章类型: Case Reports
    成人间充质干细胞引起的孤立性纤维性肿瘤(SFT)是一种罕见的盆腔血管肿瘤。虽然最初被认为局限于胸膜,心包,或者腹膜,最近的研究表明,SFT可以在身体的不同区域发展。通常,SFT生长缓慢,可能没有明显症状。在这两个案例研究中,我们描述了一名46岁的女性患者和68岁的男性患者的临床情况,他们分别抱怨持续的盆腔疼痛和泌尿症状。影像学检查显示盆腔孤立性纤维瘤,经组织病理学证实,这种类型肿瘤的不寻常位置。该病例报告着重于早期识别和治疗在处理这种罕见肿瘤中的重要性。
    Solitary fibrous tumor (SFT) arising from adult mesenchymal stem cells is an uncommon vascular tumor of pelvic cavity. While initially thought to be confined to the pleura, pericardium, or peritoneum, recent studies have revealed that SFTs can develop in different areas of the body. Typically, SFTs grow slowly and may not present noticeable symptoms. In this 2 case study, we describe the clinical situation of a 46-year-old female patient and 68 years old male patient who complained of persistent pelvic pain and urinary symptoms respectively. Imaging tests revealed solitary fibrous tumor in the pelvic cavity which was confirmed on histopathology, an unusual location for this type of tumor. This case reports focusses on importance of early recognition and treatment in dealing with this rare tumor.
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  • 文章类型: Case Reports
    恶性胃肠道神经外胚层肿瘤(GNET)是一种罕见的实体,特征为仅在胃肠道附近发生的恶性间质瘤,易发生频繁的局部复发和转移。这里,我们报告一例49岁男性出现腹痛和体重减轻.该患者有胸腺B细胞淋巴瘤的遥远病史。腹部计算机断层扫描(CT)扫描显示回肠末端局灶性壁增厚伴肠系膜淋巴结病,提示淋巴瘤。肠系膜淋巴结的芯针活检尚无定论。随后进行了右半结肠切除术。组织学上,存在丰富的多核破骨细胞样巨细胞。肿瘤细胞对S100和SOX10表现出弥漫性强阳性。通过荧光原位杂交(FISH)鉴定EWSR1-ATF1基因融合,与GNET的诊断一致。此病例强调了对罕见恶性肿瘤的诊断挑战。
    A malignant gastrointestinal neuroectodermal tumor (GNET) is a rare entity, characterized as a malignant mesenchymal neoplasm occurring exclusively near the gastrointestinal tract, prone to frequent local recurrence and metastasis. Here, we report a case of a 49-year-old male presented with abdominal pain and weight loss. The patient had a remote history of thymic B-cell lymphoma. An abdominal computed tomography (CT) scan revealed a focal wall thickening of the terminal ileum with mesenteric lymphadenopathy, suggestive of lymphoma. A core needle biopsy of the mesenteric node was inconclusive. A right hemicolectomy was subsequently performed. Histologically, abundant multinucleated osteoclast-like giant cells are present. The tumor cells show diffuse strong positivity for S100 and SOX10. EWSR1-ATF1 gene fusion was identified by fluorescence in situ hybridization (FISH), consistent with a diagnosis of GNET. This case emphasizes a diagnostic challenge of a rare malignancy.
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  • 文章类型: Journal Article
    目的:侵袭性血管黏液瘤是一种少见的间叶性肿瘤,其特点是高复发率,通常在育龄期妇女的下生殖道中观察到。
    方法:收集2007年1月至2021年12月在北京朝阳医院经病理证实的侵袭性血管黏液瘤17例。我们收集了临床数据,并总结了临床和免疫组织化学特征。
    结果:所有17名患者均为女性,年龄在23至57岁之间(平均,37.7年;中位数,42年)。14例新诊断,3例复发。肿瘤位于外阴(58.8%),阴道(23.5%),臀部(11.8%),和子宫颈(5.9%)。肿瘤最大尺寸为2至15cm(平均8±4.4cm,中位数6厘米)。有9名患者的随访数据,范围从25到124个月(平均,82个月;中位数,80个月)。在后续行动结束时,未报告其他复发或转移。免疫组织化学分析显示雌激素(10/11)和孕激素(8/11)受体具有免疫反应性,desmin(6/8),平滑肌肌动蛋白(4/10),和波形蛋白(4/4),S-100(1/8)和CD34(1/7)。5例患者的Ki67水平低于5%。
    结论:AAM是一种激素敏感,明显的罕见间充质肿瘤,局部复发发生率高。手术是首选治疗方法,完全切除是将复发风险降至最低的基本前提。
    OBJECTIVE: Aggressive angiomyxoma is an uncommon mesenchymal neoplasm characterized by a high recurrence rate, usually observed in the lower genital tract of women during their reproductive age.
    METHODS: Seventeen cases of aggressive angiomyxoma confirmed by pathology from January 2007 to December 2021 in Beijing Chao-yang Hospital were included. We collected clinical data and summarized the clinical and immunohistochemical features.
    RESULTS: All seventeen included patients were females, aged between 23 and 57 years (mean, 37.7 years; median, 42 years). Fourteen patients were newly diagnosed and three were recurrent. The tumors were located in vulva (58.8 %), vagina (23.5 %), buttock (11.8 %), and cervix (5.9 %). The tumors size were 2 to 15 cm in greatest dimension (mean 8 ± 4.4 cm, median 6 cm). Follow-up data was available for nine patients, which ranged from 25 to 124 months (mean, 82 months; median, 80 months). At the end of follow-up, no other recurrence or metastasis was reported. Immunohistochemical analysis showed immunoreactive for estrogen (10/11) and progesterone (8/11) receptor, desmin (6/8), smooth muscle actin (4/10), and vimentin (4/4), S-100 (1/8) and CD34 (1/7). The Ki67 level was less than 5 % in five cases.
    CONCLUSIONS: AAM is a hormone-sensitive, distinct rare mesenchymal neoplasm with high incidence of local recurrence. Surgery is the preferred treatment, with complete resection being an essential prerequisite for minimizing the risk of recurrence.
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  • 文章类型: Journal Article
    有一组不同的非胃肠道间质瘤(GIST),胃肠道(GI)间充质肿瘤,表现出特征性的病理学和组织发生以及可变的影像学发现和生物学行为。肿瘤遗传学的最新进展揭示了与某些肿瘤相关的特定异常,影响其分子发病机制,生物学对治疗的反应,和预后。值得注意的是,食管巨大纤维血管息肉,通过MDM2基因扩增鉴定,现在被归类为脂肪肉瘤。一些肿瘤表现出独特的疾病分布模式。血管球瘤和丛状纤维粘液瘤对胃窦表现出明显的亲和力。相比之下,胃肠道内的平滑肌肿瘤主要见于食管和结肠直肠,超过这些地点的GIST发生率。手术切除足以治疗有症状的良性肿瘤;对于坦率的肉瘤,可能需要多模式治疗。本文旨在阐明与这些肿瘤的广谱相关的横断面影像学发现,提供符合其组织病理学特征的见解。
    There is a diverse group of non-gastrointestinal stromal tumor (GIST), mesenchymal neoplasms of the gastrointestinal (GI) tract that demonstrate characteristic pathology and histogenesis as well as variable imaging findings and biological behavior. Recent advancements in tumor genetics have unveiled specific abnormalities associated with certain tumors, influencing their molecular pathogenesis, biology, response to treatment, and prognosis. Notably, giant fibrovascular polyps of the esophagus, identified through MDM2 gene amplifications, are now classified as liposarcomas. Some tumors exhibit distinctive patterns of disease distribution. Glomus tumors and plexiform fibromyxomas exhibit a pronounced affinity for the gastric antrum. In contrast, smooth muscle tumors within the GI tract are predominantly found in the esophagus and colorectum, surpassing the incidence of GISTs in these locations. Surgical resection suffices for symptomatic benign tumors; multimodality treatment may be necessary for frank sarcomas. This article aims to elucidate the cross-sectional imaging findings associated with a wide spectrum of these tumors, providing insights that align with their histopathological features.
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  • 文章类型: Case Reports
    肺孤立性纤维瘤(SFT)是一种罕见的间充质肿瘤,其组织起源不确定,未知的分子特征,和不可预测的临床行为,以NAB2-STAT6融合为特征。伴随SFT(Doege-Potter综合征)的低血糖是一种罕见的表现。我们介绍了经组织病理学证实的肺SFT病例的活检印迹涂片上的细胞形态学特征,其表现并不常见。一个76岁的不吸烟者,不含酒精,和非糖尿病男性出现了6个月的间歇性混乱发作和晕厥发作(>10次发作)的抱怨。患者没有呼吸不适,也没有体重减轻的病史。实验室调查显示,空腹血糖为38mg/dl,血清胰岛素和C肽水平较低。体格检查显示胸部左侧的空气进入减少。胸部X线显示左侧均匀混浊。胸部高分辨率计算机断层扫描(HRCT)显示左侧肺部肿块大。进行活检。活检印迹涂片是细胞的,显示肿瘤细胞成簇和碎片排列,穿过毛细血管,向椭圆形核显示单形泵。细颗粒均匀分散的染色质,规则的核膜,不显眼的核仁,和适量的纤细细胞质。注意到细胞间透明基质材料的病灶。进行了低度间充质肿瘤的细胞诊断。组织病理学显示细胞肿瘤包括紧密堆积的圆形到梭形细胞,排列在血管周围,中间有厚胶原蛋白,CD99阳性,波形蛋白,BCL2、CD34和STAT6,EMA阴性,CKAE1/AE3、S100、TLE1和SMA。熟悉细胞形态学在早期诊断这种罕见的肺部肿瘤中起着关键作用。特别是在低血糖的背景下。
    Solitary fibrous tumor (SFT) of the lung is a rare mesenchymal neoplasm of uncertain histogenesis, unknown molecular features, and unpredictable clinical behavior, characterized by NAB2-STAT6 fusion. Hypoglycemia accompanying SFT (Doege-Potter syndrome) is an uncommon presentation. We present the cytomorphological features on biopsy imprint smears of a histopathologically confirmed case of SFT of the lung with an uncommon presentation. A 76-year-old non-smoker, non-alcoholic, and non-diabetic man presented with complaints of intermittent episodes of confusion with syncopal attacks (>10 episodes) for six months. The patient had no respiratory complaints and no history of weight loss. Laboratory investigations revealed fasting blood sugar of 38 mg/dl with low serum insulin and C-peptide levels. Physical examination revealed reduced air entry on the left side of the chest. Chest X-ray showed left-sided homogenous opacity. High-resolution computed tomography (HRCT) of the chest showed a large left-sided lung mass. A biopsy was performed. Biopsy imprint smears were cellular and showed tumor cells arranged in clusters and fragments with traversing capillaries displaying monomorphic pump to oval nuclei, fine granular evenly dispersed chromatin, regular nuclear membrane, inconspicuous nucleoli, and a moderate amount of wispy cytoplasm. Foci of intercellular hyaline stromal material were noted. A cytodiagnosis of low-grade mesenchymal neoplasm was made. Histopathology revealed a cellular tumor comprising tightly packed round to fusiform cells arranged around blood vessels with intervening thick collagen, positive for CD99, vimentin, BCL2, CD34, and STAT6 and negative for EMA, CK AE1/AE3, S100, TLE1, and SMA. Familiarity with cytomorphology plays a pivotal role in clinching an early diagnosis of this rare neoplasm of the lung, particularly in the setting of presentation with hypoglycemia.
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  • 文章类型: Case Reports
    钙化性纤维性肿瘤(CFT)是一种罕见的间充质起源良性病变,可能具有与其他更常见的肿瘤相似的特征。我们介绍了一个36岁的女性,在近端空肠有肿瘤,最初怀疑是胃肠道间质瘤(GIST)。进行了手术切除,在抗肠系膜边界显示出清晰的结节,具有典型的钙化性纤维瘤的微观特征。肿瘤细胞CD34阳性,其他标志物阴性,将其与其他肿瘤区分开来。钙化性纤维性肿瘤由于其外观而与更常见的肿瘤相混淆,但是由免疫组织化学支持的准确诊断是至关重要的。完全手术切除通常是治愈性的。
    Calcifying fibrous tumor (CFT) is a rare benign lesion of mesenchymal origin that may present similar characteristics to other more common tumors. We present the case of a 36-year-old woman with a tumor in the proximal jejunum, initially suspected to be a gastrointestinal stromal tumor (GIST). Surgical resection was performed, revealing a well-demarcated nodule at the anti-mesenteric border with microscopic features typical of a calcifying fibrous tumor. The tumor cells were positive for CD34 and negative for other markers, differentiating it from other neoplasms. Calcifying fibrous tumors can be confused with more common tumors because of its appearance, but an accurate diagnosis supported by immunohistochemistry is essential. Complete surgical excision is usually curative.
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  • 文章类型: Case Reports
    孤立性纤维瘤(SFT)是罕见的间充质肿瘤,占所有软组织肿瘤的不到2%。它们通常起源于胸腔,主要在胸膜,但也可以发生在其他各种部位,如肺实质,心包,还有支气管.在这项研究中,一名49岁的非吸烟女性,有过敏史,因慢性咳嗽出现在我们的肺部诊所。探索性支气管镜检查显示左上支气管有支气管内肿块,68Ga-DOTATOC正电子发射计算机断层扫描显示类癌。随后的肺段切除术揭示了一个明确的息肉样病变,通过组织病理学和免疫组织化学评估被诊断为低级支气管SFT。患者在手术切除后无症状,在6个月的随访中没有其他病变。SFT的支气管内位置并不常见,文献中只有少数报道的病例,强调考虑各种鉴别诊断的必要性,包括类癌,粘液表皮样癌,支气管内多形性腺瘤,错构瘤,平滑肌瘤,和转移,取决于位置和成像特征。该报告强调了仔细的组织学和免疫组织化学评估在理解和适当分层与息肉样病变相关的风险方面的重要性。
    Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms constituting less than 2% of all soft tissue tumors. They typically originate in the thoracic cavity, mainly in the pleura, but can also occur in other various sites such as lung parenchyma, pericardium, and bronchus. In this study, a 49-year-old non-smoking female with a history of allergies presented to our pulmonary clinic with a chronic cough. An explorative bronchoscopy revealed an intrabronchial mass in the left superior bronchi, and a 68 Ga-DOTATOC positron emission computed tomography suggested a carcinoid tumor. Subsequent pulmonary segmentectomy unveiled a well-circumscribed polypoid lesion diagnosed as a low-grade bronchus SFT through histopathological and immunohistochemical assessments. The patient was asymptomatic after surgical excision and showed no other lesion during the 6-month follow-up. The endobronchial location of SFT is uncommon, with only a few reported cases in the literature, underscoring the necessity of considering various differential diagnoses, including carcinoid, mucoepidermoid carcinoma, endobronchial pleomorphic adenoma, hamartoma, leiomyoma, and metastasis, depending on location and imaging features. This report underscores the importance of careful histological and immunohistochemical evaluation in understanding and appropriately stratifying the risk associated with polypoid lesions.
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  • 文章类型: Case Reports
    孤立性纤维瘤是一种间充质肿瘤,在文献中首次出现为胸膜病变,但是在过去的几十年里,据报道,在许多胸外部位。肾上腺原发性孤立性纤维瘤非常罕见。它的生物学行为是可变的,但大多是良性的。我们在这里报道一个看起来健康的女人,在肢体感觉异常研究的背景下,被诊断为肾上腺偶发瘤.进行了实验室测试,没有检测到激素功能亢进。随后,进行了右肾上腺切除术,病理研究证实为孤立性纤维瘤。据我们所知,这是文献报道的第19例原发性孤立性纤维性肿瘤起源于肾上腺,特别是,西班牙第一个有记录的实例。
    Solitary fibrous tumor is a mesenchymal neoplasm that first appeared in the literature as a pleural lesion, but over the last decades, it has been reported in many extrathoracic sites. Primary solitary fibrous tumor in the adrenal gland is very uncommon. Its biological behavior is variable but mostly benign. We report here a case of an apparently healthy woman who, in the context of the study of limb paresthesias, was diagnosed with an adrenal incidentaloma. Laboratory tests were performed, and no hormone hyperfunction was detected. Subsequently, a right adrenalectomy was performed, and the pathological study confirmed a solitary fibrous tumor. To the best of our knowledge, this is the 19th case reported in the literature of a primary solitary fibrous tumor originating from the adrenal gland and, notably, the first documented instance in Spain.
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  • 文章类型: Case Reports
    胃肠道间质瘤(GIST)是胃肠道的罕见病变,对胃和小肠有强烈的易感性。我们介绍了一名89岁女性的病例,该女性最初出现急性失代偿性心力衰竭(HF)的体征和症状,后来被发现在其腹腔中有23厘米的GIST。此病例强调了大型腹膜内肿瘤的含义以及它们可能存在的独特症状。
    Gastrointestinal stromal tumors (GISTs) are rare lesions of the gastrointestinal tract that have a strong predisposition to the stomach and small intestine. We present a case of an 89-year-old female who initially presented to the emergency room with signs and symptoms of acute decompensated heart failure (HF) and was later discovered to have a 23-centimeter GIST in her abdominal cavity. This case emphasizes the implications of large intraperitoneal neoplasms and the unique constellation of symptoms they may present with.
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  • 文章类型: Journal Article
    猎人发现了成年男性自由生活的黄色Armadillo(Euphractussexcinctus),由于甲壳上有肿瘤样病变,因此被推荐进行临床评估。这只动物昏昏欲睡,身体虚弱,严重脱水,眼球突出和恶病质,并因其非常差的临床状况而被安乐死。尸检显示发白,外生性,甲壳尾部三分之一的不规则和潮湿的肿块(7×6.5×1.5厘米)。在切割截面上,质量很硬,紧凑型,不规则和发白。组织病理学显示细胞密集,膨胀,由排列在岛和索中的恶性间充质细胞组成的界限较差的肿瘤,散布着许多不规则矿化的类骨质基质区域。肿瘤细胞对波形蛋白进行了强烈的免疫标记。诊断为高级别骨肉瘤的成骨细胞亚型是基于临床,gross,组织病理学和免疫组织化学结果。这项首次报告表明,在一个Armadillo的骨肉瘤扩大了Armadillo疾病的名单,并将有助于兽医管理这些动物。
    An adult male free-living yellow armadillo (Euphractus sexcinctus) was found by hunters and referred for clinical evaluation because of a tumour-like lesion on the carapace. The animal was lethargic and weak with severe dehydration, enophthalmos and cachexia, and was euthanized because of its very poor clinical condition. Necropsy revealed a whitish, exophytic, irregular and moist mass (7 × 6.5 × 1.5 cm) in the caudal third of the carapace. On cut section, the mass was hard, compact, irregular and whitish. Histopathology revealed a densely cellular, expansive, poorly delimited neoplasm composed of malignant mesenchymal cells arranged in islands and cords, interspersed by numerous areas of irregularly mineralized osteoid matrix. Neoplastic cells were intensely immunolabelled for vimentin. The diagnosis of a high-grade osteosarcoma of osteoblastic subtype was based on the clinical, gross, histopathological and immunohistochemical findings. This first report of an osteosarcoma in an armadillo expands the list of armadillo diseases and will assist the management of these animals by veterinarians.
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