Mesenchymal tumors

间充质肿瘤
  • 文章类型: Case Reports
    我们介绍了一个巨大的孤立性纤维瘤(SFT)的病例报告,并回顾了文献,并讨论了其生物学特征和诊断。一名43岁的男子因腹痛和腹胀来到我们的急诊科,经历了两天的演变。对比增强计算机断层扫描(CT)显示,位于胰头的界限良好的半固体肿块(12cmx10cmx12cm)。经内镜超声引导下经十二指肠肿瘤活检细针穿刺获得的组织学诊断为增生的短梭形细胞,提示低度的间充质瘤形成。我们进行了Whipple手术技术。切除肿瘤的组织病理学研究证实,组织中的梭形细胞增殖,在10个高功率场(HPF)中观察到一个有丝分裂图。CD34和STAT-6的免疫染色呈阳性。组织学诊断为恶性胰腺SFT。在手术后的六个月里,病人没有反复发作的疾病。术前诊断困难,需要全面的证据,包括临床,免疫组织化学,和组织学特征。由于目前没有公认的最佳做法,我们建议全手术切除和仔细的临床监测。
    We present a case report of a giant solitary fibrous tumor (SFT) with a review of the literature and discuss its biological features and diagnosis. A 43-year-old man presented to our emergency department with abdominal pain and distension with an evolution of two days. Contrast-enhanced computed tomography (CT) showed a large, well-circumscribed semisolid mass (12 cm x 10 cm x 12 cm) localized in the pancreatic head. The histological diagnosis obtained by endoscopic ultrasound-guided trans-duodenal tumor biopsy with fine-needle aspiration showed proliferating short spindle-shaped cells, suggesting a mesenchymal neoplasia of low grade. We proceeded to a Whipple surgical technique. The histopathological study of the resected tumor confirmed proliferating spindle-shaped cells in the tissue, and one mitotic figure was observed in 10 high-power fields (HPFs). Immunostaining was positive for CD34 and STAT-6. The histological diagnosis was a malignant pancreatic SFT. In the six months posterior to the surgical procedure, the patient has been free of recurrent disease. Preoperative diagnosis is difficult and requires comprehensive evidence including clinical, immunohistochemistry, and histological features. Since there are currently no recognized best practices, we advise total surgical excision and careful clinical monitoring.
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  • 文章类型: Case Reports
    该病例报告介绍了一名42岁女性的临床细节,该女性以前没有医学问题,该女性表现为以黑素性粪便为特征的上消化道出血(UGIB)。初步检查显示轻度贫血,随后的内窥镜检查发现4厘米的粘膜下胃肿块显示近期出血指标。随后的手术病理证实2级高级别胃肠道间质瘤(GIST),复发风险增加。这种情况的意义在于强调在UGIB的鉴别诊断中考虑GIST的必要性。特别是在没有可识别的危险因素的中年人中,例如近期或慢性非甾体抗炎药(NSAID)的使用,消化性溃疡疾病,或报警症状。早期发现和及时手术干预在提高患者预后方面至关重要。虽然完全切除是治疗的基石,建议高危患者使用伊马替尼辅助治疗,以降低复发风险.
    This case report presents the clinical details of a 42-year-old female without previous medical issues who presented with upper gastrointestinal bleeding (UGIB) characterized by melanotic stools. Initial examination revealed mild anemia and subsequent endoscopy identified a 4 cm submucosal gastric mass displaying recent bleeding indicators. Subsequent surgical pathology confirmed a high-grade gastrointestinal stromal tumor (GIST) of grade 2 with a heightened risk of recurrence. The significance of this case lies in underscoring the necessity of considering GIST in the differential diagnosis of UGIB, particularly among middle-aged individuals with no identifiable risk factors such as recent or chronic non-steroidal anti-inflammatory drug (NSAID) use, peptic ulcer disease, or alarm symptoms. Early detection and prompt surgical intervention assume paramount importance in enhancing patient outcomes. While complete resection stands as the cornerstone of treatment, adjuvant imatinib therapy is recommended for high-risk patients to mitigate the risk of recurrence.
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  • 文章类型: Review
    背景:恶性外周神经鞘瘤(MPNST)是一种极为罕见且侵袭性的肿瘤,关于其管理的文献有限。在这里,我们介绍了一系列手术管理的颅脑脊髓MPNSTs,分析他们的结果,并回顾文献。
    方法:我们回顾性回顾了2005年1月至2023年5月在我们机构治疗的经手术管理的原发性颅脊髓MPNSTs。患者人口统计学,肿瘤特征,并评估治疗结果.使用Frankel等级和Karnofsky表现评分量化神经功能。描述性统计,秩和检验,进行了Kaplan-Meier生存分析.
    结果:8例患者符合纳入标准(4例男性,4女)。演示时的中位年龄为38岁(范围15-67)。大多数肿瘤位于脊柱(75%),3例患者患有1型神经纤维瘤病。最常见的症状是感觉异常(50%)和视觉变化(13%)。中位肿瘤大小为3cm,大多数肿瘤为椭圆形(50%),边界清晰(75%)。六个肿瘤为高级别(75%),5例患者实现了大体全切除,其余3例患者进行次全切除。术后放化疗6例(75%),4例(50%),分别。5例(63%)发生局部复发,2例(25%)发生远处转移。中位总生存期为26.7个月。5例(63%)患者因复发死亡。
    结论:原发性颅脊髓MPNSTs是罕见的,具有积极的临床过程。早期诊断和治疗对于治疗这些肿瘤至关重要。在这个单中心的小队列研究中,最大切除,低度病理学,年龄(<30岁),辅助放疗与生存率改善相关。
    BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is an exceedingly rare and aggressive tumor, with limited literature on its management. Herein, we present our series of surgically managed craniospinal MPNSTs, analyze their outcomes, and review the literature.
    METHODS: We retrospectively reviewed surgically managed primary craniospinal MPNSTs treated at our institution between January 2005 and May 2023. Patient demographics, tumor features, and treatment outcomes were assessed. Neurological function was quantified using the Frankel grade and Karnofsky performance scores. Descriptive statistics, rank-sum tests, and Kaplan-Meier survival analyses were performed.
    RESULTS: Eight patients satisfied the inclusion criteria (4 male, 4 female). The median age at presentation was 38 years (range 15-67). Most tumors were localized to the spine (75%), and 3 patients had neurofibromatosis type 1. The most common presenting symptoms were paresthesia (50%) and visual changes (13%). The median tumor size was 3 cm, and most tumors were oval-shaped (50%) with well-defined borders (75%). Six tumors were high grade (75%), and gross total resection was achieved in 5 patients, with subtotal resection in the remaining 3 patients. Postoperative radiotherapy and chemotherapy were performed in 6 (75%) and 4 (50%) cases, respectively. Local recurrence occurred in 5 (63%) cases, and distant metastases occurred in 2 (25%). The median overall survival was 26.7 months. Five (63%) patients died due to recurrence.
    CONCLUSIONS: Primary craniospinal MPNSTs are rare and have an aggressive clinical course. Early diagnosis and treatment are essential for managing these tumors. In this single-center study with a small cohort, maximal resection, low-grade pathology, young age (< 30), and adjuvant radiotherapy were associated with improved survival.
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  • 文章类型: Review
    背景:甲状腺间质瘤极为罕见。我们报告了甲状腺中发生的12例间充质肿瘤的细胞形态学特征,并强调了其细胞学评估中遇到的诊断困难。
    方法:从5个大型机构的细胞病理学和外科病理学档案中检索具有FNA的甲状腺间质瘤。评估了每个病例的临床病理和细胞形态学特征。
    结果:在我们的搜索中发现了12例发生在甲状腺中的间充质肿瘤。患者年龄为28至84岁(中位数,60年)。这些病例发生在7名女性和5名男性中。肿瘤大小为1.4至14厘米(中位数,3.3厘米)。肿瘤如下:血管瘤(n=4;33.3%),血管肉瘤(n=2;16.7%),神经鞘瘤(n=2;16.7%),孤立性纤维瘤(n=2,16.7%),转移性滑膜肉瘤(n=1,8.3%)和转移性多形性横纹肌肉瘤(n=1,8.3%)。肿瘤的细胞形态学特征与发生在不同部位的肿瘤相似。在6例原发性甲状腺间质病例(60%)中获得了准确的诊断。5例患者(41.7%)接受了甲状腺全切除术,3例患者接受部分甲状腺切除术(25%)。3例患者(25%)未接受甲状腺切除术,1例(8.3%)未获得后续手术信息。
    结论:甲状腺间质瘤极为少见。由于与各种上皮和非上皮甲状腺病变的形态学重叠,这些肿瘤的细胞学诊断通常具有挑战性。辅助研究如免疫组织化学和分子研究对于准确诊断至关重要。
    BACKGROUND: Mesenchymal tumors of the thyroid gland are extremely rare. We report the cytomorphologic characteristics of 12 mesenchymal tumors occurring in the thyroid gland and highlight the diagnostic difficulties encountered in their cytologic evaluation.
    METHODS: The cytopathology and surgical pathology archives from 5 large institutions were searched for thyroid mesenchymal tumors that had an FNA available for review. Clinicopathologic and cytomorphologic characteristics for each case were evaluated.
    RESULTS: Twelve cases of mesenchymal tumors occurring in the thyroid were identified in our search. Patient age ranged from 28 to 84 years (median, 60 years). The cases occurred in 7 women and 5 men. The tumor size ranged from 1.4 to 14 cm (median, 3.3 cm). The tumors were as follows: hemangioma (n = 4; 33.3%), angiosarcoma (n = 2; 16.7%), schwannoma (n = 2; 16.7%), solitary fibrous tumor (n = 2, 16.7%), metastatic synovial sarcoma (n = 1, 8.3%) and metastatic pleomorphic rhabdomyosarcoma (n = 1, 8.3%). The cytomorphologic features of the tumors were similar to those of their counterparts occurring in different sites. An accurate diagnosis was achieved in six primary thyroid mesenchymal cases (60%). Five patients (41.7%) underwent total thyroidectomy, and 3 patients received partial thyroidectomy (25%). Three patients (25%) did not receive a thyroidectomy and subsequent surgical information was not available in 1 case (8.3%).
    CONCLUSIONS: Mesenchymal tumors of the thyroid are extremely uncommon. Cytologic diagnosis of these tumors is often challenging due to the morphologic overlap with diverse epithelial and non-epithelial thyroid lesions. Ancillary studies such as immunohistochemistry and molecular studies are essential for accurate diagnosis.
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  • 文章类型: Journal Article
    BACKGROUND: To date, useful diagnostic applications of p16 IHC have been documented in gynecological pathology both for HPV-related and non-HPV-related lesions. In the present article, we reported our experience with the novel anti-p16 INK4a antibody (clone BC42), whose expression was tested across all different gynecologic neoplasms; we also compared it to the traditional E6H4 clone. Moreover, we discussed and explored all the diagnostic applications of p16 IHC in gynecologic pathology.
    METHODS: Consultation cases covering a 5-year period (2016-2020) regarding gynecological neoplastic and non-neoplastic lesions in which immunohistochemistry for p16, clone E6H4 was originally performed, were retrospectively retrieved from the files of our institution. Immunohistochemical staining for p16ink4a (BC42) [Biocare Medical group-Paceco USA; Bioptica Milan] and p16ink4a (E6H4) [Ventana Medical Systems-Arizona USA; Roche] was performed by using the Ventana automated immunostainer (Ventana Medical Systems, Tucson, AZ, USA). The immunostaining pattern was defined as negative, focal/patchy, or diffuse.
    RESULTS: A total of 196 cases, represented by 36 high-grade SIL/CIN3 of the uterine cervix, 30 cervical adenocarcinomas, 22 cervical squamous cell carcinoma, 70 endometrial carcinomas, 25 high grade serous ovarian carcinomas, 6 uterine adenomatoid tumors, and 10 uterine leiomyosarcomas were included in this study. Results showed concordant staining quality of both clones on all tested neoplastic tissues.
    CONCLUSIONS: The novel anti-p16 antibody (BC42 clone) appeared as an alternative to the current E6H4 for use in gynecological neoplasms, offering similar levels of positivity and equally reliable staining results.
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  • 文章类型: Case Reports
    BACKGROUND: Gallbladder mesenchymal tumours are rare. The more common include fibroma, lipoma or haemangioma. A gallbladder osteoma is very rare indeed there is only one other case reported in medical literature). We report a new case.
    METHODS: A 66-year-old female presented to the emergency department complaining of colicky epigastric pain and generalised abdominal discomfort for 1 month. The pain was scored 5/10 but there were no associated symptoms of fever, nausea or vomiting. Vital signs were normal as were all laboratory parameters. An abdominal ultrasound revealed a thin walled gallbladder with a solitary 3 mm polyp. Motility studies confirmed gallbladder dyskinesia. Laboratory studies were ordered reporting normal findings. Abdominal ultrasound was ordered reporting an image suggesting a gallbladder polyp and gallbladder dyskinesia. Cholecystectomy was done without any incidents and the gallbladder was sent to pathology. Pathology reported mature bone tissue in the stromal gallbladder tissue. Due to the rareness of the presence of mature bone tissue in the gallbladder we decided to report the case for the medical community.
    CONCLUSIONS: Osteomas are mesenchymal cell tumors derived from the mesoderm; the gallbladder may be the primary site of numerous types of mesenchymal tumors, although these tumors are common the location is not.
    CONCLUSIONS: We report only the second case of gallbladder osteoma. These mesenchymal tumours are common but this location is not. This case report will serve to remind readers of both benign osteomas and unusual conditions causing gallbladder disease.
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  • 文章类型: Journal Article
    OBJECTIVE: Primary leiomyosarcoma of the pancreas (PLMSP) is rare. The clinical features and outcomes are still unclear. The present study aims to identify the clinical features, outcomes, and racial differences of PLMSP.
    METHODS: PLMSP cases reported in Chinese, English, French, and Japanese journals were collected and reviewed. Data from these reports were summarized and analyzed statistically.
    RESULTS: In addition to a female patient presented to our department with PLMS, a total of 87 cases reported in the literature were included in the present study. An equal incidence in gender was observed. The mean age was 53.8 years. Common symptoms were abdominal pain and abdominal mass. At the time of diagnosis or after a period of follow-up, 37.5% of patients had distant metastasis and 31.8% of patients had regional organs/vessels invasion. The 5-year mortality rate was 77.8%. The presence of distant metastasis and the absence of radical surgery are significantly associated with poor outcomes. Regional invasion was significantly more common in East Asians.
    CONCLUSIONS: PLMSP is an aggressive tumor with a poor prognosis. Radical resection can decrease the mortality of the patients. Early detection of such tumor is recommended.
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    文章类型: Case Reports
    孤立性纤维瘤(SFT)是头颈部罕见的肿瘤,文献中仅报道了5例软腭。本文介绍了一个62岁男性的独特病例,其中TFS出现在软腭中。肿瘤是高度细胞化的,由看起来平淡随意排列的梭形细胞组成。通过免疫组织化学(IHC),信号转导和转录激活因子(STAT)-6和核β-catenin具有反应性。当前病例强调了STAT-6和β-连环蛋白作为IHC标志物与其他实体进行鉴别诊断的重要性。总之,本文介绍了第一例报道的男性患者中STAT-6和β-catenin核表达的软腭SFT病例。
    The solitary fibrous tumors (SFT) are rare tumors in the head and neck region and there have been only 5 cases reported in the literature in the soft palate. The current paper presents a unique case of a 62-year-old male with TFS arising in the soft palate. The tumor was highly cellular, composed of bland looking haphazardly arranged spindle cells. The signal transducer and activator of transcription (STAT)-6 and nuclear β-catenin were reactive by immunohistochemistry (IHC). The current case highlights the importance of the STAT-6 and the β-catenin as IHC markers to make a differential diagnosis with other entities. In summary, the paper presents the first reported case of a SFT of the soft palate in a male patient with nuclear expression of STAT-6 and β-catenin.
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  • 文章类型: Case Reports
    Desmoid tumors are benign but locally aggressive tumors of mesenchymal origin which are poorly circumscribed, infiltrate the surrounding tissue, lack a true capsule and are composed of abundant collagen. History of trauma or surgery to the site of tumor origin is elicited in up to one in four cases and they most commonly develop in the anterior abdominal wall and shoulder girdle but they can arise in any skeletal muscle. The clinical behavior and natural history of desmoid tumors are unpredictable and management is difficult with many issues remaining controversial, mainly regarding early detection, the role, type and timing of surgery and the value of non-operative therapies. We report a case of anterior abdominal wall desmoid tumor in a 40-year-old male with a previous history of surgery.
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  • 文章类型: Case Reports
    Solitary fibrous tumor (SFT) is a mesenchymal tumor typically located in the pleura, but can also be found as an asymptomatic mass in other areas, including the liver, peritoneum, kidney and salivary glands. However, SFT rarely locates in the pancreas. We present such a case of pancreatic SFT, along with a review of all reported cases. A 55-year-old man was treated surgically for an asymptomatic pancreatic mass after a rigorous preoperative control. Histologic examination of the resected specimen showed characteristics of an SFT. As only 15 cases of pancreatic SFT have been reported so far, an attempt to compare the cases was considered intriguing. We found that patients with pancreatic SFT were mainly women (81.25%), with a median age of 54 years at the time of diagnosis and a median tumor size of 5.83 cm. Pancreatic SFTs were revealed incidentally in 50% of cases, and all of them showed an enhancement through arterial computed tomography. All tumors were positive for CD34, ten were positive for Bcl-2, and twelve were negative for S100. The diagnosis of this pancreatic tumor is established by a combination of clinical suspicion, imaging procedures and histological findings, and is confirmed by immunohistochemical staining. Although the behavior of SFTs is rather benign, close clinical follow-up is recommended due to a potentially malignant nature.
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