Hemangiosarcoma

血管肉瘤
  • 文章类型: Case Reports
    背景:原发性肺血管肉瘤(PPA)是一种高度侵袭性和罕见的恶性肿瘤,起源于肺部血管的内皮细胞。PPA是一种极为罕见的亚型,迄今报告的病例不到30例。PPA不仅诊断具有挑战性,而且预后不良。通常导致诊断后一年内的高死亡率,不管治疗方法如何。
    方法:我们介绍一例33岁女性,无明显既往病史,表现为腹痛,偶然发现右侧肺门肿块伴胸腔积液和脓胸。胃溃疡穿孔手术后,她的肺部病变被进一步处理。尽管进行了广泛的诊断评估,包括成像,支气管镜检查,开胸手术,建立诊断是具有挑战性的。最终,在外科肺活检中诊断出PPA,患者开始接受帕唑帕尼和紫杉醇化疗,但由于多种并发症,1个月后过期。
    结论:该病例突出了诊断这种罕见肿瘤的困难和无论如何治疗的不良预后。需要提高对PPA的认识和更多的研究,以改善这种致命疾病的早期发现和治疗选择。
    BACKGROUND: Primary pulmonary angiosarcoma (PPA) is a highly aggressive and rare malignancy originating from the endothelial cells of blood vessels in the lungs. PPA is an extremely rare subtype, with less than 30 cases reported to date. PPA is not only challenging to diagnose but also has a poor prognosis, often resulting in a high mortality rate within a year of diagnosis, regardless of the treatment approach.
    METHODS: We present the case of a 33-year-old woman with no significant past medical history who presented with abdominal pain and was incidentally found to have a right hilar mass with pleural effusion and empyema. After undergoing surgery for a perforated gastric ulcer, her pulmonary lesions were further worked up. Despite an extensive diagnostic evaluation, including imaging, bronchoscopy, and thoracotomy, establishing a diagnosis was challenging. Ultimately, PPA was diagnosed on surgical lung biopsy, and the patient was started on pazopanib and paclitaxel chemotherapy but expired after 1 month due to multiple complications.
    CONCLUSIONS: This case highlights the difficulty in diagnosing this rare tumor and its poor prognosis regardless of therapy. Greater awareness of PPA and more research are needed to improve early detection and treatment options for this deadly disease.
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  • 文章类型: Case Reports
    原发性心脏血管肉瘤是一种罕见的,通常转移到各种器官的侵袭性恶性肿瘤。呈现的症状通常是非特异性的,因此,需要进行全面检查以及时确认诊断。此病例报告描述了一名有肿瘤史的老年患者的表现。进行超声心动图和活检,但是尽管手术切除了大量右心房肿块,病人死了。根据切除的标本对原发性血管肉瘤进行最终诊断。
    Primary cardiac angiosarcoma is a rare, aggressive malignancy that commonly metastasizes to various organs. The presenting symptoms are typically nonspecific, so a comprehensive examination is required to confirm the diagnosis promptly. This case report describes the presentation of an older patient with a history of neoplasms. Echocardiography and biopsy were performed, but despite surgical intervention to resect a large right atrial mass, the patient died. A final diagnosis of primary angiosarcoma was made based on the resected specimen.
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  • 文章类型: Journal Article
    背景:原发性乳腺血管肉瘤是一种罕见的肿瘤,仅占所有乳腺恶性肿瘤的0.05%。原发性乳腺血管肉瘤通常表现为非特异性临床表现。这很容易导致误诊。导致误诊的潜在因素包括皮肤变化,这些变化可能被错误地归因于乳房外伤引起的瘀伤和乳房肿胀,可能被误认为是炎症性疾病或其他良性肿瘤。
    方法:一名19岁女性因左乳外伤后9个月反复形成左乳肿块入院。
    方法:经血肿活检确诊为原发性乳腺血管肉瘤。
    方法:由于患者的病情,术后未给予特殊治疗。在那之后,胸壁复发了,患者接受了2个周期的化疗,导致复发性胸壁肿块的大小减少和减轻。当化疗不耐受发生时,患者选择停止治疗.
    结果:经过18个月的随访,复发的胸壁肿块增加,患者死于出血。
    结论:原发性乳腺血管肉瘤发病率低,恶性程度高,具有很高的复发率和转移率,导致预后不良。辅助化疗,放射治疗,靶向治疗,应考虑其他治疗方法,以降低局部复发率,延长患者生存期。
    BACKGROUND: Primary breast angiosarcoma is a rare tumor, accounting for only 0.05% of all malignant breast tumors. The primary breast angiosarcoma typically presents with nonspecific clinical manifestations, which can easily lead to misdiagnosis. Potential factors contributing to misdiagnosis include skin changes that may be erroneously attributed to breast trauma-induced bruising and breast swelling that may be mistaken for inflammatory diseases or other benign tumors.
    METHODS: A 19-year-old female was admitted to the hospital due to repeated lump formation in the left breast for 9 months after left breast trauma.
    METHODS: The diagnosis of primary breast angiosarcoma was confirmed on hematoma biopsy.
    METHODS: Due to the patient\'s condition, no special treatment was given postoperatively. After then, there was a recurrence in the chest wall, and the patient received 2 cycles of chemotherapy, resulting in a reduction in the size and lightening of the recurrent chest wall mass. When chemotherapy intolerance happened, the patient chose to discontinue treatment.
    RESULTS: After an 18-month follow-up, the recurrent chest wall mass increased and the patient died from bleeding.
    CONCLUSIONS: Primary breast angiosarcoma has a low incidence but high malignancy, with a high recurrence and metastasis rate, leading to a poor prognosis. The adjuvant chemotherapy, radiotherapy, targeted therapy, and other treatments should be considered to reduce the local recurrence rate and prolong patient survival.
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  • 文章类型: Journal Article
    暂无摘要。
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  • 文章类型: Journal Article
    已广泛检查了施用阿霉素(DOX)后狗的左心室功能障碍。然而,DOX对右心室(RV)功能的影响尚不清楚.因此,本研究调查了DOX化疗是否会降低RV功能.十二只狗(五只患有多中心淋巴瘤,四个患有血管肉瘤,两个患有甲状腺癌,和一个患有肺腺癌)接受至少两个剂量的DOX的前瞻性登记。在每次施用DOX之前和最后一次施用后约一个月进行超声心动图和肌钙蛋白I的测量。右心室功能通过RV面积变化和RVTei指数评估。两个(n=4),三(n=3),四(n=3),给予5个(n=2)剂量的DOX。虽然在RV面积变化中没有观察到显著差异,两剂DOX后RVTei指数明显受损。肌钙蛋白I水平在四个剂量后显著增加。DOX累积剂量与RVTei指数相关(r=0.77,P<0.001)。目前的结果表明,使用DOX进行的化学疗法治疗以剂量依赖性方式降低了狗的RV功能。
    Left ventricular dysfunction in dogs after the administration of doxorubicin (DOX) has been extensively examined. However, the effects of DOX on right ventricular (RV) function remain unknown. Therefore, the present study investigated whether the chemotherapy treatment with DOX decreases RV function. Twelve dogs (five with multicentric lymphoma, four with hemangiosarcoma, two with thyroid cancer, and one with lung adenocarcinoma) that received at least two doses of DOX were prospectively enrolled. Echocardiography and the measurement of troponin I were performed prior to each administration of DOX and approximately one month after the last administration. Right ventricular function was assessed by the RV fractional area change and RV Tei index. Two (n=4), three (n=3), four (n=3), and five (n=2) doses of DOX were administered. While no significant differences were observed in the RV fractional area change, the RV Tei index was significantly impaired after two doses of DOX. Troponin I level significantly increased after four doses. Cumulative doses of DOX correlated with the RV Tei index (r=0.77, P<0.001). The present results demonstrated that the chemotherapy treatment with DOX decreased RV function in a dose-dependent manner in dogs.
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  • 文章类型: Journal Article
    目的:面部血管肉瘤很少见,但由于其起源于血液或淋巴管的支持组织,因此存在重大的治疗挑战。实现肿瘤疗效和美学结果之间的最佳平衡需要多学科的方法,特别是在需要根治性R0切除的情况下。延误经常发生,尤其是在组织病理学检查期间,在明确的病理发现之前,这会使原发性塑料重建复杂化。
    方法:要解决此问题,我们介绍了一例使用猪源脱细胞真皮基质进行临时软组织覆盖的病例,作为面部血管肉瘤的可行选择.这在冷冻切片有丧失关键解剖结构的风险且术中诊断不可行的情况下特别有用。这种方法在诊断阶段允许令人满意的伤口覆盖和肉芽形成。为肿瘤可管理的情况和功能康复铺平道路。
    结论:在罕见和复杂情况的肿瘤手术中,用猪来源的脱细胞真皮基质临时覆盖软组织是一种有价值的选择。
    OBJECTIVE: Angiosarcomas of the face are rare but present significant treatment challenges due to their origin in the supportive tissues of blood or lymphatic vessels. Achieving optimal balance between oncological efficacy and aesthetic outcomes requires a multidisciplinary approach, particularly in cases where radical R0 resection is necessary. Delays often occur, especially during histopathological examinations, which can complicate primary plastic reconstruction before definitive pathological findings.
    METHODS: To address this issue, we present a case with the use of porcine-derived acellular dermal matrix for temporary soft tissue coverage as a viable option in a case of angiosarcoma of the face. This is particularly useful in situations where frozen sections risk the loss of critical anatomical structures and intraoperative diagnosis is not feasible. This approach allowed for satisfactory wound coverage and granulation during diagnostic phases, paving the way for oncologically manageable situations and functional rehabilitation.
    CONCLUSIONS: Temporary soft tissue coverage with porcine-derived acellular dermal matrix is a valuable option in tumor surgery of rare and complex situations.
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  • 文章类型: Case Reports
    简介:肾血管瘤是一种良性肿瘤,由于它的特点,它必须与恶性疾病区分开来。我们介绍了一例原发性肾血管肉瘤的临床病例,由于它们的相似性,最初被误认为是血管瘤。病例报告:一名58岁的男子因怀疑肺栓塞而入院。患者抱怨左侧疼痛。腹部的超声和CT扫描显示,在左肾的投影中,肿瘤肿块的大小约为20.5×17.2×15.4cm。在CT图像上,有透明细胞肾透明细胞癌(ccRCC)的数据。进行左肾切除术。然而,组织学检查显示肾血管瘤。三个月后,患者出现腹部和腰部疼痛。CT扫描显示肝脏中有多个小的低回声病灶,大小达2厘米,肺,腹内,癌症的数据最多。左肾的组织学重新验证显示肾血管肿瘤具有明显的梗塞和坏死迹象,大多数证据支持血管肉瘤。尽管治疗,病人的结局是致命的。结论:根据临床表现,放射学图像和组织学检查数据,该肿瘤最初被误诊为肾血管瘤。由于这种肿瘤的罕见,对于原发性肾血管肉瘤的治疗,目前尚无既定的治疗方案或临床指南.
    Introduction: Renal haemangioma is a benign tumour, and due to its characteristics, it must be distinguished from malignant diseases. We present a clinical case of primary renal angiosarcoma initially mistaken for haemangioma due to their similarity. Case report: A 58-year-old man was admitted to the hospital with suspicion of pulmonary embolism. The patient complained of pain on the left side. An ultrasound and CT scan of the abdomen showed a tumour mass ~20.5 × 17.2 × 15.4 cm in size in the projection of the left kidney. On CT images, there were data for clear cell renal clear cell carcinoma (ccRCC). A left nephrectomy was performed. However, histological examination revealed renal haemangioma. Three months later, the patient presented to the hospital with abdominal and lumbar pain. A CT scan showed multiple small hypoechoic foci up to 2 cm in size in the liver, lungs, and intra-abdominally, with the most data for carcinosis. Histological re-verification of the left kidney showed a renal vascular tumour with pronounced signs of infarction and necrosis with the majority of the evidence supporting angiosarcoma. Despite treatment, the patient\'s outcome was fatal. Conclusions: Based on the clinical presentation, radiological images and histological examination data, the tumour was initially misdiagnosed as kidney haemangioma. Due to the rarity of this tumour, there are no established treatment protocols or clinical guidelines for managing primary kidney angiosarcoma.
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  • 文章类型: Case Reports
    背景:血管肉瘤是一种发生在一系列组织类型中的肉瘤,在唾液腺中很少见,对老年患者的腮腺表现好感。术前诊断可能具有挑战性,尤其是细胞学,与高级别原发性唾液腺癌具有明显的形态学重叠。这种罕见的唾液腺肿瘤的分子改变也没有得到很好的表征。
    结果:我们介绍了一例73岁男性右颌下腺肿胀的病例。细针抽吸时,包括细胞块上的免疫组织化学染色,该肿瘤最初被诊断为低分化癌。下颌下腺切除术显示上皮样血管肉瘤。我们对肿瘤进行了分子检查,利用靶向下一代测序,DNA甲基化分析和荧光原位杂交。组织病理学评估显示浸润性肿瘤包含上皮样细胞的实片。肿瘤细胞与含有红细胞的胞浆内腔随意形成吻合的血管通道。关于免疫组织化学,肿瘤细胞CD31、CD34和ERG阳性。约40%的肿瘤细胞显示GATA3的核表达。在下一代测序中检测到致病性TP53R267W突变。DNA甲基化分析没有将肿瘤与任何已知的肉瘤类型进行聚类。拷贝数分析显示可能的MYC扩增和CDKN2A损失,尽管只有后者在荧光原位杂交中得到证实。
    结论:上皮样血管肉瘤是高度涎腺癌的重要鉴别诊断。特别是,在血管肉瘤和高级唾液腺癌中都可能遇到GATA3表达,并引起诊断混乱。TP53突变和CDKN2A丢失的鉴定表明与软组织血管肉瘤共有的致癌途径,应该进一步调查。
    BACKGROUND: Angiosarcoma is a sarcoma that occurs in a range of tissue types, and only rarely in the salivary glands, showing a predilection for the parotid glands of older patients. Preoperative diagnosis may be challenging, especially on cytology, with significant morphological overlap with high-grade primary salivary gland carcinomas. The molecular alterations of this rare salivary gland neoplasm are also not well-characterized.
    RESULTS: We present a case of right submandibular gland swelling in a 73-year-old male. On fine needle aspiration, including immunohistochemical stains on cell block, the tumor was initially diagnosed as poorly differentiated carcinoma. Resection of the submandibular gland revealed epithelioid angiosarcoma. We performed molecular work-up of the tumor, utilizing targeted next-generation sequencing, DNA methylation profiling and fluorescence in-situ hybridization. Histopathologic assessment revealed an infiltrative tumor comprising solid sheets of epithelioid cells. The tumor cells formed haphazardly anastomosing vascular channels with intracytoplasmic lumina containing red blood cells. On immunohistochemistry, the tumor cells were positive for CD31, CD34 and ERG. Approximately 40% of the tumor cells showed nuclear expression of GATA3. A pathogenic TP53 R267W mutation was detected on next-generation sequencing. DNA methylation analysis did not cluster the tumor with any known sarcoma type. Copy number analysis showed possible MYC amplification and CDKN2A losses, although only the latter was confirmed on fluorescence in-situ hybridization.
    CONCLUSIONS: Epithelioid angiosarcoma is an important differential diagnosis to high-grade salivary gland carcinoma. In particular, GATA3 expression may be encountered in both angiosarcoma and high-grade salivary gland carcinomas and cause diagnostic confusion. Identification of TP53 mutations and CDKN2A losses suggest shared oncogenic pathways with soft tissue angiosarcomas, and should be further investigated.
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  • 文章类型: Journal Article
    目的:本研究旨在探讨血管肉瘤(AS)患者的肿瘤结局和预后因素。
    方法:这种单中心,回顾性队列研究,分析组织病理学证实的AS病例。初步诊断,包括局部复发和转移性AS。总生存期(OS),通过Kaplan-Meier估计器评估局部控制(LC)和局部无进展生存期(LPFS).多变量Cox回归分析检测与OS和LPFS相关的因素。
    结果:总计,纳入118例中位随访6.6个月的患者。大多数患者出现局部疾病(62.7%),其次是转移性(31.4%)和局部复发(5.9%)。74名病人(62.7%)接受手术,其中29人(39.2%)只接受手术治疗,38例(51.4%)接受手术和围手术期放疗或化疗,和7(9.4%)手术,围手术期放疗和化疗。OS的多变量Cox回归显示与年龄(风险比(HR):1.03,p=0.044)和转移疾病(风险比:3.24,p=0.015)显着相关。对于LPFS,年龄每年(HR:1.04,p=0.008),报告时局部复发性疾病(HR:5.32,p=0.013),和转移性疾病(HR:4.06,p=0.009)存在显着相关性。肿瘤大小,上皮样成分,边距状态,围手术期RT和/或CTX与OS或LPFS无显著相关性。
    结论:初始表现状态的年龄和转移性疾病与OS和LPFS呈负相关。通过收集多机构数据集来克服AS的流行病学挑战,有必要进行创新和协作。对AS进行分子表征并确定新的围手术期治疗方法以改善患者预后。
    OBJECTIVE: This study sought to investigate oncological outcomes and prognostic factors for patients with angiosarcomas (AS).
    METHODS: This single-center, retrospective cohort study, analyzed histopathologically confirmed AS cases. Primarily diagnosed, locally recurrent and metastatic AS were included. Overall survival (OS), local control (LC) and local progression-free survival (LPFS) were assessed by Kaplan-Meier estimator. Multivariable Cox regression analysis was performed to detect factors associated with OS and LPFS.
    RESULTS: In total, 118 patients with a median follow-up of 6.6 months were included. The majority presented with localized disease (62.7%), followed by metastatic (31.4%) and locally recurrent (5.9%) disease. Seventy-four patients (62.7%) received surgery, of which 29 (39.2%) were treated with surgery only, 38 (51.4%) with surgery and perioperative radiotherapy or chemotherapy, and 7 (9.4%) with surgery, perioperative radiotherapy and chemotherapy. Multivariable Cox regression of OS showed a significant association with age per year (hazard ratio (HR): 1.03, p = 0.044) and metastatic disease at presentation (hazard ratio: 3.24, p = 0.015). For LPFS, age per year (HR: 1.04, p = 0.008), locally recurrent disease at presentation (HR: 5.32, p = 0.013), and metastatic disease at presentation (HR: 4.06, p = 0.009) had significant associations. Tumor size, epithelioid components, margin status, and perioperative RT and/or CTX were not significantly associated with OS or LPFS.
    CONCLUSIONS: Older age and metastatic disease at initial presentation status were negatively associated with OS and LPFS. Innovative and collaborative effort is warranted to overcome the epidemiologic challenges of AS by collecting multi-institutional datasets, characterizing AS molecularly and identifying new perioperative therapies to improve patient outcomes.
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  • 文章类型: Case Reports
    背景:本研究旨在描述一例罕见的原发性输尿管血管肉瘤,其中手术干预保留了肿瘤切除后的肾脏和输尿管。
    方法:13岁,绝育的雄性狗,重14公斤,混血儿,带着冷漠,厌食症,急性呕吐,体格检查时腹部不适。超声检查和肾盂造影显示,由于输尿管中段完全阻塞,肾盂和输尿管右侧扩张。阻塞右输尿管管腔的肿块被完全切除,进行了输尿管缝合,保持所涉及结构的完整性。组织病理学证实原发性输尿管血管肉瘤。由于肿块的局部和非侵入性,化疗尚未开始.病人的生存期大约是两年,在此期间保持了正常的肾功能。
    结论:考虑该类型肿瘤在上尿路梗阻性疾病中的鉴别诊断。此外,手术切除非侵入性肿瘤后,保留输尿管和肾脏是一种合适的治疗选择。
    BACKGROUND: This study aims to describe a rare case of primary ureteral hemangiosarcoma, in which surgical intervention preserved the kidney and ureter after tumor removal.
    METHODS: A 13-year-old, neutered male dog, weighing 14 kg, mixed-breed, presented with apathy, anorexia, acute-onset vomiting, and abdominal discomfort during the physical examination. Ultrasonography and pyelography revealed a right-sided dilation of the renal pelvis and ureter due to complete obstruction in the middle third of the ureter. A mass obstructing the lumen of the right ureter was completely resected, and ureteral suturing was performed, preserving the integrity of the involved structures. Histopathology confirmed primary ureteral hemangiosarcoma. Due to the local and non-invasive nature of the mass, chemotherapy was not initiated. The patient\'s survival was approximately two years, and normal renal function was preserved throughout this period.
    CONCLUSIONS: Considering this type of tumor in the differential diagnosis of upper urinary tract obstructive disorders. Furthermore, the preservation of the ureter and kidney is a suitable therapeutic option after surgical resection of non-invasive tumors.
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