Malignant Triton Tumor

  • 文章类型: Case Reports
    背景:恶性Triton肿瘤(MTT)包括恶性外周神经鞘瘤(MPNSTs)的一个亚组,表现出横纹肌肉瘤分化并遵循侵袭性的过程。MTT主要沿着周围神经定位。尚未报道腹壁MTT的病例。MTT的预后比经典MPNSTs差,准确的诊断需要对临床病史和复杂的鉴别诊断知识有敏锐的了解。MTT的治疗反映了MPNST的治疗,主要是手术治疗。
    方法:一名49岁的女性在咨询前3-4个月内出现了下腹壁皮下肿块和预先存在的手术疤痕。在会诊前大约5年,她曾接受过根治性子宫切除术和宫颈癌同步化疗。腹部计算机断层扫描(CT)显示下腹部有1.3厘米的中线肿块,并浸润到腹直肌。无转移征象(T1N0M0)。切开活检发现下腹部零星的MTT。进行了包括腹膜在内的3厘米边缘的全面手术切除。随后,普通外科医生采用了类似于开放式腹膜覆盖网技术的方法。患者接受了进一步的治疗,其形状为微型腹部成形术,用于治疗皮肤缺损。没有出现并发症,每年随访的CTs未显示复发或转移的迹象。
    结论:广泛切除和腹壁重建可有效治疗腹部MTT,消除了术后放疗的需要。
    BACKGROUND: Malignant triton tumors (MTTs) comprise a subgroup of malignant peripheral nerve sheath tumors (MPNSTs) that exhibits rhabdomyosarcomatous differentiation and follow an aggressive course. MTTs are primarily located along peripheral nerves. Cases of MTTs in the abdominal wall have not been reported. MTT has a poorer prognosis than classic MPNSTs, and accurate diagnosis necessitates a keen understanding of the clinical history and knowledge of its differential diagnosis intricacies. Treatment for MTTs mirrors that for MPNSTs and is predominantly surgical.
    METHODS: A 49-year-old woman presented with a subcutaneous mass in her lower abdominal wall and a pre-existing surgical scar that had grown slowly over 3-4 months before the consultation. She had previously undergone radical hysterectomy and concurrent chemo-radiotherapy for cervical cancer approximately 5 years prior to the consultation. Abdominal computed tomography (CT) showed a 1.3 cm midline mass in the lower abdomen with infiltration into the rectus abdominis muscle. There was no sign of metastasis (T1N0M0). An incisional biopsy identified sporadic MTT of the lower abdomen. A comprehensive surgical excision with a 3 cm margin inclusive of the peritoneum was executed. Subsequently, the general surgeon utilized an approach akin to the open peritoneal onlay mesh technique. The patient underwent additional treatment with an excision shaped as a mini-abdominoplasty for the skin defect. No complications arose, and annual follow-up CTs did not show signs of recurrence or metastasis.
    CONCLUSIONS: An abdominal MTT was efficaciously treated with extensive excision and abdominal wall reconstruction, eliminating the need for postoperative radiotherapy.
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  • 文章类型: Case Reports
    心肌炎是指由细菌等非传染性病原体引起的坏死的心肌炎症,真菌,或药物。已知念珠菌在健康和免疫受损的个体中引起心肌炎。由于胰岛素分泌受损或功能低下,糖尿病会导致慢性高血糖。诱导一个妥协的状态,并增加感染各种感染的风险。
    我们报告一例自发性糖尿病托里大鼠念珠菌引起的肉芽肿性心肌炎,非肥胖糖尿病模型。
    一只雄性SDT大鼠,61周龄,被安置在传统环境中。向大鼠提供商业饮食和随意的自来水。对心脏进行取样并制备苏木精-和-eosin-的标本,Sirius-red-,Giemsa-,Grocott染色.组织学上,在左心室壁中观察到大肉芽组织的形成。病灶中心显示坏死。此外,病灶周围和心肌细胞之间的炎性细胞浸润和纤维成分增加。偶尔出现在病灶中的Grocott和Giemsa染色阳性细胞团由于其特征性形式而被认为是念珠菌。
    心肌炎的发展和进展可能与糖尿病引起的损害状态有关。
    UNASSIGNED: Myocarditis refers to myocardial inflammation with necrosis caused by non-infectious of infectious agents such as bacteria, fungi, or drugs. Candida is known to cause myocarditis in healthy and immunocompromised individuals. Diabetes mellitus causes chronic hyperglycemia due to impaired secretion or hypofunction of insulin, induces a compromised state, and increases the risk of contracting various infections.
    UNASSIGNED: We report a case of granulomatous myocarditis caused by Candida in a Spontaneously Diabetic Torii rat, a non-obese diabetic model.
    UNASSIGNED: A male SDT rat, 61 weeks of age, was housed in conventional environment. The rat was provided a commercial diet and tap water ad libitum. The heart was sampled and prepared the specimen of hematoxylin-and-eosin-, Sirius-red-, Giemsa-, Grocott-stain. Histologically, formation of large granulation tissue was observed in the left ventricular wall. A center of the foci showed necrosis. Moreover, inflammatory cells infiltration and fibrous component were increased surrounding the foci and between myocardial cells. A Grocott and Giemsa staining-positive cell masses occasionally appearing in the foci were considered to be Candida because of their characteristic form.
    UNASSIGNED: The development and progression of myocarditis were potentially related to a diabetes-induced compromised state.
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  • 文章类型: Case Reports
    恶性triton肿瘤(MTT)是恶性周围神经鞘瘤(MPNST)的亚型,由周围神经或神经纤维瘤的Schwan细胞发展而来,并显示横纹肌母细胞分化。是一种罕见的软组织肿瘤,预后较差。
    我们报告了一名46岁男性患者的右肩恶性Triton肿瘤(MTT)病例,该患者于2018年6月在侯赛因国王医疗中心皇家康复中心的肌肉骨骼肿瘤诊所就诊。
    患者主诉8个月的进行性右肩疼痛和肩后外侧区肿胀。在这种情况下,准确的诊断至关重要,包括X线和磁共振成像(MRI)在内的研究表明,软组织肿瘤累及右肩区域,从而对侵袭性软组织肿瘤进行了鉴别诊断,并制定了开放切开活检的计划,以组织病理学报告为一例。恶性Triton肿瘤是一种非常罕见且侵袭性的肉瘤,起源于周围神经鞘,因为它是恶性周围神经鞘肿瘤的亚型,此后对整个肿瘤进行了辅助切除,并进行了安全化疗。
    选择的治疗方法是广泛的肿瘤切除,然后进行化疗和/或放疗,以提高5年生存率。
    UNASSIGNED: Malignant triton tumors (MTT) are subtype of malignant peripheral nerve sheath tumor (MPNST) which develop from Schwan cells of peripheral nerves or within neurofibromas, and shows rhabdomyoblastic differentiation. It is a rare soft tissue tumor with poor prognosis.
    UNASSIGNED: We report a case of Malignant Triton Tumor (MTT) arising in the right shoulder in a 46 year old male patient presented to our Musculoskeletal Oncology Clinic at Royal Rehabilitation center at King Hussein Medical Center during June 2018.
    UNASSIGNED: The patient was complaining of an 8 months long progressive right shoulder pain and swelling at the posterior lateral area of the shoulder. As accurate diagnosis is crucial in such case, investigations that included x-rays and magnetic resonance imaging (MRI) demonstrated an soft tissue tumor involving the right shoulder area leading to the differential diagnosis of aggressive soft tissue tumor which laid down the plan of an open incisional biopsy to be reported histopathological as a case of Malignant Triton Tumor which is a very rare and aggressive sarcoma originates from the peripheral nerve sheaths as it is subtype of malignant peripheral nerve sheath tumors after which excision of the entire tumor with safety margin was performed and referred for adjuvant chemotherapy.
    UNASSIGNED: The treatment of choice is radical tumor excision with wide margins followed by chemotherapy and /or radiotherapy to improve the 5 years survival rates.
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  • 文章类型: Review
    恶性Triton肿瘤(MTT)是一种高度侵袭性的恶性肿瘤,分类为具有横纹肌母细胞分化的恶性外周神经鞘瘤的变体。泌尿生殖系统发生MTT的报道很少。在本研究中,我们报告了第一次MTT发生在子宫内。一名57岁的妇女因持续2个月的阴道出血而来到急诊科。妇科触诊发现,阴道存在一个约7cm×3cm×3cm的棍棒状突起。位于子宫下段及子宫颈的肿块经妇科阴道超声及磁共振成像证实,初步诊断为宫颈癌。肿瘤穿刺活检后,病理诊断为恶性triton肿瘤。患者最终失去了随访。这是关于子宫MTT的首次报道,提示病理活检结合影像学检查对于很少的MTT诊断是必要的。
    Malignant triton tumor (MTT) is a highly aggressive malignant neoplasm, classified as a variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. There are few reports that MTT occurred in urogenital system. In the present study, we report the first MTT occurring in the uterus. A 57-year-old woman came to the emergency department due to persistent vaginal bleeding for 2 months. The gynecological palpation found that a club-shaped excrescence existed in the vagina about 7 cm × 3 cm × 3 cm. The mass located in the lower segment of the uterus and the cervix was confirmed by gynecological vaginal ultrasound and magnetic resonance imaging, which was preliminarily diagnosed as cervical carcinoma. After neoplasm punch biopsy, the pathological diagnosis was malignant triton tumor. The patient finally lost follow-up. This is the first report about MTT in the uterus and suggests that pathological biopsy combined with imaging examination is necessary for the diagnosis of rarely MTT.
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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: Journal Article
    背景:恶性Triton肿瘤(MTT)是一种罕见且侵袭性的恶性外周神经鞘瘤,通过局灶性横纹肌母细胞分化在组织学上鉴定。
    方法:一名37岁女性,既往有霍奇金淋巴瘤病史,表现为急性发作性意识错乱,认知缺陷,和弱点。脑磁共振成像显示出血性颅内肿块,后来证实为恶性triton肿瘤。该患者因多次肿瘤复发和转移而接受了两次切除和几个疗程的放化疗。不幸的是,尽管进行了广泛的治疗,她在初次出现这种肿瘤并发症后2年死亡.她的总生存期(OS)为26.7个月,是历史队列报告的两倍(OS〜13mos)。
    结论:中枢神经系统MTT的诊断和治疗是困难的,需要多学科的神经外科医生团队,放射科医生,和肿瘤学家。需要进行组织病理学分析以确认诊断。已证明肿瘤的总切除和辅助放射治疗可使患者获得最高的生存率并改善预后。进一步的研究和临床试验是必要的,以调查化疗药物如替莫唑胺的疗效,贝伐单抗,和abemaciclib。
    BACKGROUND: Malignant triton tumors (MTTs) are a rare and aggressive type of malignant peripheral nerve sheath tumor identified histologically by focal rhabdomyoblastic differentiation.
    METHODS: A 37-year-old female with a prior history of Hodgkin lymphoma presented with acute-onset confusion, cognitive deficits, and weakness. Brain magnetic resonance imaging revealed a hemorrhagic intracranial mass later confirmed to be a malignant triton tumor. The patient underwent two resections and several courses of chemoradiation for multiple tumor recurrences and metastases. Unfortunately, despite extensive treatment, she died 2 years after initial presentation from complications of this tumor. Her overall survival (OS) of 26.7 months was double that reported in historical cohorts (OS ∼13 mos).
    CONCLUSIONS: The diagnosis and treatment of central nervous system MTTs are difficult and require a multidisciplinary team of neurosurgeons, radiologists, and oncologists. Histopathological analysis is required for confirmation of diagnosis. Gross-total resection of tumor and adjuvant radiation therapy have been shown to give patients the highest rates of survival and improved outcomes. Further studies and clinical trials are warranted to investigate the efficacy of chemotherapeutic agents like temozolomide, bevacizumab, and abemaciclib.
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  • 文章类型: Case Reports
    恶性Triton肿瘤(MTT)是一种罕见且侵袭性的恶性外周神经鞘瘤(MPNSTs)亚型,常伴有1型神经纤维瘤病。此病例报告描述了一名33岁男性的颈动脉鞘内复发性散发性MTT的独特实例,该男性没有任何个人或家族性神经纤维瘤病病史。患者最初在右颈部进行了活检证实的MTT,涉及颈动脉体和臂丛神经,接受了部分切除,放射治疗,和化疗。六个月后,患者出现复发性MTT,随后接受了根治性肿瘤切除术,节段性右颈动脉切除术,和股深静脉介入。恢复并发血肿形成,患者出现声带麻痹和左声带囊肿,需要进一步的手术。每年随访8年,未发现复发。这个案例强调了全面患者评估的重要性,包括临床病史,成像,和活检结果,准确的诊断和及时的手术干预治疗这种罕见和侵袭性肿瘤。需要进一步的研究来确定新疗法并提高MTT患者的生存率。
    Malignant Triton Tumors (MTTs) are a rare and aggressive subtype of malignant peripheral nerve sheath tumors (MPNSTs), often associated with neurofibromatosis type 1. This case report describes a unique instance of recurrent sporadic MTT within the carotid sheath in a 33-year-old male without any personal or familial history of neurofibromatosis. The patient initially presented with a biopsy-confirmed MTT in the right neck, involving the carotid body and brachial plexus, and underwent partial resection, radiation therapy, and chemotherapy. Six months later, the patient presented with recurrent MTT, and subsequently underwent radical tumor resection, segmental right carotid artery resection, and deep femoral vein interposition. Recovery was complicated by hematoma formation, and the patient developed vocal fold paralysis and a left vocal fold cyst, necessitating further surgeries. Yearly follow-ups for 8 years revealed no recurrence. This case emphasizes the importance of comprehensive patient evaluation, including clinical history, imaging, and biopsy findings, for accurate diagnosis and prompt surgical intervention in managing such rare and aggressive tumors. Further research is needed to identify novel therapies and improve survival rates for patients with MTTs.
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  • 文章类型: Case Reports
    恶性\'triton\'肿瘤是恶性外周神经鞘瘤的一种极其罕见的亚型。恶性triton肿瘤由于其发病率低,知识缺乏,在手术前临床诊断困难。因此,描述和总结恶性肿瘤的CT影像学特征对早期和术前诊断有很大帮助。
    术前密切观察2例经CT扫描疑似MTT的病例。最终通过免疫化学检测证实了恶性Triton肿瘤的诊断。证实了CT扫描的推测.巨大的,不规则,通过CT扫描可以从这些患者中观察到界限清楚的分叶状团块样阴影。此外,通过CT扫描可以很好地确定肿瘤体内密度的异质性,与线性隔膜一起。同时,CT扫描显示肿瘤体边缘有明显的钙化。
    两个病例的一些CT图像特征作为MTT术前考虑的参考:(i)巨大的团块样阴影;(ii)存在明确的小叶状;(iii)明确定义的肿块内的隔膜伴有出血,坏死和囊性改变以及钙化,特别是在1型神经纤维瘤病患者中。
    Malignant \'triton\' tumor is an extremely rare subtype of malignant periphery nerve sheath tumors. Clinical diagnosis of malignant triton tumor is difficult before surgery due to its low incidence and the lack of knowledge. Therefore, to describe and summarize the CT imaging characteristics of malignant triton tumor is of great assistance for early and preoperative diagnosis.
    Two cases suspected of MTT by CT scan before operation were closely observed. The diagnosis of malignant triton tumor was eventually confirmed by immunochemical assay, which verified speculation of CT scans. Huge, irregular, well-circumscribed lobulated mass-like shadows can be observed from these patients by CT scans. Besides, heterogeneity of density within the body of tumor was well-established by CT scans, together with linear septum. Meanwhile, CT scans demonstrated that calcifications were remarkable at the margin of tumor body.
    Some CT image features from two cases were presented as a reference for the preoperative consideration of MTT: (i) enormity of mass-like shadow; (ii) presence of well-circumscribed lobulated shape; (iii) septum within the well-defined mass accompanied with hemorrhage, necrosis and cystic changes as well as calcification, especially within neurofibromatosis type 1 patients.
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  • 文章类型: Review
    暂无摘要。
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  • 文章类型: Case Reports
    这是一例在十二指肠中产生的恶性triton肿瘤,该肿瘤通过胰十二指肠切除术切除。一名62岁的女性在13年前曾因十二指肠恶性Triton肿瘤手术史的高血压随访期间,在常规血液检查中发现了肝和胆囊功能障碍。进一步检查发现转移性肝肿瘤起源于十二指肠的恶性triton肿瘤。由于放射治疗后检测到肝肿瘤的进展,通过右肝切除术完成完整切除。在一种极为罕见的十二指肠恶性triton肿瘤引起的肝转移病例中,根治性肝切除术可实现1年零5个月的无病生存。
    This is an additional case report of a malignant triton tumor arising in the duodenum that was removed by pancreatoduodenectomy. Liver and gallbladder dysfunctions were detected in a regular blood examination during a follow-up for hypertension in a 62-year-old woman with a previous surgical history for a malignant Triton tumor in the duodenum 13 years ago. Further examinations revealed a metastatic liver tumor originating from the malignant triton tumor in the duodenum. Since the progression of the liver tumor was detected after radiation therapy, complete resection was performed by right hepatectomy. Curative hepatectomy resulted in disease-free survival for 1 year and 5 months in an extremely rare case of liver metastasis derived from a malignant triton tumor in the duodenum.
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