soft tissue tumour

软组织肿瘤
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    文章类型: Case Reports
    平滑肌瘤和神经鞘瘤都是罕见的良性软组织肿瘤。平滑肌瘤更常见于下肢,而不是上肢,而神经鞘瘤是罕见的周围神经鞘瘤,可以发生在不同的解剖区域。然而,它们很少发生在隐神经。该病例研究介绍了一名41岁的女性患者,其孤立性肿块病变位于下肢前内侧软组织深处。体检显示,弹性硬,流动和非招标群众。磁共振成像(MRI)在对比增强的T1加权切片上显示椭圆形皮下肿块。最初的MRI图像显示神经鞘瘤,但在完全摘除后,该肿瘤后来被证实为平滑肌瘤。进行了免疫组织化学研究以进行鉴别诊断。下肢孤立性肿块性病变可被误认为是各种类型的肿瘤,并被误诊,需要进行组织病理学检查和良好的影像学检查以进行鉴别诊断。完整的手术切除通常是平滑肌瘤的安全有效的治疗方法。
    Leiomyomas and schwannomas are both types of rare benign soft tissue tumours. Leiomyomas are more commonly found in the lower limbs than in the upper extremities, while schwannomas are rare peripheral nerve sheath tumours that can occur in different anatomical regions. However, they rarely occur in the saphenous nerve. This case study presents a 41-year-old female patient with a solitary mass lesion located deep in the soft tissue of the anteromedial lower extremity. The physical examination revealed a palpable, elastic-hard, mobile and non-tender mass. Magnetic resonance imaging (MRI) showed an oval-shaped subcutaneous mass on contrast-enhanced T1-weighted sections. The initial MRI images suggested a schwannoma, but the tumour was later confirmed to be a leiomyoma after total enucleation. An immunohistochemical study was performed for differential diagnosis. Solitary mass lesions in the lower extremities can be mistaken for various types of tumours and misdiagnosed and require histopathological examination and good radiological imaging for differential diagnosis. Complete surgical excision is usually a safe and effective treatment for leiomyomas.
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  • 文章类型: Case Reports
    诊断头部和颈部区域的软组织肿瘤可能由于其复杂的解剖结构和多样的组织学谱而具有挑战性。此病例报告重点介绍了一名妇女的病例,该妇女在颈部后三角形出现无痛的颈部肿块。各种病理和影像学检查提示多形性腺瘤,它来自左侧椎前空间。患者通过经宫颈入路进行了完整的手术切除。颈部后三角形的多形性腺瘤极为罕见,在处理颈部软组织肿瘤时面临诊断困境。
    Diagnosing a soft tissue tumor in the head and neck region can be challenging due to its complex anatomy and diverse histological spectrum. This case report highlights the case of a woman who presented with a painless neck lump in the posterior triangle of the neck. Various pathological and imaging studies were suggestive of pleomorphic adenoma, which arises from the left prevertebral space. The patient underwent complete surgical excision via the transcervical approach. Pleomorphic adenoma in the posterior triangle of the neck is extremely rare and causes a diagnostic dilemma in managing soft tissue tumors of the neck.
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  • 文章类型: Journal Article
    在黑色素瘤(PRAME)中优先表达抗原,癌症/睾丸抗原家族的成员,是皮肤癌诊断和治疗领域的核心。作为核受体和转录调节因子,PRAME在抑制视黄酸信号中起关键作用,这对细胞分化和增殖至关重要。其在各种恶性肿瘤中的异常过表达,特别是皮肤黑色素瘤,与更具侵袭性的肿瘤表型有关,将PRAME定位为诊断和预后标志物。在黑色素瘤中,PRAME通常高度表达,与其在良性痣中的弱表达或缺失相反,从而提高鉴别诊断的准确性。PRAME的诊断价值扩展到各种病变。它在葡萄膜黑色素瘤中显著表达,与转移风险增加有关。在肢端黑色素瘤中,尤其是那些组织病理学模糊的人,PRAME有助于提高诊断准确性。然而,其在皮囊样和指甲黑素细胞病变中的表达不一致,需要全面的方法进行准确评估。在软组织肉瘤中,PRAME可能特别有助于区分黑色素瘤和透明细胞肉瘤,一个重要的区别,由于它们相似的组织学外观,但不同的治疗方法和预后,或检测去分化和未分化的黑素瘤。在非黑色素瘤皮肤癌如基底细胞癌,鳞状细胞癌,和默克尔细胞癌,PRAME的可变表达可导致诊断复杂性。尽管面临这些挑战,PRAME作为黑色素瘤治疗靶点的潜力是显著的.新兴的免疫疗法,包括针对PRAME的T细胞疗法和疫苗,正在研究利用其癌症特异性表达。对PRAME在皮肤癌中的分子作用和作用机制的持续研究继续为诊断和治疗开辟新的途径。有可能改变黑色素瘤和相关皮肤癌的管理。
    Preferentially Expressed Antigen in Melanoma (PRAME), a member of the cancer/testis antigen family, is central to the field of skin cancer diagnostics and therapeutics. As a nuclear receptor and transcriptional regulator, PRAME plays a critical role in inhibiting retinoic acid signalling, which is essential for cell differentiation and proliferation. Its aberrant overexpression in various malignancies, particularly cutaneous melanoma, is associated with more aggressive tumour phenotypes, positioning PRAME as both a diagnostic and prognostic marker. In melanoma, PRAME is typically highly expressed, in contrast to its weak or absent expression in benign nevi, thereby improving the accuracy of differential diagnoses. The diagnostic value of PRAME extends to various lesions. It is significantly expressed in uveal melanoma, correlating to an increased risk of metastasis. In acral melanomas, especially those with histopathological ambiguity, PRAME helps to improve diagnostic accuracy. However, its expression in spitzoid and ungual melanocytic lesions is inconsistent and requires a comprehensive approach for an accurate assessment. In soft tissue sarcomas, PRAME may be particularly helpful in differentiating melanoma from clear cell sarcoma, an important distinction due to their similar histological appearance but different treatment approaches and prognosis, or in detecting dedifferentiated and undifferentiated melanomas. In non-melanoma skin cancers such as basal cell carcinoma, squamous cell carcinoma, and Merkel cell carcinoma, the variable expression of PRAME can lead to diagnostic complexity. Despite these challenges, the potential of PRAME as a therapeutic target in melanoma is significant. Emerging immunotherapies, including T-cell-based therapies and vaccines targeting PRAME, are being investigated to exploit its cancer-specific expression. Ongoing research into the molecular role and mechanism of action of PRAME in skin cancer continues to open new avenues in both diagnostics and therapeutics, with the potential to transform the management of melanoma and related skin cancers.
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  • 文章类型: Case Reports
    多发性遗传性外生体综合征是一种罕见的诊断,男性发病率约为1:50000。外生骨或骨软骨瘤是良性的,但有1-5%的恶性转化潜力。有很强的遗传成分,外生症(EXT)信号通路是一个根本原因。它们可能是有症状的,以疼痛和功能缺陷为主要主诉。我们介绍了一例17岁的男性,他的左股骨下部出现疼痛和解剖畸形。磁共振成像显示多个骨软骨瘤压迫the神经血管束。以多学科方法对骨软骨瘤进行切除术以减压神经血管束。组织学检查未显示恶性肿瘤。目前,对于诊断为多发性骨软骨瘤的患者,在进一步检查和/或筛查恶性转化方面尚无共识.
    Multiple hereditary exostosis syndrome is a rare diagnosis with approximately 1:50000 incidence prevailing in males. The exostoses or osteochondromas are benign but have the potential for malignant transformation in 1-5%. There is a strong genetic component, with exostosis (EXT) signaling pathways being an underlying cause. They can be symptomatic, with pain and functional deficit as the main complaints. We present a case of a 17-year-old male who presented with pain and anatomical deformity in his left lower femur. Magnetic resonance imaging revealed multiple osteochondromas compressing the popliteal neurovascular bundle. Excision of the osteochondromas was performed to decompress the neurovascular bundle in a multidisciplinary approach. Histological examination demonstrated no evidence of malignancy. Currently, there is no consensus for patients diagnosed with multiple osteochondromas regarding further investigation and/or screening for malignant transformation.
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  • 文章类型: Journal Article
    粘液纤维肉瘤(MFS)是一种软组织肉瘤,通常发生在成年后期。它主要位于四肢的皮下软组织中,其特征在于原始部位的高复发率。头部和颈部的MFS很少见,而它在上颌骨的发生极为罕见。我们报告了一名29岁男性上颌骨MFS的非典型病例。切除肿瘤的边缘足够,随后进行术后辅助放疗。到目前为止,该患者已经被随访了2年,并且一直没有疾病。稀有,病理学的侵略性,肿瘤的范围和靠近该部位的复杂神经血管结构通常会导致不良后果。我们将讨论一例罕见的病例,该病例是一名年轻的患者,该患者有辐射暴露史,这对诊断提出了挑战。我们的病例可能会为管理上颌窦粘液纤维肉瘤提供更多的诊断和治疗经验。
    Myxofibrosarcoma (MFS) is a soft tissue sarcoma that commonly occurs in late adult life. It is mainly located in the subcutaneous soft tissues of extremities characterized by a high recurrence rate at the original site. MFS of the head and neck is rare, while it occurrence in the maxilla is extremely rare. We report an atypical case of MFS of the maxilla in a 29-year-old male. The tumour was resected with adequate margin and following which post-operative adjuvant radiotherapy was given. This patient has been followed for 2 years to date and has remained disease free. The rarity, the aggressive nature of the pathology, the extent of the tumour and the complex neurovascular structures in close proximity to the site often lead to adverse outcomes. We will be discussing a rare case of a rapidly growing high grade maxillary sinus MFS in a young patient with a history of radiation exposure which posed as a diagnostic challenge. Our case may provide additional diagnostic and treatment experience with regards to managing maxillary sinus myxofibrosarcoma.
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  • 文章类型: Case Reports
    Low-grade fibromyxoid sarcoma (LGFMS) is a soft tissue neoplasm that occurs preferentially in young, male adults as a slowly growing, asymptomatic mass. According to current literature, the most common anatomical sites where it occurs are the trunk and lower extremities, especially the thigh, perineum, and groin. The risk factors are still unknown. Surgical intervention (simple resection and wide excision) is nowadays considered the best treatment option; however, patients require a long follow-up due to the high recurrence and metastasis rates. We present a low-grade fibromyxoid sarcoma case located in the abdominal wall of a female Hispanic patient.
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  • 文章类型: Journal Article
    骨瘤是良性的,生长缓慢,分化肿瘤,主要位于颌面部骨骼区域。涉及没有骨附着的软组织的骨瘤是非常罕见的事件。一名68岁的女性,患有合并症(II型糖尿病,原发性高血压,原位起搏器)在她的右手掌的大鱼际区域出现无痛的固体肿块,这出现在大约1年半前,并在过去几个月显示出逐步扩大。在区域麻醉下,进行了切除活检,病变的组织病理学评估证实了软组织骨瘤的诊断。术后随访顺利,无任何并发症。
    Osteomas are benign, slow-growing, differentiated tumours, which are primarily located in the region of maxillofacial skeleton. Osteoma involving the soft tissues with no bony attachments is a very rare event. A 68-year-old woman with comorbidities (diabetes mellitus type II, primary hypertension, pacemaker in situ) presented with a painless solid mass in the thenar region of her right palm, which appeared almost 1 and half years ago and showed a progressive enlargement in the last few months. Under regional anaesthesia, an excisional biopsy was performed and the histopathological evaluation of the lesion confirmed the diagnosis of soft tissue osteoma. The postoperative follow-up period was uneventful without any complication.
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  • 文章类型: Journal Article
    成像在表征和描绘软组织肿瘤的范围方面至关重要,这方面有大量文献。需要一种简化的方法来表征MR上的病变,我们描述了一种简化的街道智能方法,称为SLAM(信号,location,年龄,多重性和矩阵)。
    Imaging is vital in characterising and delineating the extent of soft tissue tumours and there is abundant literature on this. A simplified approach is required to characterise the lesions on MR and we describe a simplified street-smart approach called SLAM (signal, location, age, multiplicity and matrix).
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  • 文章类型: Journal Article
    OBJECTIVE: To determine the efficacy of image-guided core needle biopsy (IGCNB) in patients presenting with suspected intra-articular soft tissue tumours or tumour-like lesions.
    METHODS: Retrospective study of patients referred to a musculoskeletal oncology service between January 2019 and May 2020 with a suspected intra-articular soft tissue tumour over a 16-month period. Data collected included patient age, gender, joint involved and maximal lesion size. Type of image-guidance (ultrasound or computed tomography), type of needle and type of anaesthesia, general anaesthetic (GA) or local anaesthetic (LA), were recorded, as was the histological diagnosis. For patients who proceeded to surgical excision, the IGCNB histology result was correlated with resection histology. Descriptive statistics were used and complications were also noted.
    RESULTS: By the termination of data collection 91 patients underwent IGCNB, 32 (35.2 %) males and 59 (64.8 %) females with a mean age of 41.4 years (age range 3-86 years). The joints involved were the knee (n = 73; 80.2 %), ankle (n = 12; 13.2 %), hip (n = 3; 3.3 %), shoulder (n = 1; 1.1 %), elbow (n = 1; 1.1 %) and wrist (n = 1; 1.1 %). Biopsy types were as follows: US-guided GA (n = 29; 31.9 %), US-guided LA (n = 37; 40.7 %), CT-guided GA, (n = 23; 25.3 %), CT-guided LA (n = 2; 2.2 %). Mean maximal tumour dimension for 76 focal lesions was 36.5 mm (range 18-113 mm). IGCNB yielded a definitive histological result in 85 of 91 cases (93.4 %), 44 of whom went on to surgical resection. Concordance between IGCNB and resection histology was achieved in 42 of 44 cases (95.5 %). The commonest diagnosis was tenosynovial giant cell tumour, with only a single malignant lesion identified. There were no recorded immediate or delayed complications.
    CONCLUSIONS: IGCNB of suspected intra-articular tumours or tumour-like lesions is a highly effective and safe technique.
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  • 文章类型: Case Reports
    Cutaneous angiosarcoma (cAS) is a rare and aggressive malignant vascular tumor, which mostly occurs in the head and neck region. The outcome of cAS is poor and timely diagnosis is paramount, but often delayed because of the slow onset and the variance in presentation. This paper reports on a case of an 88-year old woman who presented with a persisting \"hematoma\" in the left retro-auricular region. Although considered at initial differential diagnosis, no signs of malignancy were identified in histopathology and imaging in the diagnostic work-up. At first, short-term follow-up showed no progression of the lesion. But 3 months after the first presentation additional biopsies were taken, because of rapid expansion of the lesion. The initial histopathological findings were most consistent with a benign vascular lesion, with signs of hemorrhage and reactive inflammation. However, the additional immunohistochemical analysis showed the presence of MYC oncoprotein, which confirmed the clinical suspicion of angiosarcoma. Because size and location of the lesion rendered complete resection unattainable, radiotherapy was commenced, but no significant volume reduction could be achieved. Therefore, palliative irradiation was initiated. The patient passed away 1 month later. Clinical diagnosis is often difficult and little is known about imaging of cAS. Histology and immunohistochemistry can be misleading, as cAS are easily mistaken for other lesions. Most studies report that multimodality treatment with surgery and radiotherapy is preferable, but this can be challenging in the head & neck region.
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