Malignant degeneration

恶性变性
  • 文章类型: Journal Article
    背景:多发性骨软骨瘤是一种遗传性疾病,其特征是形成多个良性软骨覆盖的骨肿瘤,骨软骨瘤,在骨骼发育过程中。最可怕的并发症是继发性外周软骨肉瘤,由先前存在的骨软骨瘤的软骨帽引起的恶性软骨瘤。我们对1960年至2019年诊断和随访的患者进行了一项回顾性队列研究,以描述多发性骨软骨瘤受周围软骨肉瘤影响的个体的临床和病理特征。评估随访信息和个体结局,并将结果与文献进行比较。数据,包括年龄,性别,site,组织学分级,软骨帽厚度,手术治疗,手术切缘,我们从医院电子健康记录和多发性骨软骨瘤登记处获取了基因型突变状态和治疗细节.此外,病理学家对所有苏木精和伊红(H&E)染色切片进行了完整的组织学检查.
    结果:本研究纳入了105例筛查病例。诊断SPC的年龄范围为13至63岁,诊断时的中位年龄为34岁。最常受到恶性变性影响的部位是骨盆(46例,44%),男性患者发病率较高(男性32例,女性14例)。第二个是下肢(包括股骨,腓骨,或胫骨),在35名患者中确定。组织学信息-可用于103名患者-显示:59名患者为1级;40名患者为2级,4名患者为3级。最常见的手术治疗是完全切除,接着是剔除,截肢和部分切除。大多数病例没有复发。无病生存的结果突出表明,病程较差与组织学2级或3级以及部分切除手术有关。在大多数分析病例(94%)中,发现了致病变异。
    结论:结论:本研究概述了继发性外周软骨肉瘤,确认这种疾病代表了多个骨软骨瘤患者的影响并发症,并表明恶性转化也可能发生在年轻患者中,在一些并非无关紧要的案件中。
    BACKGROUND: Multiple osteochondromas is genetic disorder characterized by the formation of multiple benign cartilage-capped bone tumors, named osteochondromas, during skeletal development. The most feared complication is the secondary peripheral chondrosarcoma, a malignant cartilaginous neoplasm that arises from the chondroid cap of pre-existent osteochondromas. We conducted a retrospective cohort study on patients diagnosed and followed up from 1960 to 2019 to describe the clinical and pathological features of individuals affected by peripheral chondrosarcoma in multiple osteochondromas, to evaluate follow up information and individual outcome and to compare the results with literature. Data, including age, gender, site, histological grade, cartilage cap thickness, surgical treatments, surgical margins, genotype mutational status as well as treatment details were captured from the hospital electronic health records and from Registry of Multiple Osteochondromas. In addition, a complete histological review of all hematoxylin and eosin (H&E)-stained sections has been performed by expert pathologists.
    RESULTS: One hundred five of the screened cases were included in the present study. The age at diagnosis of SPC ranges from 13 to 63, with median age at diagnosis of 34 years. The site most frequently affected by malignant degeneration was the pelvis (46 patients, 44%) with higher incidence in male patients (32 males vs.14 females). The second one was lower limbs (including femur, fibula, or tibia), identified in 35 patients. Histological information - available for 103 patients - showed: 59 patients with grade 1; 40 patients had a grade 2 and 4 patients had a grade 3. The most common surgical treatment was the complete resection, followed by debulking, amputation and partial resection. Most of cases did not have recurrence of the disease. Outcome in disease-free survival highlights that a worse course of the disease was associated with histological grade 2 or 3, and partial resection surgery. In most of analyzed cases (94%) a pathogenic variant was identified.
    CONCLUSIONS: In conclusion, the present study gives an overview of the secondary peripheral chondrosarcomas, confirming that this disease represents an impacting complication for multiple osteochondromas patients and suggests that malignant transformation can occur also in younger patient, in a not irrelevant number of cases.
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  • 文章类型: Case Reports
    持续性苗勒管综合征(PMDS)是一种罕见的常染色体隐性遗传性性发育障碍,其特征是存在输卵管,XY基因型和正常男性表型的个体的子宫和阴道上部三分之一。PMDS的主要并发症是不育以及睾丸和穆勒衍生物恶性变性的罕见风险。我们报道了一个49岁的男人,在修补切口疝的过程中,偶然发现膀胱后方有子宫样结构。在过去的18个月大的时候,他因双侧隐睾而接受了双侧睾丸固定术。术中决定保留子宫样结构,并在术后做出更准确的诊断。评估显示XY染色体和成像与PMDS一致。患者被告知残余Müller导管肿瘤转化的风险,并接受了手术治疗。他拒绝了。随后的随访影像学研究,包括睾丸和盆腔超声,提示恶性睾丸和苗勒氏衍生变性的发现均为阴性。对国际文献的回顾表明,当决定删除Mullerian导数时,腹腔镜检查,尤其是机器人手术可以成功切除穆勒衍生物。每当无法移除这些结构或患者拒绝接受手术时,有必要告知患者需要进行充分的随访。患者应定期进行骨盆成像检查,MRI可能是实现此目的的更好方法。
    Persistent Müllerian Duct Syndrome (PMDS) is a rare autosomal recessive disorder of sex development characterized by the presence of fallopian tubes, uterus and upper one-third of the vagina in individuals with XY genotype and normal male phenotype. The main complications of PMDS are infertility and the rare risk of malignant degeneration of both testicular and Müllerian derivatives. We report the case of a 49-year-old man who, during repair of an incisional hernia, was incidentally found to have a uterine-like structure posterior to the bladder. In the past at the age of 18 months, he had undergone bilateral orchidopexies for bilateral cryptorchidism. The intraoperative decision was to preserve the uterine-like structure and make a more accurate diagnosis postoperatively. Evaluation revealed an XY chromosome and imaging consistent with PMDS. The patient was informed about the risk of neoplastic transformation of the residual Müller ducts and was offered surgical treatment, which he declined. Subsequent follow-up imaging studies, including testicular and pelvic ultrasound, were negative for findings suggestive of malignant testicular and Mullerian derivative degeneration. A review of the international literature showed that, when a decision is taken to remove the Mullerian derivatives, laparoscopy and especially robotic surgery allow for the successful removal of Müllerian derivatives. Whenever the removal of these structures is not possible or the patient refuses to undergo surgery, it is necessary to inform the patient of the need for adequate follow-up. Patients should undergo regular pelvic imaging examination and MRI might be a better method for that purpose.
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  • 文章类型: Journal Article
    背景:分支导管-导管内乳头状粘液性肿瘤(BD-IPMNs)是最常见的胰腺囊性肿瘤,恶性转化的风险较低。缺乏能够早期识别高风险BD-IPMN的功能,和指南目前依赖于令人担忧的特征(WF)和高风险污名(HRS)的发生。
    目的:在我们的研究中,我们的目的是使用磁共振成像(MRI)影像组学模型来识别与恶性变性高风险相关的特征,以及这些是否出现在WF和HRS发生之前。
    方法:我们回顾性评估了已知BD-IPMN的成年患者,这些患者在我们中心进行了至少两次对比增强MRI研究,并进行了24个月的最短随访时间。两次检查的MRI采集协议包括造影前和造影后阶段以及扩散加权成像(DWI)/表观扩散系数(ADC)图。根据随访结束时WF或HRS的发展将患者分为两组(第0组=无WF或HRS;第1组=WF或HRS)。我们对MRI图像进行分割,提取并比较两组之间的定量特征。然后将显示显着差异(SF)的特征包括在LASSO回归方法中,以建立基于放射学的预测模型。
    结果:我们纳入了50例患者:0组31例,1组19例。该队列中没有患者发生HRS。在基线,确定了47、67、38和68SF用于对比前T1加权(T1-W)序列,对比后T1-W序列,T2加权(T2-W)序列,和ADC图,分别。在后续行动结束时,我们发现了69、78、53和91SF,分别。基于放射学的预测模型确定了16SF:更具体地说,5SF用于预对比T1-W序列,6用于对比后T1-W序列,3对于T2-W序列,和2为ADC。
    结论:我们在接受对比增强MRI的BD-IPMNs患者中发现了与WF显著相关的影像学特征。我们基于MRI的影像组学模型可以预测WF的发生。
    BACKGROUND: Branch duct-intraductal papillary mucinous neoplasms (BD-IPMNs) are the most common pancreatic cystic tumors and have a low risk of malignant transformation. Features able to early identify high-risk BD-IPMNs are lacking, and guidelines currently rely on the occurrence of worrisome features (WF) and high-risk stigmata (HRS).
    OBJECTIVE: In our study, we aimed to use a magnetic resonance imaging (MRI) radiomic model to identify features linked to a higher risk of malignant degeneration, and whether these appear before the occurrence of WF and HRS.
    METHODS: We retrospectively evaluated adult patients with a known BD-IPMN who had had at least two contrast-enhanced MRI studies at our center and a 24-month minimum follow-up time. MRI acquisition protocol for the two examinations included pre- and post-contrast phases and diffusion-weighted imaging (DWI)/apparent diffusion coefficient (ADC) map. Patients were divided into two groups according to the development of WF or HRS at the end of the follow-up (Group 0 = no WF or HRS; Group 1 = WF or HRS). We segmented the MRI images and quantitative features were extracted and compared between the two groups. Features that showed significant differences (SF) were then included in a LASSO regression method to build a radiomic-based predictive model.
    RESULTS: We included 50 patients: 31 in Group 0 and 19 in Group 1. No patients in this cohort developed HRS. At baseline, 47, 67, 38, and 68 SF were identified for pre-contrast T1-weighted (T1-W) sequence, post-contrast T1-W sequence, T2-weighted (T2- W) sequence, and ADC map, respectively. At the end of follow-up, we found 69, 78, 53, and 91 SF, respectively. The radiomic-based predictive model identified 16 SF: more particularly, 5 SF for pre-contrast T1-W sequence, 6 for post-contrast T1-W sequence, 3 for T2-W sequence, and 2 for ADC.
    CONCLUSIONS: We identified radiomic features that correlate significantly with WF in patients with BD-IPMNs undergoing contrast-enhanced MRI. Our MRI-based radiomic model can predict the occurrence of WF.
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  • 文章类型: Journal Article
    未经证实:虽然癌变是罕见的,一些作者支持在取毛囊肿切除后需要进行组织病理学检查。今天,微创技术广泛用于治疗藏毛窦疾病,但与标准程序相反,由于没有标本,这些技术无法进行组织病理学检查。这项由两机构组成的研究的目的是评估在内窥镜消融囊肿后是否可以成功进行藏毛窦切除材料的组织病理学检查。
    UNASSIGNED:我们确定了2021年1月至2021年9月所有连续诊断为毛发窦疾病的患者,这些患者接受了视频辅助的毛发窦消融(VAAPS),然后进行组织病理学检查。
    UNASSIGNED:本研究共纳入45例患者。所有患者均为白种人,年龄在50岁以下。其中9人因PSD复发而接受了手术。在藏毛窦采样材料的组织病理学检查中未发现恶性肿瘤的证据。
    UNASSIGNED:我们能够发送藏毛窦取样材料,对所有接受微创技术治疗藏毛窦病的患者进行组织病理学检查。在45个样本中没有发现恶性肿瘤的证据。我们的发现证明,对藏毛窦进行微创消融并不排除对囊肿进行组织病理学检查。
    UNASSIGNED: Although carcinomatous degeneration is a rare occurrence, some authors support the need for a histopathological examination after pilonidal cyst excision. Today, minimally invasive techniques are widely spread for the treatment of pilonidal sinus disease but opposed to standard procedures, these techniques could not allow to perform a histopathological examination because of the absence of a specimen. The aim of this two-institutions study is to evaluate whether histopathological examination of the pilonidal sinus excision material can be successfully performed after an endoscopic ablation of the cyst.
    UNASSIGNED: We identified all consecutive patients from January 2021 to September 2021 with diagnosis of pilonidal sinus disease who underwent Video Assisted Ablation of Pilonidal Sinus (VAAPS) followed by histopathological examination.
    UNASSIGNED: A total of 45 patients were included in the study. All patients were Caucasians and aged below 50 years. Nine of them underwent surgery due to recurrence of PSD. No evidence of malignancy was detected in the histopathological examination of the pilonidal sinus sampling material.
    UNASSIGNED: We were able to send pilonidal sinus sampling material for a histopathological examination in all patients who underwent minimally invasive technique for the treatment of pilonidal sinus disease. No evidence of malignancy was found in any of the 45 samples. Our findings prove that minimally invasive ablation of pilonidal sinus does not preclude histopathological examination of the cysts.
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  • 文章类型: Journal Article
    慢性炎症,长期参与恶性肿瘤的发生,现在据了解,估计占所有癌症的25%。最相关的恶性肿瘤,整形外科医生,与慢性炎症变性相关的包括马乔林溃疡,乳房植入物相关大细胞淋巴瘤,辐射诱导的肉瘤,和卡波西肉瘤.由延长的炎症反应引起的细胞和遗传损伤由越来越了解的细胞动力学系统控制。在理解慢性炎症级联方面的进展已经产生了新的疗法和治疗靶标。
    Chronic inflammation, long implicated in the genesis of malignancy, is now understood to underlie an estimated 25% of all cancers. The most pertinent malignancies, to the plastic surgeon, associated with the degeneration of chronic inflammation include Marjolin\'s ulcer, breast implant-associated large cell lymphoma, radiation-induced sarcoma, and Kaposi\'s sarcoma. The cellular and genetic damage incurred by a prolonged inflammatory reaction is controlled by an increasingly understood cytokinetic system. Advances in understanding the chronic inflammatory cascade have yielded new therapeutics and therapeutic targets.
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  • 文章类型: Journal Article
    BACKGROUND: Although pilonidal sinus disease is common, development of associated malignancy is very rare. After surgical treatment, most surgeons send the excision material for a histopathological examination. The aim of this study was to examine whether it is necessary to routinely send the pilonidal sinus surgical excision material for this examination.
    METHODS: The data of 3146 patients were retrospectively screened, and 2486 patients with available histopathological reports of the excision material were included in the study.
    RESULTS: Of the 2486 patients included in the study, 2165 were men and 321 were women, and 94.7% of the patients were under the age of 50 years while 5.3% were 50 years or above. The rate of patients who underwent surgery due to recurrence was 1.2%. No malignancy was detected in any patient after the histopathological examination.
    CONCLUSIONS: In this study, none of the pathology results was reported as malignant. This confirms that it is necessary to ask the question whether we should routinely send the surgical excision material for a histopathological examination.
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  • 文章类型: Case Reports
    “马乔林溃疡”被称为古代烧伤疤痕的恶性变性,但是这两个词都可能导致误诊。瘢痕组织的恶性变性可以发生,并且可以在临床上有所不同,组织学表现及其预先存在的皮肤病变。我们提出了几个案例来证实我们的观察结果。“马乔林溃疡”并不是老烧伤伤疤上出现的溃疡的同义词,转化为鳞状细胞癌。
    \"Marjolin\'s ulcer\" is known as malignant degeneration of ancient burn scars, but both words can induce misdiagnosis. Malignant degeneration of scarring tissue can occur and can vary in its clinical, histological manifestations and its pre-existing skin lesions. We present several cases to substantiate our observations. \"Marjolin\'s ulcer\" is not synonym to an ulceration appeared on an old burn scar, transformed into squamous cell carcinoma.
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  • 文章类型: Case Reports
    Epitheloide angiomyolipoma (EAML) is a very rare type of benign mesenchymal angiomyolipoma. In contrast to classical angiomylipoma, lymph node metastases, local recurrence and distant metastases occur in one third of patients with EAML. We report the case of a 49-year-old patient with a large recurrence of EAML of the left kidney. According to the literature, this is the first case of a malignant EAML with local recurrence in Germany.
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  • 文章类型: Journal Article
    Hidradenitis suppurativa (acne inversa) is a chronic inflammatory follicular skin disease which leads to fistulae, abscesses and scarring. The Hurley classification is used to quantify the extent of the disease whereby stage III is associated with extensive disfiguring lesions. The most frequently used conservative treatment options are topical clindamycin, systemic antibiotics, e.g. clindamycin and rifampicin, anti-TNF antibodies and laser epilation. Complete excision of fistulae with or without plastic coverage of defects are the mainstays of surgical treatment. Preoperative and postoperative strategies to diminish tissue loss and options for recurrence prevention are discussed.
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  • 文章类型: Case Reports
    Chondrosarcoma is the second most common primary malignant bone tumor. While the majority arrive de novo, a minority arise from malignant transformation of benign neoplasms, such as osteochondromas. Rarely, chondrosarcomas have been found to originate from other preexisting lesions, such as synovial chondromatosis. A 44-year-old male with a history of a spinal osteochondroma presented with one year of left hip pain and decreased range of motion. On examination, he had a palpable, irregular fullness in the left groin that was minimally tender to palpation. Radiographs and CT of the hip showed extensive soft tissue calcifications and erosion of the femoral neck. The lesion was debulked surgically and histologically diagnosed as synovial osteochondromatosis with no evidence of atypia or cellularity. One year later, his residual disease progressed and resulted in increasingly limited range of motion. He underwent left total hip arthroplasty with simultaneous debulking and the lesion was once again diagnosed as synovial osteochondromatosis. Two months postoperatively, the patient developed a new focus of calcification around the hip joint that was thought to be recurrent disease. Six months later, due to worsening symptoms, he underwent a repeat CT scan. This scan demonstrated extensive intra-articular disease extending into the iliopsoas bursa and around total hip arthroplasty, as well as a new soft tissue nodule with foci of calcification in the left gluteus maximus. The new lesion was debulked surgically and diagnosed as a grade 1 chondrosarcoma. Chondrosarcoma arising from synovial chondromatosis is a rare presentation of the second most common primary malignant bone tumor. It typically presents as an indolent, slowly growing painful mass of large joints in middle aged men. Conventional radiography shows punctate opacities, while MRI and CT reveal diffuse soft tissue calcification and cortical erosion. Low-grade chondrosarcomas are treated with intralesional curettage and adjuvant therapy, while higher grade chondrosarcomas are treated with wide, en bloc excision. Malignant transformation should be considered in any patient presenting with worsening symptoms and a history of a benign bony lesion.
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