primary

原发性干燥综合征
  • 文章类型: Case Reports
    原发性子宫内膜鳞状细胞癌(PESCC)是一种罕见的恶性肿瘤。探讨PESCC的临床和病理特征,对福建省妇幼保健院2例PESCC病例进行回顾性分析,并进行文献复习。两例均为57-62岁的绝经妇女,临床表现为“阴道分泌物”。病例1为非角化鳞状细胞癌,具有高危型HPV感染。肿瘤浸润于深子宫肌层,伴有多灶性血管内血栓和一个盆腔淋巴结(1/15)和腹主动脉淋巴结(1/1)的宏观转移。术后36个月出现肺转移。手术切除后,没有术后补充治疗,患者至今仍无肿瘤110个月.病例2有5年的乳腺癌病史,长期摄入芳香化酶抑制剂药物而没有HPV感染。这是角化鳞状细胞癌。肿瘤也浸润在深子宫肌层,多灶性血管内血栓和一个盆腔淋巴结转移(1/18),然而,其他地方未见转移。迄今为止,患者术后存活16个月,无肿瘤。两例均表达鳞状上皮标志物P40,P63和CK5/6,但均不表达PAX8或PR。病例1具有P16、野生型P53和ER阴性的弥漫性表达。病例2P16阴性,P53突变和局灶性ER阳性。PESCC通常与HPV感染和低雌激素水平有关。然而,文献研究发现,P16的表达与HPV感染并不总是一致,这表明PESCC不能轻易归类为HPV相关或非依赖性宫颈癌。P16和P53的表达主要有两种模式,P16阳性/P53野生型和P16阴性/P53突变体,但是到目前为止,还没有看到两者的积极表达。值得注意的是,我们报道了第二例有乳腺癌病史的PESCC,患者长期服用口服芳香化酶抑制剂药物(依西美坦)以降低雌激素水平,表明低雌激素水平也可能是PESCC发病的关键因素。
    Primary endometrial squamous cell carcinoma (PESCC) is a rare malignant tumor. To investigate the clinical and pathological features of PESCC, two cases of PESCC in Fujian Maternal and Child Health Hospital were retrospectively studied and the literatures were reviewed. Both of the two cases were menopausal women aged 57-62 years, clinically presenting with \"vaginal discharge\". Case 1 was a non-keratinising squamous cell carcinoma with high-risk HPV infection. Tumor infiltrated in deep myometrium with multifocal intravascular thrombus and macro metastases to one pelvic lymph node (1/15) and abdominal aortic lymph node (1/1). Lung metastasis occurred 36 months after the surgery. After surgical resection and without postoperative supplemental therapy, the patient remained tumor-free for 110 months to date. Case 2 had a history of breast cancer for 5 years and long-term intake of aromatase inhibitor drugs without HPV infection. It was a keratinized squamous cell carcinoma. Tumor also infiltrated in deep myometrium with multifocal intravascular thrombus and one pelvic lymph node metastasis (1/18), However, no metastasis was seen elsewhere. To date, the patient survived for 16 months without tumor after surgery. Both of the two cases expressed squamous epithelial markers P40, P63, and CK5/6, but neither expressed PAX8 or PR. Case 1 had diffuse expression of P16, wild-type P53, and ER-negative. Case 2 had negative P16, mutant P53, and focal positive ER. PESCC is often associated with HPV infection and low estrogen levels. However, studies in the literatures have found that P16 expression is not always consistent with HPV infection, indicating that PESCC cannot be easily classified as HPV-associated or non-dependent like cervical cancer. There are two main patterns of P16 and P53 expression, P16-positive/P53 wild-type and P16-negative/P53-mutant, but no positive expression of both has been seen so far. It is worth noting that we reported the second case of PESCC with a history of breast cancer, where the patient had been taking the oral aromatase inhibitor drug (exemestane) for a long period of time to reduce the estrogen level, indicating the low estrogen level may be also a key factor in the pathogenesis of PESCC.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    血管瘤样纤维组织细胞瘤(AFH)是一种临床罕见的,偶尔转移的低度恶性软组织肿瘤。它占所有软组织肿瘤的0.3%,最常见于四肢,后面是后备箱,头部和颈部。肺支气管的原发性血管瘤样纤维组织细胞瘤(PAFH)很少见。在本文中,报告1例肺支气管PAFH的临床和影像学资料,并对文献进行了综述。
    一名57岁女性患者,有6个月的咳嗽史,无明显原因,以阵发性干咳为特征,胸闷,呼吸急促,随着活动的恶化。她没有发烧,发冷,胸痛,咯血,或者盗汗.实验室检测显示C反应蛋白和铁蛋白水平升高,而肿瘤标志物如AFP,CEA,CA199、CA125、CA50和T-SPOT均为阴性。胸部CT扫描显示支气管阻塞,肺不张,和肺右中叶的软组织密度。增强扫描显示支气管内结节不均匀增强。18F-FDGPET/CT扫描显示右肺支气管有结节状软组织密度影,密度不均,清晰的边界,并增加18F-FDG摄取,最大标准摄取值(SUVmax)为11.2。支气管镜检查显示结节状或息肉状肿块,黄色且坚韧。根据影像学检查结果,术前诊断有利于肺癌。然而,术后病理诊断证实肺支气管原发性血管瘤样纤维组织细胞瘤(PAFH)。
    原发性血管瘤样纤维组织细胞瘤(PAFH)的发生率很低,临床表现和影像学表现缺乏特异性,最终诊断依赖于病理学。PET/CT显像对PAFH的诊断具有一定的价值,对术前分期具有重要的应用价值。术后疗效评价,和后续监测。总之,该病例报告进一步扩大了肺和支气管肿瘤的范围。
    UNASSIGNED: Angiomatoid fibrous histiocytoma (AFH) is a clinically rare, low-grade malignant soft tissue tumor that occasionally metastasizes. It accounts for 0.3% of all soft tissue tumors and most frequently occurs in the extremities, followed by the trunk, and the head and neck. Primary angiomatoid fibrous histiocytoma (PAFH) of the pulmonary bronchus is rare. In this paper, the clinical and imaging data of a case of PAFH of the pulmonary bronchus are reported, and the literature is reviewed.
    UNASSIGNED: A 57-year-old female patient presented with a six-month history of cough without apparent cause, characterized by paroxysmal dry cough, chest tightness, and shortness of breath, which worsened with activity. She did not experience fever, chills, chest pain, hemoptysis, or night sweats. Laboratory tests revealed an elevated C-reactive protein and ferritin levels, while tumor markers such as AFP, CEA, CA199, CA125, CA50, and T-SPOT were negative. A chest CT scan showed bronchial obstruction, atelectasis, and a soft tissue density in the right middle lobe of the lung. The enhanced scan demonstrated uneven enhancement of endobronchial nodules. An 18F-FDG PET/CT scan revealed a nodular soft tissue density shadow in the right lung bronchus with uneven density, clear boundaries, and increased 18F-FDG uptake, with a maximum standard uptake value (SUVmax) of 11.2. Bronchoscopy revealed a nodular or polypoid mass that was yellow and tough. Based on imaging findings, the preoperative diagnosis favored lung cancer. However, the postoperative pathological diagnosis confirmed primary angiomatoid fibrous histiocytoma (PAFH) of the pulmonary bronchus.
    UNASSIGNED: The incidence of primary angiomatoid fibrous histiocytoma (PAFH) is very low, and its clinical manifestations and imaging findings lack specificity, with the final diagnosis relying on pathology. PET/CT imaging has a certain value in the diagnosis of PAFH and holds significant application value in preoperative staging, postoperative efficacy evaluation, and follow-up monitoring. In conclusion, this case report further expands the spectrum of lung and bronchial tumors.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Letter
    暂无摘要。
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    一名53岁的男性患者在2年的时间内表现出右侧肢体的进行性麻木和无力。磁共振成像扫描显示,髓内病变跨越颈部和胸部水平,并在病变的近端伴有脊髓空洞症。患者接受了肿瘤次全切除术。经过仔细的皮肤和眼科检查,肿瘤的组织学发现与原发性髓内恶性黑色素瘤一致,而不是最初的室管膜瘤。原发性脊髓黑色素瘤,特别是颈胸定位伴脊髓空洞症,在文献中很少报道。我们报告了一例这种罕见的肿瘤,并讨论了临床过程,诊断,和治疗。
    A 53-year-old male patient presented progressive numbness and weakness in the right limbs for a 2-year duration. Magnetic resonance imaging scans revealed an intramedullary lesion crossed over cervical and thoracic levels accompanied by syringomyelia at the proximal end of the lesion. The patient underwent subtotal resection of the neoplasm. The histological findings of the tumor were consistent with primary intramedullary malignant melanoma and not initial ependymoma after careful dermatologic and ophthalmologic re-examination. Primary melanoma of the spinal cord, particularly cervicothoracic localization with syringomyelia, is seldom reported in the literature. We report a case of this uncommon tumor and also discuss the clinical course, diagnosis, and treatment.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    原发性脾血管肉瘤(PSA)是一种罕见的肿瘤。它是来源于脾窦内皮细胞的恶性肿瘤。PSA的病因不明,高度恶性,容易早期转移,不典型的临床症状和影像学表现,和困难的早期诊断。本文报告1例PSA伴肝内转移的18F-FDGPET/CT表现,成像,和病理数据;并回顾文献。
    一名64岁男性患者于2022年3月13日出现左下腹胀痛,无明显原因。疼痛持续、迟钝,坐着吃饭后加重。血常规检查结果为RBC→3.33×1012/L,WBC^12.32×109/L,和PLT△40×109/L肿瘤标志物显示CA125^47.0U/ml,AFP(-),CEA(-),CA199(-),和CA724(-)。腹部CT非对比增强扫描显示脾脏体积明显增大,形态不规则,有多发结节和块状低密度阴影,界限不清,密度不均匀,和多个坏死区域。增强CT显示脾脏弥漫性不均匀轻度增强,增强程度随时间增加。肝脏可见多个结节状低密度阴影,18F-FDGPET/CT显示脾脏有多个结节和大量病变,并有多个坏死区。肝脏有多个结节性病变,FDG代谢水平升高,脾脏病变的SUVmax为9.0,肝脏病变的SUVmax为5.6.18F-FDGPET/CT诊断为脾恶性肿瘤伴肝转移。最后,经过多学科的讨论,决定进行腹腔镜全脾切除和门静脉灌注化疗。病理检查显示肿瘤细胞呈圆形,椭圆形,或者梭形,有明显的非典型性,排入电缆或吻合的血管腔。最终诊断为原发性脾血管肉瘤伴大量坏死。手术后,患者接受抗肿瘤联合治疗,5个月后死亡。
    PSA的发生率很低,其临床和放射学表现缺乏特异性。18F-FDGPET/CT显像对PSA有一定的诊断价值,对术前分期有显著的实用性,指导活检程序,评估术后治疗反应,监测疾病复发。在脾脏内存在显示坏死区域的占位性病变时,应考虑PSA。在对比增强扫描中显示渐进增强,并证明FDG摄取的异质性增加。
    UNASSIGNED: Primary splenic angiosarcoma (PSA) is a rare neoplasm. It is a malignant tumor derived from endothelial cells of the splenic sinuses. PSA has an unknown etiology, a high degree of malignancy, easy early metastasis, atypical clinical symptoms and imaging findings, and difficult early diagnosis. This paper reports the 18F-FDG PET/CT findings of a case of PSA with intrahepatic metastasis; summarizes its clinical, imaging, and pathological data; and reviews the literature.
    UNASSIGNED: A 64-year-old male patient presented with left lower abdominal distending pain without obvious causes on 13 March 2022. The pain was persistent and dull and worsened after sitting and eating. Blood routine examination results were RBC ↓ 3.33 × 1012/L, WBC ↑ 12.32 × 109/L, and PLT ↓ 40 × 109/L. The tumor markers indicated CA125 ↑ 47.0 U/ml, AFP (-), CEA (-), CA199 (-), and CA724 (-). Non-contrast-enhanced CT scan of the abdomen showed that the spleen was significantly enlarged in volume and irregular in shape and had multiple nodules and clumpy low-density shadows, unclear boundaries, uneven density, and multiple necrotic areas. Enhanced CT showed diffuse uneven mild enhancement of the spleen, and the degree of enhancement increased with time. Multiple nodular low-density shadows were seen in the liver, which were slightly enhanced by the enhanced scan.18F-FDG PET/CT showed multiple nodular and massive lesions in the spleen with multiple necrotic areas. There were multiple nodular lesions in the liver, the level of FDG metabolism increased, the SUVmax of the spleen lesions was 9.0, and the SUVmax of the liver lesions was 5.6. The 18F-FDG PET/CT diagnosis was splenic malignancy with liver metastasis. Finally, after a multidisciplinary discussion, it was decided to perform laparoscopic total splenectomy and portal vein infusion chemotherapy. Pathological examination showed that the tumor cells were round, oval, or fusiform, with obvious atypia, arranged into a cable or anastomosed vascular lumen. The final diagnosis was primary splenic angiosarcoma with massive necrosis. After surgery, the patient received antitumor combined therapy and died 5 months later.
    UNASSIGNED: The incidence of PSA is very low, and its clinical and radiological manifestations lack specificity. 18F-FDG PET/CT imaging has a certain diagnostic value for PSA and significant utility in preoperative staging, guiding biopsy procedures, evaluating postoperative treatment response, and monitoring disease recurrence. PSA should be considered in the presence of a space-occupying lesion within the spleen that exhibits necrotic areas, shows progressive enhancement on contrast-enhanced scans, and demonstrates heterogeneous increases in FDG uptake.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    目的:头颈部原发性结外非霍奇金淋巴瘤(PE-NHL)是结外淋巴瘤的第二常见部位,约占所有结外非霍奇金淋巴瘤(E-NHL)的三分之一。然而,近年来,在中国和世界范围内,大规模的PE-NHL案例研究很少见,也不够全面。这项工作分析了临床表现,病理特征,PE-NHL的免疫表型和诊断,以及影响治疗和预后的因素。
    方法:对74例首次诊断为头颈部PE-NHL的患者进行回顾性研究。临床表现,病理特征,并对免疫表型进行了总结,分析与治疗及预后相关的因素。
    结果:这种疾病最常见的部位是Waldeyer的戒指,其次是鼻腔。弥漫性大B细胞淋巴瘤是最常见的类型,其次是结外NKT细胞淋巴瘤鼻型。1年,2年,5年无进展生存率(PFS)为76.4%,67.9%,和59.3%。1年,2年,5年总生存率(OS)为89.4%,85.6%,和63.2%。ECOG评分≥2分、AnnArborIII或IV期以及IPI危险分层确定患者为高危人群是影响头颈部PE-NHL患者OS的独立危险因素。
    结论:这些中国患者中PE-NHL最常见的部位是Waldeyer环,但鼻腔的发病率高于西方国家的报告。放疗联合化疗疗效优于单纯化疗,预后取决于ECOG评分和临床分期。IPI在头颈部PE-NHL高危人群中有较好的预后价值。
    Primary extranodal non-Hodgkin\'s lymphoma (PE-NHL) of the head and neck is the second common site of extranodal lymphoma, accounting for approximately one-third of all extranodal non-Hodgkin\'s lymphoma (E-NHL). However, in recent years, large-scale PE-NHL case studies in China and worldwide are rare and not comprehensive enough. This work analyzed the clinical manifestations, pathological features, immunophenotypes and diagnosis of PE-NHL, as well as the factors affecting the treatment and prognosis.
    A retrospective study was performed on 74 patients who were diagnosed with head and neck PE-NHL and treated for the first time. The clinical manifestations, pathological features, and immunophenotypes were summarized, and the factors related to the treatment and prognosis were analyzed.
    The most common site of this disease was the Waldeyer\'s ring, followed by the nasal cavity. Diffuse large B-cell lymphoma was the most common type, followed by extranodal NK T-cell lymphoma nasal type. The 1-year, 2-year, and 5-year progression-free survival (PFS) rates were 76.4%, 67.9%, and 59.3%. The 1-year, 2-year, and 5-year overall survival (OS) rates were 89.4%, 85.6%, and 63.2%. ECOG score ≥ 2, Ann Arbor stage III or IV and IPI risk stratification identifying patients as the high-risk group were independent risk factors affecting the OS of patients with PE-NHL of the head and neck.
    The most common site of PE-NHL in these Chinese patients was the Waldeyer\'s ring, but the incidence in the nasal cavity was higher than that reported in Western countries. Radiotherapy combined with chemotherapy had better efficacy than chemotherapy alone, and the prognosis depended on the ECOG score and clinical stage. IPI had a better prognostic value in patients in the high-risk group of head and neck PE-NHL.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Multicenter Study
    目的:探讨原发性卵巢黏液性恶性肿瘤(POMT)和累及卵巢的转移性黏液性癌(MOMCs)的临床及MRI表现。
    方法:这项回顾性多中心研究纳入了61例患者,其中22例POMT和49例MOMC,在2014年11月至Jane2023年之间进行了病理证明。评估并比较POMT和MOMC的临床和MRI特征。进行了单变量和多变量分析,以确定两组之间的显著变量,然后将其合并到预测列线图中,随后进行ROC曲线分析以评估诊断性能.
    结果:35.9%的MOMC患者与原发癌同步发现;25.6%的MOMC患者为双侧,所有POMT患者均为单侧。生物标志物CEA在两组之间显著不同(p=0.002)。以下MRI特征存在显著差异:肿瘤大小,配置,增强模式,囊肿的数量,蜂巢标志,彩色玻璃外观,腹水,大小多样性比,信号分集比。局部大小多样性比(p=0.005,OR=1.31),和信号强度多样性比(p=0.10,OR=4.01)是MOMC的独立预测因子。上述独立标准的组合产生了0.922的最大曲线下面积,灵敏度为82.3%,特异性为88.9%。
    结论:患有MOMC的患者更常见于双侧,CEA水平更高,但并不总是有恶性肿瘤病史。对于产生粘蛋白的卵巢肿瘤,均匀的病灶大小和信号强度更能预测MOMC。
    OBJECTIVE: To investigate the clinical and magnetic resonance imaging (MRI) features for preoperatively discriminating  primary ovarian mucinous malignant tumors (POMTs) and metastatic mucinous carcinomas involving the ovary (MOMCs).
    METHODS: This retrospective multicenter study enrolled 61 patients with 22 POMTs and 49 MOMCs, which were pathologically proved between November 2014 to Jane 2023. The clinical and MRI features were evaluated and compared between POMTs and MOMCs. Univariate and multivariate analyses were performed to identify the significant variables between the two groups, which were then incorporated into a predictive nomogram, and ROC curve analysis was subsequently carried out to evaluate diagnostic performance.
    RESULTS: 35.9% patients with MOMCs were discovered synchronously with the primary carcinomas; 25.6% patients with MOMCs were bilateral, and all of the patients with POMTs were unilateral. The biomarker CEA was significantly different between the two groups (p = 0.002). There were significant differences in the following MRI features: tumor size, configuration, enhanced pattern, the number of cysts, honeycomb sign, stained-glass appearance, ascites, size diversity ratio, signal diversity ratio. The locular size diversity ratio (p = 0.005, OR = 1.31), and signal intensity diversity ratio (p = 0.10, OR = 4.01) were independent predictors for MOMCs. The combination of above independent criteria yielded the largest area under curve of 0.922 with a sensitivity of 82.3% and specificity of 88.9%.
    CONCLUSIONS: Patients with MOMCs were more commonly bilaterally and having higher levels of CEA, but did not always had a malignant tumor history. For ovarian mucin-producing tumors, the uniform locular sizes and signal intensities were more predict MOMCs.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    目的:鼻咽黑色素瘤是一种罕见的黏膜恶性黑色素瘤,复发率高,转移率和血管浸润率。在本文中,我们报告一例原发性鼻咽粘膜黑色素瘤。方法:报道1例原发性鼻咽粘膜黑色素瘤,和它的临床症状,病理特征,治疗和随访进行了详细描述。结果:本报告描述了一名59岁的男性患者,患有持续的鼻充血和可疑的恶性鼻咽肿瘤。患者在完全切除后接受手术切除和辅助放疗。影像学检查显示无组织浸润或淋巴结转移。免疫组化结果为Melan-A(+),HMB45(+),S100(+)最终诊断为恶性鼻咽黑色素瘤。经过2年的随访,预后良好,无转移或复发。讨论:鼻咽黑色素瘤是一种罕见的恶性肿瘤,预后不良,手术切除是治疗的主要手段。术后辅助治疗可提高病灶局部控制率。早期诊断和全面检查对患者的预后极为重要。
    Objective: Nasopharyngeal melanoma is a rare mucosal malignant melanoma with high recurrence rate, metastasis rate and vascular invasion rate. In this paper, we report a case of primary nasopharyngeal mucosal melanoma. Methods: A case of primary nasopharyngeal mucosal melanoma was reported, and its clinical symptoms, pathological characteristics, treatment and follow-up were described in detail. Results: This report describes a 59-year-old male patient with persistent nasal congestion and suspected malignant nasopharyngeal neoplasm. Patients receive surgical resection and adjuvant radiotherapy after complete resection. Imaging studies showed no tissue invasion or lymph node metastases. The results of immunohistochemistry were Melan-A(+), HMB45(+), and S100(+). The final diagnosis was malignant nasopharyngeal melanoma. After 2 years of follow-up, the prognosis was good, and there was no metastasis or recurrence. Discussion: Nasopharyngeal melanoma is a rare malignancy with a poor prognosis, and surgical resection is the mainstay of treatment. Postoperative adjuvant therapy can improve the rate of local control of lesions. Early diagnosis and thorough examination are extremely important for the patient\'s prognosis.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    暂无摘要。
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Case Reports
    暂无摘要。
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

公众号