medullary

髓质
  • 文章类型: Journal Article
    多发性内分泌肿瘤(MEN)综合征是罕见的常染色体显性疾病,与内分泌和非内分泌肿瘤的混合相关。传统上,有2种类型的男性具有独特的临床关联:MEN1(甲状旁腺增生,胰腺神经内分泌肿瘤,和垂体肿瘤)和MEN2(甲状腺髓样癌和嗜铬细胞瘤),进一步分为MEN2A(增加甲状旁腺腺瘤)和2B(增加神经节神经瘤和马法样习性)。许多内分泌肿瘤都是通过手术切除的,和pre,内部,所采用的术后管理策略必须考虑到与MEN肿瘤相关的高复发率.
    Multiple endocrine neoplasia (MEN) syndromes are rare autosomal dominant diseases that are associated with a mixture of both endocrine and non-endocrine tumors. Traditionally, there are 2 types of MEN that have unique clinical associations: MEN 1 (parathyroid hyperplasia, pancreatic neuroendocrine tumors, and pituitary tumors) and MEN 2 (medullary thyroid carcinoma and pheochromocytoma), which is further classified into MEN 2A (adds parathyroid adenomas) and 2B (adds ganglioneuromas and marfanoid habitus). Many of the endocrine tumors are resected surgically, and the pre, intra, and postoperative management strategies used must take into account the high recurrence rates asscioated with MEN tumors.
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  • 文章类型: Journal Article
    目的:确定甲状腺癌犬的淋巴结转移率,并评估免疫组织化学(IHC)是否发现了H&E评估之外的其他转移。
    方法:70只接受甲状腺切除术治疗的甲状腺癌患者。
    方法:狗行甲状腺切除术,同时行选择性双侧咽后内侧(MRP)±颈深淋巴结清扫术。甲状腺肿瘤和相关淋巴结由一名单板认证的病理学家进行审查。免疫组织化学用于所有原发性肿瘤(甲状腺转录因子-1和降钙素),以支持诊断滤泡或髓样癌。H&E审查后无转移迹象的淋巴结用与原发性肿瘤中更广泛摄取相关的抗体标记。
    结果:从70只狗中切除了77例甲状腺癌,包括61个(79.2%)卵泡,8(10.7%)髓质,和7(9.3%)混合滤泡/髓样癌,1(1.3%)癌肉瘤。12只狗在H&E检查后有淋巴结转移的证据。1只狗在淋巴结IHC后发现隐匿性微转移,导致70只狗中的13只(18.6%)发生转移。髓样(5/8)和滤泡/髓样癌(3/7)的转移比滤泡癌(5/61)更常见。所有MRP转移均为同侧(7/77[9.1%]),无对侧MRP转移(0/62)。41个(34.1%)深颈部淋巴结中有14个转移。
    结论:淋巴结转移在滤泡性癌中并不常见,但在>50%的甲状腺癌犬中可见,涉及髓样成分。常规淋巴结IHC似乎对甲状腺癌的产量较低。建议甲状腺切除术切除同侧MRP和可识别的颈深淋巴结,直到获得详细的术前风险分层为止。
    OBJECTIVE: To determine the rate of nodal metastasis in dogs with thyroid cancer and evaluate whether immunohistochemistry (IHC) identifies additional metastases beyond evaluation with H&E.
    METHODS: 70 prospectively enrolled client-owned dogs with thyroid cancer managed with thyroidectomy.
    METHODS: Dogs underwent thyroidectomy with concurrent elective bilateral medial retropharyngeal (MRP) ± deep cervical lymphadenectomy. Thyroid tumors and associated lymph nodes were reviewed by a single board-certified pathologist. Immunohistochemistry was used for all primary tumors (thyroid transcription factor-1 and calcitonin) to support a diagnosis of follicular or medullary carcinoma. Lymph nodes without evidence of metastasis after H&E review were labeled with the antibody associated with the wider uptake in the primary tumor.
    RESULTS: 77 thyroid cancers were resected from the 70 dogs enrolled, including 61 (79.2%) follicular, 8 (10.7%) medullary, and 7 (9.3%) mixed follicular/medullary carcinomas, with 1 (1.3%) carcinosarcoma. Twelve dogs had evidence of nodal metastasis following H&E review. Occult micrometastasis was identified in 1 dog following nodal IHC, resulting in documented metastasis in 13 of 70 (18.6%) dogs. Metastasis was more common with medullary (5/8) and follicular/medullary carcinoma (3/7) than follicular carcinoma (5/61). All MRP metastases were ipsilateral (7/77 [9.1%]), without contralateral MRP metastases (0/62). Fourteen of 41 (34.1%) deep cervical lymph nodes were metastatic.
    CONCLUSIONS: Nodal metastasis was uncommon for follicular carcinoma but was seen in > 50% of dogs with thyroid cancer involving a medullary component. Routine nodal IHC appears to be low yield for thyroid carcinoma. Extirpation of ipsilateral MRP and identifiable deep cervical lymph nodes is recommended with thyroidectomy until detailed preoperative risk stratification becomes available.
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  • 文章类型: Journal Article
    当考虑将牙种植体放置在萎缩性缺牙部位时,可能有不足的部位宽度和很少或没有垂直骨丢失。几种外科手术中的任何一种都可以扩大这些部位。皮质外增强是通过将移植材料应用于皮质骨来完成的。该技术期望祖细胞迁移到骨脊的范围之外并形成新骨。另一种方法需要脊线分裂和扩张,以创造成骨的空间,如果可能,植入物放置。这可能是一种更好的水平脊增强方法。山脊是分裂的,分离面部和舌皮质以形成完整的骨折。然后,患者的骨细胞可以从暴露的髓质骨迁移到所产生的空间中以形成骨。该技术可以优选地进行无折叠,使得完整的骨膜维持血液供应以确保适当的愈合。
    When considering placing dental implants in atrophic edentulous sites, there may be inadequate site width and little or no vertical bone loss. Any of several surgical procedures can augment these sites. Extracortical augmentation is done by applying graft material against the cortical bone. This technique expects progenitor cells to migrate outside the bony ridge\'s confines and form new bone. Another method entails ridge splitting and expansion to create space for osteogenesis and, when possible, implant placement. This may be a better method for horizontal ridge augmentation. The ridge is split, separating the facial and lingual cortices for a complete bone fracture. The patient\'s osseous cells can then migrate into the created space from the exposed medullary bone to form bone. The technique can be preferably performed flapless so the intact periosteum maintains a blood supply to ensure appropriate healing.
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  • 文章类型: Systematic Review
    背景:甲状腺髓样癌(MTC)是一种罕见的神经内分泌肿瘤,来自产生降钙素(Ct)的滤泡旁细胞。尽管目前有一些零星MTC(sMTC)的手术治疗指南,甲状腺的最佳初始手术管理,中央和外侧颈部仍然是一个争论的问题。
    方法:在PubMed和Scopus中,根据PRISMA指南对目前的sMTC手术治疗指南及其引用进行了系统评价。
    结果:确定了200篇文章,其中7人符合纳入标准。总的来说,指南对sMTC手术治疗的建议差异很大.只有一个指南建议甲状腺部分切除术治疗有限的疾病,但其余指南的42%(3/7)承认在选定病例中避免完成甲状腺切除术的可能性.大多数指南(71.4%;5/7)建议对所有患者进行预防性中央颈清扫术(CND),而其余两个指南建议基于Ct水平和肿瘤大小进行CND。42%(3/7)的指南推荐了基于术前Ct水平的预防性侧颈清扫术的作用。总的来说,这些指南是基于低质量的证据,主要是单中心回顾系列,其中一些已经超过20岁了。
    结论:应修订现行的sMTC手术管理指南,并且应该基于挑战当前建议的最新数据,基于历史,低质量的证据。甲状腺部分切除术可能成为小型手术的可行选择,有限的肿瘤。前瞻性,多中心研究可能有助于得出所有sMTC患者是否需要预防性ND的结论.
    BACKGROUND: Medullary thyroid carcinoma (MTC) is a rare neuroendocrine tumor from parafollicular cells that produce calcitonin (Ct). Despite several existing guidelines for the surgical management of sporadic MTC (sMTC), optimal initial surgical management of the thyroid, the central and the lateral neck remains a matter of debate.
    METHODS: A systematic review in PubMed and Scopus for current guidelines addressing the surgical management of sMTC and its referenced citations was conducted as per the PRISMA guidelines.
    RESULTS: Two-hundred and one articles were identified, of which 7 met the inclusion criteria. Overall, guidelines vary significantly in their recommendations for the surgical management of sMTC. Only one guideline recommended partial thyroidectomy for limited disease, but the possibility to avoid completion thyroidectomy in selected cases is acknowledged in 42% (3/7) of the remaining guidelines. The majority of guidelines (71.4%; 5/7) recommended prophylactic central neck dissection (CND) for all patients while the remaining two guidelines recommended CND based on Ct level and tumor size. The role of prophylactic lateral neck dissection based on preoperative Ct levels was recommended by 42% (3/7) of guidelines. Overall, these guidelines are based on low-quality evidence, mostly single-center retrospective series, some of which are over 20 years old.
    CONCLUSIONS: Current surgical management guidelines of sMTC should be revised, and ought to be based on updated data challenging current recommendations, which are based on historic, low-quality evidence. Partial thyroidectomy may become a viable option for small, limited tumors. Prospective, multi-center studies may be useful to conclude whether prophylactic ND is necessary in all sMTC patients.
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  • 文章类型: Editorial
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
    背景:在酪氨酸激酶抑制剂作用下进展的转移性甲状腺髓样癌(MTC)患者可受益于烷化剂如达卡巴嗪或替莫唑胺。
    方法:我们描述了两名转移性MTC患者,他们在烷化剂治疗后出现超突变表型。该表型的特征在于高肿瘤突变负荷(TMB)和指示烷化剂诱变的突变特征(单碱基取代11)。两名患者都接受了免疫检查点抑制剂,部分形态反应,临床获益和PFS分别为6个月和9个月。
    结论:根据所描述的观察结果,我们建议,在对MTC进行烷化剂治疗后,可能会诱发超突变表型,对于TMB升高的MTC患者,应进一步探索序贯使用免疫治疗作为治疗选择.
    Background: Patients with metastatic medullary thyroid cancer (MTC) who progressed under tyrosine kinase inhibitors can benefit from an alkylating agent such as dacarbazine or temozolomide. Patient Findings: We describe two patients with metastatic MTC who developed a hypermutant phenotype after alkylating agent treatment. This phenotype was characterized by a high tumor mutational burden (TMB) and a mutational signature indicative of alkylating agent mutagenesis (single-base substitution 11). Both patients received immune checkpoint inhibitors, with partial morphological responses, clinical benefit, and progression-free survival of 6 and 9 months, respectively. Summary and Conclusions: Based on the described observations, we suggest that a hypermutant phenotype may be induced after alkylating agent treatment for MTC and the sequential use of immunotherapy should be further explored as a treatment option for MTC patients with increased TMB.
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  • 文章类型: Case Reports
    甲状腺乳头状癌(PTC)和甲状腺髓样癌(MTC)起源于滤泡和神经内分泌滤泡旁C细胞,分别。PTC和MTC同时存在于包含MTC和PTC特征的肿瘤中,这种罕见的疾病称为混合髓质滤泡状甲状腺癌(MMFTC)。在本研究中,一名60岁的女性颈部左侧有一个小肿块。超声提示甲状腺左叶有一~11.9×9.7mm2高回声结节。术前血清降钙素值升高,行甲状腺全切除术和双侧中央区淋巴结清扫术。肿瘤的组织学和免疫组织化学分析显示MMFTC。在从双侧中央室分离的淋巴结中未观察到转移。鉴于MMFTC的稀有性,加强对此类肿瘤的理解和管理至关重要。
    Papillary thyroid cancer (PTC) and medullary thyroid cancer (MTC) originate from follicular and neuroendocrine parafollicular C cells, respectively. PTC and MTC simultaneously exist in tumors containing both MTC and PTC features in a rare condition known as mixed medullary-follicular thyroid carcinoma (MMFTC). In the present study, a 60-year-old female presented with a small mass on the left side of the neck. Ultrasonography indicated a hyperechoic nodule measuring ~11.9×9.7 mm2 in the left lobe of the thyroid gland. The preoperative calcitonin serum value was elevated and total thyroidectomy and bilateral central compartment lymph node dissection was performed. Histological and immunohistochemical analysis of the tumor demonstrated MMFTC. No metastasis was observed in lymph nodes isolated from the bilateral central compartment. Given the rarity of MMFTC, enhancing understanding and management of such tumors is crucial.
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  • 文章类型: Journal Article
    甲状腺癌(TC)患者的脑转移(BM)很少见,乳头状和滤泡性的发生率为1%,髓质为3%,间变性TC为10%(PTC,联邦贸易委员会,MTC和ATC)。关于TC的BM的特征和管理知之甚少。因此,我们回顾性分析了在维也纳脑转移登记处发现的经组织学证实的TC和经放射学证实的BM患者.自1986年以来,数据库中总共有20/6074名患者患有来自TC的BM,而13/20为女性。十个病人有FTC,8PTC,一个MTC和一个ATC。诊断BM的中位年龄为68岁。除一名患者外,所有患者均有症状性BM,13/20患者有单一BM。在6例患者中发现了初步诊断的同步BM,虽然PTC诊断BM的中位时间为13年(范围1.9-24),FTC为4年(范围2.1-41),MTC患者为22年。PTC诊断为BM的总生存期为13个月(范围1.8-57),FTC26个月(范围3.9-188),MTC为12年,ATC患者为3个月。总之,由TC引起的BM极为罕见,最常见的表现是有症状的单个病变。虽然BM通常构成不良预后因素,个别患者在局部治疗后经历长期生存.
    Brain metastases (BM) in patients with thyroid cancer (TC) are rare with an incidence of 1% for papillary and follicular, 3% for medullary and up to 10% for anaplastic TC (PTC, FTC, MTC and ATC). Little is known about the characteristics and management of BM from TC. Thus, we retrospectively analyzed patients with histologically verified TC and radiologically verified BM identified from the Vienna Brain Metastasis Registry. A total of 20/6074 patients included in the database since 1986 had BM from TC and 13/20 were female. Ten patients had FTC, 8 PTC, one MTC and one ATC. The median age at diagnosis of BM was 68 years. All but one had symptomatic BM and 13/20 patients had a singular BM. Synchronous BM at primary diagnosis were found in 6 patients, while the median time to BM diagnosis was 13 years for PTC (range 1.9-24), 4 years for FTC (range 2.1-41) and 22 years for the MTC patient. The overall survival from diagnosis of BM was 13 months for PTC (range 1.8-57), 26 months for FTC (range 3.9-188), 12 years for the MTC and 3 months for the ATC patient. In conclusion, development of BM from TC is exceedingly rare and the most common presentation is a symptomatic single lesion. While BM generally constitute a poor prognostic factor, individual patients experience long-term survival following local therapy.
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  • 文章类型: Case Reports
    最近,selpercatinib,RET受体酪氨酸激酶的高选择性抑制剂,已用于RET改变的甲状腺癌。我们介绍了4例接受selpercatinib治疗的晚期甲状腺癌患者。第一位患者是一名63岁的男性,患有晚期甲状腺髓样癌(MTC),接受vandetanib治疗。六个月前,他有颅内出血和吞咽困难。他开始使用经皮内镜胃造瘘术(PEG)进行selpercatinib。11个月来,PEG给药稳定观察到部分缓解(PR),无更多心血管事件.第二名患者是一名67岁的女性,患有vandetatib治疗的晚期MTC。selpercatinib治疗后,在大多数转移部位观察到PR,包括脉络膜转移.第三名患者是一名32岁的女性,患有晚期甲状腺乳头状癌(PTC),没有系统治疗史。六个月来,在她的转移部位观察到PR,并出现可控的不良事件.最后一名患者是一名59岁女性,患有晚期PTC,接受lenvatinib治疗。在lenvatinib治疗期间,由于转移,她患有恐慌症和胸膜疼痛。selpercatinib治疗后,她的疼痛和恐慌症状得到改善。面对现实世界的各种临床障碍,无论以前的治疗或转移部位如何,selpercatinib都安全地证明了显着的治疗效果。
    Recently, selpercatinib, a highly selective inhibitor of RET receptor tyrosine kinase, has been used for RET-altered thyroid cancer. We present four cases of patients with advanced thyroid cancer who were treated with selpercatinib. The first patient was a 63-year-old male with advanced medullary thyroid cancer (MTC) treated with vandetanib. Six months ago, he had an intracranial hemorrhage and swallowing difficulty. He started selpercatinib with percutaneous endoscopic gastrostomy (PEG). For 11 months, a partial response (PR) was observed stably with PEG administration without any more cardiovascular events. The second patient was a 67-year-old female with advanced MTC treated with vandetatib. After selpercatinib treatment, a PR was observed for most metastatic sites, including choroidal metastasis. The third patient was a 32-year-old female with advanced papillary thyroid cancer (PTC) without history of systematic treatment. For six months, a PR was observed at her metastatic site with manageable adverse events. The last patient was a 59-year-old female with advanced PTC treated with lenvatinib. She suffered from a panic disorder and pleural pain due to metastasis during lenvatinib treatment. After selpercatinib treatment, her pain and panic symptoms were improved. Facing varying clinical obstacles of the real world, selpercatinib safely proved remarkable therapeutic efficacy regardless of previous treatment or metastatic site.
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