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A Rare Case of Pure Primary Large Cell Neuroendocrine Carcinoma of the Gallbladder 罕见的单纯原发性胆囊大细胞神经内分泌癌
IF 0.9 Pub Date : 2022-05-21 DOI: 10.1155/2022/6956046
R. Shackelford, E. Ozluk, J. Abdulsattar, T. Lairmore, Quyen Chu, E. Wei
Primary large cell neuroendocrine carcinoma (LCNEC) of the gallbladder is a rare malignancy which is often associated with non-LCNEC histologic components. Histologically “pure” LCNECs of the gallbladder are exceedingly rare with only 15 cases reported in the medical literature. Clinically, LCNECs present with abdominal pain and jaundice and follow an aggressive course with patients surviving a median of 15 months following initial diagnosis. To our knowledge, we present the 16th case of a histologically pure LCNEC in a 62-year-old African American male who was successfully treated surgically. After discharge, he was subsequently lost to follow-up. Due to the extreme rarity of such disease entity, accurate diagnosis and proper management are essential for the best clinical outcome.
胆囊原发性大细胞神经内分泌癌(LCNEC)是一种罕见的恶性肿瘤,通常与非LCNEC组织学成分有关。组织学上“纯”胆囊LCNECs极为罕见,医学文献中仅报道了15例。临床上,LCNECs表现为腹痛和黄疸,并遵循一个积极的过程,患者在最初诊断后平均存活15个月。据我们所知,我们报告了一名62岁的非裔美国男性的第16例组织学纯LCNEC,该男性通过手术成功治疗。出院后,他随后失访。由于这种疾病非常罕见,准确的诊断和适当的管理对于获得最佳临床结果至关重要。
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引用次数: 0
A Pulmonary Zebra: Adult Primary Pulmonary Synovial Sarcoma 肺斑马:成人原发性肺滑膜肉瘤
IF 0.9 Pub Date : 2022-04-16 DOI: 10.1155/2022/8649540
Sheffield Sandra, Nwachukwu Chidi, Ashby Tracy
Primary pulmonary synovial sarcoma (PPSS) is an extremely rare tumor, with approximately 50 cases being reported in the English literature (Golota et al., 2018). Difficulties are often encountered in the diagnosis of PPSS as it can be confused with other spindle or round cell tumors, such as fibrosarcoma, hemangiopericytoma, mesothelioma, sarcomatoid carcinoma, or metastatic sarcomas. PPSS was first described by Zeren et al. in 1995. We present a case of PPSS in a 41-year-old woman, who complained of progressive dyspnea and left-sided chest pain.
原发性肺滑膜肉瘤(PPSS)是一种极为罕见的肿瘤,英国文献中报道了大约50例(Golota等人,2018)。PPSS的诊断经常遇到困难,因为它可能与其他梭形或圆形细胞肿瘤混淆,如纤维肉瘤、血管外皮细胞瘤、间皮瘤、肉瘤样癌或转移性肉瘤。PPSS由Zeren等人于1995年首次描述。我们报告一位41岁女性的PPSS病例,她主诉进行性呼吸困难和左侧胸痛。
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引用次数: 0
A Rare Case of Large-Vessel Vasculitis following Checkpoint Inhibitor Therapy and Pegfilgrastim 检查点抑制剂治疗和培非司汀治疗后罕见的大血管血管炎
IF 0.9 Pub Date : 2022-02-23 DOI: 10.1155/2022/7295305
J. Mort, Shipra Maheshwari, Nayanika Basu, P. Dillon, Kevin Brady, H. Bear, T. Millard
Checkpoint inhibitors (CPIs) and pegfilgrastim, a long-acting growth factor agent, are vital components of current cancer treatments. Immune-related adverse events (irAEs) such as colitis and pneumonitis are well-established toxicities associated with CPI therapy. However, large-vessel vasculitis secondary to CPI utilization is reported only in rare case reports and case series. Interestingly, large-vessel vasculitis has also been reported as a rare complication of pegfilgrastim use. We present a 59-year-old female with left stage IIA (cT2N0M0) triple-negative breast cancer receiving neoadjuvant decitabine and pembrolizumab prior to neoadjuvant chemotherapy (NAC). NAC included standard-of-care dose dense doxorubicin and cyclophosphamide (ddAC) supported with pegfilgrastim use followed by weekly carboplatin and paclitaxel. After receiving her second cycle of ddAC with pegfilgrastim, the patient reported five days of left shoulder and arm pain. Subsequent CT imaging demonstrated wall thickening and inflammatory changes surrounding the left subclavian artery, aortic arch, left carotid artery, proximal innominate arteries, and the mid internal carotid arteries and its branching vessels. These findings were extremely concerning for large-vessel vasculitis. Excluding CPI therapy and pegfilgrastim use, no additional inciting event or medication that the patient was exposed to was noted to be associated with large-vessel vasculitis. We present this case to report on this rare but severe complication from commonly utilized agents in cancer treatment. We also extend the possibility of large-vessel vasculitis development in relation to the COVID-19 vaccine due to shared ingredients found in both the vaccine and pegfilgrastim. It is important to outline the treatment used for such a complication as no standardized treatment has been established for large-vessel vasculitis caused by CPI therapy or pegfilgrastim use.
检查点抑制剂(CPI)和长效生长因子培非司汀是当前癌症治疗的重要组成部分。免疫相关不良事件(irAE),如结肠炎和肺炎,是与CPI治疗相关的公认毒性。然而,仅在罕见的病例报告和病例系列中报道了继发于CPI使用的大血管血管炎。有趣的是,大血管血管炎也被报道为使用培非司汀的罕见并发症。我们报道了一名患有左IIA期(cT2N0M0)三阴性癌症的59岁女性,在新辅助化疗(NAC)前接受新辅助地西他滨和pembrolizumab。NAC包括标准护理剂量的高密度阿霉素和环磷酰胺(ddAC),辅以培非司汀,然后每周使用卡铂和紫杉醇。在接受第二个周期的培非格拉司汀ddAC治疗后,患者报告左肩和手臂疼痛五天。随后的CT成像显示,左锁骨下动脉、主动脉弓、左颈动脉、无名动脉近端、颈内动脉中动脉及其分支血管周围的壁增厚和炎症变化。这些发现对于大血管血管炎非常令人担忧。除CPI治疗和培非司汀使用外,没有发现患者接触的其他刺激事件或药物与大血管血管炎有关。我们提出这个病例,报告这种罕见但严重的并发症,在癌症治疗中常用的药物。我们还扩大了与新冠肺炎疫苗相关的大血管血管炎发展的可能性,因为在疫苗和培非格拉司汀中发现了共同的成分。概述这种并发症的治疗方法很重要,因为CPI治疗或使用培非司汀引起的大血管血管炎尚未建立标准化治疗方法。
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引用次数: 3
Atezolizumab-Induced Sarcoidosis-Like Reaction in a Patient with Metastatic Breast Cancer. 阿特唑单抗诱导转移性乳腺癌患者结节病样反应
IF 0.9 Pub Date : 2022-01-27 eCollection Date: 2022-01-01 DOI: 10.1155/2022/2709062
Akira Tsunoda, Toshiro Mizuno, Shohei Iida, Katsunori Uchida, Masako Yamashita, Koshi Sukeno, Hiroki Oka, Yasutaka Tono, Mikiya Ishihara, Kanako Saito, Satoshi Tamaru, Keiichi Yamanaka, Isao Tawara

Tumor-related sarcoidosis-like reactions (SLR) have been reported with the use of immune checkpoint inhibitors (ICIs). We report a case of 50-year-old woman who observed an enlarged lymph node in the right hilar region and the appearance of a subcutaneous mass in the extremities during chemotherapy with atezolizumab plus nab-paclitaxel for metastatic triple-negative breast cancer (TNBC). Skin biopsy revealed the formation of epithelioid granulation species with the Langhans giant cell. After discontinuing atezolizumab in the treatment procedure, the hilar lymph nodes and the subcutaneous mass were reduced. A pathological examination was effective in differentiating tumor exacerbation from SLR. Owing to limited information on ICI-related SLR in breast cancer, future studies are recommended to properly manage immune-related adverse effects during cancer treatment.

使用免疫检查点抑制剂(ICIs)已报道肿瘤相关结节病样反应(SLR)。我们报告了一例50岁的女性,她在用阿特唑单抗加nab-紫杉醇治疗转移性三阴性乳腺癌(TNBC)期间,观察到右侧门区淋巴结肿大,并在四肢出现皮下肿块。皮肤活检显示朗汉斯巨细胞形成上皮样肉芽。在治疗过程中停用atezolizumab后,肝门淋巴结和皮下肿块减少。病理检查可有效鉴别肿瘤加重与单发复发。由于乳腺癌中ici相关SLR的信息有限,建议未来的研究适当地管理癌症治疗期间免疫相关的不良反应。
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引用次数: 2
Primary Eccrine Porocarcinoma of the Breast: A Case Report and Review of Literature. 乳腺原发性内分泌性多孔癌1例报告及文献复习。
IF 0.9 Pub Date : 2022-01-01 DOI: 10.1155/2022/4042298
Yi Xin Li, Mihir Gudi, Zhiyan Yan

Eccrine porocarcinoma (EPC) is a rare cutaneous neoplasm, with less than 500 reported cases worldwide since it was first described in 1963. EPC tends to affect the elderly and most commonly affects the head and neck. The mainstay of EPC treatment is surgery, with lymphadenectomy in the case of nodal involvement or presence of unfavourable characteristics. No evidence exists to guide the use of adjuvant chemotherapy or radiation. EPC is prone to misdiagnosis given its multiple clinical and histopathological mimics, especially in uncommon sites of presentation such as the breast. Herein, we report the case of a 59-year-old woman who presented with a left breast skin lump. The biopsied specimen revealed an infiltrative carcinoma involving the dermis and epidermis with positive IHC staining for P63 and CK5/6 and negative staining for ER, PR, and HER2. The tumour was resected and diagnosed as EPC with atypical features as overlapping characteristics of squamous cell carcinoma (SCC) were detected on histopathological analysis. In our case, a simple mastectomy with broad margins and axillary lymph node dissection with adjuvant radiotherapy to a dose of 60 Gy failed to achieve loco-regional control with nodal recurrence occurring 4 months postsurgery-a testament to the aggressive course of this rare malignancy.

EPC是一种罕见的皮肤肿瘤,自1963年首次报道以来,全球报告病例不足500例。EPC倾向于影响老年人,最常影响头颈部。EPC治疗的主要方法是手术,在淋巴结受累或存在不利特征的情况下进行淋巴结切除术。没有证据可以指导辅助化疗或放疗的使用。由于其多种临床和组织病理学模拟,特别是在不常见的表现部位,如乳房,EPC容易被误诊。在此,我们报告的情况下,59岁的妇女谁提出了一个左乳房皮肤肿块。活检标本显示浸润性癌累及真皮和表皮,免疫组化染色P63和CK5/6阳性,ER、PR和HER2阴性。肿瘤被切除并诊断为非典型特征的EPC,在组织病理学分析中发现鳞状细胞癌(SCC)的重叠特征。在我们的病例中,简单的乳房切除术,宽边缘和腋窝淋巴结清扫,辅助放疗剂量为60 Gy,未能达到局部区域控制,术后4个月淋巴结复发,证明了这种罕见的恶性肿瘤的侵袭性进程。
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引用次数: 0
Eosinophilic Asthma Secondary to Adjuvant Anti-PD-1 Immune Checkpoint Inhibitor Treatment in a Melanoma Patient. 黑色素瘤患者辅助抗pd -1免疫检查点抑制剂治疗继发嗜酸性哮喘
IF 0.9 Pub Date : 2022-01-01 DOI: 10.1155/2022/2658136
P Kissoonsingh, B Sutton, Syed U Iqbal, Lalit Pallan, Neil Steven, L Khoja

Background: Adjuvant immune checkpoint inhibitors are a new standard of care in melanoma. However, the immune related toxicity associated with these agents can be serious, and the long-term implications are yet to be defined especially in the adjuvant setting. We report, to our knowledge, the first case of anti-PD-1-induced eosinophilic asthma in a melanoma patient treated with adjuvant pembrolizumab. Case Presentation. A 72-year-old man commenced pembrolizumab in the adjuvant setting after resection of a stage IIIB cutaneous melanoma. The patient experienced episodes of breathlessness 4 weeks after cycle 1. These episodes were nocturnal and caused acute respiratory distress and cough, occasionally waking him up. The episodes progressed, and he was admitted after cycle 2 with a productive cough, wheeze, and breathlessness. Observations showed saturations on air of 94% and a respiratory rate of 19/min. The only laboratory abnormality was a raised eosinophil count of 1.1 × 109. Spirometry showed a FEV1 of 2.57 (91% predicted), FVC of 4.04 (108% predicted), and ratio of 64%. Peak expiratory flow rate was 94% predicted, and corrected gas transfer was 6.29 (78% predicted) with KCO 1.18 (93% predicted). FeNO was raised at 129 indicating inflammation of his airways, and peak flow was 422 l/min. CT of the chest did not show pneumonitis or other lung pathology. A diagnosis of acute eosinophilic asthma was made. Treatment with steroids and beclometasone dipropionate and formoterol inhaler produced rapid resolution of symptoms and normalisation of the eosinophil count. Pembrolizumab was safely recommenced once steroids had discontinued and symptoms had resolved.

Conclusions: Specialist respiratory input was needed for optimal patient management and is ongoing. Although a safe rechallenge with pembrolizumab was possible, treatment in the adjuvant setting is curative in intent and long-term safety follow-up is required to assess for delayed toxicity and long-term health implications. This is likely to require large regional/national/international databases to detect, monitor, and educate the wider medical community as these patients are followed up in primary care following initial specialist follow-up.

背景:辅助免疫检查点抑制剂是黑色素瘤治疗的新标准。然而,与这些药物相关的免疫相关毒性可能是严重的,长期影响尚未确定,特别是在佐剂设置中。我们报告,据我们所知,第一例抗pd -1诱导嗜酸性粒细胞哮喘的黑色素瘤患者接受辅助治疗派姆单抗。案例演示。一名72岁男性在IIIB期皮肤黑色素瘤切除术后开始使用派姆单抗作为辅助治疗。患者在第1周期后4周出现呼吸困难发作。这些发作发生在夜间,引起急性呼吸窘迫和咳嗽,偶尔惊醒他。发作进展,患者在第2周期后因咳嗽、喘息和呼吸困难入院。观察显示空气饱和度为94%,呼吸速率为每分钟19次。唯一的实验室异常是嗜酸性粒细胞计数升高1.1 × 109。肺活量测定显示FEV1为2.57(预测91%),FVC为4.04(预测108%),比值为64%。预测呼气流量峰值为94%,校正气体传递为6.29(预测78%),KCO为1.18(预测93%)。FeNO升高至129,表明气道炎症,峰值流量为422 l/min。胸部CT未见肺炎或其他肺部病变。诊断为急性嗜酸性哮喘。用类固醇、二丙酸倍氯米松和福莫特罗吸入器治疗可迅速缓解症状并使嗜酸性粒细胞计数恢复正常。一旦类固醇停用且症状消退,派姆单抗可以安全地重新使用。结论:需要专科呼吸输入以优化患者管理,并且正在进行中。尽管使用派姆单抗进行安全的再挑战是可能的,但辅助治疗的目的是治愈性的,需要长期的安全性随访来评估延迟毒性和长期健康影响。这可能需要大型的区域/国家/国际数据库来发现、监测和教育更广泛的医疗界,因为这些患者在最初的专家随访之后在初级保健中进行随访。
{"title":"Eosinophilic Asthma Secondary to Adjuvant Anti-PD-1 Immune Checkpoint Inhibitor Treatment in a Melanoma Patient.","authors":"P Kissoonsingh,&nbsp;B Sutton,&nbsp;Syed U Iqbal,&nbsp;Lalit Pallan,&nbsp;Neil Steven,&nbsp;L Khoja","doi":"10.1155/2022/2658136","DOIUrl":"https://doi.org/10.1155/2022/2658136","url":null,"abstract":"<p><strong>Background: </strong>Adjuvant immune checkpoint inhibitors are a new standard of care in melanoma. However, the immune related toxicity associated with these agents can be serious, and the long-term implications are yet to be defined especially in the adjuvant setting. We report, to our knowledge, the first case of anti-PD-1-induced eosinophilic asthma in a melanoma patient treated with adjuvant pembrolizumab. <i>Case Presentation.</i> A 72-year-old man commenced pembrolizumab in the adjuvant setting after resection of a stage IIIB cutaneous melanoma. The patient experienced episodes of breathlessness 4 weeks after cycle 1. These episodes were nocturnal and caused acute respiratory distress and cough, occasionally waking him up. The episodes progressed, and he was admitted after cycle 2 with a productive cough, wheeze, and breathlessness. Observations showed saturations on air of 94% and a respiratory rate of 19/min. The only laboratory abnormality was a raised eosinophil count of 1.1 × 10<sup>9</sup>. Spirometry showed a FEV1 of 2.57 (91% predicted), FVC of 4.04 (108% predicted), and ratio of 64%. Peak expiratory flow rate was 94% predicted, and corrected gas transfer was 6.29 (78% predicted) with KCO 1.18 (93% predicted). FeNO was raised at 129 indicating inflammation of his airways, and peak flow was 422 l/min. CT of the chest did not show pneumonitis or other lung pathology. A diagnosis of acute eosinophilic asthma was made. Treatment with steroids and beclometasone dipropionate and formoterol inhaler produced rapid resolution of symptoms and normalisation of the eosinophil count. Pembrolizumab was safely recommenced once steroids had discontinued and symptoms had resolved.</p><p><strong>Conclusions: </strong>Specialist respiratory input was needed for optimal patient management and is ongoing. Although a safe rechallenge with pembrolizumab was possible, treatment in the adjuvant setting is curative in intent and long-term safety follow-up is required to assess for delayed toxicity and long-term health implications. This is likely to require large regional/national/international databases to detect, monitor, and educate the wider medical community as these patients are followed up in primary care following initial specialist follow-up.</p>","PeriodicalId":9636,"journal":{"name":"Case Reports in Oncological Medicine","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9078828/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10252335","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 5
A Nonseminomatous Germ Cell Tumor Presenting as a Mixed Cryoglobulinemic Vasculitis. 表现为混合性冷球蛋白血症性血管炎的非半细胞性生殖细胞肿瘤。
IF 0.9 Pub Date : 2022-01-01 DOI: 10.1155/2022/3326761
Gabriel Cojuc-Konigsberg, Isabel Comte Natera, Blanca E López Graciano, Luis Gerardo Mosqueda López, José Alonso Rojo Ávila, Braulio Martínez, Juan C Ramírez-Sandoval

Background: Mixed cryoglobulinemia syndrome (MCS) is a rare entity with a variety of causes but has not been associated with testicular germ cell tumors. We present here a case of a patient with a nonseminomatous germ cell tumor (NSGCT) presenting as a type III mixed cryoglobulinemic vasculitis. Case Presentation. A 58-year-old male exhibited typical clinical features of vasculitis, including weakness, fatigue, palpable purpura, multiple mononeuropathy, and a low C4 level. An MCS diagnosis was confirmed by the presence of cryoglobulins (6%) with polyclonal IgM and IgG components and biopsy proven leukocytoclastic vasculitis. Concomitantly, a stage IIIC (TxNxM1bS1) germ tumor with marked elevation of serum beta-human chorionic gonadotropin (2764 mUI/mL) was diagnosed. An aggressive treatment was needed, including methylprednisolone pulses, plasmapheresis, rituximab, followed by orchiectomy, and chemotherapy (bleomycin/etoposide/cisplatin). After tumor resection and treatment, cryoglobulins decrease to 0%, suggesting a paraneoplastic origin of the vasculitis.

Conclusion: To the best of our knowledge, this is the first case of MCS possibly attributable to a NSGCT. This case further elaborates on the presentation of mixed cryoglobulinemia vasculitis and adds to the published literature on the topic.

背景:混合性冷球蛋白血症综合征(MCS)是一种罕见的疾病,病因多种多样,但尚未与睾丸生殖细胞肿瘤相关。我们在此报告一例非半细胞瘤性生殖细胞肿瘤(NSGCT),表现为III型混合性冷球蛋白性血管炎。案例演示。一名58岁男性,表现出典型的血管炎临床特征,包括虚弱、疲劳、可触及的紫癜、多发性单神经病变和低C4水平。MCS的诊断是通过存在含有多克隆IgM和IgG成分的冷球蛋白(6%)和活检证实的白细胞破碎性血管炎。同时,诊断为IIIC期(TxNxM1bS1)生殖肿瘤,血清β -人绒毛膜促性腺激素显著升高(2764 mUI/mL)。需要积极的治疗,包括甲基强的松龙脉冲、血浆置换、利妥昔单抗、睾丸切除术和化疗(博来霉素/依托泊苷/顺铂)。肿瘤切除和治疗后,冷球蛋白下降至0%,提示血管炎的副肿瘤起源。结论:据我们所知,这是首例可能由NSGCT引起的MCS病例。本病例进一步阐述了混合性冷球蛋白血症血管炎的表现,并增加了有关该主题的已发表文献。
{"title":"A Nonseminomatous Germ Cell Tumor Presenting as a Mixed Cryoglobulinemic Vasculitis.","authors":"Gabriel Cojuc-Konigsberg,&nbsp;Isabel Comte Natera,&nbsp;Blanca E López Graciano,&nbsp;Luis Gerardo Mosqueda López,&nbsp;José Alonso Rojo Ávila,&nbsp;Braulio Martínez,&nbsp;Juan C Ramírez-Sandoval","doi":"10.1155/2022/3326761","DOIUrl":"https://doi.org/10.1155/2022/3326761","url":null,"abstract":"<p><strong>Background: </strong>Mixed cryoglobulinemia syndrome (MCS) is a rare entity with a variety of causes but has not been associated with testicular germ cell tumors. We present here a case of a patient with a nonseminomatous germ cell tumor (NSGCT) presenting as a type III mixed cryoglobulinemic vasculitis. <i>Case Presentation</i>. A 58-year-old male exhibited typical clinical features of vasculitis, including weakness, fatigue, palpable purpura, multiple mononeuropathy, and a low C4 level. An MCS diagnosis was confirmed by the presence of cryoglobulins (6%) with polyclonal IgM and IgG components and biopsy proven leukocytoclastic vasculitis. Concomitantly, a stage IIIC (TxNxM1bS1) germ tumor with marked elevation of serum beta-human chorionic gonadotropin (2764 mUI/mL) was diagnosed. An aggressive treatment was needed, including methylprednisolone pulses, plasmapheresis, rituximab, followed by orchiectomy, and chemotherapy (bleomycin/etoposide/cisplatin). After tumor resection and treatment, cryoglobulins decrease to 0%, suggesting a paraneoplastic origin of the vasculitis.</p><p><strong>Conclusion: </strong>To the best of our knowledge, this is the first case of MCS possibly attributable to a NSGCT. This case further elaborates on the presentation of mixed cryoglobulinemia vasculitis and adds to the published literature on the topic.</p>","PeriodicalId":9636,"journal":{"name":"Case Reports in Oncological Medicine","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9803560/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10467096","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Systemic Bevacizumab for Severe Recurrent Respiratory Papillomatosis. 全身贝伐单抗治疗严重复发性呼吸道乳头状瘤病。
IF 0.9 Pub Date : 2022-01-01 DOI: 10.1155/2022/2767996
Swetha Tatineni, Zachary Warren, Mark A Applebaum, Fuad M Baroody

Recurrent respiratory papillomatosis (RRP) is the most common benign pediatric laryngeal neoplasm. Various adjuvant medical therapies have failed to reliably decrease surgical frequency in this challenging airway disease. Recently, systemic bevacizumab has shown promise in advanced, treatment-resistant papillomatosis. We describe the use of systemic bevacizumab in two children with severe RRP unresponsive to other therapies. Voice and breathing improved dramatically in both patients with minimal side effects. Both patients have not required surgery in 24 months and 16 months, respectively. Systemic bevacizumab is a promising long-term treatment for severe RRP, with oncology playing an important role in patient care.

复发性呼吸道乳头状瘤病(RRP)是最常见的小儿喉部良性肿瘤。各种辅助药物治疗都不能可靠地减少这种具有挑战性的气道疾病的手术频率。最近,全身性贝伐单抗在晚期,治疗抵抗性乳头状瘤病中显示出希望。我们描述了在两名对其他治疗无反应的严重RRP儿童中使用全身贝伐单抗。两名患者的声音和呼吸都得到了显著改善,副作用最小。两名患者分别在24个月和16个月没有需要手术。系统性贝伐单抗是严重RRP的一种有希望的长期治疗方法,肿瘤学在患者护理中起着重要作用。
{"title":"Systemic Bevacizumab for Severe Recurrent Respiratory Papillomatosis.","authors":"Swetha Tatineni,&nbsp;Zachary Warren,&nbsp;Mark A Applebaum,&nbsp;Fuad M Baroody","doi":"10.1155/2022/2767996","DOIUrl":"https://doi.org/10.1155/2022/2767996","url":null,"abstract":"<p><p>Recurrent respiratory papillomatosis (RRP) is the most common benign pediatric laryngeal neoplasm. Various adjuvant medical therapies have failed to reliably decrease surgical frequency in this challenging airway disease. Recently, systemic bevacizumab has shown promise in advanced, treatment-resistant papillomatosis. We describe the use of systemic bevacizumab in two children with severe RRP unresponsive to other therapies. Voice and breathing improved dramatically in both patients with minimal side effects. Both patients have not required surgery in 24 months and 16 months, respectively. Systemic bevacizumab is a promising long-term treatment for severe RRP, with oncology playing an important role in patient care.</p>","PeriodicalId":9636,"journal":{"name":"Case Reports in Oncological Medicine","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9726241/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10372150","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Late Lung Metastasis in a Patient with a Clear Cell Chondrosarcoma: An Indication for a Life-Long Follow-Up? 透明细胞软骨肉瘤晚期肺转移:终身随访的指征?
IF 0.9 Pub Date : 2021-12-02 eCollection Date: 2021-01-01 DOI: 10.1155/2021/7205649
Paulien West, Celine Jacobs, Michael Saerens, David Creytens, Gwen Sys, Lore Lapeire

Background: Clear cell chondrosarcoma (CCCS) is a rare subtype of chondrosarcoma and comprises between 1.6% and 2.5% of all chondrosarcoma. They are known to be chemo- and radiotherapy resistant; surgical resection is therefore the therapy of choice.

Methods: We present a 63-year-old woman with a progressive lung nodule 20 years after initial diagnosis and treatment of a clear cell chondrosarcoma of the right os naviculare.

Results: On serial CT scans of the chest, an asymptomatic, slowly growing nodule in the left upper lung lobe was detected. CT-guided transthoracic biopsy of this nodule confirmed the diagnosis of a chondrosarcoma lung metastasis. Video-assisted thoracoscopic wedge resection was performed with complete removal of the nodule. The patient recovered well from surgery and remains in good health during further follow-up.

Conclusion: Given the tendency of clear cell chondrosarcoma to recur and metastasize after extended periods of time, a long-term, possibly life-long follow-up and clinical surveillance is advisable in these patients.

背景:透明细胞软骨肉瘤(CCCS)是一种罕见的软骨肉瘤亚型,占所有软骨肉瘤的1.6% - 2.5%。已知它们具有化疗和放疗抗性;因此,手术切除是首选的治疗方法。方法:我们报告一名63岁的女性,在最初诊断和治疗20年后,出现了一个进行性肺结节右脐透明细胞软骨肉瘤。结果:胸部连续CT扫描,发现左侧肺上叶无症状,缓慢生长的结节。ct引导下经胸活检证实结节为软骨肉瘤肺转移。视频辅助胸腔镜楔形切除,完全切除结节。患者术后恢复良好,在进一步随访期间健康状况良好。结论:考虑到透明细胞软骨肉瘤在较长时间后复发和转移的趋势,建议对这些患者进行长期的,可能是终身的随访和临床监测。
{"title":"Late Lung Metastasis in a Patient with a Clear Cell Chondrosarcoma: An Indication for a Life-Long Follow-Up?","authors":"Paulien West,&nbsp;Celine Jacobs,&nbsp;Michael Saerens,&nbsp;David Creytens,&nbsp;Gwen Sys,&nbsp;Lore Lapeire","doi":"10.1155/2021/7205649","DOIUrl":"https://doi.org/10.1155/2021/7205649","url":null,"abstract":"<p><strong>Background: </strong>Clear cell chondrosarcoma (CCCS) is a rare subtype of chondrosarcoma and comprises between 1.6% and 2.5% of all chondrosarcoma. They are known to be chemo- and radiotherapy resistant; surgical resection is therefore the therapy of choice.</p><p><strong>Methods: </strong>We present a 63-year-old woman with a progressive lung nodule 20 years after initial diagnosis and treatment of a clear cell chondrosarcoma of the right os naviculare.</p><p><strong>Results: </strong>On serial CT scans of the chest, an asymptomatic, slowly growing nodule in the left upper lung lobe was detected. CT-guided transthoracic biopsy of this nodule confirmed the diagnosis of a chondrosarcoma lung metastasis. Video-assisted thoracoscopic wedge resection was performed with complete removal of the nodule. The patient recovered well from surgery and remains in good health during further follow-up.</p><p><strong>Conclusion: </strong>Given the tendency of clear cell chondrosarcoma to recur and metastasize after extended periods of time, a long-term, possibly life-long follow-up and clinical surveillance is advisable in these patients.</p>","PeriodicalId":9636,"journal":{"name":"Case Reports in Oncological Medicine","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2021-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8660253/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39719713","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Primary Intraosseous Synovial Sarcoma in the Mandible. 下颌骨原发性骨内滑膜肉瘤
IF 0.9 Pub Date : 2021-11-28 eCollection Date: 2021-01-01 DOI: 10.1155/2021/9945591
Lucas Novaes Teixeira, Eduardo Zambaldi da Cruz, Ana Cláudia Garcia Rosa, Anderson Abdo Rodrigues, Fabrício Passador-Santos, Vera Cavalcanti de Araújo, Andresa Borges Soares

Synovial sarcoma (SS) is a rare malignant mesenchymal tumor that mainly occurs in body extremities, being uncommon in the head and neck region. In the present study, we described a case of primary intraosseous SS arising in the mandible of a 22-year-old young male. The patient reported a painful swelling on the left side of the mandible for the last 7 months. Imaging exams showed the presence of an expansive and multilocular radiolucent lesion, extending from the left condyle to the mandibular body. The clinic diagnostic hypotheses were ameloblastoma or malignant neoplasm. Histologically, the lesion was characterized by a proliferation of spindle cells exhibiting vesicular nuclei and evident nucleolus. Neoplastic cells were positive for AE1/AE3, cytokeratin 7, vimentin, CD-99, and TLE-1 and negative for CD-34, S-100, SMA, and HHF-35. A combination of clinical, histologic, and immunohistochemical characteristics supported the diagnosis of SS. The patient was referred for treatment, and preoperative exams did not reveal any other tumor foci in the body of the patient. The final diagnosis was of a primary intraosseous SS of the mandible.

滑膜肉瘤(SS)是一种罕见的恶性间充质肿瘤,主要发生于人体四肢,在头颈部并不常见。在本研究中,我们描述了一例原发性骨内滑膜肉瘤病例,患者是一名 22 岁的年轻男性,病发于下颌骨。患者报告说,下颌骨左侧肿胀疼痛已持续 7 个月。影像学检查显示,患者的下颌骨左侧髁状突延伸至下颌骨体部,存在扩张性多发性放射状病变。临床诊断假设为釉母细胞瘤或恶性肿瘤。从组织学角度看,病变的特点是纺锤形细胞增生,核呈水泡状,核仁明显。肿瘤细胞的AE1/AE3、细胞角蛋白7、波形蛋白、CD-99和TLE-1呈阳性,CD-34、S-100、SMA和HHF-35呈阴性。综合临床、组织学和免疫组化特征,患者被确诊为 SS。患者被转诊接受治疗,术前检查未发现患者体内有其他肿瘤病灶。最终诊断为下颌骨骨内原发性 SS。
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引用次数: 0
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Case Reports in Oncological Medicine
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