A314 未分化胰腺癌伴破骨细胞样巨细胞:病例报告

M. K. Parvizian, D. Hurlbut, M. S. Rai
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Methods Case report and literature review. Results A 79-year-old man with atrial fibrillation on Xarelto presented with 4 months of abdominal pain, nausea, vomiting, and weight loss (22 kg). An MRI revealed a dilated main pancreatic duct and side branches with abrupt cutoff in the neck due to an ill-defined lesion. Endoscopic ultrasound confirmed a 36 mm hypoechoic ill-defined mass in the pancreatic neck. Biopsy performed with a 22-gauge needle with dry suction showed tissue fragments consistent with UCOGC (Figure 1). Unfortunately, the patient presented to hospital with worsened abdominal pain and nausea and was found to have new portal, superior mesenteric, and splenic vein thrombosis. His anticoagulant was changed to Dalteparin. A multidisciplinary cancer conference was held including input from experts in Toronto, and due to surrounding inflammation and new thrombosis he was not considered a surgical candidate at the time and neoadjuvant treatment with standard adenocarcinoma chemotherapy was planned. Unfortunately, the patient continued to decline and he was treated with palliative intent FOLFIRINOX for 2 cycles before opting for a medical assistance in dying procedure. Conclusions Treatment for UCOGC has not been well studied due to its rarity. Surgery is first-line therapy if appropriate based on patient status and cancer stage. The efficacy of chemotherapy and radiation is unclear but as a suspected adenocarcinoma variant, standard chemotherapy regimens are often used. Research is ongoing with an area of interest being PD-L1 inhibition due to high tumor PD-L1 positivity rates. 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引用次数: 0

摘要

摘要 背景 含破骨细胞样巨细胞的胰腺未分化癌(UCOGC)是一种罕见的胰腺癌,占胰腺癌病例的 1%以下。据推测,UCOGC 是胰腺腺癌的一种变异型,虽然其病理生理学尚未完全明确,但人们认为它是一种具有破骨性巨细胞趋化作用的未分化肉瘤样癌变异型。临床、放射学或生化特征无法将其与其他肿瘤可靠地区分开来。组织学检查显示非肿瘤性多核破骨巨细胞(CD68 阳性)、单核组织细胞和肿瘤性单核细胞,即可明确诊断。目的 描述一名 UCOGC 患者的病例,强调主要临床病理特征和治疗方法。方法 病例报告和文献综述。结果 一位 79 岁的男性患者因心房颤动服用沙瑞托(Xarelto)治疗 4 个月后出现腹痛、恶心、呕吐和体重下降(22 公斤)。核磁共振成像显示主胰管和侧支扩张,颈部因病变不明确而突然断流。内窥镜超声波检查证实,胰腺颈部有一个 36 毫米的低回声不明确肿块。用 22 号针头干抽吸活检显示,组织碎片与 UCOGC 一致(图 1)。不幸的是,患者因腹痛和恶心加剧而入院,并被发现有新的门静脉、肠系膜上静脉和脾静脉血栓形成。他的抗凝剂改为达肝素。由于周围炎症和新的血栓形成,当时认为他不适合手术治疗,因此计划采用标准腺癌化疗进行新辅助治疗。不幸的是,患者病情继续恶化,他在接受了两个周期的姑息性 FOLFIRINOX 治疗后,选择了医疗协助死亡程序。结论 UCOGC 的治疗方法因其罕见性尚未得到充分研究。根据患者状况和癌症分期,手术是合适的一线治疗方法。化疗和放疗的疗效尚不明确,但作为一种疑似腺癌变体,通常采用标准化疗方案。由于肿瘤 PD-L1 阳性率较高,PD-L1 抑制剂是一个值得关注的研究领域。由于这种罕见肿瘤具有独特的诊断和治疗特点,因此对其进行识别至关重要,同时还需要更多的研究来确定最佳疗法。图 1:HPS 和 CD68 染色显示 UCOGC 的病理检查 资助机构 无
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A314 UNDIFFERENTIATED PANCREATIC CARCINOMA WITH OSTEOCLAST-LIKE GIANT CELLS: A CASE REPORT
Abstract Background Pancreatic undifferentiated carcinoma with osteoclast-like giant cells (UCOGC) is a rare form of pancreatic cancer representing under 1% of cases. UCOGC is hypothesized to be a variant of pancreatic adenocarcinoma and while the pathophysiology is incompletely defined, it is felt to be an undifferentiated sarcomatoid carcinoma variant with chemotaxis of osteoclastic giant cells. Clinical, radiographic, or biochemical features do not reliably differentiate this from other tumors. Definitive diagnosis is made by histologic examination demonstrating non-neoplastic multinucleated osteoclastic giant cells (CD68 positive), mononuclear histiocytes, and neoplastic mononuclear cells. Aims To describe the case of a patient with UCOGC, highlighting key clinicopathologic features and management. Methods Case report and literature review. Results A 79-year-old man with atrial fibrillation on Xarelto presented with 4 months of abdominal pain, nausea, vomiting, and weight loss (22 kg). An MRI revealed a dilated main pancreatic duct and side branches with abrupt cutoff in the neck due to an ill-defined lesion. Endoscopic ultrasound confirmed a 36 mm hypoechoic ill-defined mass in the pancreatic neck. Biopsy performed with a 22-gauge needle with dry suction showed tissue fragments consistent with UCOGC (Figure 1). Unfortunately, the patient presented to hospital with worsened abdominal pain and nausea and was found to have new portal, superior mesenteric, and splenic vein thrombosis. His anticoagulant was changed to Dalteparin. A multidisciplinary cancer conference was held including input from experts in Toronto, and due to surrounding inflammation and new thrombosis he was not considered a surgical candidate at the time and neoadjuvant treatment with standard adenocarcinoma chemotherapy was planned. Unfortunately, the patient continued to decline and he was treated with palliative intent FOLFIRINOX for 2 cycles before opting for a medical assistance in dying procedure. Conclusions Treatment for UCOGC has not been well studied due to its rarity. Surgery is first-line therapy if appropriate based on patient status and cancer stage. The efficacy of chemotherapy and radiation is unclear but as a suspected adenocarcinoma variant, standard chemotherapy regimens are often used. Research is ongoing with an area of interest being PD-L1 inhibition due to high tumor PD-L1 positivity rates. Recognition of this rare entity is crucial due to its distinct diagnostic and therapeutic characteristics with more research needed to establish optimal therapy. Figure 1: Pathologic examination with HPS and CD68 stains demonstrating UCOGC Funding Agencies None
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