An isolated metachronous metastasis to the adrenal gland from a pancreatic neuroendocrine tumor: A case report.
An isolated metachronous metastasis to the adrenal gland from a pancreatic neuroendocrine tumor: A case report.
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DOI：
10.1016/j.ijscr.2017.10.017
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发表时间：
2017
影响因子：
0.6
通讯作者：
Lefor AK
中科院分区：
文献类型：
--
作者：
Sakuma Y;Sata N;Endo K;Yasuda Y;Yokota S;Hosoya Y;Shimizu A;Fujii H;Matsubara D;Fukushima N;Asakawa S;Shimada YK;Lefor CK;Lefor AK
这是第一例报告的pNET转移至脾脏的患者。脾脏转移是非常罕见的，通常是在疾病播散时发生。这是第一个报告的孤立异时性转移到肾上腺从pNET，这出现在90个月后。在pNET患者中从未报告过血清PIVKA水平。在该患者中，血清PIVKA水平在5/7例复发诊断时升高，并在治疗后恢复至基线水平。提示PIVKA可能是pNET患者的一个有用的肿瘤标志物，值得进一步研究。胰腺神经内分泌肿瘤是罕见的。治疗方法包括对原发灶和转移灶进行积极的局部处理，以及全身性生长抑素治疗。这是第一个报告的孤立异时转移到肾上腺从胰腺神经内分泌肿瘤，提出了90个月后的原发肿瘤。患者为53岁男性，左上象限肿块，同时转移至脾脏和肝脏（胰腺神经内分泌肿瘤T4 N 0 M1，IV期），已切除（CD 56-、突触素+、嗜铬粒蛋白+、Ki-67 < 1%）。在接下来的90个月里，他接受了5次手术治疗肝复发（2次肝切除术和3次经皮射频消融术）。6处病变中有4处在治疗前血清PIVKA水平升高，治疗后恢复至基线水平。他现在提出，无症状，与右肾上腺肿块发现常规成像和没有其他病变。血清PIVKA升高至44 mg/dL。切除肾上腺，发现转移瘤（CD 56+、突触素+、嗜铬粒蛋白+、Ki-67 15-20%）。该患者的临床病程反映了近8年来对原发病灶和三个器官（肝、脾、肾上腺）的多个转移病灶的积极局部治疗。血清PIVKA水平在胰腺神经内分泌肿瘤患者中的应用以前没有报道，需要进一步研究。该患者患有胰腺神经内分泌肿瘤，转移至脾脏、肝脏和肾上腺，PIVKA水平升高，疾病复发。这些独特的临床特征增加了这些罕见肿瘤临床表现的多样性。
This is the first patient reported with a metastasis to the spleen from a pNET. Spleen metastases are extremely rare and are usually seen with disseminated disease. This is the first report of an isolated metachronous metastasis to the adrenal gland from a pNET, which appeared after 90 months. Serum PIVKA levels have never been reported in a patient with pNET. In this patient, serum PIVKA level was elevated at the time of diagnosis of 5/7 recurrences and returned to baseline levels following treatment. This suggests that PIVKA may be a useful tumor marker in patients with pNET, and deserves further study. Pancreatic neuroendocrine tumors are rare. Treatment includes aggressive local management of the primary lesion and metastases, and systemic somatostatin. This is the first report of an isolated metachronous metastasis to the adrenal gland from a pancreatic neuroendocrine tumor that presented 90 months after the primary tumor. The patient presented as a 53yo man with a left upper quadrant mass and synchronous metastases to the spleen and liver (pancreatic neuroendocrine tumor T4N0M1, Stage IV), which were resected (CD56-, synaptophysin+, chromogranin+, Ki-67 < 1%). Over the next 90 months, he underwent five procedures to treat hepatic recurrences (2 liver resections and 3 percutaneous radiofrequency ablations). Serum PIVKA levels were elevated prior to treatment of four of six lesions and returned to baseline after therapy. He presents now, asymptomatic, with a right adrenal mass found on routine imaging and no other lesions. Serum PIVKA was elevated to 44 mg/dL. The adrenal gland was resected and shown to be a metastasis (CD56+, synaptophysin+, chromogranin+, Ki-67 15–20%). This patient’s clinical course reflects aggressive local therapy of the primary lesion and multiple metastatic lesions to three organs (liver, spleen, adrenal) over nearly eight years. The utility of serum PIVKA levels in patients with pancreatic neuroendocrine tumors is not previously reported and needs further investigation. This patient has a pancreatic neuroendocrine tumor with metastases to the spleen, liver and adrenal gland and elevated PIVKA levels with recurrent disease. These unique clinical features add to the diversity of clinical presentation of these rare tumors.