Yu Shenghui, Ge Muxi, Zhang Cheng, Chen Lang, Zhao Lei
Department of Hepatobiliary and Pancreatic Surgery, The First Hospital of China Medical University, Shenyang, Liaoning 110001, P.R. China.
Department of Pancreatic and Biliary Surgery, The First Hospital of China Medical University, Shenyang, Liaoning 110001, P.R. China.
Oncol Lett. 2024 Mar 11;27(5):202. doi: 10.3892/ol.2024.14336. eCollection 2024 May.
Glucagonomas are rare neuroendocrine neoplasms of the pancreas with malignant potential. At present, their epidemiology is not entirely clear, so clinicians are not well versed, lacking any consensus on diagnosis or comprehensive treatment. The present study reports the case of a 32-year-old woman hospitalized for recurrent glossitis, perioral dermatitis and necrolytic migratory erythema (NME) of both lower limbs. Imaging studies revealed a low-density nodule (~2 cm) in the tail of the pancreas, as well as multiple space-occupying hepatic lesions. Surgical intervention was then selected, and distal pancreatectomy, splenectomy and palliative metastasectomies were performed. Tissue examination subsequently confirmed a primary pancreatic neuroendocrine tumor (grade 2), metastatic to the liver. The NME resolved postoperatively, aided by intramuscular injections of long-acting release octreotide (30 mg) every 28 days. A series of three percutaneous ablative treatments (microwave ablation) were also undertaken within a 2-year period, targeting the liver metastases. The present condition of the patient is good, with no cutaneous relapse to date. Palliative metastasectomies, in conjunction with ablative treatments and combination somatostatin analog (SSA) use, are unique aspects of this case that, to the best of our knowledge, have yet to be documented in the literature. Surgical palliation may benefit patients with liver involvement and prolong their survival time. Likewise, ablative treatments and SSA injections delivered together not only address hepatic spread, but also control hormone-related symptoms, having a positive impact on prognosis. As glucagonomas are so rare, there is no real agreement on their management. The present study aims to guide clinical practice by adding further to the available data.
胰高血糖素瘤是一种罕见的具有恶性潜能的胰腺神经内分泌肿瘤。目前,其流行病学尚不完全清楚,因此临床医生并不十分精通,在诊断或综合治疗方面缺乏共识。本研究报告了一名32岁女性的病例,该患者因复发性舌炎、口周皮炎和双下肢坏死性游走性红斑(NME)住院。影像学检查显示胰腺尾部有一个低密度结节(约2 cm),以及肝脏多发占位性病变。随后选择了手术干预,进行了远端胰腺切除术、脾切除术和姑息性转移灶切除术。组织检查随后证实为原发性胰腺神经内分泌肿瘤(2级),已转移至肝脏。术后NME症状缓解,这得益于每28天肌肉注射一次长效奥曲肽(30 mg)。在2年时间内还针对肝转移灶进行了一系列三次经皮消融治疗(微波消融)。患者目前状况良好,至今无皮肤复发。姑息性转移灶切除术,结合消融治疗和联合使用生长抑素类似物(SSA),是本病例的独特之处,据我们所知,此前文献中尚未有记载。手术姑息治疗可能使有肝脏受累的患者受益并延长其生存时间。同样,消融治疗和SSA注射联合使用不仅能解决肝转移问题,还能控制激素相关症状,对预后有积极影响。由于胰高血糖素瘤非常罕见,其治疗尚无真正的共识。本研究旨在通过增加现有数据来指导临床实践。