胰高血糖素是胰腺的罕见神经内分泌肿瘤,具有恶性潜能。目前,他们的流行病学并不完全清楚,所以临床医生不熟悉,对诊断或综合治疗缺乏共识。本研究报告了一例因复发性舌炎住院的32岁女性,双下肢口周皮炎和坏死性游走性红斑(NME)。影像学检查显示胰腺尾部有一个低密度结节(约2厘米),以及多发性占位性肝脏病变。然后选择手术干预,和远端胰腺切除术,进行了脾切除术和姑息性转移瘤切除术。组织检查随后证实原发性胰腺神经内分泌肿瘤(2级),转移到肝脏。NME术后解决,每28天肌肉注射长效释放奥曲肽(30mg)。在2年内还进行了一系列的三次经皮消融治疗(微波消融),靶向肝转移瘤.病人目前情况良好,至今没有皮肤复发.姑息性转移瘤,与消融治疗和生长抑素类似物(SSA)联合使用,是这个案例的独特方面,据我们所知,尚未在文献中记录。手术缓解可能使肝脏受累患者受益并延长其生存时间。同样,消融治疗和SSA注射一起提供不仅解决肝脏扩散,但也控制激素相关的症状,对预后有积极影响。因为胰高血糖素是如此罕见,他们的管理没有真正的协议。本研究旨在通过进一步增加现有数据来指导临床实践。
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.