Fatima Asma, Chandra Suresh, Fatima Saubia, Izhar Mohammed Yasir, Bokhari Syed Faqeer Hussain, Iqbal Asma
Department of General Surgery, Vikarabad Government Medical College, India.
Department of Surgical Gastroenterology, Deccan College of Medical Sciences, India.
Int J Surg Case Rep. 2024 Dec;125:110647. doi: 10.1016/j.ijscr.2024.110647. Epub 2024 Nov 23.
Primary hepatic neuroendocrine tumors (PHNETs) are rare, accounting for approximately 0.3 % of all neuroendocrine tumors (NETs) and are often difficult to diagnose due to their nonspecific symptoms and imaging features. Standard diagnostic and treatment protocols are lacking due to their rarity, but imaging and immunohistochemistry (IHC) remain key tools for diagnosis.
A 68-year-old male presented with abdominal discomfort and loss of appetite. Imaging revealed a large exophytic mass in the left lobe of the liver. After ruling out extrahepatic primary sources, a left lobe hepatectomy was performed. Histopathology confirmed the diagnosis of PHNET, with positive IHC staining for synaptophysin and CK-7. Postoperative PET-CT ruled out any distant metastases. The patient had an uneventful recovery.
PHNETs are believed to originate from ectopic neuroendocrine cells in the liver, though several theories exist. Imaging alone cannot conclusively diagnose PHNETs, as they mimic other hepatic tumors like hepatocellular carcinoma. Histopathological examination, along with IHC markers like chromogranin and synaptophysin, is essential for diagnosis. Surgical resection remains the treatment of choice, with good outcomes despite the risk of recurrence. Non-surgical therapies, such as chemotherapy or ablation, are under investigation but lack consensus.
PHNETs are rare and challenging to diagnose, requiring imaging and IHC for confirmation. Surgery offers the best prognosis, making personalized, surgery-centered treatment plans essential for management. Comprehensive follow-up, including functional imaging, is necessary to monitor recurrence or metastasis.
原发性肝神经内分泌肿瘤（PHNETs）较为罕见，约占所有神经内分泌肿瘤（NETs）的0.3%，由于其症状和影像学特征不具特异性，常难以诊断。因其罕见性，缺乏标准的诊断和治疗方案，但影像学检查和免疫组织化学（IHC）仍是诊断的关键手段。
一名68岁男性出现腹部不适和食欲减退。影像学检查显示肝脏左叶有一个巨大的外生性肿块。排除肝外原发灶后，进行了左叶肝切除术。组织病理学确诊为PHNET，免疫组织化学染色突触素和细胞角蛋白7（CK-7）呈阳性。术后PET-CT排除了任何远处转移。患者恢复顺利。
尽管存在多种理论，但PHNETs被认为起源于肝脏中的异位神经内分泌细胞。仅靠影像学检查不能确诊PHNETs，因为它们可类似其他肝脏肿瘤，如肝细胞癌。组织病理学检查以及嗜铬粒蛋白和突触素等免疫组织化学标志物对于诊断至关重要。手术切除仍是首选治疗方法，尽管有复发风险，但预后良好。化疗或消融等非手术治疗方法正在研究中，但尚未达成共识。
PHNETs罕见且诊断具有挑战性，需要影像学检查和免疫组织化学检查来确诊。手术提供了最佳预后，因此制定以手术为中心的个性化治疗方案对于管理至关重要。包括功能成像在内的全面随访对于监测复发或转移是必要的。
