Successful Correction of the Misdiagnosis of a Primary Liver Neuroendocrine Tumour with a Combination of Immunohistochemical and Radiological Assessments: Report of a Rare Case from Pakistan

Abstract

Human neuroendocrine tumours arise in different organs including most frequently in the gall bladder, kidneys, and ovaries or testicles. Liver neuroendocrine tumours are rarer types that grow slowly and arise from neuroendocrine cells. A 50-year-old woman with a history of type 1 diabetes mellitus and hypertension presented with severe abdominal pain, fever, pallor and jaundice over two days. She had not history of cancer. Initially, she was treated medically with Ciproxin, Entamizole, Zentel, and Nospa, but her symptoms were not resolved, and she was referred for gastroduodenal endoscopy, radiological and blood tests. The gastroduodenal endoscopic evaluation revealed severe gastritis with mild erythema in the corpus and antrum of the stomach with no evidence of esophagitis or duodenitis. At the time of initial diagnosis, the patient was asymptomatic for a liver neuroendocrine tumour as she did not have anemia, weight loss, abdominal distension, severe abdominal pain, or a palpable right upper quadrant mass. The radiological investigations, abdominal and pelvic ultrasound and computed tomography scan, detected hepatomegaly with a mass of an internal necrotic area in left lobe and smaller lesions in the right lobe. Liver core biopsies taken from the left lobe contained hepatocellular carcinoma. After a hepatectomy of the left lobe, the patient was stable and managed with intravenous fluid, analgesics, antibiotics, and antiemetic drugs and was discharged. Hepatocellular carcinoma was diagnosed in the resection specimen. The resected tumour tissue was analysed through immunohistochemistry for presence of Sheppard, arginase, Cam5.2 and CDX-2, heppar, arginase, PAX8, Albumin ISH, CK7, CK20 and INSM1, synaptophysin, chromogranin, Ki67, Synaptophysin, Cytokeratin AE1/AE3, synaptophysin, CD56, and Glypican-3. Consequently, liver neuroendocrine cancer was diagnosed. The patient was treated with Sandostatin for 7 days to treat the neuroendocrine tumour. Following a left hepatectomy, post-surgery endoscopic retrograde cholangiopancreatography (ERCP) showed normal ampullary mucosa and successful surgical biliary drainage was observed for almost 45 days with bile drain effluent between 20-230 ml. In this case, the differentiation of this liver neuroendocrine tumour from hepatocellular carcinoma, hyper vascular hepatic lesions based upon detection of chromogranin, CK7, CK20, HepPar1, Ki67 allowed appropriate clinical management of the patient. During radiological analysis, it was confirmed that the liver mass was not metastasized from the primary tumour of the stomach or colon.