Tightening the NET in a Rare Cause of Jaundice

A 38-year-old female with no comorbid conditions had come with c/o gradually increasing abdominal distension, abdominal pain, and jaundice for the past 3 months. O/E patient was icteric and a tender right hypochondrial mass of size 7 × 5 cm was palpable. Investigations revealed a deranged liver function with total bilirubin of 15.4, direct bilirubin 10.2, SGOT: 138, SGPT: 196, ALP: 864, and INR: 2.3.USG abdomen revealed a large heteroechoic lesion in right lobe of liver infiltrating GB with multiple peritoneal deposits suggestive of HCC. CECT abdomen showed hepatomegaly with large heterogeneously enhancing mass lesion involving segments 4, 5, 6, and 2 of liver with GB infiltration with metastatic omental and pelvic deposits suggestive of HCC. Serum levels of alpha-fetoprotein, cancer antigen 125, and ferritin were normal. EUS-guided biopsy from the liver lesion confirmed neuroendocrine carcinoma based on the IHC staining. The tumor was immunoreactive for CK7, synaptophysin SATB2, CGA, INSM 1, and Ki-67 index of 95% which confirmed the diagnosis of poorly differentiated Grade 3 NEC—small cell carcinoma type. Patient underwent OGD, colonoscopy, and PET CT to investigate the primary site of NEC which did not reveal any lesion in other organs. Hence the patient was diagnosed with primary hepatic neuroendocrine carcinoma. In view of the large tumor size and poor physical condition of the patient, surgical resection was deferred and planned for chemotherapy. Liver NETs are one of the rarest subtypes constituting less than 0.3% of NETs and less than 1% of primary liver tumors with only about 150 cases reported till now. Thus, it is challenging to diagnose a case of NET as it is similar to HCC and if it is of primary or metastatic origin and should be kept in mind while investigating a liver tumor.

Publication History

Article published online:
22 April 2024