Hepatic neuroendocrine tumor

Case contributed by Ammar Ashraf

Presentation

Abdominal pain and distension. No fever, vomiting, anorexia, or weight loss.

Patient Data

Age: 55 years
Gender: Male
ultrasound

Multiple heterogeneous echogenicity lesions containing both solid and cystic components, showing no significant internal vacsularity on doppler ultrasound examination, are noted in the right lobe of the liver.

 

FINDINGS: Enlarged liver with multiple variable size heterogeneous hypodense lesions predominantly in the right hepatic lobe. These lesions are complex in nature, have both solid and cystic components, and show mild enhancement in the post-contrast study. The gross morphology of the bowel is also within normal limits. Small fat-containing left inguinal hernia. CONCLUSION: Enlarged liver with multiple variable size complex heterogeneous lesions, suspicious of metastases, with no appreciable abdominal primary lesion. Possible differential diagnosis includes biliary cystadenoma, hepatic hydatid cyst, amoebic hepatic abscesses, hepatic tuberculosis, and hepatic mesenchymal hamartoma.

I123-MIBG

Nuclear medicine

DESCRIPTION: The study revealed good physiological distribution of the injected radiotracer. Early and delayed whole-body scan showed multiple photon deficient areas in the liver (predominantly in the right hepatic lobe). No MIBG avid lesions are seen anywhere in the imaged body to suggest metastatic dissemination.  Physiological uptake of the radiotracer is seen in the salivary & thyroid glands as well as in the gut. For further evaluation with octreotide scan.

Tc-99m tekrotyd (Technetium labeled octreotide)

Nuclear medicine

DESCRIPTION: The whole body and SPECT images showed multiple areas of increased uptake of the octreotide in the liver. No abnormal radiotracer activity is seen in the rest of the imaged body to suggest any metastatic disease. Physiological uptake of octreotide is noted in the thyroid, liver, spleen, gut, kidneys, and urinary bladder. 

Case Discussion

  • The patient was diagnosed as metastatic adenocarcinoma of the liver with an unknown primary (on imaging & liver biopsy) 15 years ago in another health facility and was treated with cisplatin-based chemotherapy. Unfortunately, the previous imaging and histopathology record was not available for review. The patient had no history of pulmonary tuberculosis.
  • After abdominal ultrasound examination and triphasic CT abdomen, further investigations were done, particularly for GIT malignancy, tuberculosis, amebiasis, and hydatid disease, all of which were negative. The case was discussed in our tumor board meeting and decided to proceed with the hepatic lesion biopsy, under anthelmintic (albendazole 400 mg bid for four weeks) cover.
  • Procedure: Ultrasound-guided Tru-cut biopsy of the hepatic lesion. Microscopic Description: The tumor is well-circumscribed limited by pseudo-fibrous pseudo-capsule. The tumor proliferation is composed of the small nests of syncytial cells with abundant basophilic cytoplasm and hyperchromatic nuclei showing sometimes inconspicuous nucleoli or some pseudo-inclusions.  Some of the tumor cells show pseudo-glandular cavities and some are filled by large hyaline bodies. The stroma between the nests is very highly vascularized and in the nest, some cells are multinucleated.  Moderate atypia but no abnormal mitosis is seen.  These morphological features are suggestive of hepatic neuroendocrine tumor. Immunostaining: The immunostains with the antibody anti-Pancytokeratin (AE1/AE3), anti-CK7, and anti-Synaptophysin are diffusely positive. CK19 is focally positive. CK20 is also positive in rare cells. The immunostain with the antibody anti-Vimentin highlight the reached vascular channels surrounding the tumor nest. The immunostains with the antibody chromogranin, antibody anti-hepatocyte, anti-CD10, anti-smooth actin, alpha-fetoprotein, antibody anti-HMB45, anti-S100, and the antibody anti-Glypican are negative. The Ki67 index is 1%.  Final Diagnosis: Neuroendocrine tumor, low grade (< 2 mitoses per 10 high-power field, Ki67 <1%).
  • I123-MIBG & octreotide scans were done after the liver biopsy result. 
  • The patient was referred to the medical oncologist for further management.

 

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