Primary multicentric hepatic neuroendocrine tumor with bone and pleural metastasis: A case report and review of literature

Primary hepatic neuroendocrine carcinoma (PHNEC) is a rare form of cancer, accounting for less than 1% of all neuroendocrine neoplasms. Due to its rarity, it often presents significant diagnostic challenges and is frequently mistaken for hepatocellular carcinoma or metastatic disease. This study aims to report a unique case of multicentric primary hepatic neuroendocrine carcinoma with metastases to the bone and pleura. It highlights the difficulties encountered in both diagnosis and treatment, underscoring the necessity for tailored therapeutic approaches and the need for further investigation in this area.A 51-year-old woman experienced one month of right upper abdominal pain and loss of appetite, leading to imaging that revealed multiple liver lesions. A biopsy confirmed a primary multicentrichepatic neuroendocrine tumor, with normal tumor markers (AFP, CEA, CA19.9) and no extrahepaticlesions found on pan-endoscopy. Initially treated with Etoposide and Carboplatin, she showedprogressive disease after three cycles. Subsequent treatment with capecitabine and temozolomidewas ineffective, and she ultimately succumbed to the diseasePHNECs are exceedingly rare, complicating their diagnosis and management. Currentliterature is limited to isolated case reports, highlighting the importance of this study. Management of PHNEC requires a multidisciplinary approach, with treatment plans tailoredto disease presentation and patient health. In our study, the patient received three cycles ofan etoposide-carboplatin regimen, followed by a response assessment scan that showedprogressive disease. The patient did not respond significantly to subsequent treatment withthree cycles of the CAPTEM regimen and ultimately succumbed to the disease. PHNEC should be considered a potential differential diagnosis for liver tumors. The currentstudy provides valuable insight into the clinical presentation, diagnosis, management, andoutcomes of the disease. There is no standardized treatment protocol for metastaticPHNEC; decisions must be individualized, with options including surgery, chemotherapy,targeted therapies, or PRRT, emphasizing the need for further research.