Primary hepatic neuroendocrine carcinoma: report of two cases and literature review

Abstract

Background Primary hepatic neuroendocrine carcinoma (PHNEC) is extremely rare. The diagnosis of PHNEC remains challenging—partly due to its rarity, and partly due to its lack of unique clinical features. Available treatment options for PHNEC include surgical resection of the liver tumor(s), radiotherapy, liver transplant, transcatheter arterial chemoembolization (TACE), and administration of somatostatin analogues. Case presentation We report two male PHNEC cases and discuss the diagnosis and treatment options. Both cases presented with abdominal pain; case two also presented with symptoms of jaundice. The initial diagnosis for both cases was poorly differentiated grade 3 small-cell neuroendocrine carcinoma, based on imaging characteristics and the pathology of liver biopsies. Final diagnoses of PHNEC were arrived at by ruling out non-hepatic origins. Case one presented with a large tumor in the right liver lobe, and the patient was treated with TACE. Case two presented with tumors in both liver lobes, invasions into the left branch of hepatic portal vein, and metastasis in the hepatic hilar lymph node. This patient was ineligible for TACE and was allergic to the somatostatin analogue octreotide. This limited treatment options to supportive therapies such as albumin supplementation for liver protection. Patient one and two died at 61 and 109 days, respectively, following initial hospital admission. Conclusions We diagnosed both cases with poorly differentiated grade 3 small-cell PHNEC through imaging characteristics, immunohistochemical staining of liver biopsies, and examinations to eliminate non-hepatic origins. Neither TACE nor liver protection appeared to significantly extend survival time of the two patients, suggesting these treatments may be inadequate to improve survival of patients with poorly differentiated grade 3 small-cell PHNEC. The prognosis of poorly differentiated grade 3 small-cell PHNEC is poor due to limited and ineffective treatment options.

What is your diagnosis? Impression smear from a liver mass in a dog

“…1 The latter study also found that NSE was more sensitive (80% positive) than chromogranin-A (30% positive), whereas chromogranin-A is the preferred marker for human neuroendocrine tumors. [1][2][3] Positive staining reactions for CKAE1/AE3 have been reported in PHNEC in humans, as seen in this case. 3 Differential diagnoses, in this case, included a primary hepatic myoepithelial carcinoma and cholangiocarcinoma.…”

“…[1][2][3] Positive staining reactions for CKAE1/AE3 have been reported in PHNEC in humans, as seen in this case. 3 Differential diagnoses, in this case, included a primary hepatic myoepithelial carcinoma and cholangiocarcinoma. There is a single report of a dog with a primary hepatic myoepithelioma that was positive for CKAE1/AE3 and negative for CK19 on immunohistochemical staining.…”

Primary hepatic neuroendocrine carcinoma diagnosed by needle biopsy: a case report

“…Thus, surgical resection must be considered for curative intent [ 3 ]. Surgical resection of NEC is the most common treatment, particularly for localized primary hepatic NEC, whereas transarterial chemoembolization (TACE) is normally performed for advanced primary hepatic NECs that are poor candidates for resection [ 3 , 12 ]. TACE can be used for cytoreduction of NETs because NETs are hypervascular and sensitive to ischemia [ 3 ].…”

“…TACE can be used for cytoreduction of NETs because NETs are hypervascular and sensitive to ischemia [ 3 ]. Due to the lack of availability of substantial high-quality data, there is no standard therapy for primary hepatic NEC [ 2 , 9 , 10 , 12 ]. Therefore, selecting an appropriate treatment depending on several factors such as tumor stage and differentiation and a patient’s performance status and clinical course is preferred [ 2 , 9 – 12 ].…”

Primary Hepatic Neuroendocrine Carcinoma

“…TACE and other methods of liver protection have been utilized but do not appear to significantly extend the survival time of patients with PHNEC. 3 It is not clear whether the histology of the tumor itself deems the tissue amenable to the effects of certain chemotherapies as there have been so few available for study. For now, however, we propose that surgical resection is the best definitive treatment for these extremely rare carcinomas.…”