Mixed adenoendocrine carcinoma in the extrahepatic biliary tract: A case report and literature review

Abstract

Neuroendocrine neoplasm (NEN) comprises a group of tumors that exhibit neuroendocrine phenotypes. NEN is subclassified into neuroendocrine tumor (NET), neuroendocrine carcinoma (NEC) and mixed adenoendocrine carcinoma (MANEC), based on histopathological parameters. NEN in the extrahepatic biliary tract (EHBT) is uncommon. Little is known about its clinicopathological features and prognostic indicators. The present study presented a case of MANEC in the distal common bile duct (CBD) and reviewed previous cases of NENs in the EHBT to characterize the clinical settings of this disease entity and to identify influencing factors of survival outcomes. A 64-year-old Chinese woman presented with abdominal pain and jaundice. Imaging studies demonstrated malignant stenosis in the distal CBD. Bile duct brush cytology revealed small clusters of atypical cells. Following an initial diagnosis of distal cholangiocarcinoma (CCA), the patient underwent pancreaticoduodenectomy. Histological analysis combined with immunohistochemical investigation of the resected specimen revealed a collision tumor that was composed of poorly differentiated adenocarcinoma and NEC. Each histological component accounted for >30% of the tumor. The definitive diagnosis was a MANEC in the distal CBD. Multiple intrahepatic and pulmonary metastases were observed postoperatively over 8 months. The patient succumbed to the disease 12 months after surgery. In conclusion, NEN occurs infrequently in the EHBT, with NET being the predominant type. NEN in the EHBT is extremely challenging to diagnose preoperatively due to its tendency to mimic CCA. Patients with NEN in the EHBT exhibited extremely distinct oncology outcomes according to pathological types. Additionally, old age (>60 years) and the presence of tumor recurrence were associated with decreased survival of patients with NEN.

Mixed Neuroendocrine Non-Neuroendocrine Neoplasms: A Systematic Review of a Controversial and Underestimated Diagnosis

“…The quantitative composition of the primary tumour was described in 36 studies (n = 294) [12,15,17,19,21,[23][24][25]27,29,30,34,36,[39][40][41]45,46,[48][49][50][52][53][54]56,57,59,65,73,74,80,82,[84][85][86]92]; the two components were present in equal proportion in 27.9% (n = 82) of cases, whereas in the remaining 72.1% one of the two histologies was predominant; the neuroendocrine component in 42.2% (n = 124), the non-neuroendocrine component in 29.9% (n = 88).…”

“…Interestingly, in 43 studies (n = 61) where more than one diagnostic sample was available [11,[13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28]30,[32][33][34][36][37][38][39][41][42][43][44][45][46][47]49,50,53,55,[59][60][61][62]64,65,96], the initial diagnosis from the first sample collected (either cytological or histological) was in keeping with MiNEN or suspicion of MiNEN in 36.1% (n = 22) of cases, adenocarcinoma in 36.1% (n = 22), poorly differentiated neuroendocrine carcinoma in 21.3% (n = 13), and well differentiated neuroendocrine tumour in 6.6% (n = 4).…”

Challenges in treatment of a patient suffering from neuroendocrine tumor G1 of the hilar bile duct: a case report

“…However, Wang HH et al [9] reported that the positivity of CA19-9 was only 15.0%, CA72-4 was 7.5%, CEA was 17.5%, and AFP was 15.0% in primary hepatic neuroendocrine tumors. Furthermore, serum tumor markers are normal in most EBNET G1/G2, but they tend to be higher than normal levels in EBNET G3 or extrahepatic biliary neuroendocrine carcinomas [10][11][12][13][14]. Our patient with the hilar bile duct NETs G1 had serum tumor markers were within normal limits.…”

Rapidly progressed neuroendocrine carcinoma in the extrahepatic bile duct: a case report and review of the literature

“…We searched the PubMed database using the terms “neuroendocrine carcinoma,” “bile duct,” “endocrine carcinoma,” and “mixed adeno-neuroendocrine carcinoma.” Excluding NECs in the intrahepatic bile duct, gallbladder, and ampulla of Vater, only 24 resected cases of primary EHBD NECs were found [ 3 , 10 – 32 ]. We briefly summarized these clinical and pathological features (Tables 1 and 2 ).…”