Rare Mixed Adenoneuroendocrine Carcinoma of the Gallbladder: Case Report and Review of Literature

Abstract

Patient: Female, 58-year-old Final Diagnosis: Mixed adenoneuroendocrine carcinoma of the gallbladder Symptoms: Abdominal pain • asthenia Medication: — Clinical Procedure: — Specialty: Surgery Objective: Rare disease Background: Mixed adenoneuroendocrine carcinoma of the gallbladder (gMANEC) is an extremely rare cancer. Most of the cases are reported in Asia, North America, and Europe, with no cases reported in Latin America; this is the first report for this region, and the 24 th case reported worldwide. Case Report: A 68-year-old woman was referred to our department due to asthenia and moderate abdominal pain in the right upper quadrant for 6 months, with imaging examinations showing a solid heterogeneous expansive lesion in gallbladder topography and segment IV of the liver. The MRI displayed an expansive and heterogeneous lesion with inaccurate limits in the gallbladder affecting segment IVb of the liver, in addition to lymphadenopathy in the hepatic hilum. A cholecystectomy with resection of segments IV-B and V of the liver (radical cholecystectomy) and hepatic hilar lymphadenectomy were performed. Anatomopathological examination and immunohisto-chemistry confirmed a primary mixed adenoneuroendocrine carcinoma of the gallbladder. The patient received adjuvant chemotherapy and radiotherapy; however, after the patient reported experiencing low back pain, a CT was performed, revealing retroperitoneal metastasis, and the radiotherapy was interrupted. Currently, the patient has a stable disease, following a protocol of 5-Fluorouracil and somatostatin, and she reports having low back pain of low intensity. Conclusions: This is the 24 th gMANEC case reported in the literature. The tumor was successfully resected; however, the patient presented retroperitoneal metastasis 6 months after surgery, despite combined adjuvant therapy.

Comprehensive analysis of mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs): A SEER database analysis of 767 cases

“…The rarity of MiNENs makes it difficult to get high quality evidence from prospective randomized controlled trials to optimize its clinical care. Therefore, retrospective studies at a Increasing reports of MiNENs have been published during the last 20 years, however, the majority of them were small retrospective studies from singe centers, which likely did not show the real epidemiological trend of all of the diagnosed MiNENs (10)(11)(12)(13). Our study showed that in 2000, only 13 cases of MiNEN (composite carcinoid) were found in the SEER database, however, by 2017, it increased ten-fold.…”

Focal neuroendocrine carcinoma mixed with adenocarcinoma of the gallbladder with aggressive lymph node metastasis in a patient who did not meet the mixed neuroendocrine–non-neuroendocrine neoplasm criteria

“…Gallbladder MiNENs are extremely rare. Costa et al [ 6 ] published a literature review in 2021, and reported that there are only 24 cases of gallbladder MiNEN (which used to be called MANEC) including this case, reported across the literature. Although our case did not meet the criteria for MiNEN [ 4 ], this report supports our understanding of how rare and aggressive the gallbladder MiNEN is, and therefore, close postoperative surveillance is critical.…”