Metastatic mixed acinar-endocrine carcinoma of the pancreas treated with a multidisciplinary approach: a case report

Abstract

BackgroundPancreatic neoplasms are usually characterized by ductal, acinar, or endocrine differentiation. Mixed exocrine and endocrine pancreatic tumours are extremely rare. Here, we report a case of pancreatic mixed acinar-endocrine carcinoma (MAEC) with multiple synchronous liver metastases that were treated with surgery and transcatheter arterial chemoembolization (TACE) that later recurred in the stomach.Case presentationA 45-year-old female with severe anaemia was referred to our hospital. Computed tomography (CT) demonstrated a hypervascular tumour, 17 cm in diameter, that was in the tail of the pancreas. In addition, there were multiple hypervascular tumours in the liver. She underwent a distal pancreatectomy with splenectomy after the liver metastases were treated with TACE. Pathology confirmed that the pancreatic tumour was MAEC. After 4.5 years, a follow-up CT showed a hypervascular tumour at the upper part of the stomach. Gastric endoscopy showed a big tumefactive lesion with surface irregularities, gastric erosion, and multiple dilated vessels in the fornix and greater curvature of the stomach. She underwent a proximal gastrectomy and survived 7 years and 2 months after the start of the treatment.ConclusionsThis is the first report of a metastatic stomach tumour from pancreatic MAEC, which was successfully treated with a multidisciplinary approach. Additionally, we review the literature and discuss the treatment of MAEC.

Pancreatic mixed acinar‐neuroendocrine carcinoma, a unique diagnostic challenge on FNA cytology: A small series of two cases with literature review

“…Our search of the English‐language literature yielded 44 (including our own) well‐documented cases of MANEC (see Supporting Information, Table). Patient age ranged from 6 to 89 years; excluding the pediatric outlier, the average age was 60.5 years. The male to female ratio was 1.75:1, and no clear predilection in primary tumor location was identified.…”

“…The male to female ratio was 1.75:1, and no clear predilection in primary tumor location was identified. The liver was the most common site of metastasis (19 of 44), with distant or disseminated metastases occurring in eight of these cases . A Kaplan–Meier plot based on the available reported follow‐up data (see Supporting Information, Figure) gives a median overall survival of 17 months (Microsoft Excel).…”

Mixed Acinar-Endocrine Carcinoma (MAEC) Arising in Duodenal Pancreatic Heterotopia

“…MAEC is a rare pancreatic tumor, consisting of at least 25%–30% acinar and neuroendocrine cells [5, 19]. These tumors most commonly arise in the head of the pancreas [1, 9] and are almost always nonfunctional [3, 10]. Patients present in middle-age [1, 10] with nonspecific symptoms including abdominal pain and weight loss [2, 3].…”

Metastatic mixed acinar‐neuroendocrine carcinoma of the pancreas treated by a multidisciplinary team: A case report and brief review of the literature

“…MANEC composed of both acinar and neuroendocrine components, with each component accounting for at least 30% of the cancer cells . There have been rare reports on MANEC of the pancreas, with about 35 cases reported worldwide . The mean age of patients is 58 years at the diagnosis of MANEC .…”

“…Due to the few cases of MANEC reported to date, evidence on treatment options for it is scarce. Surgery is the first choice for patients in whom the tumor is resectable . However, there have been no standardized chemotherapy for unresectable tumors.…”