Hepatoid adenocarcinoma in Barrett’s esophagus associated with achalasia: First case report

Abstract

We report an unusual hepatoid adenocarcinoma in Barrett's esophagus with achalasia, which developed in a 44-year-old Japanese woman. The patient received an esophago-gastrectomy after diagnosis of the tumor and achalasia at the lower esophagus, 4 months before her death due to multiple metastatic tumors of the liver. The main granular tumor removed surgically was a hepatoid adenocarcinoma, mainly composed of clear cancer cells (alpha-1 antitrypsin, albumin and alpha-fetoprotein positive), with elements of choriocarcinoma and tubular adenocarcinoma. Non-neoplastic specialized columnar epithelium was present extensively near the oral side of the tumor edge in the esophagus, indicating Barrett's esophagus. This unusual tumor was therefore considered to have originated in Barrett's esophagus. The gastroesophageal reflux was presumed to have occurred for a long period, as there was a well-preserved fundic gland in the stomach and a history of frequent vomiting from the patient's youth, accounting for the appearance of achalasia.

Pancreatic Hepatoid Carcinoma: A Review of the Literature

“…Of particular interest, 2 patients (9.1%) complained of clinical symptoms referable to a concomitant pancreatic neuroendocrine tumor (1 insulinoma and 1 glucagonoma, respectively), underlining the possible association of pancreatic HC with other pancreatic neoplasms. Elevated AFP serum levels might be present at time of diagnosis in all HCs, despite tumor primary site [7,8,11,12,28,29,30,31,32,33,34]. Our review of pancreatic forms indeed reports a rate of 54.5% (12 out of 22 cases), bearing in mind that 3 papers did not report or did not assess AFP levels of their cases.…”

“…The first case was reported by Ishikura et al [4] and concerned HC in the stomach. Since then, its occurrence has been described in gonadal yolk sac as well as in several non-germ cell origin primary sites, including esophagus, ampulla of Vater, colon, lung, gallbladder, adrenal gland, kidney, urinary bladder, ovary, uterus, vagina, and testicle [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16]. Microscopic ‘stigmata' of those origins are typically detectable in tumor focal areas.…”

A Case of Alpha-Fetoprotein-Producing Adenocarcinoma of the Esophagogastric Junction in which Long-Term Survival Was Achieved by Means of Individualized Multidisciplinary Therapy

“…To date, eight cases exhibiting AFP-producing carcinomas of the EGJ have been reported in the literature (Table 1) [13][14][15][16][17][18][19][20]. Five cases were reported from Asia and three were reported from other regions.…”

“…Five cases were reported from Asia and three were reported from other regions. An association with Barrett's esophagus was reported in half of the cases [13,14,16,20], but in others no relationship was found [15,[17][18][19]. The histological subtypes were as follows: adenocarcinoma (n = 3) [16,17,19], hepatoid adenocarcinoma (n = 3) [14,15,18], adenocarcinoma/hepatoid adenocarcinoma (n = 1) [20], and mixed-type (n = 1) [13].…”

Esophageal Adenocarcinoma with Enteroblastic Differentiation Arising in Ectopic Gastric Mucosa in the Cervical Esophagus: a Case Report and Literature Review

“…Alfa-fetoprotein (AFP)-producing gastrointestinal carcinomas have been mostly reported as arising in the stomach [1][2][3]; there were few cases reported with esophageal cancers [4][5][6][7][8][9][10][11][12][13][14]. Adenocarcinoma with enteroblastic differentiation is a subtype of AFP-producing adenocarcinoma.…”