Adenocarcinoma arising in the multiple heterotopic submucosal glands of the intestine in a Satoyoshi syndrome patient: A case report

Pathol Int. 2021 Feb;71(2):147-154. doi: 10.1111/pin.13053. Epub 2020 Dec 17.


Satoyoshi syndrome is a rare multisystemic disorder of unknown etiology characterized by progressive muscle spasms, alopecia and diarrhea. Multiple protruding lesions with cystic glands, namely gastroenterocolitis cystica polyposa, manifest in the gastrointestinal tract. Since the first report of these lesions in 1977, which was unique to Satoyoshi syndrome, few studies have focused on their role, and the associated clinicopathological features are not well understood. Here, we report a 64-year-old Japanese woman with Satoyoshi syndrome who presented with multiple polypoid lesions in the stomach, duodenum, jejunum, ileum and colon. Histologically, the polypoid lesions in the intestine comprised multiple heterotopic submucosal glands containing cystically dilated glands and smooth muscle fibers in the lamina propria mucosa and/or submucosa. Additionally, we observed stromal changes, such as fibrosis, discontinuous and thinning muscularis mucosae, and diffuse neural fiber proliferation in the entire intestinal tract. Furthermore, multiple foci of adenocarcinomas were identified within several heterotopic submucosal glands. We hypothesized that multiple heterotopic submucosal glands in the present case corresponded to previously reported gastroenterocolitis cystica polyposa, suggesting that these lesions are essential in the histopathology and are a unique manifestation of Satoyoshi syndrome.

Keywords: Satoyoshi syndrome; adenocarcinoma; heterotopic submucosal gland.

Publication types

  • Case Reports

MeSH terms

  • Adenocarcinoma / diagnosis*
  • Adenocarcinoma / etiology
  • Adenocarcinoma / pathology
  • Alopecia / complications
  • Alopecia / pathology*
  • Bone and Bones / abnormalities*
  • Bone and Bones / pathology
  • Choristoma / diagnosis
  • Choristoma / etiology
  • Choristoma / pathology*
  • Diarrhea / complications
  • Diarrhea / pathology*
  • Female
  • Humans
  • Intestinal Diseases / diagnosis
  • Intestinal Diseases / etiology
  • Intestinal Diseases / pathology
  • Intestinal Mucosa*
  • Intestinal Neoplasms / diagnosis*
  • Intestinal Neoplasms / etiology
  • Intestinal Neoplasms / pathology
  • Middle Aged
  • Spasm / complications
  • Spasm / pathology*

Supplementary concepts

  • Satoyoshi syndrome