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[Deep cystic enteritis in Peutz-Jeghers syndrome (pseudoinfiltrating lesions in a patient with enteric polyps)]
J Ortíz1, F González San Martín, M Abad
1Servicio de Anatomía Patológica, Hospital Clínico Universitario, Salamanca.
Insights
This study details a rare case of Enteritis cystica profunda in a patient with Peutz-Jeghers syndrome. The condition, characterized by deep intestinal wall cysts, was found alongside typical hamartomatous polyps.
Area of Science:
- Gastroenterology
- Pathology
- Genetics
Background:
- Peutz-Jeghers syndrome is a rare inherited disorder associated with hamartomatous polyps and an increased risk of various cancers.
- Enteritis cystica profunda is an uncommon benign condition of the gastrointestinal tract characterized by cystic glands in the deep layers of the intestinal wall.
Observation:
- A patient with a known diagnosis of Peutz-Jeghers syndrome presented with intestinal invagination requiring surgical intervention.
- During surgery, hamartomatous polyps characteristic of Peutz-Jeghers syndrome were identified.
- Histopathological examination of the resected intestinal segment revealed lesions consistent with Enteritis cystica profunda.
Findings:
- The resected intestinal segment showed both hamartomatous polyps and Enteritis cystica profunda.
- Enteritis cystica profunda presented as benign encysted glands and mucous pools within the deep layers of the intestinal wall.
Implications:
- This case highlights a rare co-occurrence of Peutz-Jeghers syndrome and Enteritis cystica profunda.
- Understanding this association may improve diagnostic and management strategies for patients with Peutz-Jeghers syndrome.
- Further research into the potential link between these conditions is warranted.
Abstract:
We report a case of enteritis cystica profunda observed in a patient who, owing to both his personal and familial history, had been diagnosed and suffering from Peutz-Jeghers syndrome. The patient was operated because of a clinical picture of intestinal invagination. Together with hamartomatous polyps, anatomopathological study of the segment of the small intestine removed revealed the presence of lesions typical of Enteritis cystica profunda consisting in the presence of benign encysted glands and pools of mucous in the deep layers of the intestinal wall. The main characteristics of this interesting and rare entity are briefly commented.