Your browser doesn't support javascript.
loading
Peutz-Jeghers syndrome: an unusual autopsy finding in pregnancy
Departamento de PatologiaOliveira, Michell Frank Alves de; Departamento de PatologiaRodrigues, Maria Aparecida Marchesan.
  • Departamento de PatologiaOliveira, Michell Frank Alves de; Universidade Estadual Paulista. Faculdade de Medicina de Botucatu. Departamento de PatologiaOliveira, Michell Frank Alves de. Botucatu. BR
  • Departamento de PatologiaRodrigues, Maria Aparecida Marchesan; Universidade Estadual Paulista. Faculdade de Medicina de Botucatu. Departamento de PatologiaRodrigues, Maria Aparecida Marchesan. Botucatu. BR
Autops. Case Rep ; 11: e2021279, 2021. graf
Article in English | LILACS | ID: biblio-1285427
ABSTRACT
Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant polyposis entity that often remains undiagnosed. The major problems associated with PJS are acute complications due to (i) polyp-related intestinal obstruction, (ii) intussusception, and (iii) the risk of cancer in the long-term. We report the case of a 32-year-old female who presented at the emergency room with signs of acute abdomen and died during the clinical workup. She had a one-month history of nausea, vomiting, and diarrhea and was pregnant at about 30 weeks. There was no contributing past history except for undergoing small bowel resection in infancy. The postmortem examination revealed multiple arborizing polyps throughout the gastrointestinal tract, chiefly in the small bowel. Intestinal obstruction was found at the proximal jejunum with necrosis, perforation, and peritonitis. Histologically, the polyps were composed of tree branch-like bundles of smooth muscle covered by normal-appearing glandular epithelium, confirming the diagnosis of hamartomatous polyps. No malignant or premalignant lesions were detected in the gastrointestinal tract or other organs. This case was an opportunity to analyze the natural history and the pathological features of the Peutz-Jeghers syndrome in an adult and to investigate the presence of neoplastic lesions associated with this condition.
Subject(s)


Full text: Available Index: LILACS (Americas) Main subject: Peutz-Jeghers Syndrome / Gastrointestinal Tract / Intestinal Obstruction Type of study: Diagnostic study Limits: Adult / Female / Humans / Pregnancy Language: English Journal: Autops. Case Rep Journal subject: Anatomia / Patologia Cl¡nica / Patologia Legal Year: 2021 Type: Article Affiliation country: Brazil Institution/Affiliation country: Universidade Estadual Paulista/BR

Similar

MEDLINE

...
LILACS

LIS


Full text: Available Index: LILACS (Americas) Main subject: Peutz-Jeghers Syndrome / Gastrointestinal Tract / Intestinal Obstruction Type of study: Diagnostic study Limits: Adult / Female / Humans / Pregnancy Language: English Journal: Autops. Case Rep Journal subject: Anatomia / Patologia Cl¡nica / Patologia Legal Year: 2021 Type: Article Affiliation country: Brazil Institution/Affiliation country: Universidade Estadual Paulista/BR