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Recurrent intestinal obstruction in a patient of Peutz-Jeghers syndrome

Sundeep Kumar1, Preeti Arora2, Pabitra Goswami1

  • 1Department of General Surgery, R. G. Kar Medical College and Hospital, Kolkata, West Bengal, India.

Journal of Cancer Research and Therapeutics
|March 19, 2019
PubMed

Insights

Peutz-Jeghers syndrome (PJS) is a rare inherited disorder. This case highlights PJS

Area of Science:

  • Gastroenterology
  • Oncology
  • Genetics

Background:

  • Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder.
  • PJS is characterized by hamartomatous polyps in the gastrointestinal tract and mucocutaneous melanin spots.
  • It significantly increases the risk of various gastrointestinal and extragastrointestinal malignancies.

Observation:

  • A 45-year-old female with a history of jejunal adenocarcinoma presented with abdominal pain and melena.
  • Clinical examination revealed characteristic mucocutaneous hyperpigmentation.
  • Laparotomy identified multiple polyps in the proximal small intestine and ileoileal intussusception.

Findings:

  • Histopathology confirmed papillary adenocarcinoma in a previous jejunal growth.
  • Multiple hamartomatous polyps were observed throughout the small intestine.
  • Surgical intervention involved limited resection due to polyp burden and intussusception.

Implications:

  • This case underscores the importance of recognizing PJS for early cancer surveillance.
  • Timely diagnosis and management are crucial for improving outcomes in PJS patients.
  • Understanding the clinical spectrum of PJS aids in managing associated malignancies.

Keywords:
Intestinal hamartomatous polypsPeutz–Jeghers syndromemucocutaneous hyperpigmented maculessmall intestinal adenocarcinoma

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