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Peutz-Jegher's syndrome with ovarian serous cystadenoma: an unusual association

Grace Francis D'costa1, Shubhangi Vinayak Agale, Bhanumati Sumant Pandya

  • 1Department of Pathology, Grant Medical College, Mumbai. dr_grace@vsnl.com

Indian Journal of Pathology & Microbiology
|March 1, 2008
PubMed

Insights

Peutz-Jeghers syndrome, a genetic disorder, involves characteristic skin pigmentation and intestinal polyps. This case highlights a rare ovarian cystadenoma association, underscoring the need for gynecologic exams in affected females.

Area of Science:

  • Gastroenterology
  • Genetics
  • Gynecology

Background:

  • Peutz-Jeghers syndrome (PJS) is an inherited disorder characterized by hamartomatous polyps in the gastrointestinal tract and mucocutaneous pigmentation.
  • PJS significantly increases the risk of various cancers, necessitating vigilant monitoring and management.

Observation:

  • A 29-year-old female presented with a 1.5-year history of epigastric pain and bilious vomiting.
  • Physical examination revealed characteristic mucocutaneous pigmentation, present since birth.
  • An associated unilateral ovarian cystadenoma was identified.

Findings:

  • The patient was diagnosed with Peutz-Jeghers syndrome based on clinical presentation and endoscopic findings of intestinal polyposis.
  • The co-occurrence of ovarian cystadenoma with PJS is a rare finding.

Implications:

  • This case emphasizes the importance of a comprehensive gynecologic examination in female patients diagnosed with Peutz-Jeghers syndrome.
  • Early detection and management of associated gynecologic conditions can improve patient outcomes.
  • Understanding rare PJS associations aids in refining diagnostic and management protocols.

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