Bilateral adrenal cysts and ectopic pancreatic tissue in Beckwith-Wiedemann syndrome: Is a conservative approach acceptable?

Rahmah Rasat, J. F. Yong, N. A. Sharifa, Ursula Kuhnle

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Beckwith-Wiedemann syndrome is a common overgrowth syndrome associated with an increased risk of neoplasias which might be explained by the nature and localization of the genetic defect. While malignant tumors are often associated with hemihypertrophy, benign tumors are also found. We report a patient with the typical features of Beckwith-Wiedemann syndrome with two histologically different abdominal tumors, bilateral cystic adrenals and ectopic pancreatic tissue present at birth. In both tumors no malignancy could be detected. Ectopic pancreatic tissue is rarely seen and has been described in Beckwith-Wiedemann syndrome only once. After extirpation of the ectopic pancreatic tissue the cystic adrenals were left in situ since macroscopically no normal adrenal tissue could be identified and separated. Regular ultrasound examinations revealed complete resolution of the cystic adrenals within 24 months. Thus it seems that a conservative approach in selected tumors associated with the Beckwith-Wiedemann syndrome might be acceptable.

Original languageEnglish
Pages (from-to)909-912
Number of pages4
JournalJournal of Pediatric Endocrinology and Metabolism
Volume17
Issue number6
Publication statusPublished - 2004

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Beckwith-Wiedemann Syndrome
Pancreatic Cyst
Choristoma
Neoplasms
Parturition

Keywords

  • Adrenal cysts
  • Beckwith-Wiedemann syndrome
  • Ectopic pancreatic tissue

ASJC Scopus subject areas

  • Endocrinology
  • Pediatrics, Perinatology, and Child Health

Cite this

Bilateral adrenal cysts and ectopic pancreatic tissue in Beckwith-Wiedemann syndrome : Is a conservative approach acceptable? / Rasat, Rahmah; Yong, J. F.; Sharifa, N. A.; Kuhnle, Ursula.

In: Journal of Pediatric Endocrinology and Metabolism, Vol. 17, No. 6, 2004, p. 909-912.

Research output: Contribution to journalArticle

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