Pseudotumor of the pituitary due to PROP-1 deletion.

J Pediatr Endocrinol Metab

Department of Pediatric Endocrinology, Hopital Saint-Vincent de Paul, Paris, France.

Published: January 2002

Hypopituitarism associated with pituitary mass in childhood is most frequently the consequence of craniopharyngioma or Rathke's cleft cyst. We report a patient with an intrasellar pseudotumor associated with hypopituitarism, which led us to a misdiagnosis of intrasellar craniopharyngioma. After spontaneous involution of the mass, diagnosis was revised. DNA analysis showed a deletion in the Prophet of Pit-1 (PROP-1) gene, a pituitary transcription factor. It is important to recognize that a PROP-1 deletion can cause pituitary pseudotumor that can be mistaken for a craniopharyngioma or Rathke's pouch cyst.

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http://dx.doi.org/10.1515/jpem.2002.15.1.95DOI Listing

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