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Tischler AS, Semple J. Adrenal Medullary Nodules in Beckwith-Wiedemann Syndrome Resemble Extra-Adrenal Paraganglia. Endocr Pathol. 1996;7(4):265-272doi: 10.1007/BF02739833.
Tischler, A. S., & Semple, J. (1996). Adrenal Medullary Nodules in Beckwith-Wiedemann Syndrome Resemble Extra-Adrenal Paraganglia. Endocrine pathology, 7(4), 265-272. https://doi.org/10.1007/BF02739833
Tischler, Arthur S., and Semple, Joseph. "Adrenal Medullary Nodules in Beckwith-Wiedemann Syndrome Resemble Extra-Adrenal Paraganglia." Endocrine pathology vol. 7,4 (1996): 265-272. doi: https://doi.org/10.1007/BF02739833
Tischler AS, Semple J. Adrenal Medullary Nodules in Beckwith-Wiedemann Syndrome Resemble Extra-Adrenal Paraganglia. Endocr Pathol. 1996;7(4):265-272. doi: 10.1007/BF02739833. PMID: 12114797.
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Endocr Pathol. 1996;7(4):265-272. doi: 10.1007/BF02739833.

Adrenal Medullary Nodules in Beckwith-Wiedemann Syndrome Resemble Extra-Adrenal Paraganglia.

Endocrine pathology

Arthur S. Tischler, Joseph Semple

PMID: 12114797 DOI: 10.1007/BF02739833

Abstract

Clues to mechanisms regulating development and tumorigenesis may be provided by studies of unusual diseases. Beckwit-Wiedemann syndrome (BWS) is a rare congenital disorder apparently related to abnormal regulation of insulin-like growth factor-2 (IGF-2) production. IGF2 mRNA has been previously localized to the chief cells of extra-adrenal paraganglia and to adult, but not fetal, adrenal medulla. Expression of IGF-2 by neuroblastomas has been hypothesized to reflect extra-adrenal paraganglionic differentiation. In the adrenals of a fetus with 8W5, we have observed both increased numbers of chromaffin cells and organoid nodules resembling extra-adrenal paraganglia. Immunoreactive IGF-2 was observed in both cell types, but was also observed in chromaffin cells in the normal fetal adrenal. The findings suggest autocrine or paracrine influences of IGF-2 in regulating the number and phenotype of cells derived from sympathoadrenal precursors in the developing adrenal medulla as well as in extra-adrenal paraganglia. These results have implications for the interpretation of data from neuroblastoma studies.

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