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Ganglioneuroma of adrenal gland in a patient with Turner syndrome

Authors
Journal
Annals of Diagnostic Pathology
1092-9134
Publisher
Elsevier
Publication Date
Volume
14
Issue
2
Identifiers
DOI: 10.1016/j.anndiagpath.2009.06.007
Keywords
  • Adrenal Ganglioneuroma
  • Neurogenic Tumors
  • Turner Syndrome
Disciplines
  • Chemistry
  • Medicine

Abstract

Abstract A 15-year-old girl with Turner syndrome was unexpectedly found to have a left suprarenal mass. Extensive investigations showed a clinically and biochemically inapparent mass. Computed tomography disclosed a well-defined solid lesion in the left adrenal measuring 6.5 × 5 cm with minimal contrast enhancement. Laparoscopic adrenalectomy was done. Histologic examination revealed an encapsulated mass originated from the left adrenal medulla. Tumor tissue comprised abundant collagen fibers and spindloid cells admixed with mature ganglion cells. The tumor was diagnosed as left adrenal ganglioneuroma. According to literature, we report the eighth case of ganglioneuroma complicating Turner syndrome. Patients with this syndrome are predisposed to the development of neuroblastoma and related tumors. Reasons for this predisposition might relate to genetic and hormonal factors. Given that these tumors are often limited stage and of good prognosis, we recommend their screening in all patients with Turner syndrome.

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