Ganglioneuroma of adrenal gland in a patient with Turner syndrome.
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 x 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.
Kamoun
Mahdi
M
Endocrinology Department, Hedi Chaker Hospital, 3029 Sfax, Tunisia.
Mnif
Mouna Feki
MF
Rekik
Nabila
N
Belguith
Neila
N
Charfi
Nadia
N
Mnif
Lilia
L
Elleuch
Mouna
M
Mnif
Fatma
F
Kamoun
Thouraya
T
Mnif
Zeinab
Z
Kamoun
Hassen
H
Sellami-Boudawara
Tahia
T
Hachicha
Mongia
M
Abid
Mohamed
M
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000473:pathology* / Q000601:surgery; D000315:Adrenalectomy; D005260:Female; D005729:Ganglioneuroma / Q000150:complications* / Q000473:pathology* / Q000601:surgery; D006801:Humans; D014424:Turner Syndrome / Q000150:complications*