Ganglioneuroma of adrenal gland in a patient with Turner syndrome

Kamoun, Mahdi; Mnif, Mouna; Rekik, N.; Belguith, Neila; Charfi, Nadia; Mnif, Lilia; Elleuch, Mouna; Mnif, F.; Kamoun, Thouraya; Mnif, Z.; Kamoun, Hassen; Sellami‐Boudawara, Tahya; Hachicha, M.; Abid, M.

doi:10.1016/j.anndiagpath.2009.06.007

Cited by 18 publications

(17 citation statements)

References 17 publications

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“…They can very rarely metastasise to distant sites 6. Characteristic CT findings include non-enhanced low attenuation <40 HU, punctuate calcifications; MRI findings include low-signal intensity on T1-weighted images and high-signal intensity on T2-weighted images 5 7. Treatment may be by open or laparoscopic adrenalectomy 4.…”

Section: Discussionmentioning

confidence: 99%

Adrenal ganglioneuroma in a patient with polycystic ovarian disease (PCOD): a rare association

et al. 2013

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Adrenal ganglioneuromas are rare, benign incidentalomas of a neural crest origin. A majority of these tumours are clinically silent and discovered on imaging for unrelated reasons. Polycystic ovarian disease (PCOD) is an endocrine disorder characterised by bilateral polycystic ovaries, anovulation leading to infertility, irregular menstrual cycles and features of androgen hormone excess. Herein we report a rare case of adrenal ganglioneuroma in a 14-year-old girl with PCOD. She was referred to us by the gynaecologist after incidental detection of adrenal mass on ultrasonography. Except for raised 24 h urinary metanephrines, rest of the hormones measured were in normal range. Transperitoneal adrenalectomy was performed and histopathology was suggestive of ganglioneuroma. Postoperative recovery was excellent and she is doing well. To our knowledge it is the first such type of case to be reported.

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Section: Discussionmentioning

confidence: 99%

Adrenal ganglioneuroma in a patient with polycystic ovarian disease (PCOD): a rare association

et al. 2013

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“…Despite the suggestion that neuroblastic tumors may occur at an increased rate in TS, only 14 cases (7 ganglioneuromas, 4 neuroblastomas, 2 ganglioneuroblastomas, and 1 sympathoblastoma) have been previously reported, with only two cases reported in the last 9 years since Satg é et al completed their extensive review in 2003 (1,2,4) . Twelve of these tumors were located in the adrenal gland and two were in the retroperitoneum, with an age of diagnosis ranging from 1 month to 31 years.…”

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confidence: 83%

“…Several studies have recommended screening for neuroblastic tumors with urinary catecholamine metabolites at diagnosis of TS and before the initiation of rhGH treatment (2,3) . This does not appear to be warranted due to the rarity of these tumors and because more than 60 % of ganglioneuromas, the most common neuroblastic tumor found, are biochemically silent (10) .…”

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confidence: 99%

Ganglioneuroblastoma in a young child with Turner syndrome

Pinsker

Crudo²

2012

Journal of Pediatric Endocrinology and Metabolism

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X chromosome aneuploidy has been identified as a potential risk factor for the development of neuroblastic tumors. We report a case of a 4-year-old girl with a 45,X karyotype incidentally discovered to have a large ganglioneuroblastoma on initial screening ultrasound. The incidence of these tumors in girls with Turner syndrome as well as their possible relationship to recombinant human growth hormone treatment is discussed.

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“…When hormone activity exists, the patient may experience diarrhoea from vasoactive intestinal peptide release,3 sweating or high blood pressure 5. Although most ganglioneuromas occur sporadically, familial dispositions have been reported, as has an association with Turner syndrome and multiple endocrine neoplasia II 3 7. Unlike other adrenal lesions such as ganglioblastomas and neuroblastomas, ganglioneuromas have no documented malignant potential.…”

Section: Differential Diagnosismentioning

confidence: 99%

Adrenal ganglioneuroma: a rare incidental finding

et al. 2013

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The widespread use of imaging technology as a diagnostic tool has resulted in the identification of many previously unknown, clinically benign lesions. The current era of easy access to imaging studies places physicians in a difficult position, since many lesions are not precisely diagnosed by imaging. For example, the accurate diagnosis of non-functioning adrenal lesions remains a clinical challenge. This report describes a patient with the incidental CT finding of an uncommon adrenal ganglioneuroma. Clinical and radiological findings can guide suspicion towards this rare lesion; however, the actual diagnosis is based on histological findings. Specific characteristics of adrenal ganglioneuromas that would allow their diagnosis without invasive procedures have not been established. This case report is an attempt to add more data that will help to establish diagnostic criteria for this rare disease.

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Ganglioneuroma of adrenal gland in a patient with Turner syndrome

Cited by 18 publications

References 17 publications

Adrenal ganglioneuroma in a patient with polycystic ovarian disease (PCOD): a rare association

Adrenal ganglioneuroma in a patient with polycystic ovarian disease (PCOD): a rare association

Ganglioneuroblastoma in a young child with Turner syndrome

Adrenal ganglioneuroma: a rare incidental finding

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