Ganglioneuroma of adrenal gland in a patient with Turner syndrome

doi:10.1016/j.anndiagpath.2009.06.007

Annals of Diagnostic Pathology

Volume 14, Issue 2, April 2010, Pages 133-136

https://doi.org/10.1016/j.anndiagpath.2009.06.007 Get rights and content

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.

Introduction

Turner syndrome (TS) is a chromosomal disorder due to a complete or partial absence of an X chromosome affecting about 1 in 2000 live-born girls [1]. Cancer risks in TS have not been clearly established [1]. A higher incidence of extragonadal neoplasms with a preponderance of neurogenic tumors has been reported [2], [3], [4]. We present in this study a new case of female patient with TS and incidental discovery of a left adrenal ganglioneuroma.

Section snippets

Case report

The patient was a 15-year-old girl. She was born after an uneventful pregnancy and normal delivery. Weight and length at birth were, respectively, 3200 g and 48 cm. Her medical history was significant for lymphedema of feet at birth, spontaneously resolute, and surgical repair of aortic arch abnormalities at the age of 6 years old. Her psychomotor development was normal. She was confirmed to have monosomic TS when she underwent medical evaluation for short stature. The patient was referred to

Discussion

Ganglioneuroma is a rare mature neuroblastic tumor, typically nonmetabolically active, and therefore usually asymptomatic [5]. There are no specific features for this tumor, and definitive diagnosis is made on histologic examination [5]. However, certain characteristics can made the clinician evoke the diagnosis such as no hormonal hypersecretion, presence of punctuate calcifications, a nonenhanced attenuation of less than 40 Hounsfield units on computed tomography, and a low nonenhanced

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Case ReportGanglioneuroma of adrenal gland in a patient with Turner syndrome

Abstract

Introduction

Section snippets

Case report

Discussion

J Pediatr.

Am J Surg

J Urol

Lancet Oncol

Lancet Oncol

Cancer Genet Cytogenet

Endocr Pract

Prevalence, incidence, diagnostic delay and mortality in Turner syndrome

J Clin Endocrinol Metab

Nongonadal neoplasia in Turner's syndrome

Cancer

Nongonadal neoplasia in patients with Turner syndrome

J Environ Pathol Toxicol Oncol

Case Report
Ganglioneuroma of adrenal gland in a patient with Turner syndrome