Magnetic resonance imaging of sellar and juxtasellar abnormalities in the paediatric population: an imaging review

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Abstract

The sellar and juxtasellar regions in the paediatric population are complex both anatomically and pathologically, with magnetic resonance imaging (MRI) being the “gold standard” imaging modality due to the high contrast of detail. Assessment requires a detailed understanding of the anatomy, embryology, pathophysiology and normal signal characteristics of the pituitary gland and surrounding structures in order to appropriately characterise abnormalities. This article aims to provide an overview of the imaging characteristics of developmental/congenital and acquired disease processes which affect the sellar and juxtasellar region in the paediatric population.

Main Messages

• The sellar region is anatomically complex and covers a wide pathology spectrum.

• MRI is the key imaging modality to assess sellar and juxtasellar pathology.

• Numerous developmental anomalies may not be discovered until adulthood.

• Knowledge of pathology alerts and guides the clinician towards appropriate management.

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Most cited references 73

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Prognostic factors in Ewing's tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing's Sarcoma Study Group.

S Cotterill, S. Ahrens, M. Paulussen … (2000)

To further elaborate on prognostic factors for Ewing's sarcoma of bone and to document improvements in relapse-free survival (RFS) and trends in local therapy over the study period (1977 to 1993). A retrospective analysis was performed on a combined Gesellschaft Für Pädiatrische Onkologie und Hämatologie/Cooperative Ewing Sarcoma Study and United Kingdom Children's Cancer Study Group/Medical Research Council data set of 975 patients registered with the respective trial offices before the current collaborative European Intergroup Cooperative Ewing's Sarcoma Study trial. Both groups independently undertook studies with similar chemotherapy during the period. The key adverse prognostic factor is metastases at diagnosis (5-year RFS, 22% of patients with metastases at diagnosis v 55% of patients without metastases at diagnosis; P: or = 15 years), and period of diagnosis had significant influence on RFS (all P: <.005). RFS was superior in the period after 1985 compared with the period before 1985 for nonmetastatic patients (45% v 60%, respectively; P: <.0001) and for metastatic patients (16% v 30%, respectively; P: =.016). Patients who relapsed within 2 years of diagnosis had a less favorable prognosis than patients who relapsed later (5-year survival after relapse, 4% v 23%, respectively; P: <. 0001). There were other changes over the period; in particular, radiotherapy or amputation were more common in the period before 1986, whereas endoprosthetic surgery was widely used in the later period. Survival and RFS improved over the period. Prognostic factors are metastases at diagnosis, primary site, and age.

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Intracranial germ-cell tumors: natural history and pathogenesis.

F. Hochberg, Janine M. Jennings, R Gelman (1985)

The natural history of primary intracranial germ-cell tumors (GCT's) is defined from 389 previously published cases, of which 65% were germinomas, 18% teratomas, 5% embryonal carcinomas, 7% endodermal sinus tumors, and 5% choriocarcinomas. Intracranial GCT's display specificity in site of origin. Ninety-five percent arise along the midline from the suprasellar cistern (37%) to the pineal gland (48%), and an additional 6% involve both sites. The majority of germinomas (57%) arise in the suprasellar cistern, while most nongerminomatous GCT's (68%) preferentially involve the pineal gland (p less than 0.0001). The age distribution of afflicted patients is unimodal, centering with an abrupt surge in frequency in the early pubertal years; 68% of patients are diagnosed between 10 and 21 years of age. Nongerminomatous GCT's demonstrate an earlier age of onset than do germinomas (p less than 0.0001). Prolonged symptomatic intervals prior to diagnosis are common in germinomas (p = 0.0007), in suprasellar GCT's (p = 0.001), and among females (p = 0.02). Parasellar germinomas commonly present with diabetes insipidus, visual field defects, and hypothalamic-pituitary failure. Nongerminomatous GCT's present as posterior third ventricular masses with hydrocephalus and midbrain compression. Germ-cell tumors may infiltrate the hypothalamus (11%), or disseminate to involve the third ventricle (22%) and spinal cord (10%). Among a subpopulation of 263 conventionally treated patients, two factors were of prognostic significance: 1) histological diagnosis; germinomas were associated with significantly longer survival than nongerminomatous GCT's (p less than 0.0001); and 2) staging of the extent of disease; this emphasizes the ominous character of involvement of the hypothalamus (p = 0.0002), third ventricle (p = 0.02), or spinal cord (p = 0.01). Specific recommendations regarding the necessity of histological diagnosis and staging of the extent of disease are made in light of modern chemotherapeutic advances. The pathogenesis of GCT's may be revealed by their specificity of origin within the positive (suprasellar cistern-suprachiasmatic nucleus) and negative (pineal) regulatory centers for gonadotropin secretion within the diencephalon. The abrupt rise in age distribution at 10 to 12 years suggests that the neuroendocrine events of puberty are an "activating" influence in the malignant expression of these embryonal tumors.

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Central diabetes insipidus in children and young adults.

M Maghnie, G Cosi, E Genovese … (2000)

Central diabetes insipidus is rare in children and young adults, and up to 50 percent of cases are idiopathic. The clinical presentation and the long-term course of this disorder are largely undefined. We studied all 79 patients with central diabetes insipidus who were seen at four pediatric endocrinology units between 1970 and 1996. There were 37 male and 42 female patients whose median age at diagnosis was 7.0 years (range, 0.1 to 24.8). All patients underwent magnetic resonance imaging (MRI) and periodic studies of anterior pituitary function. The median duration of follow-up was 7.6 years (range, 1.6 to 26.2). The causes of the central diabetes insipidus were Langerhans-cell histiocytosis in 12 patients, an intracranial tumor in 18 patients, a skull fracture in 2 patients, and autoimmune polyendocrinopathy in 1 patient; 5 patients had familial disease. The cause was considered to be idiopathic in 41 patients (52 percent). In 74 patients (94 percent) the posterior pituitary was not hyperintense on the first MRI scan obtained, and 29 patients (37 percent) had thickening of the pituitary stalk. Eighteen patients had changes in the thickness of the pituitary stalk over time, ranging from normalization (six patients) or a decrease in thickness (one patient) to further thickening (seven patients) or thickening of a previously normal stalk (four patients). Anterior pituitary hormone deficiencies, primarily growth hormone deficiency, were documented in 48 patients (61 percent) a median of 0.6 year (range, 0.1 to 18.0) after the onset of central diabetes insipidus. Most children and young adults with acquired central diabetes insipidus have abnormal findings on MRI scans of the head, which may change over time, and at least half have anterior pituitary hormone deficiencies during follow-up.

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Author and article information

Contributors

Rachel Shields: rachel_shields@urmc.rochester.edu

Journal

Journal ID (nlm-ta): Insights Imaging

Journal ID (iso-abbrev): Insights Imaging

Title: Insights into Imaging

Publisher: Springer Berlin Heidelberg (Berlin/Heidelberg )

ISSN (Electronic): 1869-4101

Publication date (Electronic): 21 March 2015

Publication date PMC-release: 21 March 2015

Publication date Collection: April 2015

Volume: 6

Issue: 2

Pages: 241-260

Affiliations

Department of Imaging Sciences, University of Rochester Medical Center, 601 Elmwood Ave., Rochester, NY 14642-0001 USA

Article

Publisher ID: 401

DOI: 10.1007/s13244-015-0401-5

PMC ID: 4376809

PubMed ID: 25794595

SO-VID: bc8f13be-6cf1-4775-b0f3-54cef5ad4a91

License:

Open Access This article is distributed under the terms of the Creative Commons Attribution License which permits any use, distribution, and reproduction in any medium, provided the original author(s) and the source are credited.

History

Date received : 4 November 2014

Date revision received : 11 February 2015

Date accepted : 17 February 2015

Custom metadata

ScienceOpen disciplines: Radiology & Imaging

Keywords: magnetic resonance imaging,paediatrics,pituitary gland,pituitary diseases,sella turcica

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ScienceOpen disciplines: Radiology & Imaging

Keywords: magnetic resonance imaging, paediatrics, pituitary gland, pituitary diseases, sella turcica

Magnetic resonance imaging of sellar and juxtasellar abnormalities in the paediatric population: an imaging review

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Most cited references 73

Prognostic factors in Ewing's tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing's Sarcoma Study Group.

Intracranial germ-cell tumors: natural history and pathogenesis.

Central diabetes insipidus in children and young adults.

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