1. Incidence and Predictors for Oncologic Etiologies in Chinese Children with Pituitary Stalk Thickening.
- Author
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Li, Mario W. T., Poon, Sarah W. Y., Cheung, Claudia, Wong, Chris K. C., Shing, Matthew M. K., Chow, Terry T. W., Lee, Samantha L. K., Pang, Gloria S. W., Kwan, Elaine Y. W., Poon, Grace W. K., Yau, Ho-Chung, Tung, Joanna Y. L., and Liu, Anthony P. Y.
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PITUITARY disease complications , *GERMINOMA , *ENDOCRINE diseases , *CONFIDENCE intervals , *INTERDISCIPLINARY research , *DIABETES insipidus , *DISEASE incidence , *MAGNETIC resonance imaging , *LANGERHANS-cell histiocytosis , *RETROSPECTIVE studies , *DESCRIPTIVE statistics , *TUMOR markers , *LONGITUDINAL method - Abstract
Simple Summary: This is a review of the clinical course in 28 Chinese children with pituitary stalk thickening (PST) and endocrinopathies revealed a higher rate of germ cell tumors (GCT) compared to the literature based largely on non-Asian cohorts. Predictors for oncologic etiologies included male sex, severe stalk thickening ≥6.5 mm, central diabetes insipidus or ≥2 pituitary hormone deficiencies as presenting features. Management guidelines for PST should take into account patient ethnicity with a lower threshold of biopsy for Asian children. Multidisciplinary approach with serial monitoring of clinical, tumour markers, endocrinopathies and imaging are essential for diagnosis. Background: With the increasing use of magnetic resonance imaging (MRI) in the evaluation of children with endocrine disorders, pituitary stalk thickening (PST) poses a clinical conundrum due to the potential for underlying neoplasms and challenges in obtaining a tissue biopsy. The existing literature suggests Langerhans cell histiocytosis (LCH) to be the commonest (16%) oncologic cause for PST, followed by germ cell tumors (GCTs, 13%) (CCLG 2021). As the cancer epidemiology varies according to ethnicity, we present herein the incidence and predictors for oncologic etiologies in Hong Kong Chinese children with PST. Methods: Based on a territory-wide electronic database, we reviewed patients aged < 19 years who presented to three referral centers with endocrinopathies between 2010 and 2022. Records for patients who underwent at least one MRI brain/pituitary were examined (n = 1670): those with PST (stalk thickness ≥ 3 mm) were included, while patients with pre-existing cancer, other CNS and extra-CNS disease foci that were diagnostic of the underlying condition were excluded. Results: Twenty-eight patients (M:F = 10:18) were identified. The median age at diagnosis of PST was 10.9 years (range: 3.8–16.5), with central diabetes insipidus (CDI) and growth hormone deficiency (GHD) being the most frequent presenting endocrine disorders. At a median follow-up of 4.8 years, oncologic diagnoses were made in 14 patients (50%), including 13 GCTs (46%; germinoma = 11, non-germinoma = 2) and one LCH (4%). Among patients with GCTs, 10 were diagnosed based on histology, two by abnormal tumor markers and one by a combination of histology and tumor markers. Three patients with germinoma were initially misdiagnosed as hypophysitis/LCH. The cumulative incidence of oncologic diagnoses was significantly higher in boys and patients with PST at presentation ≥6.5 mm, CDI or ≥2 pituitary hormone deficiencies at presentation and evolving hypopituitarism (all p < 0.05 by log-rank). Conclusions: A higher rate of GCTs was observed in Chinese children with endocrinopathy and isolated PST. The predictors identified in this study may guide healthcare providers in Asia in clinical decision making. Serial measurement of tumor markers is essential in management. [ABSTRACT FROM AUTHOR]
- Published
- 2023
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