Incidence and Predictors for Oncologic Etiologies in Chinese Children with Pituitary Stalk Thickening

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 literatu...

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Main Authors: Mario W. T. Li, Sarah W. Y. Poon, Claudia Cheung, Chris K. C. Wong, Matthew M. K. Shing, Terry T. W. Chow, Samantha L. K. Lee, Gloria S. W. Pang, Elaine Y. W. Kwan, Grace W. K. Poon, Ho-Chung Yau, Joanna Y. L. Tung, Anthony P. Y. Liu
Format: Article
Language:English
Published: MDPI AG 2023-08-01
Series:Cancers
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Online Access:https://www.mdpi.com/2072-6694/15/15/3935
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author Mario W. T. Li
Sarah W. Y. Poon
Claudia Cheung
Chris K. C. Wong
Matthew M. K. Shing
Terry T. W. Chow
Samantha L. K. Lee
Gloria S. W. Pang
Elaine Y. W. Kwan
Grace W. K. Poon
Ho-Chung Yau
Joanna Y. L. Tung
Anthony P. Y. Liu
author_facet Mario W. T. Li
Sarah W. Y. Poon
Claudia Cheung
Chris K. C. Wong
Matthew M. K. Shing
Terry T. W. Chow
Samantha L. K. Lee
Gloria S. W. Pang
Elaine Y. W. Kwan
Grace W. K. Poon
Ho-Chung Yau
Joanna Y. L. Tung
Anthony P. Y. Liu
author_sort Mario W. T. Li
collection DOAJ
description 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 (<i>n</i> = 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 <i>p</i> < 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.
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spelling doaj.art-fca3a0772ed24782a7c8f560c57009972023-11-18T22:43:24ZengMDPI AGCancers2072-66942023-08-011515393510.3390/cancers15153935Incidence and Predictors for Oncologic Etiologies in Chinese Children with Pituitary Stalk ThickeningMario W. T. Li0Sarah W. Y. Poon1Claudia Cheung2Chris K. C. Wong3Matthew M. K. Shing4Terry T. W. Chow5Samantha L. K. Lee6Gloria S. W. Pang7Elaine Y. W. Kwan8Grace W. K. Poon9Ho-Chung Yau10Joanna Y. L. Tung11Anthony P. Y. Liu12Department of Paediatrics and Adolescent Medicine, The Hong Kong Children’s Hospital, Hong Kong, ChinaDepartment of Paediatrics and Adolescent Medicine, Queen Mary Hospital, Hong Kong, ChinaDepartment of Radiology, The Hong Kong Children’s Hospital, Hong Kong, ChinaDepartment of Radiology, The Hong Kong Children’s Hospital, Hong Kong, ChinaDepartment of Paediatrics and Adolescent Medicine, The Hong Kong Children’s Hospital, Hong Kong, ChinaDepartment of Paediatrics and Adolescent Medicine, The Hong Kong Children’s Hospital, Hong Kong, ChinaDepartment of Paediatrics and Adolescent Medicine, The Hong Kong Children’s Hospital, Hong Kong, ChinaDepartment of Paediatrics and Adolescent Medicine, The Hong Kong Children’s Hospital, Hong Kong, ChinaDepartment of Paediatrics and Adolescent Medicine, The Hong Kong Children’s Hospital, Hong Kong, ChinaDepartment of Paediatrics and Adolescent Medicine, Queen Mary Hospital, Hong Kong, ChinaDepartment of Paediatrics, Prince of Wales Hospital, Hong Kong, ChinaDepartment of Paediatrics and Adolescent Medicine, The Hong Kong Children’s Hospital, Hong Kong, ChinaDepartment of Paediatrics and Adolescent Medicine, The Hong Kong Children’s Hospital, Hong Kong, ChinaBackground: 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 (<i>n</i> = 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 <i>p</i> < 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.https://www.mdpi.com/2072-6694/15/15/3935thickened pituitary stalkcentral diabetes insipidusendocrinopathygerm cell tumorLangerhans cell histiocytosis
spellingShingle Mario W. T. Li
Sarah W. Y. Poon
Claudia Cheung
Chris K. C. Wong
Matthew M. K. Shing
Terry T. W. Chow
Samantha L. K. Lee
Gloria S. W. Pang
Elaine Y. W. Kwan
Grace W. K. Poon
Ho-Chung Yau
Joanna Y. L. Tung
Anthony P. Y. Liu
Incidence and Predictors for Oncologic Etiologies in Chinese Children with Pituitary Stalk Thickening
Cancers
thickened pituitary stalk
central diabetes insipidus
endocrinopathy
germ cell tumor
Langerhans cell histiocytosis
title Incidence and Predictors for Oncologic Etiologies in Chinese Children with Pituitary Stalk Thickening
title_full Incidence and Predictors for Oncologic Etiologies in Chinese Children with Pituitary Stalk Thickening
title_fullStr Incidence and Predictors for Oncologic Etiologies in Chinese Children with Pituitary Stalk Thickening
title_full_unstemmed Incidence and Predictors for Oncologic Etiologies in Chinese Children with Pituitary Stalk Thickening
title_short Incidence and Predictors for Oncologic Etiologies in Chinese Children with Pituitary Stalk Thickening
title_sort incidence and predictors for oncologic etiologies in chinese children with pituitary stalk thickening
topic thickened pituitary stalk
central diabetes insipidus
endocrinopathy
germ cell tumor
Langerhans cell histiocytosis
url https://www.mdpi.com/2072-6694/15/15/3935
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