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Endocrine manifestations of paediatric intracranial germ cell tumours: from diagnosis to long-term follow-up

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Abstract

Purpose

We examined endocrine manifestations in a cohort of paediatric patients with IC-GCTs at diagnosis and during follow-up, integrating clinical, radiological, histopathological and laboratory data.

Methods

Diabetes insipidus (DI), growth hormone deficiency (GHD), hypothyroidism, adrenal insufficiency, precocious puberty (PP)/hypogonadism were diagnosed clinically and biochemically. The prevalence of endocrine manifestations was compared to survival rates.

Results

Our population included 55 children (37 males, 18 females) diagnosed with IC-GCT with a median follow-up of 78.9 months from diagnosis (range 0.5–249.9). At tumour diagnosis, 50.9% patients displayed endocrinopathies: among them, 85.7% were affected by DI, 57.1% central adrenal insufficiency, 50% central hypothyroidism, 28.5% GHD, 10.7% hypogonadotrophic hypogonadism, 10.7% PP. These patients presented predominantly with suprasellar germinoma. If not diagnosed previously, endocrine disorders arose 15.15 months (1.3–404.2) after end of treatment (EOT) in 16.4% patients. At least one endocrinopathy was identified in 67.3% of subjects at last follow-up visit, especially GHD and adrenal insufficiency. DI, hypothyroidism, and adrenal insufficiency occurred earlier than other abnormalities and frequently preceded tumour diagnosis. Subjects with and without endocrine manifestations who survived beyond 12 months after EOT did not show significant difference in overall survival and progression-free survival (p = 0.28 and p = 0.88, respectively).

Conclusion

Endocrinopathies were common presenting symptoms in our population. If present at diagnosis, they often persisted hence after. The spectrum of endocrinopathies expanded during follow-up up to 33.7 years after EOT. Although they did not seem to affect survival rate in our cohort, close lifelong surveillance is mandatory to provide the best care for these patients.

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Acknowledgements

The authors thank Dr. Kavitha Srivatsa, Specialty Doctor, Paediatric Oncology, The Royal Marsden NHS Foundation Trust, London, UK, for her kind help in data acquisition.

Author contributions

C.P. and A.A. contributed to the study conception and design. Data collection and analysis were performed by C.P. and V.B.A. The first draft of the manuscript was written by C.P. A.A. and F.C. commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Authors and Affiliations

  1. Department of Pediatrics, IRCCS San Raffaele Scientific Institute, Milan, Italy

    Cristina Partenope, Gabriella Pozzobon & Giovanna Weber

  2. Department of Paediatric Endocrinology, Royal Marsden NHS Foundation Trust, London, UK

    Cristina Partenope & Assunta Albanese

  3. Department of Paediatric Endocrinology, Variety Children Hospital, King’s College Hospital NHS Foundation Trust, London, UK

    Ved Bhushan Arya

  4. Honorary Senior Lecturer, School of Life Sciences, King’s College London, London, UK

    Ved Bhushan Arya

  5. Paediatric and Adolescent Oncology Drug Development Team, Children and Young People’s Unit, Royal Marsden Hospital and Institute of Cancer Research, Sutton, UK

    Fernando Carceller

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  1. Cristina Partenope
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Correspondence to Cristina Partenope.

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Conflict of interest

The authors declare that there is no conflict of interest. F.C. is partly funded by the Giant Pledge via the Royal Marsden Cancer Charity. The Paediatric Neuro-Oncology and Drug Development Units receive charitable funding from the Hall-Hunter Foundation via the Royal Marsden Cancer Charity.

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This study was performed in accordance with the ethical standards of the local institutional Ethics Committees and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Partenope, C., Pozzobon, G., Weber, G. et al. Endocrine manifestations of paediatric intracranial germ cell tumours: from diagnosis to long-term follow-up. Endocrine 77, 546–555 (2022). https://doi.org/10.1007/s12020-022-03121-9

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  • DOI: https://doi.org/10.1007/s12020-022-03121-9

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