Detailed assessment of hypothalamic damage in craniopharyngioma patients with obesity

Fjalldal, Sigridur; Follin, Cecilia; Gabery, Sanaz; Sundgren, Pia C.; Björkman–Burtscher, Isabella M.; Lätt, Jimmy; Mannfolk, Peter; Nordström, Carl‐Henrik; Rylander, Lars; Ekman, Bertil; Cheong, Rachel Y.; Pålsson, Anna; Petersén, Åsa; Erfurth, Eva Marie

doi:10.1038/s41366-018-0185-z

Cited by 22 publications

(29 citation statements)

References 36 publications

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“…In the pediatric population, preoperative hypothalamic involvement ( Fig. 1) increases the likelihood of pre-and postoperative obesity, and hypothalamus damage during surgery increases the risk of postoperative weight gain [31][32][33][34]. Although not well established, postoperative weight gain in adults is also a problem and correlates with hypothalamic involvement [35].…”

Section: Neurosurgerymentioning

confidence: 99%

The Diagnosis and Treatment of Craniopharyngioma

Müller

2019

Neuroendocrinology

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Craniopharyngioma (CP) is a rare embryonic malformation of the sellar/parasellar region with a low histological grade. Clinical manifestations are related to hypothalamic/pituitary deficiencies, visual impairment, and increased intracranial pressure. Recent insight into the molecular pathogenesis of CP opens new perspectives on targeted therapy in papillary CP harboring BRAF-V600E mutations. Further research to elucidate pathogenic mechanisms and hopefully prevent hypothalamic involvement of CP is warranted. If the tumor is favorably localized, the therapy of choice is complete resection, with care taken to preserve the optical and hypothalamic functions. In patients with unfavorable tumor localization (i.e., hypothalamic involvement), the recommended therapy is a limited hypothalamus-sparing surgical strategy followed by local irradiation. Surgical treatment strategies should be based on a multidisciplinary approach involving experienced teams. Centralizing the treatment of CP in experienced "centers of excellence" and multicenter-based networks for reference assessments should be considered to assure a high standard of treatment quality. CP recurrence and progression are frequent. Irradiation has proven effective in reducing recurrences and progression. Proton beam therapy, available in a wider range in the near future, will help to avoid radiooncological side effects. Anatomical involvement and/or surgical lesions of posterior hypothalamic areas can result in serious sequelae that compromise quality of life (QoL), such as hypothalamic obesity and psychopathological symptoms. Novel insights into neuropsychological sequelae after CP occurrence should be the basis for the development of therapeutic neuropsychological interventions. CP should be managed as a frequently chronic disease, providing ongoing care of pediatric and adult patients' clinical and QoL consequences by experienced multidisciplinary teams.

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Section: Neurosurgerymentioning

confidence: 99%

The Diagnosis and Treatment of Craniopharyngioma

Müller

2019

Neuroendocrinology

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“…TGTV, tumor growth into the third ventricle. Reproduced from Fjalldal et al [ 21 ] with permission from Springer Nature.…”

Section: Figmentioning

confidence: 99%

Diagnosis, Background, and Treatment of Hypothalamic Damage in Craniopharyngioma

Erfurth

2020

Neuroendocrinology

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Craniopharyngiomas (CP) are rare brain tumors managed primarily with surgery and radiotherapy. There are 2 phenotypes of CP, i.e., one with a rather good outcome without hypothalamic damage and another with hypothalamic damage. With hypothalamic damage, progressive disease with recurrent operations and additional cranial radiotherapy often result in hypothalamic obesity, an affected psychosocial life, and cognitive dysfunction. The morbidity and mortality are increased for particularly cerebrovascular diseases. Preoperative hypothalamic involvement to predict hypothalamic damage is important for decision making for hypothalamus-sparing surgery. Also a postoperative hypothalamic damage evaluation with the use of hypothalamus volume measurement can predict hypothalamic obesity, which is important for early treatment options. The morbidity of CP includes cognitive dysfunction with attention deficits and impaired episodic memory and processing speed. Again patients with hypothalamic damage are more affected. Treatment options of hypothalamic obesity in the chronic phase are scarce and not convincingly successful. The most optimal situation is to try to hinder or stop the evolution of hypothalamic obesity. Prevention of hypothalamic damage is recommended, with special regard to hypothalamus-sparing therapeutic approaches that respect the integrity of essential nuclei located in both the medial and the posterior hypothalamic areas.

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“…Sixteen patients had received CRT, median dose 50 Grey (Gy) . Median age at first operation was 12 years in women (3-29) and 9 years in men (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22), and median age at investigation was 35 years…”

Section: Patientsmentioning

confidence: 99%

“…1 Recently we showed that reduced hypothalamus (HT) volume is a strong predictor of fat mass in these patients. 9 White matter lesions (WML) are pathological changes caused by obstruction of small cerebral vessels resulting in hypoperfusion of the brain microvasculature and are widely accepted as clinically relevant. 10 WML can be visualized on T2-weighted magnetic resonance images (MRI), [11][12][13] corresponding to myelin loss and mild gliosis.…”

Section: Introductionmentioning

confidence: 99%

Brain white matter lesions are associated with reduced hypothalamic volume and cranial radiotherapy in childhood‐onset craniopharyngioma

Fjalldal

Rylander

Westen

et al. 2020

Clinical Endocrinology

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Context: White matter lesions (WML) are caused by obstruction of small cerebral vessels associated with stroke risk. Craniopharyngioma (CP) patients suffer from increased cerebrovascular mortality. Objective: To investigate the effect of reduced HT volume and cranial radiotherapy (CRT) on WML in childhood-onset CP patients. Design: A cross-sectional study of 41 patients (24 women) surgically treated childhood-onset CP in comparison to controls. Setting: The South Medical Region of Sweden (2.5 million inhabitants). Methods: With magnetic resonance imaging (MRI), we analysed qualitative measurement of WML based on the visual rating scale of Fazekas and quantitative automated segmentation of WML lesion. Also, measurement HT volume and of cardiovascular risk factors were analysed. Results: Patients had a significant increase in WML volume (mL) (P = .001) compared to controls. Treatment with cranial radiotherapy (CRT) vs no CRT was associated with increased WML volume (P = .02) as well as higher Fazekas score (P = .001). WML volume increased with years after CRT (r = 0.39; P = .02), even after adjustment for fat mass and age. A reduced HT volume was associated with increased WML volume (r = −0.61, P < .001) and explained 26% of the variation (r 2 = 0.26). Altogether, 47% of the WML volume was explained by age at investigation, HT volume and CRT. Patients with more WML also had higher cardiovascular risk. Conclusions: CRT may be associated directly with increased WML volume or indirectly with reduced HT volume associated with higher cardiovascular risk. Risk factors should be carefully monitored in these patients.

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Detailed assessment of hypothalamic damage in craniopharyngioma patients with obesity

Cited by 22 publications

References 36 publications

The Diagnosis and Treatment of Craniopharyngioma

The Diagnosis and Treatment of Craniopharyngioma

Diagnosis, Background, and Treatment of Hypothalamic Damage in Craniopharyngioma

Brain white matter lesions are associated with reduced hypothalamic volume and cranial radiotherapy in childhood‐onset craniopharyngioma

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