MANAGEMENT OF ENDOCRINE DISEASE: Childhood-onset craniopharyngioma: state of the art of care in 2018

Müller, Hermann L.

doi:10.1530/eje-18-1021

Cited by 37 publications

(26 citation statements)

References 87 publications

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“…Hypothalamic involvement of CP and/or lesions to hypothalamic structures due to surgical and/or radio-oncological interventions are associated with severe sequelae, mainly HO, with a negative impact on the QoL of CP survivors [43][44][45][46][47][48][49][50]. 35% of CP patients present with clinical manifestations associated with hypothalamic dysfunction, such as HO, neuropsychological deficits, and disturbances of circadian rhythms at the time of diagnosis [50].…”

Section: Hypothalamic Syndromementioning

confidence: 99%

The Diagnosis and Treatment of Craniopharyngioma

Müller

2019

Neuroendocrinology

107

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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: Hypothalamic Syndromementioning

confidence: 99%

The Diagnosis and Treatment of Craniopharyngioma

Müller

2019

Neuroendocrinology

107

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“…Childhood-onset, adamantinomatous craniopharyngiomas (CP) are rare embryonal malformational tumors, originating in the sellar and parasellar region with WHO grade I malignancy. Long-term prognosis and quality of life (QOL) are frequently impaired due to sequelae caused by the anatomical location of CP close to the pituitary gland, the hypothalamus, and the optic chiasm (1)(2)(3)(4)(5). In CP patients with hypothalamic involvement both survival rates and QOL are reduced (6)(7)(8).…”

Section: Introductionmentioning

confidence: 99%

Cerebral Infarction in Childhood-Onset Craniopharyngioma Patients: Results of KRANIOPHARYNGEOM 2007

et al. 2021

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BackgroundCerebral infarction (CI) is a known vascular complication following treatment of suprasellar tumors. Risk factors for CI, incidence rate, and long-term prognosis are unknown for patients with childhood-onset craniopharyngioma (CP).MethodsMRI of 244 CP patients, recruited between 2007 and 2019 in KRANIOPHARYNGEOM 2007, were reviewed for CI. Risk factors for CI and outcome after CI were analyzed.ResultsTwenty-eight of 244 patients (11%) presented with CI based on reference assessment of MRI. One CI occurred before initial surgery and one case of CI occurred after release of intracystic pressure by a cyst catheter. 26 of 28 CI were detected after surgical tumor resection at a median postoperative interval of one day (range: 0.5-53 days). Vascular lesions during surgical procedures were documented in 7 cases with CI. No relevant differences with regard to surgical approaches were found. In all 12 irradiated patients, CI occurred before irradiation. Multivariable analyses showed that hydrocephalus and gross-total resection at the time of primary diagnosis/surgery both were risk factors for CI. After CI, quality of life (PEDQOL) and functional capacity (FMH) were impaired.ConclusionsCI occurs in 11% of surgically-treated CP cases. Degree of resection and increased intracranial pressure are risk factors, which should be considered in the planning of surgical procedures for prevention of CI.

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“…Craniopharyngioma (CP) is a rare benign embryonal malformation derived from ectoblastic remnants of Rathke's pouch [1][2][3][4]. Treatment options for CP include surgical resection with or without consecutive radiation therapy.…”

Section: Introductionmentioning

confidence: 99%

Pregnancies after Childhood Craniopharyngioma: Results of KRANIOPHARYNGEOM 2000/2007 and Review of the Literature

et al. 2020

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Background: Data on female fertility, pregnancy, and outcome of offspring after childhood-onset craniopharyngioma (CP) are rare. Study Design: Observational study on pregnancy rate and offspring outcome in female CP patients recruited in KRANIOPHARYNGEOM 2000/2007 since 2000. Results: A total of 451 CP patients (223 female) have been recruited, and 269 (133 female) were postpubertal at study. Six of 133 female CP patients (4.5%) with a median age of 14.9 years at CP diagnosis had 9 pregnancies, giving birth to 10 newborns. Three patients achieved complete surgical resections. No patient underwent postoperative irradiation. Five natural pregnancies occurred in 3 CP patients without pituitary deficiencies. Four pregnancies in 3 CP patients with hypopituitarism were achieved under assisted reproductive techniques (ART) (median 4.5 cycles, range: 3–6 cycles). Median maternal age at pregnancy was 30 years (range: 22–41 years). Six babies (60%) were delivered by caesarean section. Median gestational age at delivery was 38 weeks (range: 34–43 weeks); median birth weight was 2,920 g (range: 2,270–3,520 g), the rate of preterm delivery was 33%. Enlargements of CP cysts occurred in 2 women during pregnancy. Other complications during pregnancy, delivery, and postnatal period were not observed. Conclusions: Pregnancies after CP are rare and were only achieved after ART in patients with hypopituitarism. Close monitoring by an experienced reproductive physician is necessary. Due to a potentially increased risk for cystic enlargement, clinical, ophthalmological, and MRI monitoring are recommended in patients at risk. Severe perinatal complications, birth defects, and postnatal morbidity of mothers and offspring were not observed.

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MANAGEMENT OF ENDOCRINE DISEASE: Childhood-onset craniopharyngioma: state of the art of care in 2018

Cited by 37 publications

References 87 publications

The Diagnosis and Treatment of Craniopharyngioma

The Diagnosis and Treatment of Craniopharyngioma

Cerebral Infarction in Childhood-Onset Craniopharyngioma Patients: Results of KRANIOPHARYNGEOM 2007

Pregnancies after Childhood Craniopharyngioma: Results of KRANIOPHARYNGEOM 2000/2007 and Review of the Literature

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