About this Research Topic
Adult-onset craniopharyngioma is an intracranial neoplasm of embryonic origin that adversely impacts morbidity and mortality due to pituitary and hypothalamic dysfunctions. Even though it is a rare tumor, post-surgery metabolic complications are common, severe, and have no effective treatment.
Surgical resection of the tumor is standard of care, but recurrence is common, and each subsequent surgery further compounds damage to the hypothalamus. Limited resection of the primary tumor coupled with radiation therapy can spare the hypothalamus while minimizing the risk for progression but contributes to vasculopathies and secondary malignancies, encephalopathy, and radionecrosis. Ultimately, disease- and treatment-related sequelae leave craniopharyngioma patients with a 4-9-fold increase in age-adjusted mortality secondary to cardio- and cerebrovascular diseases, and a standardized mortality ratio of 2.9 attributed to hydrocephalus, tumor recurrence, obesity, and panhypopituitarism.
Craniopharyngioma incidence has a bimodal distribution, with a first peak in children aged 0-19 years and a second in adults aged 40-79. However, adult-onset craniopharyngioma is less well-studied overall and is limited to small sample sizes and short duration. The optimal management of these tumors is unclear and variable. There are two histologic variants of craniopharyngioma, adamantinomatous and papillary, each with distinct genetic causes and pathogenesis and impact on the biology of these tumors. The extent of surgery and use of radiotherapy while preserving hypothalamic function is difficult to balance, and patients not only face long-term metabolic complications but also reduced health-related quality of life. craniopharyngioma-specific measures are not used in these patients, making it difficult to distinguish craniopharyngioma-associated effects from those associated more generally with sellar/parasellar tumors.
Current research in craniopharyngioma focuses primarily on obesity and glycemic outcomes in childhood-onset craniopharyngioma and hypothalamic obesity. Few data are available on the deleterious effects of these metabolic complications leading to long-term impact in adult-onset craniopharyngioma and even less is understood about the biopsychosocial impact of craniopharyngioma.
This research topic aims to heighten awareness in clinicians of the significant impact on quality of life and metabolic function that adult-onset craniopharyngioma patients face long term. We aim to stimulate interest in research in adult-onset craniopharyngioma and promote translational work to address the gaps in care.
We welcome original research, reviews, and other article types. Submissions can encompass the following:
• Approaches for initial treatment, including surgical approach and adjuvant radiation vs no radiation postoperatively
• Novel therapies for recurrence
• Genetics of adult-onset craniopharyngioma, pathophysiology, transcriptomic studies.
• Precision medicine for craniopharyngioma
• Long-term sequelae of craniopharyngioma in terms of hypopituitarism, quality of life, metabolic complications, and hypothalamic obesity.
• Approach to the management of craniopharyngioma sequelae in adults
• Biopsychosocial impact of adult-onset craniopharyngioma
• Fertility implications
Surgical resection of the tumor is standard of care, but recurrence is common, and each subsequent surgery further compounds damage to the hypothalamus. Limited resection of the primary tumor coupled with radiation therapy can spare the hypothalamus while minimizing the risk for progression but contributes to vasculopathies and secondary malignancies, encephalopathy, and radionecrosis. Ultimately, disease- and treatment-related sequelae leave craniopharyngioma patients with a 4-9-fold increase in age-adjusted mortality secondary to cardio- and cerebrovascular diseases, and a standardized mortality ratio of 2.9 attributed to hydrocephalus, tumor recurrence, obesity, and panhypopituitarism.
Craniopharyngioma incidence has a bimodal distribution, with a first peak in children aged 0-19 years and a second in adults aged 40-79. However, adult-onset craniopharyngioma is less well-studied overall and is limited to small sample sizes and short duration. The optimal management of these tumors is unclear and variable. There are two histologic variants of craniopharyngioma, adamantinomatous and papillary, each with distinct genetic causes and pathogenesis and impact on the biology of these tumors. The extent of surgery and use of radiotherapy while preserving hypothalamic function is difficult to balance, and patients not only face long-term metabolic complications but also reduced health-related quality of life. craniopharyngioma-specific measures are not used in these patients, making it difficult to distinguish craniopharyngioma-associated effects from those associated more generally with sellar/parasellar tumors.
Current research in craniopharyngioma focuses primarily on obesity and glycemic outcomes in childhood-onset craniopharyngioma and hypothalamic obesity. Few data are available on the deleterious effects of these metabolic complications leading to long-term impact in adult-onset craniopharyngioma and even less is understood about the biopsychosocial impact of craniopharyngioma.
This research topic aims to heighten awareness in clinicians of the significant impact on quality of life and metabolic function that adult-onset craniopharyngioma patients face long term. We aim to stimulate interest in research in adult-onset craniopharyngioma and promote translational work to address the gaps in care.
We welcome original research, reviews, and other article types. Submissions can encompass the following:
• Approaches for initial treatment, including surgical approach and adjuvant radiation vs no radiation postoperatively
• Novel therapies for recurrence
• Genetics of adult-onset craniopharyngioma, pathophysiology, transcriptomic studies.
• Precision medicine for craniopharyngioma
• Long-term sequelae of craniopharyngioma in terms of hypopituitarism, quality of life, metabolic complications, and hypothalamic obesity.
• Approach to the management of craniopharyngioma sequelae in adults
• Biopsychosocial impact of adult-onset craniopharyngioma
• Fertility implications
Keywords: Craniopharyngioma, hypopituitarism, hypothalamic obesity, recurrence, genetics, treatment
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