craniopharyngioma

Childhood-onset craniopharyngioma linked to high mortality

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Craniopharyngioma appears to be linked to excess mortality, particularly in women and in patients with childhood-onset disease, according to recent findings.

In the nationwide, population-based study, researchers identified 307 patients with craniopharyngioma (151 men) from the Swedish National Patient Registry and the Swedish Cancer Registry to determine rates of morbidity and mortality for both childhood- and adult-onset disease.

The researchers specifically studied the following conditions: type 2 diabetes, myocardial infarction, fracture, severe infection, cerebral infarction and severe vision impairment. The mean follow-up was 9 years. Person-years at risk were determined from study enrollment to follow-up diagnosis, death or conclusion of study, and were stratified by gender, 5-year age groups and 1-year calendar periods.

The researchers found that there were 54 deaths during the study, which exceeded the expected number of 14.1. This resulted in a standardized mortality ratio (SMR) of 3.2 (95% CI, 2.2-4.7) for men and 4.9 (95% CI, 3.2-7.2) for women. Patients with childhood-onset disease (n=106) had an SMR of 17 (95% CI, 6.3-37), whereas those with adult-onset craniopharyngioma (n=201) had SMRs of 3.5 (95% CI, 2.6-4.6). The SMRs for patients with hypopituitarism (n=250) was 4.3 (95% CI, 3.1-5.8) and the SMR for patients with diabetes insipidus was 6.1 (95% CI, 3.5-9.7). SMR specific to cerebrovascular diseases was 5.1 (95% CI, 1.7-12).

The following standardized incident ratios (SIRs) were determined: 5.6 for type 2 diabetes (95% CI, 3.8-8), 7.1 for cerebral infarction (95% CI, 5-9.9), 0.7 for MI (95% CI, 0.2-1.7), 2.1 for fracture (95% CI, 1.4-3) and 5.9 for severe infection (95% CI, 3.4-9.4). All malignant tumors had an SIR of 1.3 (95% CI, 0.8-2.1).

According to the researchers, these findings reflect the complexity of craniopharyngioma and its treatment.

“[Craniopharyngioma] is a rare tumor, and the tumor itself and its management can lead to a complex clinical picture dominated by endocrine, metabolic and neuropsychological consequences,” the researchers wrote. “This first nationwide study reveals a marked excess mortality and a prominently shortened lifespan in [craniopharyngioma] patients.”

Ectopic recurrent craniopharyngioma of the frontal bone.

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Ectopic recurrence of craniopharyngioma is a rare phenomenon after transcranial resection of the primary tumor. The authors present a case of ectopic recurrent adamantinomatous craniopharyngioma of the frontal bone resected 16 years after initial transcranial resection of the primary tumor. The lesion was first radiographically described 12 years after surgery and was adjacent to the osteosynthesis plate that had been implanted at the craniotomy site. The recurrent craniopharyngioma was totally resected via a lateral eyebrow approach. No infiltration of the meninges or the brain was detected.

Only 50 cases of ectopic recurrent craniopharyngioma have been described to date, with the present case being the first one with recurrence located at the skull bone. So far 2 mechanisms have been described: contamination with tumor cells alongside the surgical tract and spreading via CSF and the subarachnoid space.

The authors reviewed the literature, provided the largest collection of cases so far, and performed basic statistical analysis regarding ectopic recurrence. Pediatric and adult patients as well as male and female ones are affected equally by this phenomenon. The mean time of ectopic recurrence after initial surgery was 7.1 years. Ectopic recurrence, although rare, should always be considered in a patient with a newly diagnosed intracranial lesion who has undergone transcranial craniopharyngioma resection before.

Source: Journal Of Neurosurgery.

 

 

 

 

 

Ectopic recurrent craniopharyngioma of the frontal bone.

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Ectopic recurrence of craniopharyngioma is a rare phenomenon after transcranial resection of the primary tumor. The authors present a case of ectopic recurrent adamantinomatous craniopharyngioma of the frontal bone resected 16 years after initial transcranial resection of the primary tumor. The lesion was first radiographically described 12 years after surgery and was adjacent to the osteosynthesis plate that had been implanted at the craniotomy site. The recurrent craniopharyngioma was totally resected via a lateral eyebrow approach. No infiltration of the meninges or the brain was detected.

Only 50 cases of ectopic recurrent craniopharyngioma have been described to date, with the present case being the first one with recurrence located at the skull bone. So far 2 mechanisms have been described: contamination with tumor cells alongside the surgical tract and spreading via CSF and the subarachnoid space.

The authors reviewed the literature, provided the largest collection of cases so far, and performed basic statistical analysis regarding ectopic recurrence. Pediatric and adult patients as well as male and female ones are affected equally by this phenomenon. The mean time of ectopic recurrence after initial surgery was 7.1 years. Ectopic recurrence, although rare, should always be considered in a patient with a newly diagnosed intracranial lesion who has undergone transcranial craniopharyngioma resection before.

Source: Journal of Neurosurgery.