Further Outpatient Care
Endocrine
Close monitoring of growth, pubertal development, and any known hormone abnormalities is essential in patients with craniopharyngioma.
Oncologic
Routine neuroimaging is the mainstay of follow-up assessment.
The expert consensus recommends brain MRI every 3 months for 1 year, followed by MRI every 6 months for 1 year, and then annual MRI until year 5. If they received radiation therapy, an MRI annually or every other year for life is recommended due to the potential risk for second malignancies.
Ophthalmologic
Routine ophthalmology evaluations are recommended. In particular, new visual field deficits may hallmark disease progression.
Neuropsychiatric
Periodic formal assessment can be useful when monitoring the long-term sequelae of surgery or radiotherapy.
In formal assessment, include comparison with preoperative and postoperative evaluations.
Inpatient & Outpatient Medications
Long-term hormone supplementation is the primary medical treatment associated with childhood craniopharyngiomas and includes the following:
-
Intranasal vasopressin
-
Corticosteroids
-
Thyroid hormones
-
Growth hormones
-
Sex hormones
Complications
A meta-analysis confirmed the high rates of endocrine, vascular, neurologic, and visual morbidities experienced by the patients posttherapy. Although the study was retrospective and had limitations, they concluded that, at least for some patients, less aggressive surgery followed by radiation therapy might result in less morbidity. [30] Further details regarding complications are below.
Endocrine Morbidity
The need for hormone replacement is common (approximately 80%) following therapy for craniopharyngiomas. Individual hormone deficits manifest with rates of 88-100% for GH, 55-88% for ACTH, 39-95% for TSH, 80-95% for FSH/LH, and 25-86% for ADH.
There have been concerns regarding the use of supplemental human growth hormone therapy for short stature in these children. Many pre-clinical brain tumor studies have demonstrated tumor growth after administration of growth hormone. [33] However, more recently, several large observational series have been unable to find an association between inferior tumor outcomes and use of growth hormone replacement. Current practice centers around continuing use of physiologic growth hormone replacement, with close imaging follow-up of these patients to monitor for tumor growth/recurrence. [34, 35, 36]
Diabetes insipidus is one of the most serious endocrinologic disturbances. Children with diabetes insipidus can become dehydrated during episodes of illness, and their summer activity must frequently be curtailed; in addition, vasopressin administration can be expensive.
Hypothyroidism, growth hormone deficiencies, steroid hormone deficiencies, and delayed or precocious puberty have also been reported. Pay particular attention to these children when they develop significant systemic illnesses; stress doses of steroids may be required.
Obesity and obesity-related complications are also well described morbidities for these patients. Hypothalamic damage, changes in metabolic expenditures, and decreased physical activity are some reasons for these often difficult to manage problems. [12]
Severe endocrine abnormalities are associated almost exclusively with radical surgery.
Ophthalmologic Morbidity
More than 50% of patients present with visual impairment at diagnosis. 40-50% of patients experience some post-surgical improvement of vision. Patients at risk for post-surgical visual impairment are those with a prechiasmatic tumor location or severe pre-surgical visual deficits. The transsphenoidal approach has been associated with improved opthhalmological outcomes.
Neurologic Morbidity
This is also frequently associated with attempts at radical tumor resection, visual loss, and hypothalamic injury (morbid obesity, hypersomnolence), which may develop following therapy for craniopharyngiomas. Sleep dysfunction has also become increasingly recognized as a complication of craniopharyngioma diagnosis and management, with almost two thirds of patients reporting sleep problems in one series. [37]
Neuropsychological Morbidity
Memory loss, behavior changes, and academic decline can result directly from neurologic damage. These neuropsychological changes can also occur indirectly as a result of other complications of therapy (eg, endocrinologic derangement).
Thrombophilia
Deep vein thromboses (DVT) have been well described as a complication postoperatively for patients with craniopharyngioma. Inadequate control of serum sodium and hormonal changes have been hypothesized as contributing to an increased risk for DVT in these patients.
One series evaluated concomitant risk factors for DVT in a small series of patients with craniopharyngioma and identified some inherited risk factors that might further increase the risk for DVT in these patients including increased factor VIII and von Willebrand factor (vWF) levels, MTHFR mutations, and mutations in plasminogen activator inhibitor-1(PAI-1). [38]
Consequences of Radiation Therapy
Although it is now more commonly used in the management of craniopharyngiomas, even for children, late effects of radiation therapy are becoming increasingly recognized.
Endocrinopathies, vasculopathies (as high as 21%), and second malignancies (1.9% at 10 years) are now being seen following radiation therapy for these tumors. [39]
Transformation of low-grade craniopharyngiomas into high-grade epithelial carcinomas has also been observed. Generally this is in association with prior radiation therapy, although 1 of 3 cases from 1 series did not have prior radiation. [40]
Prognosis
Previous studies have shown relatively good outcomes, with 10-year overall survival rates of 86-100% among patients who underwent gross total resection. Subtotal resection or recurrence treated with surgery and radiation therapy carry 10-year overall survival rates of 57-86%. More recently, the surgical mortality rate after primary surgical intervention has been reported to be 1.7-5.4%. However, mortality rate after re-resection can be as high as 25%. Gross total resection carries a greater risk of long-term neurologic and endocrinologic morbidity.
Yosef and colleagues recently looked at the impact of initial tumor size on outcomes. They looked at tumors less than 4.5 cm maximum diameter versus tumors greater than 4.5 cm in diameter. They termed the large craniopharyngiomas, “giant” craniopharyngiomas and found them to be associated with greater residual post-operative tumor and significantly decreased 2 and 5 year PFS versus smaller tumors (33.3 vs. 73.3% and 33.3 vs. 53.3% respectively). [41]
Patient Education
For additional information, see the People Living With Cancer article on craniopharyngioma.
-
This MRI sequence was obtained following the intravenous administration of gadolinium contrast. Observe the relatively homogeneous and cystic mass arising from the sella turcica and extending superiorly and posteriorly with compression of normal regional structures. Note that the lesion is sharply demarcated and smoothly contoured. This fluid-filled mass is consistent with a typical craniopharyngioma.
-
This axial CT scan image demonstrates a cystic lesion in the typical location of a craniopharyngioma. Although most of the lesion is fluid filled, a rim of enhancing soft tissue is observed following the administration of intravenous contrast.
-
Non-contrast Head CT with peripheral calcifications around a bi-lobed craniopharyngioma.
