Emma Hitt, PhD

May 14, 2012

May 14, 2012 (Florence, Italy) — Regardless of how childhood craniopharyngioma is treated, initial hypothalamic involvement resulting from surgical lesions is the major risk factor for impaired prognosis and quality of life (QoL), according to a new study.

Hermann Müller, MD, from the Klinikum Oldenburg, in Germany, and colleagues presented their findings here at the Joint 15th International Congress of Endocrinology and 14th European Congress of Endocrinology.

According to the researchers, hypothalamic obesity has a major impact on prognosis and QoL in childhood craniopharyngioma; however, the effects of initial hypothalamic involvement, compared with treatment-related hypothalamic lesions, remain unclear.

To assess this issue, Dr. Müller and colleagues recruited 120 patients from 2001 to 2007 and evaluated them for body mass index (BMI) and QoL at baseline and 3 years later. Postsurgical hypothalamic lesions using a standardized grading system (no, anterior, posterior involvement/lesion) were also assessed.

The researchers analyzed the neurosurgical treatment strategies used for these patients at 50 participating neurosurgical centers. Center size was defined as "small" (1 child with craniopharyngioma treated in 6 years), "middle" (2 to 5 children treated), or "large" (more than 5 children treated).

Patients with surgical lesions in the anterior and posterior hypothalamic areas had a larger increase in BMI 3 years after diagnosis than did patients without both of these types of lesions or with only an anterior lesion (P = .033 and P = .011, respectively). This increase in BMI negatively affected QoL in patients with posterior hypothalamic lesions, the researchers found.

Although surgical strategies varied at the 50 participating centers, patients treated at small centers (n = 23) presented with a higher rate of hypothalamic involvement than those treated at middle (n = 24) and large (n = 3) centers.

According to the researchers, treatment at large centers was less radical, and the rates of complete resection and hypothalamic surgical lesions were lower than treatment at middle and small centers. However, multivariable analysis showed that only preoperative hypothalamic involvement independently predicted risk for severe obesity (P = .002).

"Hypothalamic involvement of childhood craniopharyngioma, especially of posterior hypothalamic areas, has a major impact on the prognosis of patients," Dr. Müller told Medscape Medical News.

He explained that based on a novel grading system for hypothalamic involvement, patients at risk for severe adverse sequelae (such as hypothalamic obesity) can be identified with imaging before the initiation of treatment.

"Accordingly, treatment strategies should be risk-adapted based on this grading system and focus on a preservation of the integrity of hypothalamic structures and QoL," he said.

According to Dr. Müller, because the initial pretreatment degree of hypothalamic involvement has a major a priori impact on long-term follow-up, childhood craniopharyngioma should be recognized as a chronic disease requiring lifelong follow-up and care.

He added that the success of neurosurgical treatment strategies in childhood craniopharyngioma must not be defined by proven radical resection, but should be based on postsurgical clinical outcomes and sequelae.

"In the case of incomplete resection, external irradiation is recommended as an efficient second-line treatment modality. The appropriate time point for external irradiation is currently under investigation in a multinational randomized trial [KRANIOPHARYMGEOM 2007]," he said.

Independent commentator Ute Bartels, MD, from the Department of Hematology/Oncology at The Hospital for Sick Children in Toronto, Ontario, Canada, noted that this is the first study to prospectively evaluate the role of hypothalamic involvement in patients affected by craniopharyngioma.

"The authors document preoperative hypothalamic involvement as the only independent risk factor for severe obesity," he told Medscape Medical News.

"This study will enhance awareness of hypothalamic injury and the severe metabolic consequences in children with craniopharyngioma," he added. "Furthermore, it will stipulate surgical approaches aimed at avoiding or at least minimizing additional hypothalamic damage."

According to Dr. Bartels, children affected with craniopharyngioma should be treated by a multidisciplinary team of experts (pediatric neurosurgeons, endocrinologists, oncologists, and radiation oncologists) familiar with the potential long-term morbidities.

"This vulnerable patient population will benefit most from an individualized treatment approach aimed at avoiding further morbidities and preserving quality of life," he said.

The study was not commercially funded. Dr. Müller and Dr. Bartels have disclosed no relevant financial relationships.

Joint 15th International Congress of Endocrinology (ICE) and 14th European Congress of Endocrinology (ECE): Abstract OC1.5. Presented May 7, 2011.


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