Impact of diabetes insipidus (DI) and complex tumor treatment regimens on morbidity in adults with childhood-onset craniopharyngioma (COCP): data from KIMS (Pfizer International Metabolic Database)

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SUN 130-162-Neuroendocrinology
Sunday, June 16, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SUN-138
Kevin C.J. Yuen*1, David M. Cook2, Maria Koltowska-Häggström3, Janet L. Fox4, Peter J. Jonsson5, Mitchell E. Geffner6 and Roger Abs7
1Oregon Health & Science University, Portland, OR, 2OHSU, Portland, OR, 3Pfizer Endocrine Care, Sollentuna, Sweden, 4Pfizer, Inc., New York, NY, 5Pfizer, Endocrine Care, Sollentuna, Sweden, 6Children's Hospital Los Angeles, Los Angeles, CA, 7Antwerp Center for Endocrinology, Antwerp, Belgium
Context: Increased obesity and morbidity in patients with craniopharyngiomas are likely to be caused by hypothalamic damage due to the tumor itself &/or its treatment.

Aims: We compared clinical characteristics of KIMS patients with COCP with & without DI, & those who underwent CT regimens of the tumor to those that underwent only 1 surgery (1Sg).

Methods: Data from 180 COCP patients were analyzed.

Results: Overall, more patients had DI (n=140, 77.8%) than no DI (n=40, 22.2%), & more patients underwent 1Sg (n=107, 59.4%) than CT (n=73, 40.6%). There were no gender differences between the DI (91M/49F) & no DI (20M/20F), & between the CT (51M/22F) & 1Sg (60M/47F) groups.

A) DI vs no DI: Compared to patients without DI, those with DI had similarly impaired quality of life (QoL) (QoL AGHDA scores: 10.1±6.3 vs 8.6±7.3; normal 3.8-6.7), but higher BMIs (30.4±8.1 vs 26.5±6.3 kg/m2; P=0.008). More patients with DI had ACTH (97.1% vs 82.5; P<0.001), TSH (100% vs 92.5%; P=0.001) & gonadotropin (98.6% vs 90.0%; P=0.008) deficits, but both groups had comparable rates of visual field defect (VFD) & ophthalmoplegia.

B) CT vs 1Sg: In the CT group, 52 (71.2%) patients underwent 2 surgeries & 21 (28.8%) patients underwent ≥3 surgeries, while 45 (61.6%) patients did not undergo cranial radiotherapy & 28 (38.4%) patients underwent ≥1 cranial radiotherapy that was mainly external & stereotactic radiotherapy (85.7%). Comparable numbers of patients underwent transcranial surgery in the CT & 1Sg (76.4% vs 78.3%) groups. Compared to the 1Sg group, the CT group had similarly impaired QoL (QoL AGHDA scores: 10.4±6.0 vs 9.3±6.8), but higher BMIs (30.7±7.4 vs 28.8±8.2 kg/m2; P=0.03). Comparable numbers of patients had ACTH, TSH, gonadotropin & ADH (79.5% vs 77.1%) deficits, but more patients in the CT group had VFD (70.4% vs 44.3%; P=0.003) & ophthalmoplegia (22.5% vs 5.4%; P=0.005).

Conclusion: Despite more COCP patients undergoing 1Sg than CT, the majority of patients in both groups had DI suggesting that even 1Sg may adversely affect the neurohypophyseal system possibly by extensive transcranial surgery. The presence of DI is associated with higher rates of anterior pituitary deficiencies, while patients who underwent CT had higher rates of visual dysfunction. Conversely, QoL was impaired to a similar extent in all patients independent of DI & CT, while higher BMIs in the DI & CT groups imply a role of hypothalamic damage in impacting the morbidity of COCP patients.

Nothing to Disclose: KCJY, DMC, MK, JLF, PJJ, MEG, RA

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