Craniopharyngioma Clinical Status Scale: a standardized metric of preoperative function and posttreatment outcome

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>Controversy persists concerning the optimal treatment of craniopharyngiomas in children, and no standard outcome metric exists for comparison across treatment modalities, nor is there one that adequately reflects the multisystem dysfunction that may arise.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>The authors retrospectively analyzed the records of 86 consecutive children who underwent a uniform treatment paradigm of attempted radical resection performed by a single surgeon. Excluding 3 perioperative deaths and 3 patients with inadequate follow-up, 80 children (34 girls and 46 boys; mean age 9.56 years; mean follow-up 9.6 years) composed the study group (53 primary and 27 previously treated/recurrent tumors). Building on existing classification schemes proposed by De Vile for hypothalamic dysfunction and Wen for overall functional outcome, the authors devised a more nuanced classification system (Craniopharyngioma Clinical Status Scale [CCSS]) that assesses outcome across 5 axes, including neurological examination, visual status, pituitary function, hypothalamic dysfunction, and educational/occupational status at last follow-up (there is a 4-tiered grading scale in each domain, with increasing values reflecting greater dysfunction).</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>There was a significant increase in pituitary dysfunction following treatment—consistent with the high rates of diabetes insipidus and hypopituitarism common to the surgical management of craniopharyngiomas—and less dramatic deterioration in hypothalamic function or cognitive domains. Significant improvement in vision was also demonstrated, with no significant overall change in neurological status. Preoperative CCSS scores predicted postoperative outcome better than clinical characteristics like patient age, sex, tumor size, and the location or presence of hydrocephalus.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>Preoperative CCSS scores predicted outcome with higher accuracy than clinical or imaging characteristics. In lieu of randomized trials, the CCSS may provide a useful outcome assessment tool for comparison across treatment paradigms and surgical approaches. Long-term follow-up is critical to the analysis of outcomes of craniopharyngioma treatment, given the often-delayed sequelae of all therapies and the high recurrence rates of these tumors.</jats:p></jats:sec>

Recurrent craniopharyngioma after conformal radiation in children and the burden of treatment

“…33 Our end points were limited and we did not have quality of life data, an important outcome to measure in this population that the disease and treatments directly impact. 12,28,31 conclusions The management of recurrent craniopharyngioma in children is extremely challenging. The overall rate of recurrence in children with craniopharyngioma treated at our institution using photon CRT was nearly 20%.…”

The therapeutic efficacy of fractionated radiotherapy and gamma-knife radiosurgery for craniopharyngiomas

“…However, it remains controversial whether RT should be used immediately after surgery or at tumor recurrence. 14,[17][18][19] Because RT is essential in controlling tumor growth and overall survival after surgical resection, 8,[20][21][22][23] the timing of RT is critical. In this retrospective study, we attempted to identify whether adjunctive or salvage RT was optimal, and which modality (FRT or GKRS) was more efficacious for tumor control, with fewer morbidities.…”

Preoperative staging in childhood craniopharyngioma: standardization as a first step towards improved outcome

“…In the first group, the hypothalamus can be simply compressed and not infiltrated. Elliott et al [11] used a newly designed craniopharyngioma clinical status scale (CCSS) to show that preoperative CCSS scores predicted postoperative outcome better than clinical characteristics like patient age, sex, tumor size, and the location or presence of hydrocephalus.…”

“…Several grading systems for assessment of pre and/or postoperative location and hypothalamic involvement of CP and algorithms for specific treatment strategies based on these grading systems have been published [4][5][6][7][8][9][10][11]. Mortini et al [12] are publishing an analysis of their single center cohort of exclusively adult-onset CPs comparing three of the above-mentioned grading systems [4][5][6]9] in terms of prediction value for outcome and the development of hypothalamic syndrome.…”