The skull base chordoma patient reported outcome survey (sbCPROS): a patient-centered, disease-specific tool for assessing quality of life in chordoma patients.

PURPOSE

Chordomas are rare malignant tumors arising from the embryological notochord that present most frequently in the lumbosacral spine, followed by the skull base, with an overall 1/1,000,000 incidence. These tumors and their treatment significantly affect quality of life (QOL) due to intricate anatomical locations and aggressive treatment regimens. Despite these challenges, there are currently no disease-specific patient-reported outcome (PRO) surveys for chordomas. We aimed to develop a tool to assess QOL in patients with skull base chordomas (sbCs).

METHODS

Twenty-seven patients who underwent sbC resection were interviewed on QOL throughout their care. Grounded-theory analysis of interview transcripts generated 7 themes. We developed an initial survey with 79 items from existing general and anatomic-specific QOL assessment tools addressing these themes. Ten chordoma providers and 10 new patients completed an anonymous Qualtrics survey, rating items' relevance on a 5-point Likert scale to validate survey content. An a priori cutoff of > 3.0 was used for significant relevance. Mean relevance scores for each item were compared between providers and patients as well as between skull base respondents and 5 control patients with lumbar spine and sacral chordomas, using two-sided Mann-Whitney U-tests.

RESULTS

Seventy-four items reached the relevance threshold. These were consolidated to create the final 42-item Skull Base Chordoma Patient Reported Outcome Survey (sbCPROS). Providers significantly overvalued items related to the themes of pain (73%), sleep changes (60%), and sensory & motor symptoms (43%) relative to patients. Ten items were more relevant to skull base patients than patients with spinal tumors (p < 0.05).

CONCLUSION

The authors developed a novel patient-centered, disease-specific PRO instrument to assess change in QOL for sbC patients over time. sbCPROS may provide significant insight into the delivery of high quality care for patients with sbCs and guide patient-physician discussions about care decision-making.