The purpose of our study was to investigate the pattern of recurrence and the prognostic factors for recurrence of pediatric craniopharyngiomas after radical excision. A series of 36 patients with craniopharyngiomas (21 boys and 15 girls; age range 1-15 years; mean 7.3 years) were reviewed. All patients had undergone radical excision without radiotherapy. The mean follow-up period was 52 months (range 1-149 months). Tumors recurred in 14 patients within 83 months (mean 31.4 months). The overall 5-year recurrence-free survival rate was 55%. Regular neuroimaging follow-up detected tumor recurrence while the lesions were still small before symptoms developed (P<0.05). At the first surgical procedure, the optic nerve/chiasm (n=23) was the most common adhesion site. The most frequent sites of recurrence were the optic nerve/chiasm (n=6) and the pituitary fossa (n=6). Tumor location was the single significant clinical predictor of recurrence. The 5-year recurrence-free survival rate was 39% for those who had an intrasellar tumor component and 81% for those who did not (P<0.05). The Ki-67 labeling indices (LIs) of primary tumors did not have prognostic value for recurrence. Recurrent tumors tended to have higher Ki-67 LIs than their primary counterparts. On the basis of this study, we concluded that craniopharyngiomas with intrasellar components should be followed cautiously and the necessity for regular follow-up should be emphasized, even when the tumor is "totally" resected.
Background
Leptomeningeal carcinomatosis (LMC) is frequently associated with hydrocephalus, which quickly devastates the performance of the patient. Cerebrospinal fluid (CSF) shunt is a widely accepted treatment of choice, but the clinical outcomes in patients with LMC are not well studied. This study aimed to examine the efficacy of a CSF shunt in patients with LMC.
Methods
Seventy patients with LMC confirmed by cytology or magnetic resonance imaging (MRI) underwent ventriculoperitoneal (VP) or lumboperitoneal (LP) shunt surgery. We retrospectively analyzed the clinical characteristics of patients, symptom improvement after the shunt, rate of complications associated with the surgery, and overall survival.
Results
Fifty-five patients had systemic cancer as a preceding disease, including lung cancer (45), breast cancer (6), and others (4). Primary brain tumors were mainly glioma (7) and medulloblastoma (5). Fifty-one patients had VP shunt, and 19 had LP shunt. After surgery, preoperative symptoms “improved” in 35 patients (50%) and were “normalized” in 24 of those patients (34%). Shunt malfunction occurred in eight patients, and infection occurred in eight patients. Seventeen patients underwent revision due to infection, shunt malfunction, or over-drainage. There were no complications associated with peritoneal seeding during a median follow-up of 3.3 months after surgery. The median overall survival was 8.7 months (95% confidence interval, 6.0–11.4) from LMC diagnosis and 4.1 months from shunt surgery.
Conclusion
VP or LP shunt is effective for patients with hydrocephalus from LMC in terms of symptom improvement and prolonging of overall survival with an acceptable rate of procedure-related complications.
Trial registration
This study was approved by the Institutional Review Board (IRB) of the National Cancer Center (retrospectively registered,
NCC2018-0051
).
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