Resumen:
Craniopharyngiomas are rare lesions derived from cell remnants of Rathke’s pouch, usually localized in the sellar and suprasellar areas.
Objectives: To analyze clinical, endocrine and histological features in a group of patients from Buenos Aires city. To assess efficacy, number of surgeries and complications after surgery or other treatments.
Material and methods: Multicentric, retrospective, transversal study. Adult patients with craniopharyngiomas were included. We assessed clinical, biochemical, MRI features, at diagnosis and after treatments, the follow-up and recurrence of tumors.
Results: Ninety-seven patients, 49 women. Mean age at diagnosis: 41.4±16.04. Symptoms at diagnosis: visual field defects (VFD): 47.4%, headache: 14%, both symptoms: 14%, in this group with mass effect, the VFD reached 61.8%. Other symptoms at diagnosis: incidental findings: 8,2%, diabetes insipidus (DI) 5.2%, and others 11.2% (n:10): 4 hypopituitarism, 2 oligomenorrhea, 2 neurocognitive deficit, 1 behavior disorder, 1 intracranial hypertension. Supraselar extension was present in 88.7%. Endocrine tests showed: hyperprolactinemia 27.8% (Prolactin levels: 61.6 ng/ml (21-156). DI and panhypopituitarism (PHP) were present in 30.5 and 26% respectively; VFD 78.5%: hemianopsia (60.9%): 50/82, quadrantopsia 19% and blindness 10.5% (9/85). Basal BMI was 28.6±7.6. Surgical treatment (95/98): transcranial approach was performed in 71.1%, transsphenoidal approach was used in 25.8%; gross total resection was reached in 46.4%, subtotal 37.1% and partial resection 14.4%. Adamantinomatous type was found in 58.8%, papillary type in 13.4%. In the post-surgical assessment: 71.1% developed (PHP) (p .002) and DI 64.9% (p.002). VFD were 45.4% (40/88): hemianopsia 36.4%, quadrantopsia 9.5% and unilateral or bilateral amaurosis 9% (P 0.002 vs pre surgery). Tumor remnants were observed in 46.8% and recurrence rate was 19.6%. A second surgery was performed in 42.3%. Radiotherapy was indicated in 9.6%. Median time of follow-up was 57 months (4-427). Nine patients died, 4 related to the craniopharyngioma.
Conclusions: In this cohort of adult patients with craniopharyngiomas, VFD was the main symptom. Transcranial approach was the predominant surgery; Adamantinomatous type was the most frequent. In the post-surgical assessment, there was significant improvement in the visual field, but a significant increase in the BMI of patients and in the number of subjects with PHP. A second surgery was performed in 42.3% and 9.6% was treated with radiotherapy. These patients with long-term morbidities need life-long follow-up by a multidisciplinary team.
Atribución 4.0 Internacional (CC BY 4.0)