Childhood Craniopharyngioma – a Rare Disease Necessitating a Multidisciplinary Approach. The Experience of the Expert Centre for Rare Endocrine Diseases in Varna
Author: Bazdarska, Yuliya R. ,1 Galcheva, Sonya V. ,1 2 Shefket, Sevim A. 3,4, Novakova, Marianna M.5, Halvadzhiyan, Irina B. 6,7, Iotova, Violeta M. 1,2
Abstracts:
Craniopharyngioma (CP) is a rare benign tumor, with about half of the cases diagnosed before the age of 18 years.
Objective: to present summarized data for the patients with CP followed up in the Expert Center for Rare Endocrine Diseases in Varna (VECRED).
Patients and methods: We present a retrospective study (2013-2023) of all CP patients followed > 1 year. Demographic, clinical, laboratory and imaging data, type and consequences of treatment were processed.
Results: For the indicated period, 7 patients were treated in the center (mean age 8,4±3,9 years, 71,4% boys), observed for 42,7±36,4 months. The average duration of symptoms before diagnosis was 5,8±9,5 months. The most common were headache, visual disturbances and vomiting (57,1%), decreased growth velocity (42,9%), etc.; 71,4% initially had consultation with an ophthalmologist. MRI was performed in all cases (in five after a previous CT scan). More than half of the patients (57,1%) had suprasellar and sellar formation; 100% have adamantimatous type of CP. The operations were done in six different neurosurgical clinics, followed by radiotherapy in three cases. Total resection of the tumor was achieved in 71,4%. All patients developed postoperative panhypopituitarism, most frequently hypothyroidism and diabetes insipidus (100% each). The mean age at the start of replacement therapy with human recombinant growth hormone (hrGH) was 2,75±2,1 years after completion of treatment. At the moment, there are no patients with relapse of CP.
Conclusion: Craniopharyngioma in childhood is still diagnosed relatively late. The first line treatment is surgi cal removal, which should be carried out in a reference center with a sufficient volume of cases and with a broad multidisciplinary team. Growth hormone replacement therapy is proven to be safe and effective without increasing the risk of relapse.
