The Price of Success-The Long-Term Outcomes of Children with Craniopharyngioma-Two Institutions' Experience

Abstract

An analysis of patients below 21 years old treated due to craniopharyngioma in the years 1979-2022 was performed with the aim of evaluating the long-term outcome and treatment side-effects. The standard statistical tests were used, and 56 patients with a median age of 11 years were evaluated. Surgery was the primary treatment in 55 patients; however, in only 29 it was the only neurosurgical intervention. Eighteen children were treated with radiotherapy (RTH) in primary treatment. The most common neurosurgical side effects observed were visual and endocrine deficits and obesity, which were diagnosed in 27 (49%), 50 (91%), and 25 (52%) patients, respectively. Complications after RTH were diagnosed in 14 cases (32%). During the median follow-up of 8.4 years (range: 0.4-39.8 years), six patients died and the 5- and 10-year overall survival was 97% and 93%, respectively. Five-year progression-free survival for gross total resection, resection with adjuvant RTH, and non-radical resection alone was 83%, 68%, and 23%, respectively (p = 0.0006). Surgery combined with RTH provides comparable results to gross tumor resection in terms of oncologic outcome in craniopharyngioma patients. Adjuvant irradiation applied in primary or salvage treatment improves disease control. The rate of complications is high irrespective of improved surgical and radiotherapeutic management.

Keywords: craniopharyngioma; endocrinopathies; long-term complications; pediatric oncology.

Figure 1

Examples of magnetic resonance imaging of treated patients. Case 1 (upper row) Six-year old boy treated with partial surgery. Conventionally fractionated irradiation was applied due to the progression of the size of the tumour. Magnetic resonance imaging (MRI) performed before the surgery (A), 3 days after the surgery (B) and six months later (C). Initial tumour dimensions were 56 × 49 × 39 mm and patient suffered from preoperative visual deficits. After the surgery endocrine deficits were diagnosed. After the irradiation the size of tumour is stable and no complications were present. Currently he is alive in follow-up and increase in body mass index is observed. Case 2 (middle row) Fourteen-year old boy treated with partial surgery and postoperative conventionally fractionated irradiation. Magnetic resonance imaging (MRI) performed before the surgery (D), 2 days after the surgery (E) and three and half months later (F). Initial tumour dimensions were 35 × 30 × 34 mm and patient suffered from preoperative headaches, visual deficits and increase in weight. After the surgery endocrine deficits were diagnosed. After the irradiation the size of tumour is stable and no complications were present. Currently he is alive in follow-up. Case 3 (lower row) Four-year old boy treated with partial surgery. Single fraction (16 Gy) of stereotactic radiotherapy was applied due to the progression of the size of the tumour. Magnetic resonance imaging (MRI) performed before the surgery (G), 7 days after the surgery (H) and one year later (I). Initial tumour dimensions were 23 × 36 × 28 mm and patient suffered from preoperative visual deficits (blindness of left eye) and endocrine deficits. After the surgery endocrine deficits were diagnosed. After the irradiation the size of tumour is stable and no complications were present. Currently he is alive in follow-up.

Figure 2

Progression-free survival and overall survival.