Clinical Situation, Therapy, and Follow-Up of Adult Craniopharyngioma

Abstract

Context Craniopharyngioma is a rare neoplastic entity of the central nervous system. Childhood-onset craniopharyngioma is the subject of frequent research whereas the information on adult-onset craniopharyngioma is scarce. Objective The objective of this study was to examine the level of daily impairment in adult patients suffering from craniopharyngioma. Design Noninterventional patient registry indexed as PV4842 with the local ethics committee. Setting The study is set in a hospitalized and ambulatory setting. Patients 148 patients with adult-onset craniopharyngioma were recruited from 8 centers, 22 prospectively and 126 retrospectively. Mean follow-up was 31 months. Interventions No interventions performed. Main Outcome Measures Complications, symptoms, body mass index (BMI), and quality of life (QoL; EORTC QLQ C30 and BN20) were recorded preoperatively and at follow-up. The hypotheses tested were generated after data collection. Results Complications were more frequent after transcranial than transsphenoidal approaches (31 % vs. 11%; P < 0.01). Preoperative obesity was present in 0% papillary and in 38% of all adamantinomatous craniopharyngiomas (P = 0.05), and diabetes insipidus was more frequent for papillary craniopharyngioma (36.8% vs. 16,7%; P < 0.05). Hormone deficits at follow-up were reduced in 16.9%, equal in 31.4%, and increased in 63.6% (P < 0.001). BMI increased from 28.7 ± 7.4 kg/m2 before surgery to 30.2 ± 7.4 kg/m2 at follow-up (P < 0.001). In QoL, a decrease of future uncertainty (62.5 vs. 36.8; P = 0.02) and visual disorders (38.9 vs. 12.0; P = 0.01) were observed in the prospective collective after surgery. Conclusions Adult craniopharyngioma is associated with a complex sociological and psychological burden and hypothalamic dysfunction, warranting further investigation and emphasizing the need for a wider treatment approach.

Coexistence of craniopharyngioma and meningioma

“…Craniopharyngioma usually leads to hormonal disorder mediated by pituitary gland injury. [ 17 – 19 ] It is now well-documented that meningiomas have endogenous and exogenous sex hormonal susceptibility. [ 20 – 22 ] Three patients presented hormonal disorder, and 4 cases showed craniopharyngioma of adamantinomatous type.…”

Diagnosis and Treatment of Parasellar Lesions

“…As mentioned above, CPs are usually low-grade tumors (grade I WHO) burdened by low mortality, but considerable morbidity [14], currently defined into two clinicopathological variants with different phenotypes and distinctive mutations (adamantinomatous and papillary). Adamantinomatous CPs (ACPs) are composed of well-differentiated epithelium, organized in different architectural patterns: cords, lobules, nodular whorls, and irregular trabeculae surrounded by palisading columnar epithelium.…”

Revived Attention for Adult Craniopharyngioma

“…Overall, CP represent 2-5 % of all intracranial tumors [6] with an incidence of 0.5-2 new cases per 1 000 000 people [3] Although craniopharyngioma is rated as a benign neoplasm according to the World Health Organization (WHO) Grade I, the implications for daily living for adults can be substantial [7]. Initial presenting symptoms are highly variable including foremost visual disturbances and headaches but also hormone deficiencies, psychosocial alterations as well as eating disorders and morbid obesity [3,7]. In some cases, even symptoms like gait disturbance, mnestic deficien-cies and incontinence due to tumor mass effect and resulting hydrocephalus are found [8,9].…”

“…Craniopharyngioma (CP) is a rare extra-axial tumor of the sella region thought to originate from remnant cells of Rathke's pouch [1,2], peaking almost evenly divided (40-50 % childhood onset [3]) in children (5-14 years) and adults (40-44 years and within the 6 th decade [4,5]). Overall, CP represent 2-5 % of all intracranial tumors [6] with an incidence of 0.5-2 new cases per 1 000 000 people [3] Although craniopharyngioma is rated as a benign neoplasm according to the World Health Organization (WHO) Grade I, the implications for daily living for adults can be substantial [7]. Initial presenting symptoms are highly variable including foremost visual disturbances and headaches but also hormone deficiencies, psychosocial alterations as well as eating disorders and morbid obesity [3,7].…”

“…Headaches (unspecific or due to compression of the Foramen of Monroi with resulting hydrocephalus) and visual deficits due to suprasellar, infrachiasmatic tumor masses with chiasm compression are commonly observed symptoms leading to diagnosis in the adult population [7,12], other cranial nerve affection, e. g. oculomotor palsy is less frequently observed and occurs only in tumors displacing the cavernous sinus [13].…”