Clinical outcome in children with craniopharyngioma treated with primary surgery and radiotherapy deferred until relapse

Kalapurakal, John A.; Goldman, Stewart; Hsieh, Y. C.; Tomita, Tadanori; Marymont, Maryanne Hoffman

doi:10.1002/mpo.10247

Cited by 119 publications

(91 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…New hypothalamic dysfunction occurred in 35% of children and obesity in 15%; both of these rates are comparable to reported incidence rates for tumors of all sizes. 14,[40][41][42]49,63,80,84,88,95 No patients developed severe obesity (> +3 SDs). These results contrast greatly with the results published by Muller et al 56 reporting severe obesity in 44% of 185 children with a concomitant decrease in quality of life.…”

Section: Surgical Success and Functional Outcomementioning

confidence: 99%

Surgical management of giant pediatric craniopharyngiomas

Elliott¹,

Wisoff

2010

PED

View full text Add to dashboard Cite

Object Prior work by the authors' group and reports of other authors suggest worse functional outcomes and decreased survival in children with larger craniopharyngiomas. The purpose of this study was to assess the oncological, endocrinological, and functional outcomes in children who underwent radical resection of giant craniopharyngiomas (defined as 5 cm or greater in largest diameter). Methods Between 1986 and 2006, 26 children under the age of 18 (14 boys, 12 girls; mean age 10.5 years) underwent radical resection of giant craniopharyngiomas performed by the senior author. Data were retrospectively collected to assess the outcome of surgical treatment. Results Twenty (77%) of 26 patients underwent gross-total resection (GTR) confirmed by intraoperative inspection and postoperative imaging. All primary tumors (17 of 17) and 3 (33%) of 9 recurrent tumors were treated with GTR. There was no operative mortality, and 18 of 26 patients (69%) were alive at a mean follow-up of 8.9 years (median 9.3 years). Disease control was achieved in 21 (84%) of the 25 patients followed up for more than 6 months and was more successful in patients who underwent GTR (95%) than in those who underwent STR (50%, p = 0.03). New-onset diabetes insipidus (DI) occurred in 63.2% of patients (73% of patients had DI postoperatively). New or worsened deficits in visual acuity and visual fields occurred in 16% and 28%, respectively, of the 25 patients for whom postoperative visual data were available. Five patients (19%) experienced significant, permanent neurological deficits, and 5 (19%) had mild to moderate deficits. New or worsened hypothalamic disturbance occurred in 35% and 22% of patients, respectively, but obesity developed in only 15%. Conclusions In this retrospective series, radical resection of giant craniopharyngiomas in children was found to lead to excellent rates of disease control with acceptable or good functional outcomes but slightly higher rates of neurological complications compared with rates in patients with smaller tumors. Radical resection is less successful in recurrent tumors that reach very large sizes, especially previously irradiated tumors, with resultant diminished survival.

show abstract

Section: Surgical Success and Functional Outcomementioning

confidence: 99%

Surgical management of giant pediatric craniopharyngiomas

Elliott¹,

Wisoff

2010

PED

View full text Add to dashboard Cite

show abstract

“…Despite a benign histology, their management presents an ongoing challenge given lesional proximity to critical structures. Disease progression or surgical intervention can result in neuro-endocrinological symptoms, visual disturbances, and obstructive hydrocephalus, which impacts on quality of life [9,13,22]. The apparent futility of chemotherapy [10], and concerns regarding adverse sequelae from adjuvant radiotherapy, particularly for the developing brain, compound the dilemma [14,20].…”

Section: Introductionmentioning

confidence: 99%

Papillary craniopharyngioma in a 4-year-old girl with BRAF V600E mutation: a case report and review of the literature

et al. 2018

View full text Add to dashboard Cite

Introduction Craniopharyngiomas are one of the most frequently diagnosed hypothalamo-pituitary tumors in childhood. The adamantinomatous histological subtype accounts for most pediatric cases, while the papillary variant is almost exclusively diagnosed in adults. Here, we report a case of papillary craniopharyngioma in a very young child, confirmed by molecular tissue analysis. Case report A 4-year-old girl was being investigated for symptomatic central hypothyroidism. Brain MR imaging revealed a large solid/cystic suprasellar mass, splaying the optic chiasm and measuring 3 × 1.9 × 2.3 cm. The patient underwent a transsphenoidal near total resection of the lesion, which was encased within a tumor capsule. Post-operatively, the patient developed transient diabetes insipidus but otherwise recovered well. The pathology of the lesion was consistent with a papillary craniopharyngioma with regions of stratified squamous epithelium accompanied by superficial goblet cells and ciliated cells. Subsequent next-generation sequencing analysis of the lesion confirmed the presence of a BRAF V600E mutation (BRAFc.1799T>A p. (Val600Glu). To date, she remains free from progression 1 year following surgery. Conclusion This is the youngest case published to date of papillary craniopharyngioma with a confirmed BRAF V600E mutation. The case encourages discussion about the most appropriate adjuvant therapy for tumor progression in such cases, given the risks of radiotherapy to the developing brain and the increasing availability of oral BRAF inhibitor therapy.

show abstract

“…However, the surgical outcomes for large craniopharyngiomas remain controversial. 1,6,10,11,[15][16][17]19,[22][23][24][27][28][29]31,[34][35][36][37][38][39][40][41][42]44,45) Furthermore, most reports on craniopharyngioma have focused primarily on children 10,[15][16][17]22,27,41,42) or mixed series, 1,19,23,24,31,34,36,[38][39][40]45) and studies of only adults are limited. 6) The postoperative endocrinological state of patients treated for craniopharyngioma is critical to the quality of life.…”

Section: Introductionmentioning

confidence: 99%

Spontaneous Resolution of Diabetes Insipidus After Pituitary Stalk Sectioning During Surgery for Large Craniopharyngioma-Endocrinological Evaluation and Clinical Implications for Surgical Strategy-

Nishizawa

Ohta

Osuga

2006

Neurol. Med. Chir.(Tokyo)

View full text Add to dashboard Cite

The mechanism of spontaneous resolution of diabetes insipidus (DI) was investigated after surgery for large craniopharyngioma. Twenty-two adult patients (mean age 48.9 years old), who underwent surgery via the anterior interhemispheric trans-lamina terminalis approach, were divided into three groups: Group I, the entire pituitary stalk was preserved (n ＝ 2); Group II, the stalk was dissected distally from the tumor but ultimately sacrificed (n ＝ 9); Group III, the stalk was not identified and was sacrificed (n ＝ 11). All patients were discharged without neurological deficits 1 month after surgery. Four patients underwent gamma-knife treatment for residual tumor or recurrence. Postoperative endocrinological functions were normal in Group I, and no replacement therapy was required. Hormonal replacement for pan-hypopituitarism and DI was necessary in Groups II and III (mean follow-up period 5.9 years). DI resolved at 2.7 ± 1.3 years after surgery in four patients in Group II, and a hypertonic saline infusion test revealed production of small amounts of intrinsic antidiuretic hormone (ADH). Urine osmolarity was high in the morning, and a significant increase in urinary osmolarity was noted after Pitressin injection. These results indicate induction of hypersensitivity of the distal renal tubules to small amounts of intrinsic ADH, resulting in decreased urinary output. Recovery from DI can be expected, despite permanent impairment of anterior pituitary function, if the pituitary stalk is dissected as distally as possible.

show abstract

Clinical outcome in children with craniopharyngioma treated with primary surgery and radiotherapy deferred until relapse

Cited by 119 publications

References 20 publications

Surgical management of giant pediatric craniopharyngiomas

Surgical management of giant pediatric craniopharyngiomas

Papillary craniopharyngioma in a 4-year-old girl with BRAF V600E mutation: a case report and review of the literature

Spontaneous Resolution of Diabetes Insipidus After Pituitary Stalk Sectioning During Surgery for Large Craniopharyngioma-Endocrinological Evaluation and Clinical Implications for Surgical Strategy-

Contact Info

Product

Resources

About