Pediatric symptomatic Rathke cleft cyst compared with cystic craniopharyngioma

Hayashi, Yasuhiko; Kita, Daisuke; Fukui, Issei; Sasagawa, Yasuo; Oishi, Masahiro; Okajima, Michiko; Tachibana, Osamu; Nakada, Mitsutoshi

doi:10.1007/s00381-016-3160-9

Cited by 12 publications

(11 citation statements)

References 30 publications

(58 reference statements)

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“…Several retrospective studies have investigated the clinical presentation of RCCs in a paediatric population, including at least 5% of patients in the transition age. In these patients, as in adults, of non‐endocrine symptoms, headache is the most frequent manifestation with an incidence of 15%‐85%, whereas activity loss, fatigue and anorexia are reported in 13%‐18% of cases, and visual disturbances are reported in 3%‐18%, occurring more rarely than in adults 192,193,197‐205 . Pituitary hormone deficits, involving one or more axes, are common.…”

Section: Rathke Cleft Cystmentioning

confidence: 99%

A clinical approach to parasellar lesions in the transition age

Sbardella

Puliani

Feola

et al. 2021

J Neuroendocrinology

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Many reviews have summarised the pathology and management of the parasellar region in adult patients, although an analysis of these aspects in the transition years, from puberty onset to the age of peak bone mass, has been lacking. A comprehensive search of English‐language original articles, published from 2000 to 2020, was conducted in the MEDLINE database (December 2019 to March 2020). We selected all studies regarding epidemiology, diagnosis and management of the following parasellar lesions: germinoma, craniopharyngioma, Langerhans cell histiocytosis, optic glioma, hypothalamic hamartoma, tuber cinereum hamartoma, cranial chordoma, Rathke cleft cyst, hypophysitis and hypothalamitis during the transition age from childhood to adulthood. In the present review, we provide an overview of the principal parasellar lesions occurring in the transition age. Symptoms are usually a result of the mass effect of the lesions on nearby structures, as well as anterior pituitary deficits. Diabetes insipidus occurs frequently in these patients. In this age group, pubertal developmental disorders may be more evident compared to other stages of life. Parasellar lesions in the transition age mostly include neoplastic lesions such as germinomas, hamartomas, optic gliomas, craniopharyngiomas Langerhans cell histiocytosis and chordomas, and rarely inflammatory lesions (hypophysitis, hypothalamitis). There are limited data on the management of parasellar lesions in the transition age. Endocrine evaluation is crucial for identifying conditions that require hormonal treatment so that they can be treated early to improve the quality of life of the individual patient in this complex age range. The clinical approach to parasellar lesions involves a multidisciplinary effort.

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Section: Rathke Cleft Cystmentioning

confidence: 99%

A clinical approach to parasellar lesions in the transition age

Sbardella

Puliani

Feola

et al. 2021

J Neuroendocrinology

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“…The majority of the surgical cases in the literature have been approached via the transsphenoidal corridor, although 7 patients, described in 4 different series, have undergone a transcranial approach for RCC fenestration. 6,10,12,28 Pooled analysis of all surgically managed cases suggests that preoperative endocrinopathy may improve in 25% of patients, while new or worsening anterior pituitary dysfunction may occur in 27% of cases, with a postoperative DI rate of 27%. 3,6,8,[10][11][12][13][15][16][17]22,26,28 Complete cyst fenestration was achieved in 82% of reported cases in the literature, with an 18% recurrence rate over a 33-month median followup period.…”

Section: Surgical Outcomesmentioning

confidence: 99%

“…9 Unlike craniopharyngiomas, which are much more frequently encountered in the pediatric population, RCCs typically lack contrast enhancement and do not have evidence of calcification. 10,20 As for adults, headaches, hormonal disturbance, and visual loss have been commonly reported for pediatric patients with RCCs. 10,13,28 While the natural history and surgical outcomes of RCCs in adults have been described in larger series, the natural history and optimal management of pediatric RCCs is less clear owing to the rarity with which these lesions have been reported.…”

mentioning

confidence: 99%

“…10,20 As for adults, headaches, hormonal disturbance, and visual loss have been commonly reported for pediatric patients with RCCs. 10,13,28 While the natural history and surgical outcomes of RCCs in adults have been described in larger series, the natural history and optimal management of pediatric RCCs is less clear owing to the rarity with which these lesions have been reported. 1,4,9,10,13,28 Indeed, to date, a total of less than 200 cases of RCC have been described in the literature, and only 60 surgical cases have been described.…”

mentioning

confidence: 99%

“…10,13,28 While the natural history and surgical outcomes of RCCs in adults have been described in larger series, the natural history and optimal management of pediatric RCCs is less clear owing to the rarity with which these lesions have been reported. 1,4,9,10,13,28 Indeed, to date, a total of less than 200 cases of RCC have been described in the literature, and only 60 surgical cases have been described. 3,5,6,8,[10][11][12][13][15][16][17][18][19]22,23,25,26,28 Only 2 prior dedicated surgical series on pediatric RCC have been published, and no study has compared the presentation and outcomes of surgically versus conservatively managed pediatric patients with RCCs.…”

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confidence: 99%

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Presentation and outcomes in surgically and conservatively managed pediatric Rathke cleft cysts

Shepard¹,

Elzoghby

Kiehna

et al. 2018

Journal of Neurosurgery: Pediatrics

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OBJECTIVERathke cleft cysts (RCCs) are sellar lesions that are commonly encountered in adults but infrequently diagnosed in the pediatric population. As a result, the optimal management of pediatric RCCs remains a subject of controversy. Only 2 prior surgical series have been published on pediatric RCCs and no study has compared the presentation and outcomes of surgically versus conservatively managed cases. The authors therefore performed a comparative analysis of pediatric cases of RCC in which patients were treated with surgery or managed in a conservative manner.METHODSAll cases involving pediatric patients diagnosed with an RCC at the University of Virginia between 2000 and 2016 were included in this study. Patient medical records, operative notes, and neuroimaging findings were reviewed. Patients who developed visual field deficits, radiographic evidence of chiasmal compression, or medically refractory headaches were considered candidates for surgical intervention. All patients who were selected for surgery underwent an endoscopic endonasal approach with cyst fenestration.RESULTSA total of 24 pediatric patients were diagnosed with an RCC over a 16-year period. Seven patients ultimately underwent transsphenoidal cyst fenestration, and 17 were managed conservatively. The patients’ age at diagnosis, cyst size, and pituitary function at the time of RCC diagnosis were similar in the conservatively and surgically managed cohorts. At diagnosis, 19 of 24 patients endorsed headaches that led to neuroimaging. All patients in the surgical cohort endorsed severe headaches at diagnosis compared with 71% in the conservative group. For the 7 patients treated with surgery, complete cyst evacuation was achieved in 86% of cases. Transient postoperative endocrinopathy occurred in 4 (57%) of 7 surgically treated individuals and resolved in all cases. In the conservative cohort, 1 patient developed a delayed pituitary-related endocrinopathy. Headache resolution occurred in 5 (71%) of the 7 patients who underwent surgery and 7 (58%) of the 12 who were treated without surgery. Cyst recurrence was documented in 1 individual in the surgical cohort who underwent a subtotal cyst fenestration that ultimately required re-intervention. In the conservative cohort, spontaneous cyst shrinkage occurred in 35% of patients with a median time to regression of 23.5 months.CONCLUSIONSPediatric RCCs are benign sellar lesions that often present with headaches. While cyst fenestration mitigates headaches in most patients, the majority of conservatively managed pediatric patients with RCCs will have spontaneous headache resolution. Furthermore, spontaneous RCC regression occurs in a substantial number of individuals. Thus, in the absence of optic compression, visual field deficit, or diagnostic uncertainty, many pediatric cases of RCC can be managed conservatively.

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The Gist of a Rathke’s Cleft Cyst

McKean

Alter

2021

Diabetes Insipidus in Children

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Pediatric symptomatic Rathke cleft cyst compared with cystic craniopharyngioma

Cited by 12 publications

References 30 publications

A clinical approach to parasellar lesions in the transition age

A clinical approach to parasellar lesions in the transition age

Presentation and outcomes in surgically and conservatively managed pediatric Rathke cleft cysts

The Gist of a Rathke’s Cleft Cyst

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