Endoscopic endonasal transsphenoidal surgery for sellar tumors in children

doi:10.1016/j.ijporl.2010.08.009

International Journal of Pediatric Otorhinolaryngology

Volume 74, Issue 11, November 2010, Pages 1298-1302

https://doi.org/10.1016/j.ijporl.2010.08.009 Get rights and content

Abstract

Objective

Endoscopic endonasal transsphenoidal surgery (EETS) is still rarely used in pediatric subjects compared with adults. Reports on EETS in children appeared only recently in the literature, usually regarding small series. The aim of the study is to assess the actual role and the limits of EETS in children with sellar tumors by reporting a two-centers experience.

Methods

Twenty-seven children (mean age: 12.2 years) were operated on during the last decade. Seventeen patients harbored a sellar mass, 7 a suprasellar tumor, and 3 a clival mass. Laboratory investigations revealed hypopituitarism in 6 children and hormone hypersecretion in 9. All the operations were carried out by a team including both ENT surgeon and neurosurgeon using a dedicated 18-cm long rigid endoscope (2.7 mm and 4 mm diameter) through the direct paraseptal or the transethmoidal or the transpterygoid route.

Results

Overall, 29 operations were performed. Gross total tumor resection was obtained in 22 children (81.5%) while a subtotal and a partial removal in 2 (7.5%) and 3 cases (11%), respectively. Pituitary adenoma was the most common histotype (12 cases), followed by craniopharyngioma (5) and Rathke's cleft cyst (4). No surgical mortality or neurological morbidity or late nasal complications were observed. Postoperative CSF fistula occurred in 3 patients. All children are alive at current follow-up (average: 8.6 years). Preoperative hypopituitarism disappeared or improved in 4 cases and was stable in the remaining 2 (no new hormone deficits appeared).

Conclusion

EETS is a safe and effective surgical option also in children. As for adults, it allows to manage most of the tumor lesions of the sellar region with stable long-term results.

Introduction

In the last two decades, neuroendoscopy underwent an impressive development in terms of techniques and sophisticated surgical tools that currently permits to treat a wide range of diseases. Endoscopic endonasal transsphenoidal surgery (EETS) has become a relatively common surgical procedure to treat the anterior skull base tumors, especially in adult patients. EETS is less utilized in the pediatric population mainly because of the higher prevalence of such tumors in adults (namely pituitary adenomas and meningiomas) and the less favorable anatomical conditions in children (minute nostrils, narrow nasal cavities, reduced/absent pneumatization of the sphenoid sinus). However, some studies concerning EETS in small series of children have recently appeared, showing an effectiveness of the procedure comparable to that of the older counterpart [1], [2], [3], [4].

We describe the results obtained with EETS in the largest series of children with tumors of the sellar and clival region reported so far. The goal of this report is to evaluate whether EETS is a suitable surgical option in pediatric subjects.

Section snippets

Series

The series is composed by 27 pediatric patients (11 boys and 16 girls) operated on at the Neurosurgical Unit of the San Matteo Hospital, Pavia, Italy, and at the Pediatric Neurosurgery of the A. Gemelli Hospital, Rome, Italy, during the last decade. The mean age at surgery was 12.2 years, ranging from 4 to 18 years. Seventeen patients harbored a purely sellar mass, 7 a sellar/supra- and para-sellar tumor, and 3 a clival mass. The conchal variant of the sphenoid sinus was found in one case.

All

Results

Overall, 29 surgical procedures were performed (2 children required a second EETS; see below). The approach was carried out through a direct paraseptal route in 24 cases (bilateral in 18 and monolateral in 6), through a transethmoidal approach in 4 and through a transpterygoid approach in the remaining one. Two children underwent EETS as secondary procedure following a previous transcranial approach.

Based on MRI, a gross total resection of the tumor was obtained in 81.5% of the cases (22

Discussion

In spite of the increasing application of EETS in the pediatric population, only a few studies concerning this subject are currently available in the literature. Most of them consider small pediatric series, alone or as part of mixed cohorts, or series where the young patients are treated by combined approaches (e.g. transnasal and/or transcranial endoscopically assisted surgery) [1], [3], [4], [8], [9]. Among the series of 22 children with craniopharyngioma operated on by transsphenoidal route

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National UK guidelines for the management of paediatric craniopharyngioma
2023, The Lancet Diabetes and Endocrinology
Although rare, craniopharyngiomas constitute up to 80% of tumours in the hypothalamic–pituitary region in childhood. Despite being benign, the close proximity of these tumours to the visual pathways, hypothalamus, and pituitary gland means that both treatment of the tumour and the tumour itself can cause pronounced long-term neuroendocrine morbidity against a background of high overall survival. To date, the optimal management strategy for these tumours remains undefined, with practice varying between centres. In light of these discrepancies, as part of a national endeavour to create evidence-based and consensus-based guidance for the management of rare paediatric endocrine tumours in the UK, we aimed to develop guidelines, which are presented in this Review. These guidelines were developed under the auspices of the UK Children's Cancer and Leukaemia Group and the British Society for Paediatric Endocrinology and Diabetes, with the oversight and endorsement of the Royal College of Paediatrics and Child Health using Appraisal of Guidelines for Research & Evaluation II methodology to standardise care for children and young people with craniopharyngiomas.
Outcomes of Endoscopic Transsphenoidal Approach for More Aggressive Pediatric Pituitary Adenomas: Early- and Late-Term Results from a Single Center Experience
2023, World Neurosurgery
Pediatric pituitary adenomas are rare lesions and account for approximately 3% of all supratentorial tumors in children. There is a paucity of reports on endoscopic transsphenoidal surgery in children. The aim of this study was to assess the early/late outcomes of endoscopic pediatric pituitary adenoma surgery at a high-volume tertiary center, as well as to characterize the factors associated with aggressive growth, including the histopathological features.
Between August 1997 and June 2022, a total of 3256 patients underwent endoscopic transsphenoidal surgery for pituitary adenoma at the Department of Neurosurgery and Pituitary Research Center of the Kocaeli University School of Medicine. Of these, 70 (2.1%) pediatric patients (25 males, 45 females) (age ≤18 years) with a pathological diagnosis of pituitary adenoma were retrospectively reviewed.
The mean age of patients was 15.5 ± 2.3 years. Among the hormone-secreting adenomas, 19 (34.5%) were adrenocorticotrophic hormone secreting, 13 (23.6%) were growth hormone secreting, 19 (34.5%) were prolactin secreting, and 4 (7.2%) were both growth hormone-prolactin secreting. Gross total resection was achieved in 93.3% of nonfunctional tumors. The early/late surgical remission rates for hormone-secreting adenomas were 61.5%/46.1% (mean follow-up: 63.7 ± 49.3 months) for acromegaly, 78.9%/68.4% (47.8 ± 51.0 months) for Cushing disease, 57.8%/31.5% (72.2 ± 59.5 months) for prolactinoma, and 25%/25% (35.2 ± 31.4 months) for growth hormone-prolactin–secreting adenomas. Five sparsely granulated corticotroph tumors, 5 sparsely granulated somatotroph tumors, and 11 densely granulated lactotroph tumors were classified as aggressive histopathological subtypes.
The unique characteristics of the pediatric population and the aggressiveness of the disease in this population pose considerable therapeutic challenges. To increase treatment success, current adjuvant therapies that are appropriate for the morphological and biological characteristics of the tumor are required in addition to surgical treatment.
Combined Neuroendoscopic Techniques in the Management of Pediatric Brain and Skull Base Tumors: A Single-Institutional Case Series
2022, World Neurosurgery
Central nervous system tumors encompass the leading cause of cancer-related death in the pediatric population. Neuroendoscopic techniques have been optimized over the years, becoming an important tool for the management of brain tumors. Our study aims to review the indications for neuroendoscopic procedures and the feasibility of combined interventions.
This is a single-center, self-adjudicated, retrospective experience. Inclusion criteria consisted of pediatric patients (≤18 years old) who underwent management of brain tumor or related diseases with the employment of neuroendoscopy.
A total of 47 patients undergoing 51 procedures met inclusion criteria. The mean age was 9.8 ± 4.6 years, and the majority were female (55.3%). Common indications for endoscopic intervention were hydrocephalus management (n = 24; 16 endoscopic third ventriculostomies and 9 septostomies), tumor biopsy (n = 19), cyst fenestration (n = 16), and tumor resection (n = 9). In one third of the cases, combined interventions occurred during a single operative session. Hydrocephalus was successfully managed in 74.4% of cases; tumor biopsy confirmed the diagnosis in 95.8% of cases, and gross total resection was achieved in 88.9% of cases. Cyst fenestration required reintervention in 3 cases: one case associated with initial cyst enlargement and 2 cases with the development of new tumor cysts separated from the originally fenestrated cyst. The overall complication rate was 6.3%, with only one major safety event, which was successfully managed.
Neuroendoscopy is an important minimally invasive tool for diagnosing and treating pediatric patients with brain tumors, permitting to address multiple problems in a single surgery.
Endoscopic endonasal transsphenoidal approach for pediatric craniopharyngiomas: A case series
2020, International Journal of Pediatric Otorhinolaryngology
Citation Excerpt :
Nonetheless, although the EETA associated with a lesser risk of complications, the incidence of endocrine dysfunction is high in both (transcranial and endonasal) approaches [9]. Therefore, patients subjected to craniopharyngioma resection are later followed by an endocrinologist [10]. To increase the chances for optimal results using the EETA for craniopharyngioma resection, it is important that the anatomy (spatial relationship of the posterior wall of the sphenoid sinus and the anterior/posterior walls of the sella turcica) and pneumatization of the sphenoid sinus are studied preoperatively.
This study aims to analyze our series of pediatric patients who underwent craniopharyngioma resection using the endoscopic endonasal transsphenoidal approach (EETA).
We collected clinical and surgical data from the charts of 20 children who underwent craniopharyngioma removal surgery using the EETA from 2007 to 2017. From the charts, we collected demographic information, results of imaging tests (size and extension of the tumor), and information regarding the surgical procedure and postoperative complications.
From the 20 patients included in this series (12 women and eight men), 17 underwent EETA as a primary procedure, and the remaining three underwent EETA as a secondary procedure due to a relapsing tumor following previous transcranial surgery. The mean age of the patients at the time of the surgical procedure was 7.5 years (range 3–18 years). Regarding their location, 12 tumors were in the sellar and suprasellar regions, three extended into the third ventricle, and five were exclusively intrasellar. We achieved a gross total resection (GTR) of the tumor in 14 patients (70%), subtotal in five (25%), and partial in one (5%). One patient (5%) developed a cerebrospinal fluid fistula after the surgical procedure. In the postoperative follow-up period (mean time = 5.3 years; range = 2–9 years), 11 (55%) patients developed panhypopituitarism, and a relapsing tumor was later found in three (15%) patients. Regarding visual impairment, four patients had visual abnormalities preoperatively (amaurosis, n = 2; bilateral visual acuity decrease, n = 1; bilateral visual field defect, n = 1), and those did not improve or worsened postoperatively. None of the patients who did not have vision problems before the surgery developed those postoperatively.
Our results showed that the EETA is a safe and effective approach for removing craniopharyngiomas in children, as it associated with low operative morbidity and complication rates. Also, our data demonstrated that the EETA may be performed regardless of the size of the nasal cavity, pneumatization of the sphenoid sinuses, and location or extension of the tumors.
Outcome of Endoscopic Endonasal Surgery in Pediatric Craniopharyngiomas
2020, World Neurosurgery
Citation Excerpt :
Similar to those in adults, the main limits of this approach are asymmetric extension of the tumor, the pure intraventricular location, and encasement of the major vessels.2-18 Children present with some particular age-related limitations.18 Specifically, the anatomic features of the nose and paranasal sinuses are an absolute contraindication to treating children younger than 3 years.
In the last years, few reports have shown the feasibility of the endoscopic endonasal approach (EEA) for craniopharyngiomas in pediatric patients. For these tumors, recent studies have suggested less aggressive surgery, favoring the preservation of the patient's quality of life.
The aim of this study was to assess the outcome of the EEA in a large series with specific attention on the long-term functional sequelae.
All consecutive pediatric craniopharyngiomas operated on through this approach since 2000 were included in the study. Preoperative and postoperative operative clinical, radiologic, and pathologic features were retrieved from patient records (mean follow-up, 72 ± 67 months).
The series included 25 patients (12 female; mean age, 8.9 ± 4.1 years). Most of the tumors presented with a supradiaphragmatic extension (88%). Removal was radical in 23 patients (92%). Complications consisted of 6 cerebrospinal fluid leaks (24%). One patient (4%) died of postoperative respiratory complications. Most patients (92%) developed panhypopituitarism and visual disturbances normalized or improved in 6 patients (43%). At follow-up, 9 patients (36%) were overweight/obese (6 were already overweight before surgery). The tumor recurrence rate was 19%.
EEA can be an effective approach for midline craniopharyngiomas in children older than 3 years. It gives a satisfactory exposure of the suprasellar region and an adequate assessment of the brain–tumor interface. Its main limitations are age-related anatomic features of nasal/paranasal sinuses and the risk of cerebrospinal fluid leak.
Surgical outcomes and follow-up results of 53 pediatric craniopharyngioma cases: A single-center study
2024, Journal of Neurosurgery: Pediatrics

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Endoscopic endonasal transsphenoidal surgery for sellar tumors in children

Abstract

Objective

Methods

Results

Conclusion

Introduction

Section snippets

Series

Results

Discussion

Int. J. Pediatr. Otorhinolaryngol.

The role of the endoscopic transsphenoidal approach in pediatric neurosurgery

Childs Nerv. Syst.

The transsphenoidal resection of pediatric craniopharyngiomas: a case series

J. Neurosurg. Pediatr.

Endoscopic approaches to the cranial base: perspectives and realities

Childs Nerv. Syst.

Application of endoscopy to the surgical management of craniopharyngiomas

Childs Nerv. Syst.

The endoscopic diving technique in pituitary and cranial base surgery: technical note

Neurosurgery

Different surgical approaches to the sellar region: focusing on the “two nostrils four hands technique”

Rhinology

Endoscopic approach for the treatment of relapses in cystic craniopharyngiomas

Childs Nerv. Syst.

Variations on the standard transsphenoidal approach to the sellar region, with emphasis on the extended approaches and parasellar approaches: surgical experience in 105 cases

Neurosurgery

Combined endoscopic and microscopic management of pediatric pituitary region tumors through one nostril: technical note with case illustrations

Childs Nerv. Syst.

The role of the endoscope in the transsphenoidal management of cystic lesions of the sellar region

Neurosurg. Rev.

Pediatric sellar tumors: diagnostic procedures and management

Neurosurg. Focus

Long-term results of the surgical treatment of craniopharyngioma: the experience at the Policlinico Gemelli, Catholic University, Rome

Childs Nerv. Syst.

Diagnosis and management of pediatric sellar lesions

Front. Horm. Res.

Transnasal surgery for infradiaphragmatic craniopharyngiomas in pediatric patients

Neurosurgery

Endonasal transsphenoidal approach for pituitary adenomas and other sellar lesions: an assessment of efficacy, safety, and patient impressions

J. Neurosurg.

Surgical management of craniopharyngiomas—experience with a pediatric series

J. Pediatr. Endocrinol. Metab.