scholarly journals Pediatric Pituitary Adenoma: Case Series, Review of the Literature, and a Skull Base Treatment Paradigm

2018 ◽  
Vol 79 (01) ◽  
pp. 091-114 ◽  
Author(s):  
Avital Perry ◽  
Christopher Graffeo ◽  
Christopher Marcellino ◽  
Bruce Pollock ◽  
Nicholas Wetjen ◽  
...  

Background Pediatric pituitary adenoma is a rare skull base neoplasm, accounting for 3% of all intracranial neoplasms in children and 5% of pituitary adenomas. Compared with pituitary tumors in adults, secreting tumors predominate and longer disease trajectories are expected due to the patient age resulting in a natural history and treatment paradigm that is complex and controversial. Objectives The aims of this study were to describe a large, single-institution series of pediatric pituitary adenomas with extensive long-term follow-up and to conduct a systematic review examining outcomes after pituitary adenoma surgery in the pediatric population. Methods The study cohort was compiled by searching institutional pathology and operative reports using diagnosis and site codes for pituitary and sellar pathology, from 1956 to 2016. Systematic review of the English language literature since 1970 was conducted using PubMed, MEDLINE, Embase, and Google Scholar. Results Thirty-nine surgically managed pediatric pituitary adenomas were identified, including 15 prolactinomas, 14 corticotrophs, 7 somatotrophs, and 4 non-secreting adenomas. All patients underwent transsphenoidal resection (TSR) as the initial surgical treatment. Surgical cure was achieved in 18 (46%); 21 experienced recurrent/persistent disease, with secondary treatments including repeat surgery in 10, radiation in 14, adjuvant pharmacotherapy in 11, and bilateral adrenalectomy in 3. At the last follow-up (median 87 months, range 3–581), nine remained with recurrent/persistent disease (23%).Thirty-seven publications reporting surgical series of pediatric pituitary adenomas were included, containing 1,284 patients. Adrenocorticotropic hormone (ACTH)-secreting tumors were most prevalent (43%), followed by prolactin (PRL)-secreting (37%), growth hormone (GH)-secreting (12%), and nonsecreting (7%). Surgical cure was reported in 65%. Complications included pituitary insufficiency (23%), permanent visual dysfunction (6%), chronic diabetes insipidus (DI) (3%), and postoperative cerebrospinal fluid (CSF) leak (4%). Mean follow-up was 63 months (range 0–240), with recurrent/persistent disease reported in 18% at the time of last follow-up. Conclusion Pediatric pituitary adenomas are diverse and challenging tumors with complexities far beyond those encountered in the management of routine adult pituitary disease, including nuanced decision-making, a technically demanding operative environment, high propensity for recurrence, and the potentially serious consequences of hypopituitarism with respect to fertility and growth potential in a pediatric population. Optimal treatment requires a high degree of individualization, and patients are most likely to benefit from consolidated, multidisciplinary care in highly experienced centers.

Author(s):  
Kent Tadokoro ◽  
Colten Wolf ◽  
Joseph Toth ◽  
Cara Joyce ◽  
Meharvan Singh ◽  
...  

Abstract Objectives Ki-67/MIB-1 is a marker of cellular proliferation used as a pathological parameter in the clinical assessment of pituitary adenomas, where its expression has shown utility in predicting the invasiveness of these tumors. However, studies have shown variable results when using Ki-67/MIB-1 association with recurrence. The purpose of this study is to determine if a high Ki-67/MIB-1 labeling index (LI) is predictive of recurrence in pituitary adenomas. Methods A retrospective chart review was performed for patients undergoing pituitary adenoma resection with at least 1 year of follow-up. Additionally, systematic data searches were performed and included studies that correlated recurrence rate to Ki-67/MIB-1 LI. Our institutional data were included in a synthesis with previously published data. Results Our institutional review included 79 patients with a recurrence rate of 26.6%. We found that 8.8% of our patients had a high Ki-67/MIB-1 LI (>3%); however, high Ki-67/MIB-1 was not associated with recurrence. The systematic review identified 244 articles and 49 full-text articles that were assessed for eligibility. Quantitative analysis was performed on 30 articles including our institutional data and 18 studies reported recurrence by level of Ki-67/MIB-1 LI. Among studies that compared Ki-67/MIB-1 ≥3 vs. <3%, 10 studies reported odds ratios (OR) greater than 1 of which 6 were statistically significant. A high Ki-67/MIB-1 had higher odds of recurrence via the pooled odds ratio (OR = 4.15, 95% confidence interval [CI]: 2.31–7.42). Conclusion This systematic review suggests that a high Ki-67/MIB-1 should prompt an increased duration of follow-up due to the higher odds of recurrence of pituitary adenoma.


2013 ◽  
Vol 11 (3) ◽  
pp. 227-241 ◽  
Author(s):  
Srinivas Chivukula ◽  
Maria Koutourousiou ◽  
Carl H. Snyderman ◽  
Juan C. Fernandez-Miranda ◽  
Paul A. Gardner ◽  
...  

Object The use of endoscopic endonasal surgery (EES) for skull base pathologies in the pediatric population presents unique challenges and has not been well described. The authors reviewed their experience with endoscopic endonasal approaches in pediatric skull base surgery to assess surgical outcomes and complications in the context of presenting patient demographics and pathologies. Methods A retrospective review of 133 pediatric patients who underwent EES at our institution from July 1999 to May 2011 was performed. Results A total of 171 EESs were performed for skull base tumors in 112 patients and bony lesions in 21. Eighty-five patients (63.9%) were male, and the mean age at the time of surgery was 12.7 years (range 2.3–18.0 years). Skull base tumors included angiofibromas (n = 24), craniopharyngiomas (n = 16), Rathke cleft cysts (n = 12), pituitary adenomas (n = 11), chordomas/chondrosarcomas (n = 10), dermoid/epidermoid tumors (n = 9), and 30 other pathologies. In total, 19 tumors were malignant (17.0%). Among patients with follow-up data, gross-total resection was achieved in 16 cases of angiofibromas (76.2%), 9 of craniopharyngiomas (56.2%), 8 of Rathke cleft cysts (72.7%), 7 of pituitary adenomas (70%), 5 of chordomas/chondrosarcomas (50%), 6 of dermoid/epidermoid tumors (85.7%), and 9 cases of other pathologies (31%). Fourteen patients received adjuvant radiotherapy, and 5 received chemotherapy. Sixteen patients (15.4%) showed tumor recurrence and underwent reoperation. Bony abnormalities included skull base defects (n = 12), basilar invagination (n = 4), optic nerve compression (n = 3) and trauma (n = 2); preexisting neurological dysfunction resolved in 12 patients (57.1%), improved in 7 (33.3%), and remained unchanged in 2 (9.5%). Overall, complications included CSF leak in 14 cases (10.5%), meningitis in 5 (3.8%), transient diabetes insipidus in 8 patients (6.0%), and permanent diabetes insipidus in 12 (9.0%). Five patients (3.8%) had transient and 3 (2.3%) had permanent cranial nerve palsies. The mean follow-up time was 22.7 months (range 1–122 months); 5 patients were lost to follow-up. Conclusions Endoscopic endonasal surgery has proved to be a safe and feasible approach for the management of a variety of pediatric skull base pathologies. When appropriately indicated, EES may achieve optimal outcomes in the pediatric population.


2013 ◽  
Vol 3 (1) ◽  
pp. 18-23
Author(s):  
Shamsul Alam ◽  
ATM Mosharef Hossain ◽  
AN Wakil Uddin ◽  
Tariqul Islam ◽  
Rezaul Amin ◽  
...  

Background: The use of endoscope for the management of pituitary adenoma is not new. The better magnification and illumination provided by the endoscope gives better outcome than microscopic pituitary surgery. Objective: To find out the benefits of endoscope in relation to microscopic surgery. Materials and Methods: We performed 45 cases of pituitary adenoma surgery by endoscopic endonasal approach from July 2008 to July 2010. Results: Forty five cases underwent endoscopic transsphenoidal approach. Gross total removal was done in 35 cases and subtotal removal was done in 10 cases. Residual tumours were seen in 10 cases (22%) in postoperative follow-up MRI scan. Visual improvement was satisfactory, and hormonal improvement of functional adenoma was nice. Postoperative visual acuity and visual field were improved in 75% cases. There were 37% cases of temporary diabetes insipidus and about 4.5% cases of permanent diabetes insipidus. The average duration of follow-up was 20 months. One patient required reexploration to correct visual deterioration in the immediate postoperative period. There were 4.5% cases of CSF leak and 6.6% mortality. Mortality was due to electrolyte imbalance and improper management of infection and hydrocephalus. Conclusion: Endoscopic endonasal pituitary surgery now has become a gold standard surgery for most of the pituitary adenomas because of its better advantages in relation to microscopic surgery and less complications and less hospital stay. DOI: http://dx.doi.org/10.3329/jemc.v3i1.13869 J Enam Med Col 2013; 3(1): 18-23


Author(s):  
Emma M. H. Slot ◽  
Kirsten M. van Baarsen ◽  
Eelco W. Hoving ◽  
Nicolaas P. A. Zuithoff ◽  
Tristan P. C van Doormaal

Abstract Background Cerebrospinal fluid (CSF) leakage is a common complication after neurosurgical intervention. It is associated with substantial morbidity and increased healthcare costs. The current systematic review and meta-analysis aim to quantify the incidence of cerebrospinal fluid leakage in the pediatric population and identify its risk factors. Methods The authors followed the PRISMA guidelines. The Embase, PubMed, and Cochrane database were searched for studies reporting CSF leakage after intradural cranial surgery in patients up to 18 years old. Meta-analysis of incidences was performed using a generalized linear mixed model. Results Twenty-six articles were included in this systematic review. Data were retrieved of 2929 patients who underwent a total of 3034 intradural cranial surgeries. Surprisingly, only four of the included articles reported their definition of CSF leakage. The overall CSF leakage rate was 4.4% (95% CI 2.6 to 7.3%). The odds of CSF leakage were significantly greater for craniectomy as opposed to craniotomy (OR 4.7, 95% CI 1.7 to 13.4) and infratentorial as opposed to supratentorial surgery (OR 5.9, 95% CI 1.7 to 20.6). The odds of CSF leakage were significantly lower for duraplasty use versus no duraplasty (OR 0.41 95% CI 0.2 to 0.9). Conclusion The overall CSF leakage rate after intradural cranial surgery in the pediatric population is 4.4%. Risk factors are craniectomy and infratentorial surgery. Duraplasty use is negatively associated with CSF leak. We suggest defining a CSF leak as “leakage of CSF through the skin,” as an unambiguous definition is fundamental for future research.


2021 ◽  
Vol 108 (Supplement_2) ◽  
Author(s):  
B Kewlani ◽  
I Hussain ◽  
J Greenfield

Abstract The hallmark symptom of spontaneous intracranial hypotension (SIH) is orthostatic headaches which manifests secondary to cerebrospinal fluid (CSF) hypovolaemia. Well-recognised aetiologies include trauma which includes procedures such as lumbar punctures and spinal surgery. More recently, structural defects such as bony osteophytes and calcified or herniated discs have been attributed to mechanically compromising dural integrity consequently resulting in CSF leak and symptom manifestation. A thorough literature review noted only a handful of such cases. We report the case of a thirty-two-year-old Asian female who presented with a one-month history of new-onset progressively worsening orthostatic headaches. Workup included MRI of the thoracic spine which revealed an epidural collection of CSF consequently prompting a dynamic CT-myelogram of the spine which not only helped to confirm severe cerebral hypotension but also suggested the underlying cause as being a dorsally projecting osteophyte-complex at level T2-3. Conservative and medical management including bed rest, analgesia, mechanical compression, and epidural blood patches failed to alleviate symptoms and a permanent surgical cure was eventually sought. The surgery involved T2-T3 laminectomy and osteophytectomy and at a 3-month follow-up, complete resolution of symptoms was noted.


2019 ◽  
Vol 24 (3) ◽  
pp. 246-257 ◽  
Author(s):  
Jennifer L. Quon ◽  
Lily H. Kim ◽  
Peter H. Hwang ◽  
Zara M. Patel ◽  
Gerald A. Grant ◽  
...  

OBJECTIVETransnasal endoscopic transsphenoidal approaches constitute an essential technique for the resection of skull base tumors in adults. However, in the pediatric population, sellar and suprasellar lesions have historically been treated by craniotomy. Transnasal endoscopic approaches are less invasive and thus may be preferable to craniotomy, especially in children. In this case series, the authors present their institutional experience with transnasal endoscopic transsphenoidal approaches for pediatric skull base tumors.METHODSThe authors retrospectively reviewed pediatric patients (age ≤ 18 years) who had undergone transnasal endoscopic transsphenoidal approaches for either biopsy or resection of sellar or suprasellar lesions between 2007 and 2016. All operations were performed jointly by a team of pediatric neurosurgeons and skull base otolaryngologists, except for 8 cases performed by one neurosurgeon.RESULTSThe series included 42 patients between 4 and 18 years old (average 12.5 years) who underwent 51 operations. Headache (45%), visual symptoms (69%), and symptoms related to hormonal abnormalities (71%) were the predominant presenting symptoms. Improvement in preoperative symptoms was seen in 92% of cases. Most patients had craniopharyngiomas (n = 16), followed by pituitary adenomas (n = 12), Rathke cleft cysts (n = 4), germinomas (n = 4), chordomas (n = 2), and other lesion subtypes (n = 4). Lesions ranged from 0.3 to 6.2 cm (median 2.5 cm) in their greatest dimension. Gross-total resection was primarily performed (63% of cases), with 5 subsequent recurrences. Nasoseptal flaps were used in 47% of cases, fat grafts in 37%, and lumbar drains in 47%. CSF space was entered intraoperatively in 15 cases, and postoperative CSF was observed only in lesions with suprasellar extension. There were 8 cases of new hormonal deficits and 3 cases of new cranial nerve deficits. Length of hospital stay ranged from 1 to 61 days (median 5 days). Patients were clinically followed up for a median of 46 months (range 1–120 months), accompanied by a median radiological follow-up period of 45 months (range 3.8–120 months). Most patients (76%) were offered adjuvant therapy.CONCLUSIONSIn this single-institution report of the transnasal endoscopic transsphenoidal approach, the authors demonstrated that this technique is generally safe and effective for different types of pediatric skull base lesions. Favorable effects of surgery were sustained during a follow-up period of 4 years. Further refinement in technology will allow for more widespread use in the pediatric population.


2019 ◽  
Vol 130 (3) ◽  
pp. 861-875 ◽  
Author(s):  
Andrew Conger ◽  
Fan Zhao ◽  
Xiaowen Wang ◽  
Amalia Eisenberg ◽  
Chester Griffiths ◽  
...  

OBJECTIVEThe authors previously described a graded approach to skull base repair following endonasal microscopic or endoscope-assisted tumor surgery. In this paper they review their experience with skull base reconstruction in the endoscopic era.METHODSA retrospective review of a single-institution endonasal endoscopic patient database (April 2010–April 2017) was undertaken. Intraoperative CSF leaks were graded based on size (grade 0 [no leak], 1, 2, or 3), and repair technique was documented across grades. The series was divided into 2 epochs based on implementation of a strict perioperative antibiotic protocol and more liberal use of permanent and/or temporary buttresses; repair failure rates and postoperative meningitis rates were assessed for the 2 epochs and compared.RESULTSIn total, 551 operations were performed in 509 patients for parasellar pathology, including pituitary adenoma (66%), Rathke’s cleft cyst (7%), meningioma (6%), craniopharyngioma (4%), and other (17%). Extended approaches were used in 41% of cases. There were 9 postoperative CSF leaks (1.6%) and 6 cases of meningitis (1.1%). Postoperative leak rates for all 551 operations by grade 0, 1, 2, and 3 were 0%, 1.9%, 3.1%, and 4.8%, respectively. Fat grafts were used in 33%, 84%, 97%, and 100% of grade 0, 1, 2, and 3 leaks, respectively. Pedicled mucosal flaps (78 total) were used in 2.6% of grade 0–2 leaks (combined) and 79.5% of grade 3 leaks (60 nasoseptal and 6 middle turbinate flaps). Nasoseptal flap usage was highest for craniopharyngioma operations (80%) and lowest for pituitary adenoma operations (2%). Two (3%) nasoseptal flaps failed. Contributing factors for the 9 repair failures were BMI ≥ 30 (7/9), lack of buttress (4/9), grade 3 leak (4/9), and postoperative vomiting (4/9). Comparison of the epochs showed that grade 1–3 repair failures decreased from 6/143 (4.1%) to 3/141 (2.1%) and grade 1–3 meningitis rates decreased from 5 (3.5%) to 1 (0.7%) (p = 0.08). Prophylactic lumbar CSF drainage was used in only 4 cases (< 1%), was associated with a higher meningitis rate in grades 1–3 (25% vs 2%), and was discontinued in 2012. Comparison of the 2 epochs showed increase buttress use in the second, with use of a permanent buttress in grade 1 and 3 leaks increasing from 13% to 55% and 32% to 76%, respectively (p < 0.001), and use of autologous septal/keel bone as a permanent buttress in grade 1, 2, and 3 leaks increasing from 15% to 51% (p < 0.001).CONCLUSIONSA graded approach to skull base repair after endonasal surgery remains valid in the endoscopic era. However, the technique has evolved significantly, with further reduction of postoperative CSF leak rates. These data suggest that buttresses are beneficial for repair of most grade 1 and 2 leaks and all grade 3 leaks. Similarly, pedicled flaps appear advantageous for grade 3 leaks, while CSF diversion may be unnecessary and a risk factor for meningitis. High BMI should prompt an aggressive multilayered repair strategy. Achieving repair failure and meningitis rates lower than 1% is a reasonable goal in endoscopic skull base tumor surgery.


Author(s):  
Erin Mamuyac Lopez ◽  
Zainab Farzal ◽  
Kelly Marie Dean ◽  
Craig Miller ◽  
Justin Cates Morse ◽  
...  

Objectives: The frequency of endoscopic skull base surgery in pediatric patients is increasing. This study’s aim is to systematically review the literature for endoscopic skull base surgery outcomes in children/adolescents ages 0-18 years. Design: A systematic review of the literature was performed in PubMed and SCOPUS databases querying studies from 2000-2020 using PRISMA guidelines. Final inclusion criteria included: case series with 10+ patients with pediatric patients age ≤18 years, endoscopic or endoscopic-assisted skull base surgery, and outcomes reported. Setting: Tertiary care medical center Participants: Children/adolescents ages 0-18 years who underwent endoscopic skull base surgery Main Outcome Measures: Patient demographics, pathology, reconstructive technique, intra-operative findings, intra-operative and post-operative surgical complications. Results: Systematic literature search yielded 287 publications. Of these, 12 studies discussing a total of 399 patients age 0-18 years met inclusion criteria for final analysis. 7 of 12 studies discussed a single pathology. The most common pathology was a skull base defect causing CSF leak. The majority of skull base repairs were made with free tissue grafts. The most common post-operative complication was CSF leak (n=40). Twelve cases of meningitis occurred post-operatively with two of these episodes resulting in death. Conclusions: Endoscopic skull base surgery has been performed recently in the pediatric population in a variety of disease states. Inconsistent individual-level data and reporting standards are present in existing studies posing challenges for comparative analysis. Standardized reporting will aid future reviews and meta-analysis for rare skull base pathology.


2012 ◽  
Vol 32 (6) ◽  
pp. E2 ◽  
Author(s):  
Grace Lam ◽  
Vivek Mehta ◽  
Gabriel Zada

Object Spontaneous and medically induced CSF leaks are uncommonly associated with pituitary adenomas. The aim of this study was to further characterize the clinical scenarios most closely associated with this phenomenon. Methods A review of the literature was conducted to identify all cases of nonsurgical CSF leaks associated with pituitary adenomas. The authors aimed to identify associated risk factors and management strategies used to treat these tumors and repair the CSF leak site. Results Fifty-two patients with spontaneous or medically induced CSF leaks in the setting of a pituitary adenoma were identified from 29 articles published from 1980 through 2011. In 38 patients (73%), CSF rhinorrhea developed following initiation of medical therapy, whereas spontaneous CSF leakage developed as the presenting symptom in 14 patients (27%). The patients' mean age was 42.8 years (range 23–68 years). There were 35 males and 17 females. Forty-two patients (81%) had a prolactinoma, with the remaining patients having the following tumors: nonfunctioning pituitary adenoma (6 patients), growth hormone–secreting adenoma (2 patients), mammosomatotroph cell adenoma (1 patient), and ACTH-secreting adenoma (1 patient). Infrasellar tumor invasion into the paranasal sinuses was specifically reported in 56% of patients. The medical agents associated with CSF leakage were dopamine agonists (97%) and somatostatin analogs (3%). The average time from initialization of medical treatment to onset of rhinorrhea was 3.3 months (range 3 days–17 months). Nonsurgical management was successful in 4 patients, including 1 who had successful resolution with a temporary lumbar drain. Forty-six patients (88%) underwent surgical intervention to treat the CSF leak and/or resect the tumor. In 2 patients, surgery was not performed due to medical contraindications or patient preference. Conclusions Nonsurgical development of CSF rhinorrhea may occur in the setting of pituitary adenomas, especially following favorable response of invasive prolactinomas to initiation of dopamine agonist therapy. Additional cases have been reported as the presenting symptom of a pituitary adenoma and are likely to be related to decreased tumor volume due to intrinsic infarction or hemorrhage, ongoing invasion, and/or increases in intracranial pressure. Surgical repair, preferentially via a transsphenoidal approach, is the recommended initial treatment for definitive repair of the CSF leak and achievement of maximal tumor resection.


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