Elsevier

World Neurosurgery

Volume 83, Issue 4, April 2015, Pages 644-651
World Neurosurgery

Peer-Review Report
Pituitary Tumor Apoplexy in Adolescents

https://doi.org/10.1016/j.wneu.2014.12.026Get rights and content

Objective

The aim of this study was to determine whether there are differences in pituitary apoplexy and subclinical apoplexy secondary to adenoma hemorrhage in the adolescent population with regard to symptomatology, neuroimaging features, pathology, and outcomes compared with adults.

Methods

A retrospective series of 9 consecutive patients with a diagnosis of pituitary hemorrhage who were surgically treated at Rady's Children's Hospital San Diego, between 2008 and 2013 were evaluated for clinical, endocrine, neuroradiographic, and pathologic features in association with clinical outcomes.

Results

Nine patients (6 girls, age 14−21 years) presented to our institution with headache (9/9), nausea (3/9), dizziness (4/9), and visual disturbances (6/9) in the setting of a sellar hemorrhagic tumor on magnetic resonance imaging (MRI). Three patients presented with apoplexy and 6 with subclinical apoplexy. Duration of symptoms ranged from 3 days to 1 year. MRI revealed hemorrhage (9/9), rim enhancement (6/9), sphenoid sinus mucosal thickening (2/9), mass effect on the optic chiasm (8/9), and sellar remodeling (9/9). The percentage of hemorrhage preoperatively observed on MRI ranged from 50% to greater than 95%. On presentation, hyperprolactinemia was recorded in 7 patients, 6 of whom had galactorrhea and/or amenorrhea. Open transsphenoidal decompression was performed in 8/9 patients; 7 of 9 were diagnosed with prolactinoma. Biopsy specimens revealed 10%−90% hemorrhage and no infarction in any of the cases. All patients treated showed improvement of symptoms after surgery (average follow-up, 28.2 months). Postoperative complications included transient diabetes insipidus (n = 5), persistent cerebrospinal fluid rhinorrhea (n = 3), and meningitis (n = 1). Five patients had long-term endocrine sequelae of hyperprolactinemia requiring ongoing medical treatment.

Conclusions

Pituitary hemorrhage resulting in apoplexy or subclinical apoplexy in adolescents may represent a distinct entity with a more indolent symptomatology and more favorable neurologic and endocrine outcome compared with adults that is worthy of further validation in a multi-institutional cohort.

Introduction

Pituitary apoplexy can occur as the result of infarction, hemorrhage, or a combination of hemorrhagic infarction in a pituitary tumor. It is a clinical syndrome identified by a rapid onset of signs and symptoms caused by either hemorrhage or infarction of a pituitary tumor 4, 16, 32. Contributing to pituitary apoplexy is relatively rapid enlargement of a pituitary tumor resulting in compression of surrounding structures, potentially causing pituitary insufficiency 2, 4, 5, 6, 7, 9. Potential predisposing factors that have been documented in the literature include coagulation disorder, dopamine agonist therapy, coronary artery bypass surgery, anticoagulant therapy, radiotherapy, head trauma, diabetes mellitus, pregnancy, hypophyseal dynamic testing, carotid angiography, or upper respiratory tract infections (33). Some authors prefer to distinguish pituitary apoplexy from subclinical presentations, where magnetic resonance imaging (MRI) indicates isolated areas of hemorrhage or infarction that is later confirmed intraoperatively and on pathologic analysis 7, 13, 14, 22, 33. Therefore, the incidence of this clinical syndrome varies depending on definitions used by the reporting authors. In the adult population subclinical hemorrhage has been reported in 14%−22% of pituitary macroadenomas (31). However, symptomatic pituitary apoplexy is seldom seen, occurring in 0.6%−9% of the population 16, 31.

The overwhelming majority of data and information for this clinical presentation comes from the adult literature. Patients typically experience a rapid onset of symptoms that varies from headache with or without endocrine dysfunction, visual deficits, ophthalmoplegia, altered mental status, or coma 4, 20, 28, 31, 34. Literature regarding pituitary apoplexy in the pediatric or adolescent population is restricted to case reports or individual cases that are part of a larger series discussing pituitary adenomas in this population 18, 19, 21, 23. There is no reported patient series dedicated to pituitary apoplexy or subclinical apoplexy in the pediatric or adolescent population after our review of the literature. Therefore, there remain gaps in our knowledge of the differences between adults and children in the presentation, severity of symptoms, and outcomes of this disease between the 2 groups. In this study, from a single pediatric neurosurgical center, we review the clinical presentation, radiologic features, histopathology, and outcomes of 9 adolescent patients treated for pituitary apoplexy and subclinical apoplexy secondary to hemorrhage during a 6-year period.

Section snippets

Clinical Materials and Methods

The retrospective series includes patients with pituitary mass hemorrhage who were treated at Rady Children's Hospital San Diego (RCHSD), California, by a single neurosurgeon between 2008 and 2013. Chart reviews were performed in accordance to University of California San Diego and RCHSD Human Research Protections Program guidelines. The diagnosis of pituitary apoplexy or subclinical apoplexy was determined from the clinical presentation and neuroimaging features. Patient with clinical (acute)

Presenting Symptoms

All patients presented with a Glasgow Coma Scale of 15. Duration of symptoms ranged from 3 days to 1 year in our group. Time to presentation to RCHSD neurosurgery department ranged from 1 to 39 days. Headache was the most common symptom present in 9 patients (100%), followed by visual impairment seen in 6 patients (66%). Four patients (44%) had dizziness, and 3 had nausea (33%) (Table 1). No patients in the study presented with nonfunctional vision or blindness. From the 6 patients with

Discussion

Although pituitary tumors are common intracranial neoplasms in the adult population constituting 8%−12% of all intracranial neoplasms 12, 23, 27, 36, they are uncommon in children. In the largest series of pituitary adenomas in patients younger than 20 years of age, Mindermann and Wilson (19) reported an incidence of 6.1% from all intracranial neoplasms. Brougham et al. (3), in 1950, were the first to establish the diagnosis of pituitary apoplexy in the literature backed by pathologic

Conclusions

Pituitary apoplexy remains a clinical condition that has the potential for serious neurologic and endocrine symptoms with the possibility of disabling sequalae. The spectrum of this condition can be quite heterogeneous. Subclinical apoplexy is a well known clinical entity in adults and based on our results manifests as a similar phenotype in adolescents. On the basis of our results, it is possible that this age group is less susceptible to tumor infarction and more resilient to tumor hemorrhage

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    Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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