Pituitary region tumors comprise ‘significant proportion’ of pediatric brain tumors
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Nearly 20% of pediatric brain tumors occur in the pituitary region, and more than two-thirds of pituitary region tumors were present in girls, according to study findings published in Pituitary.
“Although pituitary tumors have been extensively studied in the adult population, there has been a lack of data specific to the pediatric population, despite — as we found — pituitary region tumors not being wholly uncommon in pediatric patients,” Luz Castellanos, MD, a pediatric endocrinology fellow at Massachusetts General Hospital, told Healio. “Pituitary region tumors comprise a significant proportion of pediatric brain tumors, particularly in adolescent girls, and should be considered in the differential for pediatric patients with symptoms of hypothalamic-pituitary axis dysfunction.”
Castellanos and colleagues analyzed data from the National Cancer Database of pediatric patients aged 21 years and younger who presented with a pituitary region tumor from 2004 to 2017. A microscopic diagnosis or biopsy and/or a surgical procedure were used for pathological confirmation. Children were placed into the infant/toddler age group if diagnosed at younger than 2 years, early childhood if diagnosed at 2 to 5 years, middle childhood for diagnoses at 6 to 11 years, early adolescence if diagnosed at 12 to 18 years and late adolescence for diagnoses at 19 to 21 years.
Pituitary tumors more common in girls
Researchers compared the prevalence of pituitary region tumors in children with data from adult patients between 2010 and 2017. A greater proportion of children in the study presented with a pituitary region tumor compared with adults (19.7% vs. 12.4%; P < .001). Although there was very little difference in the prevalence of pituitary region tumors in adult men vs. women, a larger proportion of girls had pituitary region tumors compared with boys in the pediatric group (27.2% vs. 12.2%; P < .001).
“We were not entirely surprised to see that young females were more likely to present with pituitary adenomas, given that this finding has been previously observed in institutional series and cancer registry data,” Castellanos said. “This female predominance is partly attributed to females exhibiting more obvious symptoms of hypothalamic-pituitary axis dysfunction than males do.”
Age at diagnosis
Of 7,653 children in the study data presenting with a pituitary tumor, 46.9% were diagnosed in early adolescence, 34.8% in late adolescence, 13.3% in middle childhood, 4.1% in early childhood and 0.9% as infants or toddlers, with 68.2% of cases occurring in girls, the researchers wrote. Most tumors were identified as pituitary adenoma (77.9%), followed by craniopharyngiomas (18.1%) and germ cell tumors (1.6%). The prevalence of pituitary adenomas increased during puberty for girls, with 88.8% of pituitary tumors in early adolescent girls and 95.2% in late adolescent girls identified as pituitary adenoma.
When broken down by ethnicity, Asian and Pacific Islander children presented with a pituitary tumor at a mean age of 13.9 years, more than 1 year younger than white, Black and Hispanic adolescents. Those in the Asian/Pacific Islander cohort also had the highest proportion of children with germ cell tumors at 10.2%. The white, Black and Hispanic groups had a germ cell tumor prevalence of 3.1% or less.
Later diagnosis, odds for surgery without insurance
Of the total study cohort, 5.5% did not have insurance. Those without insurance presented at a mean age of 17.9 years, 2.3 years older than those with private insurance, 3.66 years older than patients with Medicaid and 2.19 years older than those with other government insurance (P < .001 for all). Patients were more likely to have surgery if they had private insurance (adjusted OR = 1.93; 95% CI, 1.47-2.52; P < .001), Medicaid (aOR= 1.51; 95% CI, 1.14-2; P = .004) or other government insurance (aOR = 1.99; 95% CI, 1.35-2.94; P = .001).
“When seeing a female adolescent patient with headaches, vision changes and hormonal abnormalities, it is important to include pituitary adenoma in the differential and workup,” Castellanos said. “In Asian/Pacific Islander children with pituitary masses, in particular, germ cell tumors should be considered. Uninsured pediatric patients that have pituitary tumors are less likely to be diagnosed earlier in their disease course.”
Bryan Iorgulescu, MD, FCAP, an instructor in the department of pathology at Brigham and Women’s Hospital and Dana-Farber Cancer Institute, told Healio that the findings are the first step to better understand the challenges pediatric patients with pituitary tumors have regarding access to care.
“Building on our findings, certainly more health services research is needed to understand the specific socioeconomic and care setting barriers that pediatric patients with pituitary tumors face in their access to timely and standard-of-care management; as well as how these barriers affect their outcomes,” Iorgulescu said.
For more information:
Luz Castellanos, MD, can be reached at lcastellanos2@mgh.harvard.edu.
Bryan Iorgulescu, MD, FCAP, can be reached at jiorgulescu@bwh.harvard.edu.
Perspective
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Pediatric pituitary tumors, an overall rare entity, are often identified when children undergo imaging studies of the hypothalamic-pituitary axis for a suspected endocrine disorder. Castellanos and colleagues provide important additional insight into the etiology and distribution of these tumors. Consistent with prior studies, they identify a higher frequency of pituitary adenomas among older compared to younger children, as well as a female predominance. It is important to note that compared to adults, non-functioning adenomas are not common in the pediatric age range. The most common functional adenomas in pediatrics are associated with hypersecretion of prolactin (female predominance, typically seen in adolescence), ACTH (male predominance prior to puberty, typically seen in adolescence), and growth hormone (male predominance). When identified, an association with possible genetic syndromes, including multiple endocrine neoplasia (MEN1), neurofibromatosis, McCune Albright Syndrome, and others, should be sought in the appropriate clinical context.
The increasing use of neuroimaging for evaluation of headaches, neurologic findings or neurocognitive delays leads to an increasing incidence of pituitary incidentalomas, defined by the endocrine society as “a previously unsuspected pituitary lesion that is discovered on an imaging study performed for an unrelated reason.” These incidentalomas, which can include pars intermedia cysts, pituitary microadenomas or pituitary hypertrophy, similarly have a female predominance and predilection for adolescence compared to younger ages. Microadenomas may be noted in up to 20% of children with diagnosed growth hormone deficiency or precocious puberty, where their causality is debatable in light of their frequent spontaneous resolution on subsequent imaging. Among adolescent girls, it is important to recognize that a physiologic increase in pituitary size and a change in imaging characteristics are normal and must be differentiated from adenomas to the degree possible.
Pituitary tumors, particularly adenomas, pose a clinical conundrum in which clinicians and families must weigh the risks of invasive diagnostic or surgical procedures against the value of tissue diagnosis. The association between germ cell tumors and Asian/Pacific Islander populations in this study and others, as well as both germline and somatic genetic testing, provide valuable insight toward the most appropriate course of action for diagnosis and care.
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