Original ArticlePlurihormonal ACTH-GH Pituitary Adenoma: Case Report and Systematic Literature Review
Introduction
Pituitary adenomas are usually benign, monoclonal tumors arising from adenohypophyseal cells.1, 2 They represent approximately 15% of all intracranial tumors.2 Functioning pituitary adenomas, the most frequent type, produce clinical syndromes due to pituitary hormonal oversecretion and/or a mass effect.1 The large majority of functioning pituitary adenomas exhibits single hormone production, but the introduction of immunocytochemical analysis has revealed the existence of pituitary lesions with multiple hormonal expression.3 Histologically, plurihormonal pituitary adenomas (PHAs) represent 10%–15% of all pituitary tumors4 (up to 1% of pituitary lesions in unselected autopsy series), but only a small number clinically secrete multiple hormones.5, 6 The most frequent associations are with prolactin (PRL) and growth hormone (GH), and other molecular combinations are rare.5, 7
Here we report a case of a patient with a functioning adrenocorticotropic hormone (ACTH)-GH pituitary adenoma, along with the results of a systematic literature review of PHA.
Section snippets
Systematic Literature Review
The literature review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement and PRISMA checklist (Figure 1). We searched PubMed from May 1972 to October 2017 for published articles with the search terms “(plurihormonal OR ACTH and GH) adenoma” and pertinent variations; a language restriction to English papers was applied.
Case Presentation
A 60-year-old female was referred to our pituitary clinic (Spedali Civili, Brescia, Italy) with a diagnosis of a
Systematic Literature Review
On the initial PubMed search, a total of 1234 articles were retrieved, including 692 with “ACTH GH pituitary adenoma,” 396 with “acromegaly AND Cushing's disease,” and 146 with “plurihormonal pituitary adenoma.” After removal of duplicates, 662 articles remained. Of the 35 full-text articles that were selected, 1 was excluded for language constraints (Chinese) (Figure 1).
After full-text evaluation, 17 studies, with a total of 20 patients (13 females and 7 males; mean age, 48 years; range, 5–78
Discussion
PHAs are rare tumors that exhibit cellular immunoreactivity for more than 1 pituitary hormone. Some studies have suggested a prevalence as high as 31%–36% in surgically removed tumors.22 Histologically, PHAs are subclassified as monomorphous (i.e., different hormones expressed from a single morphological cell type) or plurimorphous (i.e., different hormones are expressed by morphologically divergent cells).7
Our PubMed systematic review of the 662 initially screened articles identified only 20
Conclusions
ACTH-GH PHAs are rare entities. Our systematic literature review documented only 20 cases reported to date. Of these, only 4 (20%) presented with clinical signs of both acromegaly and Cushing's disease. We report an additional case in which signs of both conditions were subtle but present.
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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.