Objectives: Patients with acromegaly usually harbor macroadenomas measuring between 10 and 30 mm in maximal diameter. Giant (adenoma size ≥40 mm) GH-secreting pituitary tumors are rarely encountered and the aim of this study is to analyze different methods for managing them.
Design And Methods: We have identified 34 patients (15 men and 19 females) with giant adenomas among 762 subjects (4.5%) with acromegaly in our records, and characterized their clinical characteristics and response to treatment.
Results: Mean age at diagnosis was 34.9±12.5 years (range, 16-67 years). Mean adenoma size was 49.4±9.4 mm (range, 40-80 mm); 30 adenomas showed cavernous sinus invasion and 32 had suprasellar extension. Twenty-nine (85%) patients had visual field defects. Mean baseline IGF1 was 3.4±1.8×ULN. All patients except one underwent pituitary surgery (one to three procedures), but none achieved hormonal remission following first surgery. Among the 28 subjects with visual disturbances, 14 recovered post-operatively and 13 improved. Treatment with somatostatin analogs was given to all patients after surgical failure. Six achieved remission, nine others were partially controlled (IGF1<1.5×ULN; 3/9 when combined with cabergoline), and 17 did not respond (two were lost). Nine patients were treated with pegvisomant, alone (n=4) or in combination with somatostatin analogs (n=5); five are in remission and two are partially controlled. Pasireotide-LAR achieved hormonal remission in one of the six patients. Currently, after a mean follow-up period of 8.9 years, 17 patients are in biochemical remission, eight are partially controlled, and seven are uncontrolled (two were lost to follow-up).
Conclusions: Giant GH-secreting adenomas are invasive, uncontrolled by surgery, and respond poorly to medical treatment. Aggressive multimodal therapy is critical for their management, enhancing control rate and biochemical remission.
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http://dx.doi.org/10.1530/EJE-14-1117 | DOI Listing |
Expert Rev Endocrinol Metab
January 2025
Department of Medicine, Vagelos College of Physicians & Surgeons, Columbia University, New York, NY, USA.
Introduction: Acromegaly is due in almost all cases to a GH-secreting pituitary tumor. GH and IGF-1 excesses lead to its multi-system clinical manifestations and comorbidities. Acromegaly is under-diagnosed and typically presents with advanced disease.
View Article and Find Full Text PDFSci Rep
January 2025
Department of Neurosurgery, Bahcesehir Universty School of Medicine, Istanbul, Turkey.
The nature of somatotroph adenomas has not been clearly revealed in studies. We consider that there are macroscopic differences in intraoperative tumor consistency in acromegaly patients. We aimed to determine whether there is a relationship between intraoperative tumor consistency and histopathological subtypes by planning a prospective study to determine whether these differences are significant.
View Article and Find Full Text PDFClin Endocrinol (Oxf)
December 2024
Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy.
Objective: Many review articles have explored data regarding the coexistence of specific types of pituitary adenomas (PAs) and polycystic ovary syndrome (PCOS), particularly focusing on the potential pathogenesis of this intersection and overlapping features. However, a comprehensive evaluation encompassing the full spectrum of PAs and their association with PCOS remains lacking. This review aims to provide a broad assessment of the interactions between these entities, emphasizing pathophysiological mechanisms, clinical presentations, diagnostic challenges and therapeutic implications.
View Article and Find Full Text PDFEur J Endocrinol
January 2025
Endocrinology Unit, Department of Internal Medicine and Medical Specialties (DIMI), University of Genova, 16132 Genova, Italy.
Immunohistochemistry (IHC) of somatostatin receptor subtype 2 can predict response to first-generation somatostatin receptor ligands (fg-SRLs) in acromegaly. Recently, we validated an open-source digital image analysis (DIA) to quantify somatostatin receptor subtype 2 (SSTR2) expression. We aimed to validate the DIA also on somatostatin receptor subtype 5 (SSTR5) in a new cohort of growth hormone (GH)-secreting pituitary tumors, with IHC performed in a different laboratory, and to correlate fg-SRL response with SSTs expression.
View Article and Find Full Text PDFNeurochirurgie
December 2024
Lille University Hospital, Neurosurgery Department, PRISM Lab Inserm U1192, CHU LilleF-59000, Lille France.
Context: Pituitary Neuroendocrine Tumors are rare in children, but challenging, implying the two purposes to cure the child, and preserve pituitary function. In this paper, we describe our population of children who underwent endoscopic endonasal resection of a pituitary adenoma, in Lille University Hospital.
Patients And Methods: Between 2007 and 2021, all children undergoing EEN surgery for pituitary adenoma were included.
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