Giant GH-secreting pituitary adenomas: management of rare and aggressive pituitary tumors

Abstract

Objectives: Patients with acromegaly usually harbor macroadenomas measuring between 10 and 30 mm in maximal diameter. Giant (adenoma size R40 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 34.9G12.5 years (range, 16-67 years). Mean adenoma size was 49.4G9.4 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 3.4G1.8!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 (nZ4) or in combination with somatostatin analogs (nZ5); 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.

Giant Prolactinoma in Men: Clinical Features and Therapeutic Outcomes

“…Giant prolactinomas [ ≥ 4 cm (gPRLoma)] are rare tumors, predominantly diagnosed in males, with a male to female ratio of about 9:1 [4][5][6][7][8]. They are usually accompanied by endocrine symptoms, as a consequence of hormonal pituitary deficiencies, and frequent neuro-ophthalmological complications.…”

“…To date, a small number of series have evaluated patients with gPRLomas [4][5][6][7][8][11][12][13][14][15]. On the other hand, little is known about the differences in relation to the clinical features and therapeutic response in macroprolactinomas depending on their size.…”

Surgical treatment of aggressive pituitary adenomas and pituitary carcinomas

“…The dependency of surgical results on the size of the lesion and on infiltration of the sphenoid sinus were already described by Hardy [32] many years ago and confirmed in numerous more recent publications. Giant adenomas have a low remission rate of remission of hormonal oversecretion but even partial resection may improve the effect of postoperative medical treatments [33]. At least, this has been shown for tumors causing acromegaly by comparing the action of somatostatin analogs pre-and postoperatively [4].…”

A Unilateral Nasal Mass With Generalized Seizures: Potential Diagnostic Pitfalls in Giant Pituitary Adenoma

“…The reason why certain adenomas behave in a locally aggressive fashion is yet unknown. Based on a cohort study, 4 giant pituitary somatrotroph adenomas have poor response to surgery, radiotherapy, or medical therapy and required aggressive multimodal management strategy to achieve disease control. Thus, a multidisciplinary approach consisting of neurosurgery, otorhinolaryngology, ophthalmology, and endocrinology specialties is required to achieve better outcome.…”