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Concept: Pituitary adenoma

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The objective of this study was to evaluate outcomes of endoscopic transsphenoidal surgery using a single-surgeon technique as an alternative to the more commonly employed two-surgeon, three-hand method. Three hundred consecutive endoscopic transsphenoidal procedures performed over a 5 year period from 2006 to 2011 were reviewed. All procedures were performed via a binasal approach utilizing a single surgeon two handed technique with a pneumatic endoscope holder. Expanded enodnansal cases were excluded. Surgical technique, biochemical and surgical outcomes, and complications were analyzed. 276 patients underwent 300 consecutive surgeries with a mean follow-up period of 37 ± 22 months. Non-functioning pituitary adenoma (NFPA) was the most common pathology (n = 152), followed by growth hormone secreting tumors (n = 41) and Rathke’s cleft cysts (n = 30). Initial gross total cyst drainage based on radiologic criteria was obtained in 28 cases of Rathke’s cleft cyst, with 5 recurrences. For NFPA and other pathologies (n = 173) gross total resection was obtained in 137 cases, with a 92 % concordance rate between observed and expected extent of resection. For functional adenoma, remission rates were 30/41 (73 %) for GH-secreting, 12/12 (100 %) for ACTH-secreting, and 8/17 (47 %) for prolactin-secreting tumors. Post-operative complications included transient (11 %) and permanent (1.4 %) diabetes insipidus, hyponatremia (13 %), and new anterior pituitary hormonal deficits (1.4 %). CSF leak occurred in 42 cases (15 %), and four patients required surgical repair. Two carotid artery injuries occurred, both early in the series. Epistaxis and other rhinological complications were noted in 10 % of patients, most of which were minor and diminished as surgical experience increased. Fully endoscopic single surgeon transsphenoidal surgery utilizing a binasal approach and a pneumatic endoscope holder yields outcomes comparable to those reported with a two-surgeon method. Endoscopic outcomes appear to be better than those reported in microscope-based series, regardless of a one or two surgeon technique.

Concepts: Pituitary adenoma, Hospital, Surgery, Physician, Growth hormone, Common carotid artery, Anterior pituitary, Rathke's pouch

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OBJECTIVE: Gonadotroph pituitary adenomas are common, but the overwhelming majority are classified as non-functioning adenomas. Approximately one-third secrete follicle-stimulating hormone (FSH) and/or luteinizing hormone (LH), resulting in an increase in serum levels; however, the sex hormone levels are typically not elevated and the secreted gonadotropin hormones are thought to be biologically inactive. Rarely, gonadotroph adenomas induce an ovarian hyperstimulation syndrome in females. In males, an increase in testosterone level is even more rare. The authors report the case of a male patient with a gonadotroph pituitary adenoma causing hypersecretion of testosterone and review the medical literature. CASE REPORT: A 45-year-old man had progressive bilateral visual loss over a 4-year period. Bitemporal hemianopsia was diagnosed by an ophthalmologist, but no other symptoms were reported. Neurosurgical evaluation was otherwise normal. MRI of the brain showed a large enhancing sellar and suprasellar tumor consistent with pituitary macroadenoma. Levels of FSH, LH, α subunit, testosterone, free testosterone, and prolactin were elevated. The patient underwent transsphenoidal resection of the pituitary tumor. Pathological analysis confirmed the diagnosis of pituitary adenoma, and immunohistochemistry demonstrated a population of cells with cytoplasmic staining for FSH but not for LH. The postoperative hormonal profile showed normalization of FSH and low LH, testosterone, morning cortisol level, and free thyroxine. The patient was treated with levothyroxine and hydrocortisone therapy for replacement. His vision subjectively improved, with formal visual testing follow-up to monitor the degree of improvement. CONCLUSIONS: Gonadotroph adenomas are considered a type of non-functioning pituitary tumors, since the commonly associated increase in serum FSH and LH does not typically result in elevated sex hormone level. Herein, we report a rare case of high testosterone level caused by a gonadotroph adenoma successfully treated with transsphenoidal resection. Interestingly, in adult males the increase in sex hormones does not seem to lead to a recognizable clinical syndrome, and the clinical presentation remains similar to that of non-functioning adenomas.

Concepts: Pituitary adenoma, Hormone, Luteinizing hormone, Types of cancer, Testosterone, Neuroendocrinology, Follicle-stimulating hormone, Bitemporal hemianopsia

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DNA methylation is one of several epigenetic modifications that together with genetic aberrations are hallmarks of tumourigenesis including those emanating from the pituitary gland. In this study we examined DNA methylation across 27578 CpG sites spanning more than 14000 genes in the major pituitary adenoma subtypes. Genome-wide changes were first determined in a discovery cohort comprising, non-functioning (NF), growth hormone (GH), prolactin secreting (PRL) and corticotroph (CT) adenoma relative to post-mortem pituitaries. Using stringent cut-off criteria we validated increased methylation by pyrosequencing in 12 of 16 (75%) genes. Overall these criteria identified 40 genes in NF, 21 in GH, 6 in PRL and 2 in CT hat were differentially methylated relative to controls. In a larger independent cohort of adenomas, for genes where hypermethylation had been validated, different frequencies of hypermethylation were apparent; where the KIAA1822 and TFAP2E genes were hypermethylated in 12 of 13 NF adenomas whereas the COL1A2 gene showed increase in 2 of 13 adenomas. For genes showing differential methylation across and between adenoma subtypes pyrosequencing confirmed these findings. For 3 of 12 genes investigated, an inverse relationship between methylation and transcript expression was observed where increased methylation of, EML2, RHOD and HOXB1 is associated with significantly reduced transcript expression. This study provides the first, genome-wide survey of adenoma, subtype-specific epigenomic changes and will prove useful for identification of biomarkers that perhaps predict or characterise growth patterns. The functional characterisation of identified genes will also provide insight of tumour aetiology and identification of new therapeutic targets.

Concepts: DNA, Hypothalamus, Gene expression, Pituitary gland, Epigenetics, Pituitary adenoma, Growth hormone, DNA methylation

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We report 2 bilateral cases that presented as primary ocular hypertension and primary angle-closure glaucoma, respectively; however, they were subsequently discovered to be harboring secretory pituitary tumors. After transsphenoidal tumor resection, intraocular pressures (IOPs) in all 4 eyes returned to normal levels. Sudden rise in IOP then again served as a primary manifestation of relapse in the second patient with growth hormone secreting pituitary tumor. It was not found feasible for resurgery; thus, patient needed trabeculectomy in both eyes to achieve an optimum control of intraocular tension. We conclude that pituitary adenomas may mimic primary glaucoma without producing vertical hemianopia and cause a reversible rise in IOP. Furthermore, a careful ongoing expert ophthalmologic assessment may serve as a useful clinical marker for early relapse in these tumors.

Concepts: Pituitary adenoma, Growth hormone, Neuroendocrinology, Intraocular pressure, Glaucoma, Ophthalmology, Optic nerve, Bitemporal hemianopsia

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Abstract A 34-year-old African-American man was referred for eyelid swelling and ocular discomfort. He was found to have floppy hypertrophic eyelids and marked bilateral mechanical ptosis that was present since childhood. Systemic examination was significant for furrows on his forehead and scalp, coarse facial features, and enlarged hands and feet with clubbing of the fingers and toes. Radiographic imaging of the long bones demonstrated periostosis, and MRI of the head revealed a pituitary macroadenoma. Pituitary and thyroid hormone levels were normal. The patient was diagnosed with pachydermoperiostosis and a non-secreting pituitary macroadenoma. Bilateral upper lid tightening via wedge resection was followed by bilateral external levator advancement ptosis repair in a staged manner. The patient achieved symptom relief and improved lid position postoperatively.

Concepts: Head and neck, Medical terms, Pituitary adenoma, Thyroid, Eyelid, Ptosis, Levator palpebrae superioris muscle, Dermatochalasis

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Clinically nonfunctioning pituitary adenoma (NFPA), remain the only pituitary tumor subtype for which no effective medical therapy is available or recommended. We evaluated dopamine agonist (DA) therapy for preventing growth of post-surgical pituitary tumor remnants.

Concepts: Medicine, Oncology, Pituitary adenoma, Types of cancer, Neuroendocrinology, Prolactinoma, Dopamine agonist

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Background Esthesioneuroblastoma is an uncommon malignant neoplasm that arises from the olfactory neuroepithelium. In this article we report a case of esthesioneuroblastoma presenting concomitantly with a growth-hormone (GH)-secreting pituitary macroadenoma. Results A 52 year old woman underwent surgery for suspected nasal polyps. Intralesional debulking of an intranasal tumor disclosed a low-grade esthesioneuroblastoma. Magnetic resonance imaging (MRI) demonstrated a large nasal and intracranial tumor, in addition to a separate sellar and suprasellar tumor. The patient was frankly acromegalic. She underwent a first-stage gross total resection of the esthesioneuroblastoma via a combined extended subfrontal and extended endonasal approach, followed by focused radiation therapy. She then returned for endoscopic removal of the GH-secreting pituitary macroadenoma. Conclusion The combined open and endoscopic management of this patient is described and a review of the literature presented. To our knowledge this is the first case of synchronous esthesioneuroblastoma and macroadenoma, in this case GH secreting, described in the literature.

Concepts: Cancer, Brain, Oncology, X-ray, Pituitary adenoma, Brain tumor, Nuclear magnetic resonance, Magnetic resonance imaging

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ABSTRACT Purpose: To evaluate retinal nerve fiber layer (RNFL) thickness in acromegalic patients. Methods: A study group of 29 patients with acromegaly and a control group of 38 age-matched healthy individuals were enrolled in a cross-sectional study. The study group was further divided by tumor size into two subgroups, a macroadenoma group and a microadenoma group. Serum growth hormone (GH) and insulin-like growth factor-1 (IGF-I) levels were detected at the time of ophthalmological examination in the study group. In both the study and control group, the RNFL thickness in the four quadrants was measured by optical coherence tomography. The relationship between GH and IGF-I levels and RNLF thickness was also evaluated. Results: The difference in mean RNLF thickness in all quadrants between the study and control groups was not statistically significant. In acromegalic patients with macroadenoma, the mean RNLF thickness of the inferior quadrant decreased significantly compared to both patients with microadenoma and healthy individuals (p = 0,032 and p = 0,046). GH and IGF-1 levels were not significantly correlated with the RNLF thickness in the study group. Conclusions: Excessive GH and IGF-1 levels do not affect the optic nerve or RNLF thickness, whereas the RNLF becomes thinner in the inferior quadrant in acromegalic patients with macroadenoma as a result of the chiasmal compression.

Concepts: Optics, Pituitary adenoma, Insulin-like growth factor 1, Growth hormone, Retina, Optic nerve, Nerve fiber layer, Acromegaly

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To present the clinical and radiographic outcomes of 140 patients with pituitary adenomas treated by an endoscopic endonasal approach (EEA) over a period of 4 years.

Concepts: Pituitary adenoma, Types of cancer, Neuroendocrinology, Endoscopic retrograde cholangiopancreatography, Endoscopy

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Granulomatous mastitis (GM) is a relatively uncommon inflammatory breast lesion with multiple suggested etiologies. Although most GM cases show association with lactation and pregnancy, a minority of cases have been linked to hyperprolactinemia caused by either dopamine antagonist medications or with intracranial lesions, such as pituitary adenoma. The goal of this study is to review the GM cases reported in the literature with a specific emphasis on those cases associated with hyperprolactinemia and prolactinomas and to identify cases of GM seen at the Cleveland Clinic Florida which demonstrate co-occurrences of GM and intracranial lesions. CoPath and Epic data bases at Cleveland Clinic Florida were searched for cases describing inflammatory breast lesions in patients with pituitary pathology. Chart reviews were conducted and pertinent medical history was extracted for case reports. H&E-stained paraffin-embedded sections retrieved from Cleveland Clinic Florida pathology storage were evaluated by light microscopy. Four cases showing a co-occurrence of GM and hyperprolactinemia were consequently identified. A prolactin-secreting pituitary adenoma was present in two of the three GM cases. The third case demonstrated a concomitant craniopharyngioma, which was also associated with a rise in serum prolactin. This phenomenon was presumably attributable to compression, resulting in compromised transport of dopamine to the adenohypophysis and subsequent disinhibition of prolactin secretion by lactotrophs. The fourth patient with GM had a similar history of elevated prolactin. Classical histopathological features of GM were found in all four cases, including noncaseating granulomas, multinucleated giant cells, epithelioid histiocytes, and chronic inflammation. Intriguingly, complete resolution of inflammatory breast lesions along with normalization of prolactin levels occurred following the surgical excision of the craniopharyngioma, suggesting that intracranial lesion-induced hyperprolactinemia might be directly causal in GM. Therefore, the authors would suggest screening for pituitary tumors and evaluate prolactin levels in the workup of GM patients without a recent history of lactation and pregnancy and no other identified etiology.

Concepts: Hypothalamus, Prolactin, Pituitary adenoma, Anatomical pathology, Types of cancer, Neuroendocrinology, Prolactinoma, Galactorrhea