Objective: To determine the effect of transsphenoidal surgery on quality of life and sleep in patients with pituitary adenomas depending on tumor type and compression of the optic chiasm. Methods: In this prospective study, patients with pituitary adenomas who were scheduled for transsphenoidal surgery completed the Short Form 36 Questionnaire, Pittsburgh Sleep Quality Index, and Epworth Sleepiness Scale preoperatively and 7.5 (±1.5) months after surgery. Patients were analyzed based on tumor type and compression of the optic chiasm. Results: Significant improvements with large effect sizes were seen for patients with Cushing’s disease in general health (Z = −2.37; p = 0.018), vitality (Z = −2.05; p = 0.041), and mental health (Z = −2.06; p = 0.040). A significant deterioration with large effect size occurred in physical functioning (Z = −2.02; p = 0.043) in patients with acromegaly. A significant improvement with medium effect size was seen in subjective sleep quality, (Z = −2.24; p = 0.025), sleep duration (Z = −2.11; p = 0.035), and habitual sleep efficiency (Z = −2.26; p = 0.024) after decompression of the optic chiasm. Multiple significant correlations were observed between sleep parameters and Short Form 36 subscales before and after treatment. Conclusions: Changes in quality of life during the follow-up period depend on tumor type. Circadian rhythm disturbances may resolve promptly after decompression of the optic chiasm. Quality of life in pituitary adenoma patients is associated with quality of sleep in many dimensions, thus implying that developing strategies to improve sleep quality could increase overall well-being and everyday functioning in pituitary adenoma patients.
The differentiation of cystic lesions located in the sellar-suprasellar region is a significant problem in clinical practice because of the similarities in their clinical, radiological, and even histopathological picture. Arriving at the right diagnosis is vital for taking appropriate therapeutic decisions. The most frequent clinical manifestation of lesions located in the sellar-suprasellar region is headache. It often co-exists with symptoms of anterior pituitary gland insufficiency or hyperprolactinaemia caused by compression of the pituitary stalk. Diabetes insipidus, obe-sity, mental disorders, and circadian rhythm disorders may be associated with lesions penetrating the suprasellar space. It is extremely important to rule out the possible coexistence of pituitary microadenoma and Rathke's cleft cyst, which became possible with the use of ¹¹C-methionine positron emission tomography/computed tomography (C-MET PET/CT). Reports from literature indicate that pituitary microadenoma may coexist with Rathke's cleft cyst in 10% of patients. Cystic lesions of the sellar-suprasellar region should also be differentiated from a cystic pituitary adenoma or abscess. The first-choice therapy in symptomatic cystic lesions of the sellar-suprasellar region is neurosurgery, which usually relieves headache and improves vision impairment, while less frequently restores normal pituitary function. In suprasellar lesions, neurosurgery may trig-ger or aggravate pre-existing symptoms of damage to the hypothalamus. Patients undergoing neurosurgery for cystic lesions located in the sellar-suprasellar region should be monitored for a few years due to their high recurrence rate, potential malignant transformation of these lesions, and possible adenoma development through metaplasia. The advent of targeted therapy of the BRAF/MEK pathway is associated with new therapeutic opportunities for patients with craniopharyngiomas.
Background: Adrenocorticotropic Hormone (ACTH)-dependent Cushing's Syndrome (CS) is most often caused by a pituitary adenoma. Although rarely, it can also result from pituitary corticotroph cell hyperplasia (CH). Reports on concomitant pituitary lesions including ACTH-producing adenomas and Rathke's cleft cysts (RCCs) have been published. Positron emission tomography (PET), using 11 C-labelled-methionine (MET) as a tracer and co-registered with magnetic resonance imaging (MRI) has been shown to be useful in the diagnosis of pituitary collision lesions, however, its role is still under investigation. In this work we present the case of a patient in whom CS was caused by non-adenomatous CH within the wall of an RCC. Case Summary: In 2015 a patient with signs and symptoms of CS was referred to our Department. Biochemical studies repeatedly showed elevated midnight serum cortisol and ACTH levels. Magnetic resonance imaging of the sellar region revealed an RCC and MET-PET/MR showed heterogeneous labelled-methionine metabolism in the vicinity of the cyst's wall. Transsphenoidal surgery resulted in rapid, complete and lasting relief of symptoms. Histopathological examination demonstrated an RCC and CH. Conclusions: Concomitance of pituitary focal lesions is a rare phenomenon. Methionine-labelled PET/MR may be useful in the diagnosis of collision sellar lesions, including CH. Corticotroph cell hyperplasia can present as mild and fluctuating hypercortisolaemia.
Simpson–Golabi–Behmel syndrome (SGBS) is a rare genetic condition and is inherited in an X‐linked recessive manner. The disease is caused by a change in the nucleotide sequence of an X‐linked gene encoding glypican 3, a protein belonging to the heparan‐sulfate membrane proteoglycan family. SGBS case studies are almost entirely restricted to the pediatric population. Scarce literature describing SGBS course in adults may be due to both the high mortality of SGBS patients in childhood and low rate of SGBS diagnosis in adults. We present a case of a 39‐year‐old man with an initial diagnosis of acromegaly. Genetic tests revealed a hitherto unreported deletion in the GPC3 gene. SGBS manifestations in our patient included tall stature, dysmorphic features, and central nervous system (CNS) anatomical pathology. MRI of the head visualized abnormalities of median line structures, a feature consistent with SGBS: an unclosed craniopharyngeal canal, a sellar‐suprasellar cyst, dysmorphic pituitary gland, and a cyst of the septum pellucidum. Moreover, cardiomyopathy complicated by life‐threatening paroxysmal ventricular tachycardia was diagnosed. Although various cardiac anomalies are often found in SGBS, their pathogenesis is unclear and may be multifactorial. We believe that the presented case contributes to a better understanding of SGBS and may help clinicians in introducing prophylaxis and treatment for its comorbidities.
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