BACKGROUND: Transsphenoid meningoencephalocele is a congenital anomaly formed by herniation of an ependyma delimited sac through a bony defect into the sphenoid sinus. The sac contains cerebrospinal fluid and neurovascular structures. The prevalence of transsphenoid meningoencephalocele in the adult population is rare. It usually manifests as nasal liquorrhoea. METHODS AND RESULTS: This case report presents an adult male who underwent surgery due to suspected pituitary macroadenoma. The surgery was performed endoscopically via the transnasal approach with a surprising finding of true transsphenoid meningoencephalocele. Ectopic solid tissue was found in the sphenoid sinus in which pituitary adenoma was histologically confirmed. CONCLUSION: This paper presents a previously unpublished combination of true transsphenoid meningoencephalocele and pituitary adenoma in an adult individual.

• Klíčová slova
• endoscopic transnasal approach, nasal liquorrhoea, pituitary adenoma, transsphenoid meningoencephalocele,
• MeSH
• adenom * komplikace   patologie   chirurgie   MeSH
• dospělí MeSH
• encefalokéla etiologie   patologie   chirurgie   MeSH
• endoskopie metody   MeSH
• lidé MeSH
• meningokéla * diagnostické zobrazování   patologie   chirurgie   MeSH
• nádory hypofýzy * komplikace   patologie   chirurgie   MeSH
• sinus sphenoidalis patologie   chirurgie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

Enlargement of the pituitary gland is heterogenous in the etiology. Common causes of pituitary enlargement are physiological hypertrophy during pregnancy, primary and secondary tumors, autoimmune hypophysitis including side effects of anticancer therapy with check-point inhibitors. Terms like hypertrophy, hyperplasia, sellar expansion and hypophysitis are commonly used to describe enlargement of the pituitary gland on MR scan regardless its etiology. The most common pathology causing pituitary gland enlargement is pituitary adenoma. Magnetic resonance imaging can differentiate pituitary tumors from diffuse enlargement due to hypophysitis in most but not all cases. Changes on imaging during time or response to pharmacotherapy might help determine the final diagnosis in uncertain cases. We present a case report of a young woman with sellar expansion due to prolonged untreated peripheral hypothyroidism mimicking pituitary adenoma. Interdisciplinary cooperation of endocrinologist, radiologist and neurosurgeon is crucial in determining the diagnosis.

• Klíčová slova
• Hypothyroidism, Pituitary adenoma, checkpoint inhibitors, hypophysitis, hypothyroidism, immune checkpoint inhibitors, pituitary tumor, sellar expansion,
• MeSH
• adenom * komplikace   diagnóza   MeSH
• diferenciální diagnóza MeSH
• hypertrofie MeSH
• hypofýza diagnostické zobrazování   patologie   MeSH
• hypofyzitida * MeSH
• hypotyreóza * komplikace   MeSH
• lidé MeSH
• magnetická rezonanční tomografie MeSH
• nádory hypofýzy * komplikace   diagnóza   MeSH
• nemoci hypofýzy * komplikace   diagnóza   MeSH
• těhotenství MeSH
• Check Tag
• lidé MeSH
• těhotenství MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

Not required for Clinical Vignette.

• Klíčová slova
• Cushing´s disease, acromegaly, immunohistochemistry, somatostatin analogues, somatostatin receptor subtype,
• MeSH
• adenom * komplikace   farmakoterapie   MeSH
• akromegalie * MeSH
• lidé MeSH
• nádory hypofýzy * komplikace   farmakoterapie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: Targeted stereotactic radiosurgery (SRS) with sparing of the residual pituitary is the traditional radiosurgical method for pituitary adenomas. Whole-sella SRS is an alternative choice for radiologically indeterminate or large adenomas, the safety and efficacy of which has yet to be determined. OBJECTIVE: To determine if whole-sella SRS in acromegaly would have comparable radiographic and biochemical control to targeted SRS. We performed a multicenter, retrospective matched cohort study to compare outcomes between groups. METHODS: We conducted a retrospective review of acromegalic patients who underwent SRS from 1990 to 2016 at 10 centers participating in the International Radiosurgery Research Foundation. Whole-sella and targeted SRS patients were then matched in a 1:1 ratio. RESULTS: A total of 128 patients were eligible for inclusion. Whole-sella patients had a higher pre-SRS random serum growth hormone, larger treatment volume, and higher maximum point dose to the optic apparatus. The rates of initial/durable endocrine remission, new loss of pituitary function, and new cranial neuropathy were similar between groups. Mortality and new visual deficit were higher in the whole-sella cohort, though not statistically significant. CONCLUSION: There was no difference in biochemical remission or recurrence between treatment groups. Although not statistically significant, the higher rates of tumor regression and lower rates of mortality and new visual deficit may suggest consideration of targeted SRS over whole-sella SRS in acromegaly treatment. Further research is needed to determine the association between visual deficits and mortality with whole-sella SRS.

• Klíčová slova
• Acromegaly, Endocrinopathy, Gamma Knife, Hypopituitarism, Pituitary adenoma, Stereotactic radiosurgery,
• MeSH
• adenom komplikace   chirurgie   MeSH
• akromegalie etiologie   chirurgie   MeSH
• dospělí MeSH
• kohortové studie MeSH
• lidé středního věku MeSH
• lidé MeSH
• lokální recidiva nádoru chirurgie   MeSH
• mladý dospělý MeSH
• nádory hypofýzy komplikace   chirurgie   MeSH
• radiační poranění epidemiologie   etiologie   MeSH
• radiochirurgie škodlivé účinky   metody   MeSH
• retrospektivní studie MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH

Acromegaly is a rare disorder usually caused by a benign tumour of the pituitary gland. Long-term presence of elevated growth hormone (GH) and insulin like growth factor 1 (IGF1) levels accompanying this disease is associated with complications such as cardiomyopathy, diabetes mellitus, sleep apnoea and arthropathy. Incidence of acromegaly is 3-4 patients per million per year. Klinefelter syndrome (KS) is the most common sex chromosome disorder occuring in about 1/500 live male births. Common physical features include particularly small testes, among other symptoms are tall stature, reduced muscle tone, delayed pubertal development, lack of secondary male sex characteristics and gynecomastia. We present a 32-year-old man suffering from both acromegaly and 47, XXY Klinefelter syndrome. The patient with typical acromegalic features. Laboratory tests revealed high level of GH which was not suppressed after glucose administration, high level of IGF1, low testosterone concentration with high concentation of luteinizing hormone and follicle stimulating hormone. A magnetic resonance imaging scan revealed a 25 × 18 × 18 mm macroadenoma involving the pituitary gland. A diagnosis of acromegaly was established. After this examination trans-sphenoidal resection was performed. Histopathologic and immunohistochemical findings revealed growth hormoneproducing pituitary adenoma. The presence of infertility with clinical features such as small testes, lack of secondary male sex characteristics and laboratory findings revealed hypergonadotropic hypogonadism that could not be explained by the diagnosis of acromegaly. A chromosomal karyotyping revealed a 47, XXY, confirming the diagnosis of KS. Testosterone replacement therapy wasn´t begun because of patient disagreement Postoperatively elevated plasma concentration of GH and IGF1 levels persist. Treatment by somatostatin analogues (lanreotid) was initiated at dose 120 mg every 28 days. Control magnetic resonance imaging of the sella demonstrated a residue of pituary adenoma size 14 × 14 × 7 mm. The patient is currently undergoing endoscopic revision of the residue. acromegaly - growth hormone - IGF1 - Klinefelter syndrome - testosterone.

• MeSH
• adenom * komplikace   diagnóza   genetika   MeSH
• akromegalie * komplikace   diagnóza   genetika   MeSH
• dospělí MeSH
• insulinu podobný růstový faktor I MeSH
• Klinefelterův syndrom * komplikace   diagnóza   genetika   MeSH
• lidé MeSH
• lidský růstový hormon MeSH
• nádory hypofýzy * komplikace   diagnóza   genetika   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• insulinu podobný růstový faktor I MeSH
• lidský růstový hormon MeSH

BACKGROUND: Stereotactic radiosurgery (SRS) is a treatment option for persistent or recurrent acromegaly secondary to a growth hormone secreting pituitary adenoma, but its efficacy is inadequately defined. OBJECTIVE: To assess, in a multicenter, retrospective cohort study, the outcomes of SRS for acromegaly and determine predictors. METHODS: We pooled and analyzed data from 10 participating institutions of the International Gamma Knife Research Foundation for patients with acromegaly who underwent SRS with endocrine follow-up of ≥6 mo. RESULTS: The study cohort comprised 371 patients with a mean endocrine follow-up of 79 mo. IGF-1 lowering medications were held in 56% of patients who were on pre-SRS medical therapy. The mean SRS treatment volume and margin dose were 3.0 cm3 and 24.2 Gy, respectively. The actuarial rates of initial and durable endocrine remission at 10 yr were 69% and 59%, respectively. The mean time to durable remission after SRS was 38 mo. Biochemical relapse after initial remission occurred in 9%, with a mean time to recurrence of 17 mo. Cessation of IGF-1 lowering medication prior to SRS was the only independent predictor of durable remission (P = .01). Adverse radiation effects included the development of ≥1 new endocrinopathy in 26% and ≥1 cranial neuropathy in 4%. CONCLUSION: SRS is a definitive treatment option for patients with persistent or recurrent acromegaly after surgical resection. There appears to be a statistical association between the cessation of IGF-1 lowering medications prior to SRS and durable remission.

• Klíčová slova
• Acromegaly, Gamma Knife, Growth hormone, Insulin-like growth factor-1, Pituitary adenoma, radiosurgery,
• MeSH
• adenom hypofýzy vylučující růstový hormon komplikace   chirurgie   MeSH
• adenom komplikace   chirurgie   MeSH
• akromegalie etiologie   chirurgie   MeSH
• dospělí MeSH
• kohortové studie MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• radiochirurgie škodlivé účinky   metody   MeSH
• retrospektivní studie MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• výsledek terapie MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH

OBJECTIVE: To assess the diagnostic and therapeutic options in the care of patients with primary hyperparathyreosis in outpatient practice.Cohort and methods: The study included all the patients with primary hyperparathyroidism treated at the 2nd Internal Medicine Department, Masaryk University and the University Hospital of St. Anne in Brno in the period from Jan 1, 2008 to Dec 31, 2013. The sample consisted of 218 patients, including 41 men and 177 women. Patients with secondary hyperparathyroidism, especially patients with underlying hypovitaminosis D, renal insufficiency and those taking medications with possible effects on parathyroid hormone levels, have not been included in the study. A special attention was paid to differences between the normocalcaemic and hypercalcaemic patients. Ultrasound scanning was performed in all patients, while scintigraphy was indicated in patients who are considered for possible surgical treatment. RESULTS: In the group of 218 patients, serum calcium levels at the baseline were pathologically elevated in 31 patients (14 %) and normal in 187 patients (86 %). One fifth of patients with normocalcaemic primary hyperparathyroidism developed long-term hypercalcaemia - within two years in two thirds of the patients from the onset of the disease and sporadically also after more than four years of follow-up. Parathyroid adenoma was found and removed in 30 hypercalcemic patients (in 97 % of all 31 hypercalcemic patients operated on) and in 2 normocalcemic patients (40 % of all 5 the normocalcemic patients operated on). Pharmacological treatment was administered to 22 patients, of which 9 patients received long-term treatment and 13 patients received pharmacotherapy only during the preoperative preparation for patients with very high serum calcium levels. CONCLUSION: The results support the opinion that primary hyperparathyroidism is a biphasic disease. The initial normocalcemic period is often asymptomatic or associated with symptoms of little importance. Severe complications, however, may already be present also in normocalcemic patients. The decision of when patients with normocalcemic primary hyperparathyroidism should be monitored and when initiation of treatment is needed should also require more detailed information.Key words: hypercalcaemia - hyperparathyroidism asymptomatic and primary - normocalcaemia - outpatient care - parathyroid hormone - surgery and pharmacotherapy.

• MeSH
• adenom komplikace   diagnóza   chirurgie   MeSH
• asymptomatické nemoci MeSH
• hyperkalcemie etiologie   terapie   MeSH
• lidé MeSH
• nádory příštítného tělíska komplikace   diagnóza   chirurgie   MeSH
• parathormon MeSH
• primární hyperparatyreóza diagnóza   etiologie   terapie   MeSH
• vápník MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• parathormon MeSH
• vápník MeSH

Vast majority of thyroid function disturbances have primary (peripheral) etiology due to thyroid gland disorders. Rarely, dysfunction of central regulatory structures, hypothalamus and pituitary, can be a cause of both, hyperthyroidism and hypothyroidism. Despite being very rare, it is important to be aware of them not to misdiagnose their etiology. Early and correct etiological diagnosis is necessary for proper cure and decrease of morbidity and mortality of affected patients. Present review article summarizes basics and specific features of central disturbances of thyroid function, their clinical signs, diagnosis, differential diagnosis and treatment.Key words: hypothalamus - hyperthyroidism - hypothyroidism - pituitary - thyrotropinoma.

• MeSH
• adenom komplikace   diagnóza   metabolismus   terapie   MeSH
• hypertyreóza diagnóza   etiologie   terapie   MeSH
• hypotyreóza diagnóza   etiologie   terapie   MeSH
• lidé MeSH
• nádory hypofýzy komplikace   diagnóza   metabolismus   terapie   MeSH
• nemoci hypofýzy komplikace   diagnóza   terapie   MeSH
• nemoci hypotalamu komplikace   diagnóza   terapie   MeSH
• thyreotropin metabolismus   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH
• Názvy látek
• thyreotropin MeSH

Adenomas which secrete thyrotropic hormone (thyrotropinomas) are rare and constitute less than 3 % of pituitary adenomas. In laboratory studies there is a typical elevation of thyroid hormones with nonsupressible TSH. In differential diagnostics it is necessary to distinguish above all the syndrome of resistance to thyroid hormones. Clinical symptoms are usually mild and correspond to symptoms of hyperthyroidism. Goiter is a common finding. In 80 % of cases thyrotropinomas are diagnosed in a stage of invasively growing macroadenoma. The primary treatment is neurosurgical removal adenoma which results in cure in 40 % of patients. Other treatment options include radiation therapy and medical treatment (treatment with somatostatin analogues). With regard to the risk of adenoma recurrence, the long-term follow-up is similar to that of cases of other pituitary adenomas necessary.Key words: resistance to thyroid hormone - TSH secreting adenoma - thyreotropin.

• MeSH
• adenom komplikace   metabolismus   chirurgie   MeSH
• diferenciální diagnóza MeSH
• lidé MeSH
• lokální recidiva nádoru epidemiologie   MeSH
• nádory hypofýzy komplikace   metabolismus   chirurgie   MeSH
• neurochirurgické výkony MeSH
• struma etiologie   MeSH
• thyreotropin metabolismus   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• thyreotropin MeSH

Currently, primary hyperparathyroidism is the third most common endocrine disorder worldwide. In the last years, though, the numbers of patients with this diagnosis have been clearly increasing. Females are affected more often, and the age of patients is usually over 50 years. The diagnosis is guided by clinical symptoms of the patient and by biochemical and imaging methods results. [1] When parathyroid adenoma is identified as the source of primary hyperparathyroidism, the necessary extent of resection is performed. The present case involved a rapid diagnostic process necessary to stabilize the patient, together with localization of the adenoma for the indicated surgery. Ultrasound-guided exploration found a surprisingly large tumour of the parathyroid gland causing the acute clinical condition with symptoms suggestive of primary hyperparathyroidism with discrete mechanical compression of the upper mediastinum.Key words: critical diagnosis of hyperparathyroidism acute-active parathyroid adenoma extirpation of a huge parathyroid tumour - normalization of the clinical condition.

• MeSH
• adenom komplikace   diagnostické zobrazování   patologie   chirurgie   MeSH
• dospělí MeSH
• jednofotonová emisní výpočetní tomografie MeSH
• lidé MeSH
• mediastinum MeSH
• nádory příštítného tělíska komplikace   diagnostické zobrazování   patologie   chirurgie   MeSH
• paratyreoidea diagnostické zobrazování   chirurgie   MeSH
• primární hyperparatyreóza diagnóza   etiologie   chirurgie   MeSH
• radiofarmaka MeSH
• SPECT/CT MeSH
• technecium 99mTc sestamibi MeSH
• tumor burden MeSH
• ultrasonografie MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• radiofarmaka MeSH
• technecium 99mTc sestamibi MeSH