Papillary craniopharyngioma: a type of tumor primarily impairing the hypothalamus. A comprehensive anatomo-clinical characterization of 350 well-described cases

Objective: Papillary craniopharyngiomas (PCPs) represent a rare histological variant of craniopharyngiomas (CPs) usually involving the hypothalamus. This study systematically analyzes the clinical-anatomical correlation between tumor topography and symptoms related to hypothalamic dysfunction in the largest series of PCPs ever gathered. Methods: From 5,346 CP reports published from 1856 to 2021, we selected 350 well-described cases of the squamous-papillary type. Clinical presentation, tumor topography, severity of hypothalamic adhesion, patient outcome and tumor recurrence were thoroughly analyzed. Results: PCPs predominantly occur in adult (96.3%), male (61.7%) patients presenting with headache (63.4%), visual alterations (56.2%) and psychiatric disturbances (50.4%). Most PCPs are solid (50%), round (50%) lesions that occupy the third ventricle (3V, 94.8%) and show low-risk severity adhesions to the hypothalamus (66.8%). Two major topographical categories can be found: strictly 3V (57.5%), growing above an intact 3V floor (3VF) and not-strictly or infundibulo-tuberal (32.9%), expanding at the infundibulum and/or tuber cinereum. The hypothalamic syndrome predominated among strictly 3V PCPs (p<0.001). Psychiatric symptoms (p<0.001) and high-risk hypothalamic attachments (p=0.031) related to unfavorable postoperative outcomes among patients treated from 2006 onwards. The not-strictly 3V topography was identified as the major predictor of high-risk hypothalamic attachments (71.2% correctly predicted), which, along with incomplete tumor removal (p=0.018), underlies the higher tumor recurrence of this topography (p=0.001). Conclusions: This systematic review evidences that PCP topography is a major determinant of hypothalamic-related symptoms, type of hypothalamic attachments and tumor recurrence rate. Accurate preoperative definition of PCP-hypothalamus relationships is essential for the judicious, safe management of these complex lesions.

Female Factors of Infertility in a Couple

The problem of infertility is relevant to the whole world, that is explaned both by the prevalence (at least 50 million couples on the planet are diagnosed) and by the enormous medical, ecoomic, social and psychological significance. Another important aspect of infertility is the heterogeneity of its causes – about 40% of infertile marriages are caused by the female factor, 35% – male, 20% – a combination of male and female factors and 5% – have no identified factor. In 2019, the American Collegue of Obstetricians and Gynecologists updated the guidelines for the timing and scope of examinations of infertile couples. In particular, if a woman is 35–40 years old, examination and elimination of the probable factor of infertility should be started after 6 months of non-pregnancy, and in the case of age older than 40 years – immediately after the couple’s accost. The expected tactics should not be used if the patient has oligo- or amenorrhea, known uterine and fallopian tube abnormalities, grade III or IV severity of endometriosis, and the couple has known male infertility factors. Ovulation disorders as a factor of infertility include hypothalamic syndrome, polycystic ovary syndrome, premature ovarian failure and hyperprolactinemia, which differ in the content of gonadotropic hormones and ovarian hormones. The adhesion process of the pelvic organs, which restricts the transport of sperm and fertilized egg through the fallopian tubes, is a consequence of endometriosis and inflammatory diseases caused mainly by sexually transmitted pathogens. Endometriosis, in addition to the formation of adhesions in the pelvic cavity, which is characteristic of stage III and IV of the disease, is also a factor in infertility due to elevated concentrations of prostaglandins and proinflammatory cytokines, failed endometrial reciprocity. Among the uterus abnormalities in the violation of fertility are the uterine membrane, leiomyoma with submucosal localization of the node and uterine synechiae. As part of a comprehensive examination of patients with infertility, it is necessary to take into account the study of thyroid function.

CHANGES IN LIPID PROFILE AND LIPOTOXICITY IN PEOPLE WITH HYPOTALAMIC (MORBID) OBESITY

Accumulation of visceral fat causes a lipotoxic effect on the hypothalamus. Hypothalamic inflammation closes a vicious circle, aggravating the disturbance of neuroendocrine mechanisms to regulate metabolic balance, including coordination of energy consumption (during basic metabolism, physical activity, thermogenesis) and proper feeding behavior, the study group included 62 individuals (women) with obesity. Each patient met the diagnostic criteria for idiopathic hypothalamic syndrome – the neuroendocrine form with obesity.The current study shows that obesity of hypothalamic origin is associated with a dyslipidemia that progresses simultaneously with the increase of the body mass index.

Markers of endothelial dysfunction in adolescent and young patients with hypothalamic syndrome

Background: Endothelial dysfunction is the first but reversible stage of atherosclerosis. A change in the functional state of the vascular endothelium, especially of a growing organism, can be the basis for the development of many diseases in adulthood. The study of the structural and functional state of peripheral vessels of adolescents with hypothalamic syndrome is extremely important for understanding of the mechanisms of formation of cardiometabolic risks.Aims: to compare the structural and functional state of the vascular wall of young men with hypothalamic syndrome and constitutionally exogenous obesity.Methods: During the study 360 males were examined (average age 21.27±2.44 years) and divided into 3 groups: group 1 -with hypothalamic syndrome (n=242); group 2 - with constitutionally exogenous obesity (n=98); control group - practically healthy individuals (n=20). Hypothalamic syndrome was verified in the presence of a symptom complex, including obesity and pink striae. Cross-group comparative analysis of results of clinical, laboratory and instrumental examination was carried out.Results: In the group of patients with hypothalamic syndrome, endothelium-dependent vasodilatation (9.44±1.26 versus 10.37±1.21%; p=0.001) and endothelium-independent vasodilatation (10.29±1.28 versus 11.29±1.14%; p=0.001) is worse than in the group of patients with constitutionally exogenous obesity, and the rate of endothelium-dependent vasodilatation is lower than the generally accepted norm. In addition, among patients with hypothalamic syndrome, the stiffness of the vascular wall is higher than among patients with constitutionally exogenous obesity (15.47±2.58 versus 13.24±3.84%; p=0.001). Statistically significant correlations were revealed between the structural and functional state of peripheral arteries and hemodynamic, hormonal, metabolic changes, and the level of C-reactive protein (p<0.05).Conclusions: The obtained data indicate a deterioration of endothelial function and an increase in vascular wall stiffness in obese patients, regardless of its etiology. The patients with hypothalamic syndrome have more pronounced structural and functional changes in the vascular wall are observed than patients with constitutionally exogenous obesity.

Clinical aspects of autoimmune hypothalamitis, a variant of autoimmune hypophysitis: Experience from one center

Objective Autoimmune hypothalamitis (AHT) is a rare inflammatory disorder that involves the hypothalamus. It remains unclear whether autoimmune hypophysitis (AH) and AHT represent different diseases or different aspects of the same disease. Thus, further investigation of AHT is required. Methods A retrospective review of medical and pathological records of AHT patients from the Chinese PLA General Hospital were examined from January 1, 2005 to May 1, 2017. Clinical data, treatments, and outcomes were investigated. Results Five female patients were identified (median age, 42.6 years). Symptoms included central diabetes insipidus, hypopituitarism, hyperprolactinemia, headache, and hypothalamic syndrome. The following hormonal deficits were noted: follicle-stimulating hormone, luteinizing hormone, adrenocorticotropic hormone, thyroid stimulating hormone, and growth hormone. One patient underwent high-dose methylprednisolone pulse treatment (HDMPT) and azathioprine plus intensity modulated radiation therapy (IMRT), and two patients underwent HDMPT and two rounds of replacement therapy. During follow-up, one patient died because of non-compliance with therapy and the others were in remission or they recovered. Conclusions AHT had similar MRI results, pathology, and treatment compared with AH. Thus, it may be a subtype of AH, and AHT patients may also show hypothalamic syndrome.

Posterior hypothalamus-sparing surgery improves outcome after childhood craniopharyngioma

Objective Quality of life (QoL) is frequently impaired in childhood-onset craniopharyngioma (CP) by hypothalamic syndrome. The debate, whether pretreatment hypothalamic involvement (HI) has apriori prognostic impact or surgical hypothalamic lesions (HL) determine outcome, is controversial. Design Survival and outcome of CPs recruited between 2007 and 2014 in KRANIOPHARYNGEOM 2007 were analyzed with regard to reference-confirmed presurgical HI and surgical HL. Methods Radiological findings, BMI and QoL were assessed at diagnosis and during follow-up. QoL was assessed using Pediatric Quality of Life (PEDQOL) questionnaire. Results One hundred sixty-nine CPs were included presenting with no HI (n = 11), anterior (n = 49) and anterior + posterior (a + p) HI (n = 109) prior to surgery. The latter 109 were analyzed for postoperative HL (no lesion: n = 23, anterior HL: n = 29, a + pHL: n = 57). Progression-free survival (PFS) was higher after complete resection. The highest PFS was observed in CP with a + pHL, especially when compared between non-irradiated subgroups (P = 0.006). Overall survival (OS) rates were 1.0 in all subgroups. CP with a + pHL developed higher BMI (P ≤ 0.001) during follow-up compared between subgroups. 55/109 pts with a + pHI completed PEDQOL at diagnosis (48/109 at 3 years follow-up). QoL was worse for a + pHL patients in terms of physical, social and emotional functionality when compared with the anterior HL and no HL subgroup. BMI development and QoL during follow-up were similar for patients with anterior HL and without HL. Conclusions Posterior hypothalamus-sparing surgical strategies are associated with higher QoL, decreased development of obesity and lower PFS in CP.

Maternal obesity and child health at different ages

In recent years, the number of obese women of childbearing age has increased significantly. The aim of this study was to reveal the influence of maternal obesity during pregnancy on the longterm health of the offspring. This study was performed in the outpatient clinic in St. Petersburg with 76 adolescents with chronic diseases aged from 6 to 17 years. The mean age of the examined was 12.67 ± 3.19 years; the ratio of girls to boys was 6 : 7. Children were divided into 2 groups: the main group included 26 adolescents whose mothers were obese before and during pregnancy. 50 teen agers from mothers with normal BMI during pregnancy presented comparison group. The main group and the comparison group did not differ in age and sex. The children were examined by a pediatrician. Data on the transferred diseases are obtained from an outpatient card. It has been established that maternal obesity may be considered a risk factor for miscarriage, having low birth weight babies or babies weighing more than 4 kg, as well as a risk factor for rickets, parathrophies and functional constipation in the first year of life. In adolescentes, obesity, euthyroid goiter, hypothalamic syndrome and other endocrinopathies are typical for children born from obese mothers. Several medical conditions related to obesity such as chronic pancreatitis, hiatal hernia, iron deficiency anemia and orthopaedic foot and ankle pathology are commonly seen in children of obese mothers. Maternal obesity is associated with diseases of children not only in the period of newborn, but also in adolescence.