Background: Craniopharyngioma is a benign tumor originating from the sellar region. Damages in this area caused by the tumor itself, surgery, or radiotherapy may result in severe hypothalamic-pituitary dysfunction (HPD) and eventually lead to a significant impairment in the long-term quality of life of patients. This study aimed to investigate the characteristics of HPD in patients with adamantinomatous craniopharyngioma (ACP) or papillary craniopharyngioma (PCP) and to identify the factors affecting HPD after surgery.Methods: In this single-center retrospective study, a total of 742 patients with craniopharyngioma were included. The neuroendocrine function of these patients before and after surgery was investigated. The differences in hypothalamic-pituitary function between the ACP and PCP groups were compared. The factors influencing the aggravation of HPD after surgery were identified.Results: The median follow-up after surgery was 15 months. Before surgery, the proportion of patients with diabetes insipidus (DI) and hyperprolactinemia in the PCP group was significantly higher than that in the ACP group (P<0.01), and the proportion of patients with adrenocortical hypofunction in the PCP group was significantly lower than that in the ACP group (P=0.03). Most cases of ACP originated in the sellar region, while most cases of PCP originated in the suprasellar region (P<0.01). More patients experienced adenohypophyseal hypofunction, DI, and hypothalamic obesity at postoperative follow-up than at onset in both the ACP and PCP groups (both P<0.01), with a higher increase observed in the ACP group (P<0.01). Older age at CP onset, tumor recurrence or progression, and ACP type were risk factors for postoperative aggravation of HPD in CP patients.Conclusion: Surgical treatment significantly aggravated HPD in both the ACP and PCP groups, but the specific characteristics and risk factors leading to aggravation were different between the two groups.

Background: The pineal lesion affecting melatonin is a rare cause of central precocious puberty by decreasing the inhibition of hypothalamic–pituitary–gonadal axis. Germ cell tumor secreting human chorionic gonadotropin is a rare cause of peripheral puberty. Case presentation: A 5.8-year-old male presented facial hair and phallic growth, deepened voice, and accelerated growth velocity for 6 months. The elevated human chorionic gonadotropin level with undetectable gonadotropin levels indicated peripheral precocious puberty. Brain imaging revealed a pineal mass and further pathology indicated the diagnosis of teratoma. During chemoradiotherapy with operation, the elevated human chorionic gonadotropin level reduced to normal range, while the levels of gonadotropins and testosterone increased. Subsequently, progressing precocious puberty was arrested with gonadotrophin-releasing hormone analog therapy. Previous cases of transition from peripheral precocious puberty to central precocious puberty were reviewed. The transitions were caused by the suddenly reduced feedback inhibition of sex steroid hormones on gonadotropin releasing hormone and gonadotropins. Conclusions: For patients with human chorionic gonadotropin-secreting tumors, gonadotropin levels increase prior to sex steroid decrease, seems a sign of melatonin-related central PP related to melatonin.

Intracranial germ cell tumors (iGCTs) are rare malignant neoplasms that mainly affect children and adolescents. The incidence, clinical presentation, and prognosis of iGCTs exhibit high heterogeneity. Previous studies have primarily focused on eliminating tumors, reducing tumor recurrence, and improving survival rates, while neglecting the impact of the tumors and their treatment on neuroendocrine function. Throughout the entire course of the disease, neuroendocrine dysfunction may occur and is frequently overlooked by oncologists, neurosurgeons, and radiologists. Endocrinologists, however, are more interested in this issue and have varying priorities at different stages of the disease. From onset to the diagnostic phase, most patients with iGCTs may present with symptoms related to impaired neuroendocrine function, or even experience these symptoms as their first indication of the condition. Particularly, a minority of patients with sellar/suprasellar lesions may exhibit typical imaging features and elevated tumor markers long after the onset of initial symptoms. This can further complicate the diagnosis process. During the peritumor treatment phase, the neuroendocrine function shows dynamic changes and needs to be evaluated dynamically. Once diabetes insipidus and dysfunction of the hypothalamic–pituitary–adrenal and hypothalamic–pituitary–thyroid axes occur, hormone replacement therapy should be administered promptly to ensure successful tumor treatment for the patient. Subsequently, during the long-term management phase after the completion of tumor treatment, the evaluation of growth and development as well as corresponding hormone replacement therapy are the most concerning and complex issues. Thus, this paper reviews the interest of endocrinologists in iGCTs at different stages.

Long-term stimulation of thyroid follicular epithelium by high growth hormone (GH) and insulin-like growth factor-1 (IGF-1) in patients with acromegaly can lead to thyroid dysfunction, goiter, thyroid nodules, and even thyroid cancer and thyroid-associated ophthalmopathy (TAO). Excessive GH/IGF-1 promotes goiter and thyroid nodule formation, which can be reversed by normalizing the IGF-1 levels with surgery or medical treatment. Whether patients with acromegaly have an increased risk of thyroid cancer remains controversial, and routine thyroid ultrasonography and regular cancer screening are recommended in such cases, especially when the nodules possess malignant propensity. TAO is an autoimmune disease and newer treatments are being discovered against it. Recent studies have reported that the IGF-1 receptor (IGF-1R) plays an important role in the pathogenesis of TAO, and the IGF-1R inhibitor teprotumumab involves significantly improved disease endpoints in patients with active TAO. Thyroid-stimulating hormone (TSH) receptor (TSHR) and IGF-1R co-immunoprecipitate in orbital and thyroid tissues to form a functional complex; thus, combined therapy targeting TSHR and IGF-1R may be more effective than single therapy.

Objective. Intracranial germ cell tumors with isolated pituitary stalk involvement are rare. Early recognition and long-term monitoring deserve further exploration. Methods. A retrospective study reviewing eleven intracranial germ cell tumor patients with isolated pituitary stalk involvement was performed. Results. Seven boys and four girls who presented with a hyperintense pituitary stalk on postcontrast T1-weighted magnetic resonance imaging without a posterior pituitary signal were included. The average maximum width of the pituitary stalk was 5.2±1.6mm. Polydipsia and polyuria occurred in all patients, followed by growth retardation, fatigue, and amenorrhoea. Eight patients (72) had concomitant partial anterior pituitary hormone deficiency. Seven patients initially had elevated human chorionic gonadotropin levels. After chemoradiotherapy, ten patients attended follow-up. Central diabetes insipidus remained in all survivors, and four (40) of them had concomitant partial anterior pituitary hormone deficiency, primarily of growth hormone and insulin-like growth factor-1. The causes of the delayed diagnosis of previous studies were mainly negative tumor markers and the initial pathological diagnosis of autoimmune diseases. Conclusion. Isolated pituitary stalk lesions could be a signal of intracranial germ cell tumors, especially coexisting with diabetes insipidus, hypopituitarism, and a worse response to glucocorticoid therapy. Negative results of tumor markers and pathology could not exclude the diagnosis. Chemoradiotherapy is an effective therapy, leaving mild-t-moderate hypothalamus-pituitary dysfunction. This rare neuroimaging feature may be used as a factor to predict long-term neuroendocrine outcomes.

Children with intracranial germ cell tumors may present premature sexual development via either GnRH-dependent cause or GnRH-independent cause. We conducted a single-center retrospective study on thirty-seven precocious puberty patients with intracranial germ cell tumors and twenty-five age-matched prepubertal patients with elevated human-chorionic-gonadotropin levels. Classification of precocious puberty was derived from human-chorionic-gonadotropin, gonadotropin, sex steroid levels and their changes. Five boys were assigned to GnRH-dependent group (G1). Thirty-one boys and one girl were assigned to GnRH-independent group (G2) with a median human-chorionic-gonadotropin of 76.75 (8.29-2747) IU/L. Seven boys and eighteen girls were conducted as controls, with a median human-chorionic-gonadotropin of 17.12 (2.91-1062) IU/L. Patients in G1 had constant pubertal LH and testosterone levels after tumor complete response. Patients in G2 had hCG levels that decreased simultaneously with testosterone/estradiol levels, prior to tumor regression. The differences in hCG levels and the gender ratio were significant between G2 and controls (p=0.006 and p<0.001, separately). Among intracranial germ cell tumor patients with positive human-chorionic-gonadotropin, boys with significantly higher human-chorionic-gonadotropin levels more easily developed precocious puberty. Our results suggest that, GnRH-independent precocious puberty commonly regress together with tumor regression. In comparison, results were inconclusive in tying tumor regression to the regression of GnRH-dependent PP.