Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant hereditary disorder caused by germline mutation of the MEN1 gene. It is characterized by tumors of the anterior pituitary gland, parathyroid glands, and endocrine pancreas. Thymic carcinoid tumor is uncommon and associated with a high mortality, but its natural history has not been investigated yet. We report a case of asymptomatic MEN 1 with a thymic carcinoid tumor. A 37-year-old man underwent a routine medical checkup and upper gastrointestinal endoscopy revealed a duodenal neuroendocrine tumor (NET). Further studies showed the coexistence of pancreatic tumor, parathyroid hyperplasia, pituitary adenoma, and thymoma. The patient underwent duodenal endoscopic mucosal resection, distal pancreatectomy, subtotal parathyroidectomy, and thymectomy. The pathological test revealed a duodenal NET, pancreatic NET, parathyroid hyperplasia, and thymic carcinoid tumor. He was treated for MEN 1. We report this asymptomatic case of MEN 1 with a literature review.
Adult ; Carcinoid Tumor ; Endoscopy, Gastrointestinal ; Germ-Line Mutation ; Humans ; Hyperparathyroidism ; Hyperplasia ; Islets of Langerhans ; Mortality ; Multiple Endocrine Neoplasia Type 1 ; Multiple Endocrine Neoplasia ; Natural History ; Neuroendocrine Tumors ; Pancreatectomy ; Parathyroid Glands ; Parathyroidectomy ; Pituitary Gland, Anterior ; Pituitary Neoplasms ; Thymectomy ; Thymoma

Multiple endocrine neoplasia type 1 (MEN1) syndrome is characterized by combined occurrence of tumors of endocrine glands including the parathyroid, the pancreatic islet cells, and the anterior pituitary gland. Parathyroid involvement is the most common manifestation and usually the first clinical involvement inMEN1 syndrome, followed by gastroentero-pancreatic neuroendocrine tumors (NETs). Here we present a case where the patient initially presented with metastatic gastric NET and a single parathyroid adenoma was detected incidentally on ⁶⁸Ga-DOTANOC PET/CT done as part of post ¹⁷⁷Lu-DOTATATE therapy (PRRT) follow-up. Further ¹⁸F-fluorocholine PET/CT showed four adenomas for which the patient subsequently underwent subtotal parathyroidectomy.
Adenoma ; Endocrine Glands ; Follow-Up Studies ; Gastrinoma ; Humans ; Hyperparathyroidism ; Islets of Langerhans ; Multiple Endocrine Neoplasia Type 1 ; Neuroendocrine Tumors ; Parathyroid Neoplasms ; Parathyroidectomy ; Pituitary Gland, Anterior ; Positron-Emission Tomography and Computed Tomography ; Receptors, Somatostatin ; Somatostatin

Pituitary stalk interruption syndrome (PSIS) involves the occurrence of a thin or absent pituitary stalk, hypoplasia of the adenohypophysis, and ectopic neurohypophysis. Diagnosis is confirmed using magnetic resonance imaging. Patients with PSIS have a variable degree of pituitary hormone deficiency and a wide spectrum of clinical manifestations. The clinical course of the disease in our patient is similar to that of a syndrome of inappropriate antidiuretic hormone secretion. This is thought to be caused by failure in the suppression of vasopressin secretion due to hypocortisolism. To the best of our knowledge, there is no case report of a patient with PSIS presenting with hyponatremia as the first symptom in Korean children. Herein, we report a patient with PSIS presenting severe recurrent hyponatremia as the first symptom, during adolescence and explain the pathophysiology of hyponatremia with secondary adrenal insufficiency.
Adolescent ; Adrenal Insufficiency* ; Child ; Delayed Diagnosis* ; Diagnosis ; Humans ; Hyponatremia* ; Hypopituitarism ; Inappropriate ADH Syndrome ; Magnetic Resonance Imaging ; Pituitary Gland* ; Pituitary Gland, Anterior ; Pituitary Gland, Posterior ; Vasopressins

Metastasis to the pituitary gland from systemic cancer is a rare condition. The breast and lung are the most common sites of primary tumor metastasis. Most often, they occur in the setting of widespread metastatic disease, which most frequently occurs in elderly patients. However, an increase in the incidence of solitary pituitary metastasis of breast cancer as the first recurrence has been reported. Diabetes insipidus is the most frequent symptom at presentation, and visual field defects or cranial nerve deficits are common symptoms of pituitary metastasis. Unlikely these symptoms, deficiencies of anterior pituitary hormones may only become evident in critical situation because symptoms are of an insidious onset and sometimes nonspecific. We report here on a rare case of solitary pituitary metastasis from breast cancer presenting as hyponatremia without other symptoms.
Aged ; Breast Neoplasms* ; Breast* ; Cranial Nerves ; Diabetes Insipidus ; Epidermal Growth Factor* ; Humans ; Hyponatremia ; Incidence ; Lung ; Neoplasm Metastasis* ; Pituitary Gland ; Pituitary Hormones, Anterior ; Receptor, Epidermal Growth Factor* ; Recurrence ; Visual Fields

OBJECTIVETo observe the effect of Bushen Tiaojing Recipe (BTR) and Xiaoyao Pill (XYP) on the morphology and sex hormones secretion of adenohypophysis and ovaries in androgen-induced sterile rats (ASR).

METHODSFifty 9-day old SD female rats randomly recruited from total 60 rats were subcutaneously injected with testosterone propionate to establish the ASR model. And the rest 10 rats were recruited as the normal group. Thirty successfully modeled rats were recruited and randomly divided into the model group, the BTR group (administered with BTR suspension), and the XYP group (administered with XYP suspension), 10 in each group. Five weeks later, rats were decapitated in the proestrus. Serum levels of estradiol (E2), progesterone (P), testosterone (T), follicle-stimulating hormone (FSH), and luteinizing hormone (LH) were detected by radioimmunoassay. The morphologies of adenohypophysis and ovary were observed after HE staining.

RESULTSCompared with the normal group, serum E2 and T levels increased, while FSH and LH levels decreased in the model group (all P < 0.01). The morphology of adenohypophysis and ovary was abnormal in the model group. Compared with the model group, serum E2 and T levels decreased, while FSH and LH levels increased in the BTR group and the XYP group (P < 0.05, P < 0.01). Besides, E2 and T levels in the BTR group and FSH levels in the XYP group restored to normal (all P > 0.05). The damaged structure of adenohypophysis and ovary got restored to different degrees.

CONCLUSIONBTR and XYP both could improve ovulation failure.

Androgens ; adverse effects ; Animals ; Drugs, Chinese Herbal ; pharmacology ; Estradiol ; blood ; Female ; Follicle Stimulating Hormone ; blood ; Infertility, Female ; chemically induced ; drug therapy ; Luteinizing Hormone ; blood ; Ovary ; drug effects ; Ovulation ; Pituitary Gland, Anterior ; drug effects ; Progesterone ; blood ; Rats ; Rats, Sprague-Dawley ; Testosterone ; blood

Functional defects of the pituitary gland are a rare cause of pubertal delay. The pituitary stalk is an important structure that connects the hypothalamus and pituitary gland. A defect in fusion of the pituitary stalk and anterior pituitary gland will block the function of the anterior pituitary gland. A 28-year-old man was referred to our clinic with poorly developed secondary sexual characteristics. He had undeveloped facial, axillary, and pubic hair and was Tanner stage I. Laboratory tests gave random serum testosterone < 0.025 ng/mL, luteinizing hormone (LH) < 0.1 mIU/mL, follicle-stimulating hormone (FSH) 0.626 mIU/mL, thyroid-stimulating hormone (TSH) 6.85 microIU/mL, and fT4 6.96 pmol/L. Sella magnetic resonance imaging (MRI) showed no pituitary stalk enhancement. The response in the combined pituitary function test revealed multiple hormonal defects, while the TSH response to thyrotropin-releasing hormone (TRH) was exaggerated and delayed. Therefore, we concluded that pituitary stalk dysgenesis had led to hypothalamic-type panhypopituitarism.
Adult ; Follicle Stimulating Hormone ; Hair ; Humans ; Hypopituitarism ; Hypothalamus ; Luteinizing Hormone ; Magnetic Resonance Imaging ; Pituitary Function Tests ; Pituitary Gland ; Pituitary Gland, Anterior ; Puberty, Delayed ; Testosterone ; Thyrotropin ; Thyrotropin-Releasing Hormone

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant hereditary disorder characterized by the combined occurrence of tumors of the parathyroid gland, exocrine pancreas, and anterior pituitary gland. Calcitonin-secreting pancreatic endocrine tumors are rare, and calcitonin-secreting pancreatic endocrine tumors with MEN1 have not been reported in Korea. A 46-year-old woman was admitted for a right breast cancer operation. Abnormal blood chemistry findings were hypercalcemia and elevated calcitonin. The patient was diagnosed with a calcitonin-secreting pancreatic endocrine tumor, left thyroid papillary carcinoma, right breast invasive ductal carcinoma, a thymic carcinoid tumor, left adrenal adenoma, uterine myoma, and adenomyosis by computed tomography scan, and with pituitary macroadenoma by brain magnetic resonance imaging. We present this case with a review of the literature, because it is the first reported calcitonin-secreting pancreatic endocrine tumor with MEN 1 in Korea.
Adenoma ; Adenomyosis ; Brain ; Breast ; Breast Neoplasms ; Calcitonin ; Carcinoid Tumor ; Carcinoma, Ductal ; Carcinoma, Papillary ; Female ; Humans ; Hypercalcemia ; Korea ; Magnetic Resonance Imaging ; Middle Aged ; Multiple Endocrine Neoplasia ; Multiple Endocrine Neoplasia Type 1 ; Myoma ; Pancreas, Exocrine ; Parathyroid Glands ; Pituitary Gland, Anterior ; Thyroid Gland

Growth hormone (GH) is produced in a select population of cells, somatotropes, located in the anterior pituitary gland. GH is released into the general circulation where it interacts with multiple peripheral tissues through its receptor, GH receptor, to regulate growth and metabolic function. GH-releasing hormone (GHRH) and somatostatin are the primary positive and negative regulators of GH secretion, respectively. More recently, ghrelin has emerged as an additional stimulatory hormone for GH release. In humans, GH levels decrease in states of nutrient excess, such as obesity, and increase in response to nutrient deprivation, such as fasting, type 1 diabetes, and anorexia nervosa. Considering that GH regulates metabolism of carbohydrate, lipid, and protein, clarifying the mechanisms by which metabolic changes alter pituitary GH synthesis and secretion will increase our knowledge on the pathophysiology and treatment of metabolic diseases. In this review, the effect of nutrient excess and nutrient deficiency on GH-axis function in humans and other mammals will be summarized, with particular emphasis on studies exploring the direct effects of systemic signals, including insulin-like growth factor 1 (IGF-1) and insulin, on somatotrope function. Additionally, new mouse models with somatotrope-specific knockout of IGF-1 and insulin receptors generated by using the Cre/loxP system will be discussed.
Animals ; Anorexia Nervosa ; Fasting ; Ghrelin ; Growth Hormone ; Humans ; Insulin ; Insulin-Like Growth Factor I ; Mammals ; Metabolic Diseases ; Mice ; Obesity ; Pituitary Gland, Anterior ; Receptor, Insulin ; Somatostatin

Most of the patients with hemorrhagic fever with renal syndrome (HFRS) by Hantaviruses recover completely. However, the prevalence of hypopituitarism as sequel of HFRS may be approximately 10%. However, it is rare for hyponatremia to present as a manifestation of hypopituitarism due to HFRS. A 42-year-old Asian woman presented with anorexia, nausea, and vomiting. She had a history of HFRS 7 years ago. During her first hospital admission, her serum sodium was 103 mmol/L. The rapid adrenocorticotropic hormone (ACTH) stimulation test showed no abnormal findings suggesting the possibility of adrenal insufficiency. After she underwent fluid therapy for 5 days, her serum sodium level was slightly increased to 131 mmol/L, and her clinical symptoms were improving. The next day after she was discharged, she suffered from the same symptoms and was admitted to our hospital again. On the day of her second admission, her serum sodium was 117 mmol/L. A combined pituitary function test showed diminished function of the anterior pituitary gland, which led to secondary adrenal insufficiency. Magnetic resonance imaging findings of her sella were compatible with empty sella turcica. After the replacement of prednisolone, her serum sodium levels returned to normal. Pituitary dysfunction should be included in the differential diagnosis of severe hyponatremia, especially in patients who suffered HFRS. This case also suggests that the rapid ACTH stimulation test has limitations with regard to the differential diagnosis of primary or secondary adrenal insufficiency.
Adrenal Insufficiency ; Adrenocorticotropic Hormone ; Adult ; Anorexia ; Asian Continental Ancestry Group ; Diagnosis, Differential ; Empty Sella Syndrome ; Female ; Fluid Therapy ; Hantavirus ; Hemorrhagic Fever with Renal Syndrome ; Humans ; Hyponatremia ; Hypopituitarism ; Magnetic Resonance Imaging ; Nausea ; Pituitary Function Tests ; Pituitary Gland, Anterior ; Prednisolone ; Prevalence ; Sodium ; Vomiting

Acute encephalopathies can be defined as an acute central nervous system (CNS) insult, due to an underlying pathology. The clinical symptoms almost always include an acute state of confusion and cognitive impairment. Toxic encephalopathies can occur acutely or chronically depending on the toxic drugs and other substances as well as the individual metabolism of the drug. The organs acutely affected include the heart, lung and kidneys. However, the brain, spinal cord and sympathetic nerves can be affected chronically. If the toxic substance passes through the bloodbrain barrier into the hypothalamus and the posterior pituitary gland, the result can be diabetes insipidus. If the substance affects the anterior pituitary gland, the result can include hormone dysfunction, impaired immune function and altered cognition or personality. We report a patient that developed acute toxic encephalopathy after the prescribed dose of oxycodone was exceeded.
Brain ; Central Nervous System ; Cognition ; Diabetes Insipidus ; Heart ; Humans ; Hypothalamus ; Kidney ; Lung ; Neurotoxicity Syndromes ; Oxycodone ; Pituitary Gland, Anterior ; Pituitary Gland, Posterior ; Spinal Cord