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Key Clinical Message: Prompt and precise diagnosis of pituitary hyperplasia secondary to primary hypothyroidism (PHPH) is crucial to avoid unwarranted pituitary surgery and potential permanent impairment. Although PHPH may present similarly to pituitary adenoma, it is responsive to thyroxine therapy, underscoring the critical role of differential diagnosis in the treatment of adolescent patients. [ABSTRACT FROM AUTHOR]

Acromegaly due to ectopic secretion of growth hormone-releasing hormone (GHRH) is a rare disorder. The signs and symptoms of ectopic acromegaly are indistinguishable from acromegaly due to a somatotroph adenoma. A 35-yearold female presented with secondary amenorrhea for 10 years, intermittent headache, and reduced vision in both eyes for 4 years, which worsened over 4 months before presentation. Additionally, she was diagnosed with uncontrolled diabetes mellitus. On examination, she had coarse facial features, a fleshy nose, and acral enlargement. She had diminished visual acuity (left>right) and bitemporal hemianopia on perimetry. Biochemical investigations revealed elevated IGF-1 [588 ng/ml, reference range (RR) 100--242], markedly elevated basal growth hormone (>80 ng/ml; RR, 0.12--9.88), and hyperprolactinemia in the tumoral range (832 ng/ml; RR, 5--25). MRI sella demonstrated a 22x30x34mm sellar-suprasellar mass with T2 hypointensity. Chest imaging revealed a 75x87x106mm left lung mass, which was found to be a welldifferentiated neuroendocrine tumor (NET) on biopsy. Plasma GHRH levels were elevated [38,088 ng/l; RR, <250--300], and a diagnosis of ectopic acromegaly secondary to lung neuroendocrine tumor was considered. During workup, the patient developed in-hospital pituitary apoplexy, which improved with medical management. After a left pneumonectomy, her clinical features of acromegaly improved, her diabetes underwent remission, and there was a marked reduction in plasma GHRH and pituitary size. Histopathology was suggestive of a neuroendocrine tumor, with immunohistochemistry positive for GHRH and negative for prolactin. Her final diagnosis was ectopic acromegaly due to GHRH secreting a lung NET with pituitary somatotroph and lactotroph pituitary hyperplasia and apoplexy in the hyperplastic pituitary. [ABSTRACT FROM AUTHOR]

Thyrotroph hyperplasia is commonly present but remains largely undiagnosed in primary hypothyroidism. It is easily reversible with thyroid replacement therapy. If imaging is performed prior to biochemical evaluation, then patients may undergo pituitary surgery unnecessarily. We present the case of a 30-year-old man with thyrotroph hyperplasia caused by profound primary hypothyroidism leading to hypopituitarism that resolved after levothyroxine replacement therapy. We will discuss the current literature regarding pituitary hyperplasia in primary hypothyroidism in adults. [ABSTRACT FROM AUTHOR]

Purpose: In a patient with elevated thyroid stimulating hormone (TSH, >50 µIU/ml) with sellar mass, it is crucial to differentiate isolated pituitary hyperplasia (IPH) from primary hypothyroidism coexisting with nonfunctioning pituitary adenoma (PHCNFPA) pre-operatively to avoid unwarranted surgery in the former condition. Here, we describe patients having pituitary mass/enlargement with markedly elevated TSH (>50 µIU/ml) and attempt to find the differentiating features between IPH and PHCNFPA. Methods: This is a retrospective study conducted at a tertiary care center. Case records of patients presenting between January 2020 and December 2022 with elevated TSH (>50 µIU/ml) for whom magnetic resonance imaging (MRI) of the sella was available were reviewed. Demographic details, symptomatology, clinical examination findings, thyroid function tests, data on pituitary hormonal excess and deficiencies, MRI findings, and details regarding levothyroxine supplementation were noted. Based on the final diagnosis, the patients were categorized into two groups: PHCNFPA and IPH. Results: Five and 11 patients were diagnosed with PHCNFPA and IPH, respectively. The median (IQR) age at presentation of patients with PHCNFPA was significantly higher than that of IPH patients [37 (28–60.5) vs. 21 (10–21.5) years, p: 0.002]. A longer duration of hypothyroid symptoms was noted in the IPH group whereas visual field defects and corticotropin deficiency were more frequent and the pituitary lesion size was greater in PHCNFPA. Thyroid function tests were not different between the two groups. The pituitary enlargement in IPH was initially an increase in pituitary height that progressed to symmetrical nipple-, dome- or tent-shaped enlargement. Besides this characteristic enlargement pattern, isointense appearance on T1-weighted and T2-weighted images, homogeneous contrast enhancement, and prompt regression of pituitary lesion with levothyroxine replacement were characteristic of IPH whereas heterogeneous enhancement, cystic/hemorrhagic change, and ≥Knosp III invasion were characteristic of PHCNFPA. Peripheral rim enhancement and Knosp I-II parasellar extension were not uncommon in patients with IPH and did not distinguish it from PHCNFPA. Conclusions: The present study reports the radiological evolution of IPH and a unique series of PHCNFPA along with the distinguishing characteristics between them. [ABSTRACT FROM AUTHOR]

Key Clinical Message Prompt and precise diagnosis of pituitary hyperplasia secondary to primary hypothyroidism (PHPH) is crucial to avoid unwarranted pituitary surgery and potential permanent impairment. Although PHPH may present similarly to pituitary adenoma, it is responsive to thyroxine therapy, underscoring the critical role of differential diagnosis in the treatment of adolescent patients.

Acromegaly due to ectopic secretion of growth hormone-releasing hormone (GHRH) is a rare disorder. The signs and symptoms of ectopic acromegaly are indistinguishable from acromegaly due to a somatotroph adenoma. A 35-year-old female presented with secondary amenorrhea for 10 years, intermittent headache, and reduced vision in both eyes for 4 years, which worsened over 4 months before presentation. Additionally, she was diagnosed with uncontrolled diabetes mellitus. On examination, she had coarse facial features, a fleshy nose, and acral enlargement. She had diminished visual acuity (left>right) and bitemporal hemianopia on perimetry. Biochemical investigations revealed elevated IGF-1 [588 ng/ml, reference range (RR) 100–242], markedly elevated basal growth hormone (>80 ng/ml; RR, 0.12–9.88), and hyperprolactinemia in the tumoral range (832 ng/ml; RR, 5–25). MRI sella demonstrated a 22×30×34mm sellar-suprasellar mass with T2 hypointensity. Chest imaging revealed a 75×87×106mm left lung mass, which was found to be a well-differentiated neuroendocrine tumor (NET) on biopsy. Plasma GHRH levels were elevated [38,088 ng/l; RR,

Hypothyroidism is a frequently diagnosed endocrine disorder. Common signs and symptoms include fatigue, cold intolerance, hoarseness, dry skin, constipation, a slow relaxation phase of deep tendon reflexes, and bradycardia. However, some patients may exhibit atypical signs and symptoms, which can result in diagnostic confusion. Pituitary hyperplasia resulting from longstanding primary hypothyroidism was first described by Niepce in 1851. It is usually asymptomatic, but sometimes, in addition to symptoms of overt hypothyroidism, patients may complain of headaches, hypopituitarism, visual field impairment, and hyperprolactinemia. Furthermore, on imaging, pituitary hyperplasia can be mistaken for a pituitary adenoma. Distinguishing between the two is crucial, as their management differs; the former often responds to thyroid hormone replacement therapy, while the latter might need treatment with surgery and/or radiotherapy. Here we describe a patient who developed pituitary hyperplasia in the setting of longstanding uncompensated primary hypothyroidism due to a lack of compliance with levothyroxine replacement therapy. We also review the clinical, laboratory, and radiologic findings of the case reports available in the literature up to now in order to improve the knowledge and the care of the disease. [ABSTRACT FROM AUTHOR]

We describe a 13-year-old girl who presented at her local hospital with a diffuse goiter and had discrepant thyroid function test (TFT) of elevated free T4 (FT4), free T3 (FT3) levels with mildly elevated thyroid-stimulating hormone (TSH) and a pituitary magnetic resonance imaging (MRI) report of a pituitary hyperplasia. She was referred to our hospital where a repeat TFT found low FT4 and high TSH levels, and high levels of antithyroid peroxidase (anti-TPO) and anti-thyroglobulin (anti-TG) antibodies, leading to the diagnosis of Hashimoto’s thyroiditis (HT) with overt primary hypothyroidism. The girl had a good response after daily 100 µg levothyroxine treatment for 8 months with decreased goiter size along with disappearance of the pituitary mass. However, her FT4 and FT3 levels were elevated while the TSH was in the high normal range, although at this time there were no signs of hyperthyroidism. A genetic study confirmed our provisional diagnosis that the patient had a p.Pro453Thr monoallelic loss-of-function mutation of the thyroid hormone receptor beta (THRB) gene, suggesting the diagnosis of coexisting resistance to thyroid hormone-β (RTHβ) and HT in this patient.

A carotid-cavernous fistula is a rare abnormal communication between carotid vasculature and the cavernous sinus. Development of a carotid-cavernous fistula often results from trauma, but may be spontaneous in the setting of predisposing risk factors. Suspicion for a spontaneous fistula is understandably low on routine non-contrast imaging. In this article, we present a case of a carotid-cavernous fistula initially presenting with the potentially underrecognized imaging manifestation of diffuse pituitary enlargement identified on a non-contrast CT, later revealed to be due to the presence of the fistula.

We describe a 14-year-old girl who was referred for management of a prolactin-secreting pituitary adenoma as she had persistent milky discharge from her nipples, an elevated prolactin level and pituitary enlargement. Upon reviewing the medical history, it was noted that she had a history of secondary amenorrhea for 1 year, and had undergone an oophorectomy for twisted left ovarian cyst 5 months earlier. The physical examination found that she had a goiter, short stature and was relatively overweight. Based on these findings, it was thought that the patient likely had longstanding overt hypothyroidism. A thyroid function test (TFT) revealed a free thyroxine (FT4) level of 0.2 ng/dL and thyroid stimulating hormone (TSH) >100 mU/L, with high levels of antithyroid peroxidase (anti-TPO) and anti-thyroglobulin (anti-TG) antibodies, leading to the diagnosis of Hashimoto’s thyroiditis. After 8 months of levothyroxine treatment, the galactorrhea had disappeared, the pituitary enlargement had resolved and her menstruation had resumed normally, along with a 4-kg weight loss and 3-cm height gain. In summary, when evaluating a girl with ovarian cyst(s), especially if accompanied by other clinical findings like goiter, short stature, or menstrual irregularities, the physician should include hypothyroidism in the differential diagnosis. Early diagnosis and treatment of hypothyroidism can have a positive impact on the overall health and well-being of these patients, potentially preventing further complications related to both the thyroid disorder and ovarian cyst(s).

Hypothyroidism is a frequently diagnosed endocrine disorder. Common signs and symptoms include fatigue, cold intolerance, hoarseness, dry skin, constipation, a slow relaxation phase of deep tendon reflexes, and bradycardia. However, some patients may exhibit atypical signs and symptoms, which can result in diagnostic confusion. Pituitary hyperplasia resulting from longstanding primary hypothyroidism was first described by Niepce in 1851. It is usually asymptomatic, but sometimes, in addition to symptoms of overt hypothyroidism, patients may complain of headaches, hypopituitarism, visual field impairment, and hyperprolactinemia. Furthermore, on imaging, pituitary hyperplasia can be mistaken for a pituitary adenoma. Distinguishing between the two is crucial, as their management differs; the former often responds to thyroid hormone replacement therapy, while the latter might need treatment with surgery and/or radiotherapy. Here we describe a patient who developed pituitary hyperplasia in the setting of longstanding uncompensated primary hypothyroidism due to a lack of compliance with levothyroxine replacement therapy. We also review the clinical, laboratory, and radiologic findings of the case reports available in the literature up to now in order to improve the knowledge and the care of the disease.

In children, hypothyroidism usually presents non-specific symptoms; symptoms can emerge gradually, compromising a timely diagnosis. We report the case of a 13-year-old male, who was admitted to the hospital due to swelling of the torso and neck. Besides these symptoms, the child was healthy, except for a significant growth delay. Ultrasound evaluation and blood tests led to the diagnosis of myxedema secondary to severe hypothyroidism, which was due to autoimmune thyroiditis. Further investigations revealed pericardial effusion and pituitary hyperplasia, with hyper-prolactinemia. Treatment with levothyroxine led to edema regression and clinical, hemato-chemical and radiological improvement. After 6 months, growth velocity increased, although the recovery of growth already lost was not guaranteed. Brain MRI showed regression of pituitary hyperplasia. The diagnostic delay in this case was probably due to the patient's apparent good health, and the underestimation of growth restriction. This report underlines the importance of growth monitoring in adolescence, a critical period for identifying endocrine conditions; if undiagnosed, these conditions can lead to serious complications, such as myxedema in hypothyroidism, with potential effects beyond growth on multiple organs. [ABSTRACT FROM AUTHOR]

Objective: Childhood hydrocephalus patients treated by ventriculo-peritoneal (v.-p.) shunting are sometimes referred years after this therapy for evaluation of suspicious pituitary enlargement. Since pituitary size has been shown to depend on cerebrospinal fluid (CSF) pressure, we assume this phenomenon to be caused by shunt overdrainage. Therefore, we studied pituitary size and morphology in shunted hydrocephalus patients with radiological signs of high CSF drainage. Patients and methods: Retrospective study of pituitary size and morphology in 15 shunted patients with non-tumoral hydrocephalus and 7 shunted hydrocephalus patients due to childhood brain tumor compared to a population mean. In five brain tumor patients also pre- and postsurgical comparisons were performed. Results: Pituitary mid-sagittal size and pituitary volume were significantly higher in both hydrocephalus groups, compared to the population mean (midsagittal size t = 5.91; p < 0.001; pituitary volume, t = 3.03; p = 0.006). In patients available for pre- and postoperative comparison, there was also a significant increase in pituitary size and volume postoperatively (mean preoperative midsagittal height 2.54 ± 1.0 mm vs. 6.6 ± 0.7 mm post-surgery; mean pre-operative pituitary volume 120.5 ± 69.2 mm3 vs. 368.9 ± 57.9 mm3 post-surgery). Conclusion: Our results confirmed a significant increase in pituitary size and volume, mimicking pituitary pathology, after v.-p. shunt insertion. This phenomenon can be explained by the Monro–Kellie doctrine, stating that intracranial depletion of CSF—as caused by v.p. shunting—leads to compensatory intracranial hyperemia, especially in the venous system, with the consequence of engorged venous sinuses, most likely responsible for enlargement of the pituitary gland. [ABSTRACT FROM AUTHOR]

ObjectiveThis study aims to summarize the clinical characteristics of one teenager with autoimmune polyglandular syndrome (APS) type III C + D to improve the understanding of APS III C + D and its effect of thyroid function.MethodsThis article reported the clinical manifestations, laboratory examinations, treatment methods, and outcomes of an adolescent with anemia admitted to the Pediatrics Department of Tianjin Medical University General Hospital in July 2020 and reviewed the literature.ResultsA girl, aged 13 years and 1 month, was admitted to the hospital due to anemia for more than 4 years and episodic abdominal pain for 1 week. Four years ago, the girl went to a local hospital for “vitiligo”, and a routine blood test revealed anemia. The lowest hemoglobin (HGB) was 61 g/L, and the blood test revealed iron deficiency anemia. She had no menstrual cramps for 2 months. Urine routine showed protein 3+∼4+ and 258 red blood cells (RBCs)/high-power field. Urine protein was 3,380 mg/24 h. Free thyroxine was low, thyroid-stimulating hormone was >100 uIU/ml, thyroid peroxidase antibody was >1,000 IU/ml, and thyroglobulin antibody and thyrotropin receptor antibody were negative. Pituitary magnetic resonance imaging showed a mass in the sellar region with a uniform signal and a maximum height of about 15.8 mm. The result of the antinuclear antibody was 1:80 homogeneous type, and anti-dsDNA and anticardiolipin antibodies IgA and IgM were slightly higher. Thyroxine and iron were given for 1 month, menstruation resumed, and urine protein and RBC count decreased. After 5 months of treatment, free thyroid function, HGB, RBCs in urine, and pituitary returned to normal. Later, a renal biopsy showed changes in focal proliferative glomerulonephritis, and the girl was diagnosed with lupus glomerulonephritis type III. After 3 days of shock therapy with methylprednisolone, prednisone, mycophenolate mofetil, and other treatments were administrated for 1 year. At the time of writing, urine protein was 280 mg/24 h.ConclusionCo-occurrence of Hashimoto's thyroiditis, vitiligo, anemia, pituitary hyperplasia, and lupus nephritis is rare. It is very important to pay attention to the screening of thyroid function.

Sellar gangliocytomas (SGs) are rare, well-differentiated, low-grade neoplasias that commonly present along with a pituitary adenoma (PA). We describe a case of a 52-year-old woman with a 2-year history of headache, body weight increase, and recent onset of arterial hypertension and type 2 diabetes mellitus. Work-up tests revealed a normal hypophyseal profile, except for mild ACTH elevation, and a sellar mass on magnetic resonance imaging (MRI). A diagnosis of an enlarging pituitary macroadenoma was established, and to prevent symptom progression, the tumour was resected. Pathology showed 2 cell populations: ganglion and corticotrope cells. Three years after surgery, the patient no longer had a headache but persisted with arterial hypertension and type 2 diabetes mellitus. A literature review produced 207 cases of SGs. They typically present in women at 40 years of age and the most common clinical presentation are symptoms of acromegaly. Of the documented cases, 74 and 93% were treated with surgery alone or combined treatments (radiotherapy, radiosurgery, or pharmacotherapy), respectively. The majority of deaths associated with a SG came from the first half of the 20th century. In conclusion, this patient presented with a silent SG with likely pituitary hyperplasia. SGs are a challenging diagnosis, have a benign course, and may provide insights into PA tumourigenesis.

Introduction: Ectopic acromegaly is a rare condition caused most frequently by growth hormone releasing hormone (GHRH) secretion from neuroendocrine tumors. The diagnosis is often difficult to establish as its main symptoms do not differ from those of acromegaly of pituitary origin. Objectives: To determine most common clinical features and diagnostic challenges in ectopic acromegaly. Patients and Methods: A search for ectopic acromegaly cases available in literature was performed using PubMed, Cochrane, and MEDline database. In this article, 127 cases of ectopic acromegaly described after GHRH isolation in 1982 are comprehensively reviewed, along with a summary of current state of knowledge on its clinical features, diagnostic methods, and treatment modalities. The most important data were compiled and compared in the tables. Results: Neuroendocrine tumors were confirmed in 119 out of 121 patients with histopathological evaluation, mostly of lung and pancreatic origin. Clinical manifestation comprise symptoms associated with pituitary hyperplasia, such as headache or visual field disturbances, as well as typical signs of acromegaly. Other endocrinopathies may also be present depending on the tumor type. Definitive diagnosis of ectopic acromegaly requires confirmation of GHRH secretion from a tumor using either histopathological methods or GHRH plasma concentration assessment. Hormonal evaluation was available for 84 patients (66%) and histopathological confirmation for 99 cases (78%). Complete tumor resection was the main treatment method for most patients as it is a treatment of choice due to its highest effectiveness. When not feasible, somatostatin receptor ligands (SRL) therapy is the preferred treatment option. Prognosis is relatively favorable for neuroendocrine GHRH-secreting tumors with high survival rate. Conclusion: Although ectopic acromegaly remains a rare disease, one should be aware of it as a possible differential diagnosis in patients presenting with additional symptoms or those not responding to classic treatment of acromegaly. [ABSTRACT FROM AUTHOR]

Abstract Pituitary hyperplasia due to hypothyroidism can be treated medically. This condition should not be mistaken for adenoma and surgery should be avoided.

IntroductionEctopic acromegaly is a rare condition caused most frequently by growth hormone releasing hormone (GHRH) secretion from neuroendocrine tumors. The diagnosis is often difficult to establish as its main symptoms do not differ from those of acromegaly of pituitary origin.ObjectivesTo determine most common clinical features and diagnostic challenges in ectopic acromegaly.Patients and MethodsA search for ectopic acromegaly cases available in literature was performed using PubMed, Cochrane, and MEDline database. In this article, 127 cases of ectopic acromegaly described after GHRH isolation in 1982 are comprehensively reviewed, along with a summary of current state of knowledge on its clinical features, diagnostic methods, and treatment modalities. The most important data were compiled and compared in the tables.ResultsNeuroendocrine tumors were confirmed in 119 out of 121 patients with histopathological evaluation, mostly of lung and pancreatic origin. Clinical manifestation comprise symptoms associated with pituitary hyperplasia, such as headache or visual field disturbances, as well as typical signs of acromegaly. Other endocrinopathies may also be present depending on the tumor type. Definitive diagnosis of ectopic acromegaly requires confirmation of GHRH secretion from a tumor using either histopathological methods or GHRH plasma concentration assessment. Hormonal evaluation was available for 84 patients (66%) and histopathological confirmation for 99 cases (78%). Complete tumor resection was the main treatment method for most patients as it is a treatment of choice due to its highest effectiveness. When not feasible, somatostatin receptor ligands (SRL) therapy is the preferred treatment option. Prognosis is relatively favorable for neuroendocrine GHRH-secreting tumors with high survival rate.ConclusionAlthough ectopic acromegaly remains a rare disease, one should be aware of it as a possible differential diagnosis in patients presenting with additional symptoms or those not responding to classic treatment of acromegaly.

Pituitary hyperplasia occurs as a result of an increase in pituitary cell subtypes. It can be a consequence of either a physiological or pathological condition. In our case, a 31-year-old pregnant woman at 16 weeks gestation presented with headaches and vision changes. Visual field testing demonstrated bitemporal hemianopsia, and magnetic resonance imaging (MRI) brain showed enlargement of the pituitary with compression of the optic chiasm. She was treated with cabergoline and steroids, and her symptoms improved. In a subsequent pregnancy, the patient developed similar symptoms, and with cabergoline treatment, her symptoms resolved. A postpartum MRI of her brain revealed a decrease in pituitary size back to baseline with normal pituitary hormone levels. This patient’s likely diagnosis was physiologic pituitary hyperplasia. Pituitary hyperplasia can be difficult to diagnose since there are no explicit guidelines. Through deduction of imaging findings and hormonal levels, diagnosis of pituitary hyperplasia becomes a more manageable task. [ABSTRACT FROM AUTHOR]

This case report describes a woman with no psychiatric history and previously diagnosed Hashimoto's thyroiditis who presented to the psychiatric emergency department with a first episode of psychosis. The initial workup for organic causes of psychosis revealed an astronomically high thyroid stimulating hormone (TSH) (> 1,000 μIU/mL) out of proportion to the patient's minimal physical symptoms of hypothyroidism. Additionally the patient's head imaging showed an enlarged pituitary, a rare, but reversible, presentation of chronically untreated primary hypothyroidism. The patient was transferred to a medical unit to receive IV thyroid hormone replacement as well as an adjunctive antipsychotic to assist with remission of her distressing auditory hallucinations and persecutory delusions. This case highlights the importance of a thorough medical workup for causes of new onset psychosis and the need for further consensus in the literature regarding choice of antipsychotic and duration of treatment for psychosis secondary to hypothyroidism. [ABSTRACT FROM AUTHOR]

This case report describes a woman with no psychiatric history and previously diagnosed Hashimoto’s thyroiditis who presented to the psychiatric emergency department with a first episode of psychosis. The initial workup for organic causes of psychosis revealed an astronomically high thyroid stimulating hormone (TSH) (> 1,000 μIU/mL) out of proportion to the patient’s minimal physical symptoms of hypothyroidism. Additionally the patient’s head imaging showed an enlarged pituitary, a rare, but reversible, presentation of chronically untreated primary hypothyroidism. The patient was transferred to a medical unit to receive IV thyroid hormone replacement as well as an adjunctive antipsychotic to assist with remission of her distressing auditory hallucinations and persecutory delusions. This case highlights the importance of a thorough medical workup for causes of new onset psychosis and the need for further consensus in the literature regarding choice of antipsychotic and duration of treatment for psychosis secondary to hypothyroidism.

In children, hypothyroidism usually presents non-specific symptoms; symptoms can emerge gradually, compromising a timely diagnosis. We report the case of a 13-year-old male, who was admitted to the hospital due to swelling of the torso and neck. Besides these symptoms, the child was healthy, except for a significant growth delay. Ultrasound evaluation and blood tests led to the diagnosis of myxedema secondary to severe hypothyroidism, which was due to autoimmune thyroiditis. Further investigations revealed pericardial effusion and pituitary hyperplasia, with hyper-prolactinemia. Treatment with levothyroxine led to edema regression and clinical, hemato-chemical and radiological improvement. After 6 months, growth velocity increased, although the recovery of growth already lost was not guaranteed. Brain MRI showed regression of pituitary hyperplasia. The diagnostic delay in this case was probably due to the patient’s apparent good health, and the underestimation of growth restriction. This report underlines the importance of growth monitoring in adolescence, a critical period for identifying endocrine conditions; if undiagnosed, these conditions can lead to serious complications, such as myxedema in hypothyroidism, with potential effects beyond growth on multiple organs.

The Van Wyk-Grumbach syndrome (VWGS) is characterized by severe hypothyroidism, peripheral precocious puberty, delayed bone age, hyperestrogenism, prepubertal luteinizing hormone, and elevated follicle-stimulating hormone. Patients with Down syndrome have a high susceptibility and prevalence of thyroid disorders. However, the coexistence of VWGS and trisomy 21 is uncommon. We present a case of a 5-year-old Mexican girl with Down syndrome, severe autoimmune hypothyroidism, pituitary enlargement, hyperprolactinemia, peripheral precocious puberty, multiple ovarian cysts, and delayed bone age, with a clinical diagnosis of VWGS. The patient presented with a remission of these manifestations after treatment with levothyroxine. Patients with Down syndrome, precocious puberty, hyperestrogenism, prepuberal luteinizing hormone, high follicle-stimulating hormone, and delayed bone age should be evaluated with a thyroid profile due to the possibility of VWGS. [ABSTRACT FROM AUTHOR]

Sellar gangliocytomas (SGs) are rare, well-differentiated, low-grade neoplasias that commonly present along with a pituitary adenoma (PA). We describe a case of a 52-year-old woman with a 2-year history of headache, body weight increase, and recent onset of arterial hypertension and type 2 diabetes mellitus. Work-up tests revealed a normal hypophyseal profile, except for mild ACTH elevation, and a sellar mass on magnetic resonance imaging (MRI). A diagnosis of an enlarging pituitary macroadenoma was established, and to prevent symptom progression, the tumour was resected. Pathology showed 2 cell populations: ganglion and corticotrope cells. Three years after surgery, the patient no longer had a headache but persisted with arterial hypertension and type 2 diabetes mellitus. A literature review produced 207 cases of SGs. They typically present in women at 40 years of age and the most common clinical presentation are symptoms of acromegaly. Of the documented cases, 74 and 93% were treated with surgery alone or combined treatments (radiotherapy, radiosurgery, or pharmacotherapy), respectively. The majority of deaths associated with a SG came from the first half of the 20th century. In conclusion, this patient presented with a silent SG with likely pituitary hyperplasia. SGs are a challenging diagnosis, have a benign course, and may provide insights into PA tumourigenesis. [ABSTRACT FROM AUTHOR]

Introduction: Pituitary hyperplasia following primary hypothyroidism in pediatric age group population is considered rare with reports of unnecessary neurosurgical intervention for this medically treatable condition. Given the paucity of information on this topic, it is timely to provide clinicians with a comprehensive summary of available research. Methods: A search of published studies in Pubmed, PsychInfo and Cochrane Database with the terms "pituitary hyperplasia" or "pituitary hypertrophy" and "hypothyroidism" was performed and the results filtered for English language, pediatric (0-18 years) population and CT or MRI confirmed findings. 55 studies met the inclusion criteria. Data for a total of 110 patients with pituitary hyperplasia following primary hypothyroidism were extracted. The study population included 29 males and 81 females (M: F= 0.35:1). Patient age varied from 3 weeks to 18 years with a mean age of 10.22 years. Results: The most common clinical presentations included growth retardation, constipation and features of myxedema which were present in 78, 36 and 18 percent of children included in our review. Neuroimaging showed the mean (SD) pituitary height being 13.48 mm (4.72 mm). All of the patients achieved resolution of their pituitary mass and clinical as well as biochemical abnormalities 1 to 26 months after initiation of thyroid hormone replacement therapy, with an average time interval of 7.22 months. Our review has tried to delve in the pathophysiology as well as clinical, biochemical and radiological aspects of pediatric pituitary hyperplasia secondary to primary hypothyroidism and provide recommendations for treatment and follow-up. This may help anyone concerned gain a substantial knowledge on this topic. [ABSTRACT FROM AUTHOR]

Pituitary hyperplasia due to hypothyroidism can be treated medically. This condition should not be mistaken for adenoma and surgery should be avoided. [ABSTRACT FROM AUTHOR]

We describe three cases of primary hypothyroidism which presented initially to neurosurgery department with pituitary hyperplasia. We have found a novel pattern of ‘dome-shaped’ enlargement of pituitary in MRI of these patients. Out of these 3 patients, in two of them, the planned surgery was deferred when endocrinologists were consulted and the pituitary hyperplasia completely resolved with levothyroxine treatment. In the third case, pituitary surgery was already performed before endocrinology consultation and histopathology revealed thyrotroph hyperplasia. The hyperplastic lesions described typically have a homogenous symmetrical ‘dome’ shaped architecture unlike the non-functioning pituitary adenoma (NFPA), which usually might often be of varying shapes and homogeneity. Analysis of pituitary images from similar case reports published in literature, also showed this typical ‘dome’ shaped pituitary enlargement. This imaging characteristic can be a clue to look for underlying hormone deficiency, especially in primary hypothyroidism. Therefore, a thorough endocrine evaluation especially looking for primary hypothyroidism in such dome-shaped pituitary lesions is mandatory to prevent unwarranted neuro-surgical intervention as treatment of primary hypothyroidism may result in resolution of the abnormal enlargement.

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 11C-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.

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 11C-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. [ABSTRACT FROM AUTHOR]

Abstract Severe primary hypothyroidism should be considered in the differential diagnosis of pituitary enlargement. Thyroid hormone replacement therapy should lead to regression of pituitary hyperplasia.

The article presents a case study of a 15.3-year-old female with late-onset Van Wyk Grumbach syndrome, characterized by severe hypothyroidism, hyperprolactinemia, and multicystic ovaries. Topics discussed include the complex hormonal interaction in VWG syndrome pathogenesis and the importance of re-evaluation after levothyroxine treatment to avoid unnecessary tests and surgical interventions.

Ectopic ACTH-syndrome is the most diagnostically challenging variant of endogenous hypercortisolism. Particularly difficult differential diagnosis of this syndrome is from Cushing's disease (CD), as currently there is no single test sufficiently accurate to differentiate accurately ectopic ACTH production from the pituitary. The main functional tests are based on the fact that the vast majority of ectopic ACTH production is autonomous and suppresses one from pituitary. But in some cases this is not observed, and then the data obtained all necessary laboratory and instrumental research evidence in favor of central genesis of CD in a patient with ACTH ectopic secretion, which can lead to inappropriate treatment. If you confirm the ectopic ACTH-syndrome, it may take quite a long time of searching for the pathological focus, as there is no sufficiently precise imaging and diagnostic method for determining the localization of ectopic source of ACTH production. Thus, the differential diagnosis of ACTH-dependent hypercortisolism and localization of the ectopic tumor is the cornerstone of early and radical treatment of patients. We present a difficult clinical case of a patient having a pituitary hyperplasia with excessive ACTH expression with primary ectopic ACTH syndrome caused by lung carcinoid.

Autoimmune hypothyroidism is a common medical condition. Its revelation by thyrotrophic hyperplasia is an unusual and may be misdiagnosed as a pituitary adenoma. A 35-year-old man is referred to us for endocrinological assessment before surgery of a pituitary macroadenoma with bitemporal hemianopsia. Biological data reveal profound primary hypothyroidism. With thyroid hormone substitution, the thyroid function was normalized. Follow-up magnetic resonance imaging, showed the complete shrinkage of the pituitary mass attesting of a thyrotrophic pituitary hyperplasia. This case highlights the importance of a multidisciplinary assessment of pituitary masses to avoid unnecessary surgery and to prevent consequences of sellar masses.

Unlabelled: Hashimoto's thyroiditis is a well-known cause of growth retardation in adolescence. It is less frequently seen in children and rarely seen in infants. A 4-year-old girl was referred to our clinic for a second opinion before starting growth hormone (GH) treatment. Linear growth had markedly declined in the past 2 years, with height -3.4 standard deviations. GH deficiency was complete. She had dry, gray-sallow skin and bloated abdomen, but no goiter. The parents reported fatigue and constipation. Hormonal evaluation revealed TSH 629.5 mIU/ml, free T4 0.08 ng/dl, and prolactin 17.2 ng/ml. Bone age was 2 years. Antibodies to thyroglobulin and thyroid peroxidase were positive, suggesting Hashimoto's thyroiditis. Brain magnetic resonance imaging showed anterior pituitary hyperplasia. After 3 years of L-thyroxine therapy, she was symptomless, her height was -0.6 standard deviations, and the TSH level was normal. Brain magnetic resonance imaging showed regression of the pituitary hyperplasia.Conclusions: This report describes a patient with Hashimoto's thyroiditis and pituitary hyperplasia, both quite rare in very young children. Acquired hypothyroidism may appear after neonatal screening and therefore should not be overlooked in investigations of short stature, even when clinical signs of hypothyroidism are absent.What Is Known: • Hashimoto's thyroiditis and pituitary hyperplasia are rare in very young children. • Acquired hypothyroidism can appear after negative neonatal screening and should not be overlooked. What is New: • Short children should be evaluated for growth hormone deficiency but only after excluding other causes, particularly hypothyroidism, as we report a child with this disease but no clinical signs of it. [ABSTRACT FROM AUTHOR]

Objective - We aim to emphasize the importance of extensive endocrine workup in cases of pituitary masses. Case report - We report on a case of pituitary thyrotrophic hyperplasia in a 12-year-old girl who was thought to have a pituitary macroadenoma with suprasellar extension. The main complaint was headache, while other symptoms of hypothyroidism were present, but weren't recognised. Hormonal testing revealed low total thyroxine (<12.8 nmol/l) and high TSH (310.5 mIU/l) levels, and hyperprolactinemia (prolactin level at 1680 mIU/l). Based on the clinical history, laboratory data, and MRI, a diagnosis of pituitary hyperplasia secondary to primary hypothyroidism, consequent to chronic autoimmune thyroiditis, was made. Therapy with levothyroxine was initiated at 50 μg/day and gradually increased to 75 μg daily. After three months of thyroxine replacement, she was clinically and biochemically euthyroid. A follow-up MRI, 4 months after thyroxine replacement was initiated, showed complete resolution of the mass, and normal pituitary gland. Conclusion - Primary hypothyroidism should be considered in the differential diagnosis of pituitary masses. Multidisciplinary assessment in these cases will help to avoid delays in diagnosis and prevent unnecessary surgery. [ABSTRACT FROM AUTHOR]

Pituitary adenomas are a common feature of a subset of endocrine neoplasia syndromes, which have otherwise highly variable disease manifestations. We provide here a review of the clinical features and human molecular genetics of multiple endocrine neoplasia (MEN) type 1 and 4 (MEN1 and MEN4, respectively) and Carney complex (CNC). MEN1, MEN4, and CNC are hereditary autosomal dominant syndromes that can present with pituitary adenomas. MEN1 is caused by inactivating mutations in the MEN1 gene, whose product menin is involved in multiple intracellular pathways contributing to transcriptional control and cell proliferation. MEN1 clinical features include primary hyperparathyroidism, pancreatic neuroendocrine tumours and prolactinomas as well as other pituitary adenomas. A subset of patients with pituitary adenomas and other MEN1 features have mutations in the CDKN1B gene; their disease has been called MEN4. Inactivating mutations in the type 1α regulatory subunit of protein kinase A (PKA; the PRKAR1A gene), that lead to dysregulation and activation of the PKA pathway, are the main genetic cause of CNC, which is clinically characterised by primary pigmented nodular adrenocortical disease, spotty skin pigmentation (lentigines), cardiac and other myxomas and acromegaly due to somatotropinomas or somatotrope hyperplasia. © 2015 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Acromegaly is diagnosed clinically by the universal presence of acral enlargement and typical coarse facies. We report a short, elderly female developing acromegalic facies for last 10 years without acral overgrowth. She is a patient of primary hypothyroidism, well controlled for last 20 years. Acromegaly was proven by high level of serum insulin like growth factor-1 (IGF-1) and elevated and nonsuppressed level of growth hormone (GH), with other hormonal profile being undisturbed. She had mild insulin resistance and systemic hypertension in absence of any visual field defect. Magnetic resonance imaging (MRI) of brain revealed pituitary hyperplasia without any detectable adenoma. No source of ectopic secretion of GH or growth hormone releasing hormone (GHRH) could be localized. Therefore, atypical presentation of acromegaly needs high degree of suspicion even if some of the common features are lacking. Here, we have biochemically proved acromegaly with typical facies, short stature but no acral overgrowth and pituitary adenoma despite longstanding disease activity, and thus eluding diagnosis for years.

Asymptomatic reversible pituitary hyperplasia is common in patients with untreated primary hypothyroidism. Occurrence of empty sella (ES) in this scenario is extremely rare (only three reports till the date) and panhypopituitarism has not been reported in such patients. We report a 27 year man with severe short stature (height-133 cm; standard deviation score-7.36) and delayed puberty who had symptoms suggestive of hypothyroidism along with chronic persistent headache since 6 years of age. Pituitary imaging done for headache at 11 years age showed pituitary hyperplasia. He was diagnosed of primary hypothyroidism for the 1 st time at 21 year age, a diagnosis which was likely missed for 15 years. Levothyroxine therapy leads to resolution of all symptoms and a height gain of 28 cm over last 6 years. Evaluation for lack of progression of puberty along with chronic nausea, vomiting, fatigue and weight loss for the last 1 year revealed secondary hypocortisolism (9 am cortisol-4.8 mcg/dl; ACTH-3.2 pg/ml), growth hormone deficiency (IGF-1: 65 ng/ml; normal: 117-325 ng/ml) and hypogonadotrophic hypogonadism (9 am testosterone: 98 ng/dl; [280-1500] LH-0.01 mIU/L [1.14-5.75]) with ES on magnetic resonance imaging (MRI) brain. Uncontrolled thyrotroph hyperplasia due to chronic untreated primary hypothyroidism for 15 years may have been damaging the adjacent corticotrophs, somatotrophs and gonadotrophs resulting in panhypopituitarism and empty sella. This is perhaps the first report of panhypopituitarism with empty sella syndrome developing in a patient with pituitary hyperplasia, a sequel of chronic untreated primary hypothyroidism.

Diffuse and reactive pituitary gland enlargement secondary to primary hypothyroidism is an uncommon occurrence and that can masquerade many pituitary disorders. In present article, we report a case of 19 year female severe hypothyroidism presenting with diffuse enlargement of pituitary gland and hyperprolactinemia and review the clinical importance of this entity. Knowledge of this entity is very important to avoid unnecessary surgery and irreversible complications in this sub-group of patients.

Background: Pituitary adenomas are the most common cause of pituitary enlargement and can potentially warrant surgical intervention. However, there are physiological causes of pituitary enlargement that can be reversed with hormone replacement alone., Observations: A 29-year-old female presented with acute onset paranoia to the psychiatry department. A computed tomography scan of the head revealed a 2.3 cm sellar mass with confirmation on magnetic resonance imaging. Testing showed a markedly elevated thyroid-stimulating hormone 1,600 µIU/mL (0.470-4.200 µIU/mL), suggesting pituitary hyperplasia. Treatment with levothyroxine replacement resulted in marked improvement in symptoms and resolution of pituitary hyperplasia on four month follow up., Lessons: This rare presentation of severe primary hypothyroidism highlights the importance of evaluating for physiological causes of pituitary enlargement.