-
Clinical Diabetes and Endocrinology Oct 2023Pituitary adenomas (PPAs) are uncommon in childhood and adolescence, accounting for 2-6% of all intracranial neoplasms. Delayed puberty, growth retardation, galactorrhea...
BACKGROUND
Pituitary adenomas (PPAs) are uncommon in childhood and adolescence, accounting for 2-6% of all intracranial neoplasms. Delayed puberty, growth retardation, galactorrhea and weight gain are common features at presentation in pediatric patients. Functional tumors constitute a vast majority (90%) of PPAs, with the most frequent being prolactinomas.
CASE PRESENTATION
A retrospective review of the clinical features and outcomes of 7 pediatric patients with pituitary macroadenomas was conducted. We included PPAs in patients under 18 years at diagnosis with diameters larger than 10 mm by magnetic resonance (MRI). Six patients were males (85%), with age at diagnosis ranging from 8 to 15 (median 14 ± 2.8SDS). The primary symptoms that led to medical attention were growth retardation, gigantism and secondary amenorrhea. The visual field was reduced in three cases (42%). Suprasellar extension was present in 3 subjects, and one had a giant adenoma. Adenomas were clinically functioning in 6 patients (85%) (three prolactinomas, two somatropinomas, one secreting FSH and one no-producer). The prolactinomas responded to treatment with cabergoline. For the rest, one required transsphenoidal surgery and the other three both surgery and radiotherapy. All patients undergoing radiotherapy had secondary panhypopituitarism. In relation to the genetic studies, two patients presented a pathogenic mutation of the AIP gene and one of the MEN1.
DISCUSION AND CONCLUSION
Pediatric pituitary macroadenomas are a distinct entity, mostly found in males and with a predominance of functional tumors leading to detrimental effects on growth and puberty in addition to neuro-ophthalmological manifestations. It is important to perform genetic studies in patients with macroadenomas appearing under the age of 18 years as genetic and syndromic associations are more frequent in this age group.
PubMed: 37908013
DOI: 10.1186/s40842-023-00153-6 -
Annals of Medicine and Surgery (2012) Dec 2023Secretory pituitary macroadenoma also known as prolactinoma are benign neoplasm comprising very minimal cases of intracranial masses. Among the various presentation...
INTRODUCTION AND IMPORTANCE
Secretory pituitary macroadenoma also known as prolactinoma are benign neoplasm comprising very minimal cases of intracranial masses. Among the various presentation suggestive of panhypopituitarism, psychosis, and features of schizophrenia is very rarely seen. In the majority of cases, neurosurgical intervention for the excision of tumor is considered a standard treatment modality but conservative management with dopamine agonist and steroids have also been shown to provide an optimal level of care also improving the quality of level of patient.
CASE PRESENTATION
A 42-year-old Asian male presented with a history of talking to self, delusion of persecution, over talkativeness, hallucination, increased suspiciousness, and history of lost and found in the streets where he was working as a migrant worker. The patient was initially managed in line of schizophrenia with the antipsychotics drug of choice. On further assessment there was no improvement of psychiatric symptoms but they further deteriorated with additional neuropsychiatric symptoms; hence, MRI brain was carried out. Following which, the diagnosis of pituitary macroadenoma was confirmed and further more hormonal analysis was done, which showed findings suggestive of panhypopituitarism. The patient was then managed conservatively with dopamine agonist and steroids, which showed rapid improvement of psychiatric symptoms with a massive reduction in the size of the pituitary macroadenoma.
CLINICAL DISCUSSION
With the incidence of 100 per million cases pituitary adenomas are considered locally invading with the characteristic compression of the surrounding structure, presenting as visual hallucinations, olfactory hallucinations, episodes of losing time, apathy, and features suggestive of adrenal insufficiency, hypogonadotrophic hypogonadism, and symptoms secondary to hormonal imbalance such as hypothyroidism. Psychiatric symptomatic presentations are considered a very rare presentation in cases of pituitary macroadenoma. Also, psychiatric features and symptoms of psychosis are associated with prolcatinomas through idiopathic mechanism and the basic casualty has not been established. Surgical intervention such as trans-sphenoidal resection of the mass can be undertaken in case where mass effects is present but long-term remission and prognosis is found not to be fruitful. Conservative treatment with dopamine agonist such as cabergoline and steroids also plays a meaningful role in abrupt management in such cases.
CONCLUSION
Pituitary macroadenoma presenting as a patient of schizophrenia is noted very rarely in medical literature; hence, investigations in view of neurosurgical diagnosis in cases presenting as psychosis should be considered for ideal holistic management. Conservative management can also be a breakthrough treatment modality in complete recovery of pituitary macroadenoma.
PubMed: 38098557
DOI: 10.1097/MS9.0000000000001413 -
Radiology Case Reports Oct 2023A 38-year-old man who was delivered in a breech position presented with delayed development of secondary sexual characteristics and malaise. He was diagnosed with...
A 38-year-old man who was delivered in a breech position presented with delayed development of secondary sexual characteristics and malaise. He was diagnosed with panhypopituitarism caused by interruption of the pituitary stalk due to perinatal complications. Brain magnetic resonance imaging findings for pituitary stalk interruption syndrome are well-documented; however, reports of the imaging findings of the bones and several organs related to the effects of panhypopituitarism are limited. In this patient with anterior pituitary dysfunction, imaging revealed diverse sequelae, including delayed skeletal maturation, osteopenia, genital atrophy, fatty liver, and adrenal atrophy. Radiologists may find it difficult to discern complex imaging findings unless they are informed of the clinical course of the patient. Therefore, radiologists should coordinate with clinicians to arrive at a diagnosis.
PubMed: 37547790
DOI: 10.1016/j.radcr.2023.07.039 -
AACE Clinical Case Reports 2024Pituitary abscess is an uncommon life-threatening disease that could lead to panhypopituitarism. It is important to suspect its prevalence in regions with endemic...
BACKGROUND/OBJECTIVE
Pituitary abscess is an uncommon life-threatening disease that could lead to panhypopituitarism. It is important to suspect its prevalence in regions with endemic infectious diseases.
CASE REPORT
A 55-year-old man, a farmer, with a background of consumption of unpasteurized dairy products, presented with headache, impaired consciousness, and fever that started in February 2023. Initial test results were consistent with neuroinfection. Brain MRI showed ventriculitis; the pituitary gland was heterogeneous with the presence of an 8 × 8 mm abscess. The pituitary hormone axis was evaluated, and it showed results compatible with the results of panhypopituitarism with central hypothyroidism, central hypocortisolism, central hypogonadism, and growth hormone deficiency. Hormone replacement treatment with hydrocortisone and levothyroxine was started. The Rose Bengal test for and 2-mercaptoethanol Brucella agglutination test showed positive results. After neurobrucellosis (NB) was diagnosed, antibiotic treatment was commenced. The patient was discharged 6 weeks later and treatment with prednisone, levothyroxine, recombinant somatropin, testosterone, as well as doxycycline, and rifampin was continued for another 4 months.
DISCUSSION
NB and pituitary abscess are rare manifestations of brucellosis and are challenging to diagnose due to their nonspecific clinical presentation and cerebrospinal fluid (CSF) findings. NB diagnosis relies on neurologic symptoms and serological evidence of Brucella infection. Magnetic resonance imaging is the preferred diagnostic tool for pituitary abscesses. Medical management may be sufficient, while transsphenoidal drainage is not always necessary. Hormonal deficits typically remain permanent.
CONCLUSION
Pituitary abscess could be suspected in patients presenting with symptoms of neuroinfection, panhypopituitarism, and heterogenous image in the magnetic resonance imaging differential diagnosis. Opportune management can lead to reduced mortality and improved recovery of the pituitary hormone function.
PubMed: 38303770
DOI: 10.1016/j.aace.2023.10.005 -
Endocrine, Metabolic & Immune Disorders... Apr 2024Pituitary apoplexy (PA) can arise from haemorrhage or ischaemia of pituitary tissue and is characterized by abrupt onset of headache, visual impairment and...
BACKGROUND
Pituitary apoplexy (PA) can arise from haemorrhage or ischaemia of pituitary tissue and is characterized by abrupt onset of headache, visual impairment and hypopituitarism. COVID-19 may be associated with various degrees of vascular complications and, recently, its relationship with PA has been suggested. Cases Presentation Case 1: A 64-year-old male with type 2 diabetes, hypertension and coronary heart disease was admitted to the ER, after several days of asymptomatic COVID-19 infection, with symptoms of PA of a known non-functioning pituitary macroadenoma. The hormonal panel was consistent with anterior panhypopituitarism and the sellar MRI showed haemorrhagic changes of macroadenoma tissue. Transsphenoidal resection of the pituitary lesion was carried out seven days after admission. Although a volumetric reduction of the lesion was apparent during follow-up, some degree of visual symptoms endured. Case 2: An 18-year-old, otherwise healthy, female presented to the ER with symptoms of PA of a recently-diagnosed non-functioning pituitary macroadenoma, after ten days of asymptomatic COVID-19 infection. Central hypocortisolism and hypothyroidism were diagnosed and, after six days, the lesion was surgically resected. At two months follow-up, clinical symptoms had completely resolved, and the hormonal panel was normal.
CONCLUSION
Alongside known risk factors (hypertension, anticoagulation, pregnancy, surgery, etc.), COVID-19 infection might represent an emerging predisposing factor for PA onset. The two cases hereby presented are both significant: the first confirms the role of "classic" vascular predisposing factors for PA, while the second demonstrates that PA might arise also in young patients without known risk factors.
PubMed: 38676521
DOI: 10.2174/0118715303315494240419060719 -
Internal Medicine (Tokyo, Japan) Aug 2023Cryptococcal meningitis is a critical disease that occasionally involves immunosuppressed patients. We herein report a 79-year-old Japanese man who received low-dose...
Cryptococcal meningitis is a critical disease that occasionally involves immunosuppressed patients. We herein report a 79-year-old Japanese man who received low-dose prednisolone therapy for neurosarcoidosis and panhypopituitarism. He presented a 10-day history of a fever and altered mental status. The FilmArray Meningitis/Encephalitis Panel and serum cryptococcal antigen tests were both negative, but the cerebrospinal fluid sample became positive for Cryptococcus neoformans after seven-day incubation. After the diagnosis of cryptococcal meningitis, we successfully treated the patient with a recommended treatment regimen. When an immunocompromised patient presents with a subacute fever accompanying any central nervous symptoms, cryptococcal meningitis should be screened for.
Topics: Male; Humans; Aged; Meningitis, Cryptococcal; Cryptococcus neoformans; Sarcoidosis; Prednisolone; Meningitis
PubMed: 36575015
DOI: 10.2169/internalmedicine.0879-22 -
Neuropathology : Official Journal of... Dec 2023Tuberculosis of the hypothalamo-pituitary axis is extremely uncommon. The presentation of panhypopituitarism in a case of sellar tuberculosis is an even rarer...
Tuberculosis of the hypothalamo-pituitary axis is extremely uncommon. The presentation of panhypopituitarism in a case of sellar tuberculosis is an even rarer occurrence. We present a case of a 44-year-old man who presented with complaints of headache and right-sided diminution of vision for six months. A hormone profile showed abnormal anterior pituitary assay suggestive of panhypopituitarism. Magnetic Resonance imaging of the brain showed a sellar mass measuring 1.8 × 1.5 × 1.3 cm with suprasellar extension suggestive of a pituitary adenoma. Histopathological examination showed multiple epithelioid cell granulomas along with Langhans giant cells and mixed inflammatory infiltrates against a necrotic background. Zeihl Neelson stain demonstrated the presence of acid-fast bacilli. Thus, a final diagnosis of pituitary tuberculoma was made, and the patient started on antitubercular therapy. It is extremely important to correctly diagnose sellar tuberculosis as the treatment is entirely different, and the patient usually responds well to therapy.
Topics: Male; Humans; Adult; Pituitary Neoplasms; Pituitary Diseases; Hypopituitarism; Tuberculoma; Magnetic Resonance Imaging; Adenoma
PubMed: 37254443
DOI: 10.1111/neup.12925 -
Endocrine Journal Jul 2023Lymphocytic hypophysitis (LYH) is a rare chronic inflammatory disease characterized by lymphocytic infiltration of the anterior or posterior pituitary gland and...
Lymphocytic hypophysitis (LYH) is a rare chronic inflammatory disease characterized by lymphocytic infiltration of the anterior or posterior pituitary gland and hypothalamus. LYH is subdivided into lymphocytic adenohypophysitis (LAH), lymphocytic infundibulo-neurohypophysitis (LINH), and lymphocytic panhypophysitis (LPH) depending on the primary site. Most cases occur in adults, with few cases reported in children, and it is especially important to distinguish LYH from suprasellar malignancies, such as germ cell tumors and other neoplastic diseases. Although a biopsy is necessary for definitive diagnosis, it is desirable to be able to diagnose the disease without biopsy if possible, especially in children, because of the surgical invasiveness of the procedure. Recently, serum anti-rabphilin-3A antibodies have attracted attention as diagnostic markers for LYH, especially in LINH, but there are only a few reports on pediatric patients. In the present study, we experienced two children with LPH and LAH, respectively, who tested positive for anti-rabphilin-3A antibodies. This is the first report of children with LYH other than LINH positive for anti-rabphilin-3A antibodies, and anti-rabphilin-3A antibodies may be a useful non-invasive diagnostic marker not only for LINH but also for LYH in general. We also discuss the sensitivity and specificity of anti-rabphilin-3A antibody testing in cases where histological diagnosis has been made.
Topics: Adult; Humans; Child; Autoimmune Hypophysitis; Hypopituitarism; Pituitary Diseases; Pituitary Gland, Posterior
PubMed: 37045780
DOI: 10.1507/endocrj.EJ22-0637 -
Clinical Neurology and Neurosurgery Jan 2024Radiotherapy (RT) is the corner stone of nasopharyngeal carcinoma (NPC) treatment but it exposes to late effects especially hypothalamic pituitary deficiency (HPD). In...
PURPOSE
Radiotherapy (RT) is the corner stone of nasopharyngeal carcinoma (NPC) treatment but it exposes to late effects especially hypothalamic pituitary deficiency (HPD). In this article,we aimed to assess the impact of RT on pituitary function in NPC survivors.
METHODS
We included 55 patients treated in the radiation oncology department, of Farhat Hached Hospital in Sousse, Tunisia.
RESULTS
All patients received facio-cervical RT with a mean dose of 73.3 Gy to the nasopharynx. After a mean follow up of 9.56 years, 34 patients (61.8%) presented HPD. Associated peripheral involvement was seen in 18.2%. The most prevalent deficiency was of the GH axis in 50.9% followed by secondary adrenal insufficiency in 20%. Panhypopituitarism was noted in 8.8%. The development of HPD wasn't related to RT dose (OR: 0.41(0.05-2.92), p = 0.36) but was significantly associated with male gender (OR: 1.67 (1.21-2.37), p = 0.01).
CONCLUSION
HPD post RT is a common phenomenon. Therefore, we recommend regular assessment of pituitary function amongst patients treated with RT for NPC because identification of deficits is crucial to allow early and appropriate hormone replacement therapy in order to improve patients quality of life.
Topics: Humans; Male; Nasopharyngeal Carcinoma; Quality of Life; Hypothalamo-Hypophyseal System; Pituitary Gland; Pituitary Diseases; Nasopharyngeal Neoplasms
PubMed: 38218060
DOI: 10.1016/j.clineuro.2023.108100 -
Frontiers in Endocrinology 2023Pituitary stalk interruption syndrome (PSIS) is a congenital disease commonly found in patients with combined pituitary hormone deficiency (CPHD). Most PSIS patients... (Review)
Review
BACKGROUND
Pituitary stalk interruption syndrome (PSIS) is a congenital disease commonly found in patients with combined pituitary hormone deficiency (CPHD). Most PSIS patients manifest growth retardation and delayed puberty. We report a rare case of PSIS with tall stature, liver cirrhosis and diabetes, possibly caused by an inactivating gene mutation.
CASE PRESENTATION
A 37-year-old female patient initially presented with liver cirrhosis and diabetes, without any secondary sexual characteristics. Endocrine investigation indicated CPHD. Small anterior pituitary, invisible pituitary stalk and no eutopic posterior lobe hypersignal in the sella turcica viewed in magnetic resonance imaging (MRI) confirmed the diagnosis of PSIS. Despite receiving no growth hormone or sex hormone therapy, she reached a final height of 186 cm. Liver histopathology revealed nonalcoholic fatty cirrhosis. Genetic testing identified a heterozygous p.Arg301Cys mutation in the gene.
CONCLUSION
This is a rare case of PSIS with liver cirrhosis and diabetes associated with an inactivating gene mutation. It's supposed that early hyperinsulinism caused by the gene mutation, as well as delayed epiphyseal closure due to estrogen deficiency, contributed to the patient's exceptionally tall stature. Untreated growth hormone deficiency (GHD) resulted in increased visceral fat, leading to nonalcoholic fatty liver disease (NAFLD) and cirrhosis. The decline in β cell function with age, combined with NAFLD, may have played a role in the development of diabetes.
Topics: Female; Humans; Adult; Non-alcoholic Fatty Liver Disease; Pituitary Diseases; Pituitary Gland; Diabetes Mellitus; Mutation; Liver Cirrhosis
PubMed: 38152125
DOI: 10.3389/fendo.2023.1297146