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Clinical Case Reports Nov 2023Ectopic pituitary adenoma is a rare neoplasm located in the clivus and could mimic other clival tumors. Diagnosis and treatment could be challenging. It should be...
KEY CLINICAL MESSAGE
Ectopic pituitary adenoma is a rare neoplasm located in the clivus and could mimic other clival tumors. Diagnosis and treatment could be challenging. It should be considered in the differential diagnosis of clival tumors.
ABSTRACT
Ectopic pituitary adenomas (EPAs) are isolated adenomas that can be located in variable locations outside the sella turcica and have a normal-appearing pituitary gland. These tumors are rare and are thought to often arise from embryological remnants along the route of Rathke's pouch migration. EPAs are associated with a wide range of clinical manifestations depending on hormonal activity and involvement of adjacent structures, which can represent a challenge in making the diagnosis and deciding on the most appropriate management. In this case study, we report a 47-year-old male who presented with visual disturbances, a headache, and generalized weakness. Magnetic resonance imaging showed a 2 cm mass located in the clivus invading the sphenoid sinus with an intact pituitary gland. The patient underwent endoscopic transsphenoidal surgery to eradicate the mass while maintaining the integrity of the pituitary gland, which was successful and uneventful. Pathological studies were consistent with prolactinoma, with no cytological malignant features. Post-surgery, symptoms notably improved, and serum prolactin levels significantly dropped, The patient's condition was satisfactory on follow-up with no long-term complications reported. This paper contributes to the existing literature by sharing the clinical management of a challenging and uncommon case.
PubMed: 38028100
DOI: 10.1002/ccr3.8255 -
Cureus Oct 2023Objective To describe our experience with the microsurgical technique of the suboccipital transtentorial (SOTT) approach in the removal of posterior fossa lesions...
Objective To describe our experience with the microsurgical technique of the suboccipital transtentorial (SOTT) approach in the removal of posterior fossa lesions located in the posterior incisural space. Method Between 2002 and 2020 we reviewed all patients who underwent microsurgical resection of lesions of the posterior incisural space at the Department of Neurosurgery, Essex Neuroscience Centre, London, England (eight patients, male to female 3:5, mean age: 51, range 35-69). We describe the preoperative symptoms, radiological findings, surgical techniques, histology and postoperative outcomes in this cohort of patients. Results Eight patients with tumours located in the posterior incisural space underwent surgery during the study period including four meningiomas (50%), two haemangioblastomas (25%), one metastasis (13%) and one giant prolactinoma (13%). Gross or near total resection was achieved in six patients (75%): the giant prolactinoma could not be radically removed and one of the meningiomas required a small fragment to be left in place to protect the Vein of Galen. No patient developed a visual field deficit due to occipital lobe retraction. One patient developed a temporary trochlear nerve palsy (13%). Five patients had mild disability (Glasgow Outcome Scale (GOS) = 5), and four had moderate disability (GOS = 4). Conclusion In our series, the SOTT approach provided excellent access for all cases of tumours in the posterior incisural space. The tumour's size and relationship to the deep venous system contributed to the choice of approach and in one patient who had previously undergone surgery via the supracerebellar route, the SOTT approach enabled the avoidance of gliotic scar tissue. Success is dependent on careful case selection, though from our series of 8 patients, we conclude that this approach allows safe access to the posterior incisural space, with acceptable outcomes with regard to postoperative disability and cranial nerve palsy. As such, the approach should be in the armamentarium of any neurosurgeon who regularly deals with posterior fossa pathology.
PubMed: 38021782
DOI: 10.7759/cureus.47705 -
Journal of Neurosurgery. Case Lessons Nov 2023Double pituitary adenomas are rare presentations of two distinct adenohypophyseal lesions seen in <1% of surgical cases. Increased rates of recurrence or persistence are...
BACKGROUND
Double pituitary adenomas are rare presentations of two distinct adenohypophyseal lesions seen in <1% of surgical cases. Increased rates of recurrence or persistence are reported in the resection of Cushing microadenomas and are attributed to the small tumor size and localization difficulties. The authors report a case of surgical treatment failure of Cushing disease because of the presence of a secondary pituitary adenoma.
OBSERVATIONS
A 32-year-old woman with a history of prolactin excess and pituitary lesion presented with oligomenorrhea, weight gain, facial fullness, and hirsutism. Urinary and nighttime salivary cortisol elevation were elevated. Magnetic resonance imaging confirmed a 4-mm3 pituitary lesion. Inferior petrosal sinus sampling was diagnostic for Cushing disease. Primary endoscopic endonasal transsphenoidal resection was performed to remove what was determined to be a lactotroph-secreting tumor on immunohistochemistry with persistent hypercortisolism. Repeat resection yielded a corticotroph-secreting tumor and postoperative hypoadrenalism followed by long-term normalization of the hypothalamic-pituitary-adrenal axis.
LESSONS
This case demonstrates the importance of multidisciplinary management and postoperative hormonal follow-up in patients with Cushing disease. Improved strategies for localization of the active tumor in double pituitary adenomas are essential for primary surgical success and resolution of endocrinopathies.
PubMed: 38011690
DOI: 10.3171/CASE23485 -
International Journal of Molecular... Nov 2023Pituitary neuroendocrine tumors (PitNETs) are divided into multiple histological subtypes, which determine their clinical and biological variable behavior. Despite their...
Pituitary neuroendocrine tumors (PitNETs) are divided into multiple histological subtypes, which determine their clinical and biological variable behavior. Despite their benign evolution, in some cases, prolactin (PRL) and growth hormone (GH)-secreting PitNETs may have aggressive behavior. In this study, we investigated the potential predictive role of ER∝, alongside the clinicopathological classification of PitNETs (tumor diameter, tumor type, and tumor grade). A retrospective study was conducted with 32 consecutive cases of PRL- and mixed GH- and PRL-secreting PitNETs (5 patients with prolactinomas and 27 with acromegaly, among them, 7 patients with GH- and PRL- co-secretion) who underwent transsphenoidal intervention. Tumor specimens were histologically and immunohistochemical examined: anterior pituitary hormones, ki-67 labeling index, CAM 5.2, and ER∝; ER∝ expression was correlated with basal PRL levels at diagnosis (rho = 0.60, < 0.01) and postoperative PRL levels (rho = 0.58, < 0.001). In our study, the ER∝ intensity score was lower in female patients. Postoperative maximal tumor diameter correlated with Knosp grade ( = 0.02); CAM 5.2 pattern (densely/sparsely granulated/mixed densely and sparsely granulated) was correlated with postoperative PRL level ( = 0.002), and with ki-67 ( < 0.001). The IGF1 level at diagnosis was correlated with the postoperative GH nadir value in the oral glucose tolerance test (OGTT) (rho = 0.52, < 0.05). Also, basal PRL level at diagnosis was correlated with postoperative tumor diameter ( = 0.63, < 0.001). At univariate logistic regression, GH nadir in OGTT test at diagnostic, IGF1, gender, and invasion were independent predictors of remission for mixed GH- and PRL-secreting Pit-NETs; ER∝ can be used as a prognostic marker and loss of ER∝ expression should be considered a sign of lower differentiation and a likely indicator of poor prognosis. A sex-related difference can be considered in the evolution and prognosis of these tumors, but further studies are needed to confirm this hypothesis.
Topics: Humans; Female; Adenoma; Retrospective Studies; Ki-67 Antigen; Pituitary Neoplasms; Prolactinoma; Prolactin; Neuroendocrine Tumors
PubMed: 38003353
DOI: 10.3390/ijms242216162 -
Journal of Diabetes and Metabolic... Dec 2023The Dopamine-2 receptor agonists, Bromocriptine and Cabergoline, were originally introduced for prolactinomas, pituitary tumors, and parkinson's disease but have... (Review)
Review
BACKGROUND
The Dopamine-2 receptor agonists, Bromocriptine and Cabergoline, were originally introduced for prolactinomas, pituitary tumors, and parkinson's disease but have glucose-lowering effects. This paper systematically reviewed the significance of their effects on lowering blood glucose level and conducted a comprehensive systematic search to identify relevant clinical trials of dopamine 2 agonists on glycated hemoglobin (HbA1c) and fasting blood sugar (FBS).
METHOD
We conducted a systematic review search in the databases (PubMed, Google Scholar, Cochrane Library, Registers, and Citations) until November 30, 2022, using the PRISMA 2020 statement. The Oxford quality score (Jadad score) was used to assess the study's quality. The present study protocol was registered on the PROSPERO database with ID: CRD42023389582. The study included studies with full abstracts, predefined doses, clear interventions, and blood glucose measurements.
RESULT
Data were synthesized from 23 clinical studies that recruited 6125 study subjects. The pooled effect analysis of the clinical trials revealed that dopamine 2 agonists improved HbA1c [SMD = -1.26; 95% CI (-1.60, -0.93), < .00001], and FBS [SMD = -1.84; 95% CI (-2.61, -1.07), < .00001]. Each drug's pooled effect analysis indicates bromocriptine significantly improved HbA1c [SMD = -1.25; 95% CI (-1.64, -0.87), < .00001] and FBS [SMD = -1.90; 95% CI (-2.79, -1.01), < .00001] and similarly, cabergoline significantly improved HbA1c [SMD = -1.29; 95% CI (-1.96, -0.62), < .00001] and FBS [SMD = -1.62; 95% CI (-2.82, -0.41), < .00001]. The pooled and individual analyses demonstrated that dopamine 2 agonists have a significant ability to lower blood glucose levels in clinical studies.
CONCLUSION
This study shows that dopamine 2 agonists significantly lowered FBS and HbA1c levels without causing severe negative effects. Even though the results are promising, additional research is necessary to establish the appropriate antihyperglycemic dosage, frequency of daily use, side effects, and potential product interactions when employing dopamine 2 receptor agonists for their antihyperglycemic effect.
PubMed: 37975084
DOI: 10.1007/s40200-023-01230-4 -
Frontiers in Endocrinology 2023The occurrence of prolactinomas in sex hormone treated patients with central hypogonadism is extremely rare.
INTRODUCTION
The occurrence of prolactinomas in sex hormone treated patients with central hypogonadism is extremely rare.
CASE PRESENTATION
We present a Caucasian male patient who was diagnosed with Kallmann syndrome (KS) at age 15 years. Testosterone treatment was started. At age 26 the patient presented with mild headache. MRI revealed two separate pituitary adenomas along with the absence of the olfactory bulbs. Given the presence of marked hyperprolactinemia (17x upper limit of the reference range) the diagnosis prolactinoma was made and treatment with cabergoline was started which resulted in a complete biochemical response and in marked reduction of both adenomas in size. Hypogonadism persisted and testosterone replacement therapy was continued. Genetic testing of genes associated with pituitary tumors, Kallmann syndrome and idiopathic hypogonadotropic hypogonadism was negative. Mild concomitant hypercalcemia in accordance with familial hypocalciuric hypercalcemia (FHH) prompted mutation analysis of the calcium receptor ( gene which yielded a pathogenic inactivating variant.
DISCUSSION/CONCLUSION
The presence of two separate prolactinomas in a patient with KS has not yet been reported in the literature. The effect of sex hormone treatment of KS patients on the possible development of prolactinoma is unknown at present. The occurance of multiple prolactinomas in our patient suggests increased susceptibility. Although CaSR is expressed in GnRH neurons in mouse brain and CaSR deficient mice have a reduced hypothalamic GnRH neuronal population, the relevance of the gene variant in our patient for the KS phenotype is unclear at present.
Topics: Humans; Male; Animals; Mice; Adolescent; Adult; Hypercalcemia; Kallmann Syndrome; Prolactinoma; Hypogonadism; Hyperparathyroidism; Gonadotropin-Releasing Hormone; Pituitary Neoplasms; Testosterone; Gonadal Steroid Hormones
PubMed: 37964948
DOI: 10.3389/fendo.2023.1248231 -
European Heart Journal. Case Reports Nov 2023Heart failure concomitant with prolactinoma is extremely rare.
BACKGROUND
Heart failure concomitant with prolactinoma is extremely rare.
CASE SUMMARY
We present the case of a 29-year-old man who had acute decompensated heart failure concomitant with visual loss in his right eye. Transthoracic echocardiography indicated severely decreased left ventricular (LV) function. A massive tumour on the sella turcica was detected by brain computed tomography. The findings of the laboratory tests showed hyperprolactinaemia with hypopituitarism, and the antigen test for coronavirus disease 2019 was positive as an incidental finding. Medication for heart failure and cabergoline therapy were started immediately. His LV function significantly improved, and he had no symptoms after a year.
DISCUSSION
Prolactinoma in men, which can cause visual loss and hypopituitarism, is frequently substantial when diagnosed. The cardiac manifestation of prolactinoma is uncommon. It is believed that a major contributing component to the pathogenesis of peripartum cardiomyopathy is hyperprolactinaemia. Hyperprolactinaemia may cause endothelial damage and cardiomyocyte dysfunction, eventually resulting in LV dysfunction. The success of LV reverse remodelling may be significantly impacted by heart failure and hormone treatments. Heart failure and endocrine therapy should be administered concurrently to patients who have prolactinoma and congestive heart failure.
PubMed: 37954559
DOI: 10.1093/ehjcr/ytad487 -
JCEM Case Reports Jul 2023Olfactory neuroblastomas, or esthesioneuroblastomas, are rare and aggressive malignant tumors that typically arise from the olfactory neuroepithelium in the upper nasal...
Olfactory neuroblastomas, or esthesioneuroblastomas, are rare and aggressive malignant tumors that typically arise from the olfactory neuroepithelium in the upper nasal cavity. In rare instances, they can be ectopic originating from areas outside the upper nasal cavity such as the sellar region. These tumors, also known as primary sellar neuroblastomas, may be mistaken for pituitary macroadenomas. We present a rare case of a primary sellar neuroblastoma in a 30-year-old woman with a prior diagnosis of presumed prolactinoma, status post transsphenoidal resection, with residual visual deficits, who presented with worsening vision and headaches. Pituitary magnetic resonance imaging showed a large sellar mass causing compression of the optic chiasm, and invasion of the right cavernous sinus and bilateral cavernous internal carotid arteries. The patient underwent a second transsphenoidal resection. Postoperatively, she developed central adrenal insufficiency, central hypothyroidism, central hypogonadism, and transient syndrome of inappropriate antidiuretic hormone secretion. Owing to rapid tumor regrowth, she underwent a craniotomy with plans for radiation treatment. This condition is challenging to diagnose and has poorly defined clinical management guidelines. An early, aggressive approach with surgical intervention is recommended.
PubMed: 37908997
DOI: 10.1210/jcemcr/luad081 -
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 -
Cureus Sep 2023Prolactinomas are benign pituitary tumors also known as prolactin-secreting adenomas (PSA). These tumors cause excessive secretion of prolactin (hyperprolactinemia), a...
Prolactinomas are benign pituitary tumors also known as prolactin-secreting adenomas (PSA). These tumors cause excessive secretion of prolactin (hyperprolactinemia), a hormone responsible for lactation. Diagnosing hyperprolactinemia relies on measuring prolactin levels in the blood, and elevated serum levels of prolactin are typically indicative of prolactinoma. The hook effect occurs in immunological tests such as the prolactin level test. When the amount of prolactin present in the sample is too high and exceeds the binding capacity of the antibodies being used, the test result may indicate falsely low levels of prolactin, which is the hook effect. The present study describes the case of a male patient who presented with neck pain and difficulty swallowing. MRI revealed a giant (>40mm) extradural tumor affecting the clivus, anterior fossa, pterygopalatine, and bilateral infratemporal fossae as well as the petrous apex and bilateral cavernous sinuses. Endocrinological investigation yielded no specific abnormalities. An occipitocervical fixation (arthrodesis) was proposed with simultaneous extended endoscopic endonasal resection. Surgery succeeded in resecting a portion of the clival tumor and the anterior fossa. Measurement of prolactin levels several weeks post-surgery found them to be extremely high, confirming the hook effect.
PubMed: 37905282
DOI: 10.7759/cureus.46194