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ABSTRACT Objective: Data regarding rare FPAs from India, a resource limited setting, are limited. We describe a case series of rare FPAs from a single center in western India. Materials and methods: This was a retrospective case record review of patients diagnosed between January 2010 and July 2022. The diagnosis was based on biochemical (inappropriately elevated serum FSH/LH) and pathologic (positive immunostaining for FSH/LH) features in patients with FGA, and elevated serum thyroid hormones and normal/elevated TSH in patients with TSHomas. Results: We identified 11 patients with a total of six FGAs (median age 43.5 years, five men, one FGA cosecreting TSH, median largest dimension 40 mm, range 33–60 mm) and six TSHomas (median age 34.5 years, four women, two TSHomas cosecreting GH, median largest dimension 42.5 mm, range 13–60 mm). Symptoms of sellar mass effects led to pituitary imaging in most patients with FGA. Patients with TSHomas had symptoms of excess hormone secretion (GH/TSH) or sellar mass effects. The TSHomas that cosecreted GH/FSH were larger than those secreting only TSH. Transsphenoidal resection was the most common first-line therapy but significant residual disease was frequent (3 out of 6 FGAs and 4 out of 5 TSHomas). Conclusion: This is the first and second case series of FGAs and TSHomas, respectively, from India. In this study, TSHomas presented at younger age, were larger and had low surgical cure rates.

A 31-year-old gentleman presented with clinical hyperthyroidism, goiter, bitemporal hemianopia, and discordant thyroid function test. The high α-subunit and absence of throtropin (thyroid-stimulating hormone; TSH) response to thyrotropin-releasing hormone stimulation indicated a TSH-secreting pituitary tumor. Elevation in prolactin and growth hormone as well as secondary hypogonadism were also observed. A sellar-suprasellar mass displacing the optic chiasm was revealed upon magnetic resonance imaging. The patient was rendered euthyroid with carbimazole and Lugol's iodine before undergoing endoscopic transsphenoidal surgery. Due to the incomplete tumor excision during surgery, patient experienced recurrence of symptoms and rising thyroxine (T4) with nonsuppressed TSH. The histopathology examination demonstrated a plurihormonal tumor with positive immunohistochemical stain for TSH, growth hormone, and prolactin, with invasive and proliferative features. Subsequently he was initiated with a long-acting somatostatin analogue and underwent stereotactic radiosurgery. To date, his symptoms have improved, with a reduction of insulin-like growth factor 1 and normalization of other pituitary hormones as well as a slightly reduced size of the pituitary tumor. [ABSTRACT FROM AUTHOR]

Thyrotoxicosis, which characteristics are increased excitability, emotional lability, tachycardia episodes, increasing of free fractions of tetraiodothyronine (T4) and triiodothyronine (T3) is one of the most common endocrinological syndromes. However, during the interpretation of thyroid status it is very important to take into account the possibility that a patient has TSH-secreting pituitary adenoma. Timely diagnosis of TSH-secreting adenomas plays prominent role in guiding the treatment course since it is associated with an improvement of long-term prognosis and an increase of the patient’s total life expectancy. Needed to underline that in some patients with TSH-secreting adenomas manifestations of the other pituitary hormones hypersecretion (first of all — somatotropin and prolactin) come to the fore, that lead to the development of acromegaly and hyperprolactinemia accordingly. Our work basing on two clinical cases presents main principles of diagnosis and specific clinical manifestations of TSH-secreting pituitary adenomas and demonstrates efficacy of somatostatin analogues in the treatment of this pathology.

Ectopic thyroid-stimulating hormone (TSH)oma located outside the sella turcica is exceedingly rare and can be associated with significant diagnostic delay. The clinical presentation depends on the anatomical location and size of the ectopic tumor and the degree of thyrotoxicosis. A 71-year-old woman presented with goiter and thyrotoxicosis. Initial investigations revealed elevated free thyroxine (fT4) and tri-iodothyronine (fT3) with inappropriately highnormal TSH. Assay interference was unlikely, pituitary magnetic resonance imaging (MRI) scan was reported as "normal," and germline sequencing was negative for thyroid hormone receptor ß pathogenic variants. One year later, total thyroidectomy for enlarging symptomatic goiter and suspicious nodule revealed multifocal microscopic papillary thyroid carcinoma. Six years later, she presented to an ear, nose, and throat surgeon with nasal congestion, and a sphenoid bone mass was discovered on nasoendoscopy and imaging. Ectopic TSHoma was confirmed on surgical resection, and a review of the initial pituitary MRI scan revealed the mass which had initially been missed. This is the first reported case of an ectopic TSHoma located in the sphenoid bone. Ectopic TSHoma should be considered in patients with inappropriate TSH secretion when more common differentials are excluded including thyroid hormone resistance or pituitary TSHoma. [ABSTRACT FROM AUTHOR]

Ectopic thyroid-stimulating hormone (TSH)oma located outside the sella turcica is exceedingly rare and can be associated with significant diagnostic delay. The clinical presentation depends on the anatomical location and size of the ectopic tumor and the degree of thyrotoxicosis. A 71-year-old woman presented with goiter and thyrotoxicosis. Initial investigations revealed elevated free thyroxine (fT4) and tri-iodothyronine (fT3) with inappropriately high-normal TSH. Assay interference was unlikely, pituitary magnetic resonance imaging (MRI) scan was reported as “normal,” and germline sequencing was negative for thyroid hormone receptor ß pathogenic variants. One year later, total thyroidectomy for enlarging symptomatic goiter and suspicious nodule revealed multifocal microscopic papillary thyroid carcinoma. Six years later, she presented to an ear, nose, and throat surgeon with nasal congestion, and a sphenoid bone mass was discovered on nasoendoscopy and imaging. Ectopic TSHoma was confirmed on surgical resection, and a review of the initial pituitary MRI scan revealed the mass which had initially been missed. This is the first reported case of an ectopic TSHoma located in the sphenoid bone. Ectopic TSHoma should be considered in patients with inappropriate TSH secretion when more common differentials are excluded including thyroid hormone resistance or pituitary TSHoma.

Pituitary tumours are a significant diagnostic problem in clinical practice. Considering all the rarity of thyrotropic hormone secreting adenoma it is a diagnostic and therapeutic challenge in part due to the lack of the preoperative standards. TSH-oma, besides hyperthyroidism symptoms, might be a cause of other endocrine disorders, most often dysfunction of other tropic axes and may cause neurological symptoms associated with sella expansion. We report a case of a 49-year-old male patient with macroadenoma, overt clinical hyperthyroidism, secondary adrenal insufficiency and hypogonadotropic hypogonadism. The diagnosis of thyrotropinoma was confirmed by blood tests that revealed elevated FT3 and FT4 levels together with high TSH. Thyrotropin-releasing hormone (TRH) test was performed and magnetic resonance imaging (MRI) imaging revealed the presence of macroadenoma. Histopathology examination confirmed the diagnosis of thyrotropic cell pituitary adenoma. The appropriate therapy for thyrotropin pituitary adenoma has not been elaborated in reports of this domain. Preoperatively the patient was prepared by long-acting somatostatin analogue (Sandostatin LAR) injections to reduce tumor size with good therapeutic effect afterwards underwent surgical transsphenoidal treatment successfully. However, specific treatment guidelines for pituitary thyrotropic tumors need to be elaborated more in details.

Abstract Background Graves’ disease is the commonest cause of thyrotoxicosis whilst thyrotropin (TSH)-producing pituitary adenomas (thyrotropinomas, TSHomas) are very rare and account for just 1–2% of all pituitary adenomas. Coexistence of a TSHoma and Graves’ disease has been very rarely reported. Here, we report a case of a patient whose initial presentation with primary thyrotoxicosis due to Graves’ disease, was subsequently followed by a relapse of thyrotoxicosis due to a probable TSHoma. Case A sixty-eight year old woman was referred to our department with classical features of thyrotoxicosis. Initial biochemistry confirmed hyperthyroxinaemia [free thyroxine (fT4) 20.4 pmol/L (reference range 7.0–16.0)] and a suppressed TSH [

INTRODUCTION: TSH-secreting pituitary adenomas (TSHoma) or thyrotropinoma are rare pituitary tumors that secrete an abnormal Thyroid Stimulating Hormone (TSH) which constitutes up to 1%–2% of all pituitary adenomas. Patients often remain misdiagnosed for years. This case is reported to emphasize the importance of diagnosing it early and accurately to ensure timely management and relief of symptoms before perpetual damage is done. CASE DESCRIPTION: We report a case of a 29 years old patient from Peshawar, Pakistan who was referred to Endocrine unit Hayatabad Medical Complex Peshawar for the review of his persistently raised TSH despite optimal dosing of thyroxine for 2 years. He was initially diagnosed and treated for hyperthyroidism with Neomercazole and then with radioactive iodine (RAI). Post-RAI he developed Hypothyroidism and was subsequently treated with optimal doses of Thyroxine. Despite optimal doses of thyroxine, TSH remains elevated. Previous thyroid function tests also showed persistently raised T3, T4 and raised TSH. Patient was clinically thyrotoxic. MRI findings reveals macro adenoma and was resected by neurosurgeon and advised Endocrine follow up visit in OPD after surgery. CONCLUSION: TSHoma is highly under diagnosed. It is important to consider the pitfalls in diagnosis where normal or low alpha sub unit levels could lead to dubious differentials. The use of advance technology i.e. functional MRI scans to confirm diagnosis should be carried out early on leading to timely treatment options including radiotherapy and surgery.

Despite the fact that pituitary adenomas are among the most frequent brain tumours, TSH-secreting pituitary adenomas (thyrotropinomas) are less than 1% of all adenomas. Due to the increase in the free fractions of thyroid hormones at normal or elevated TSH levels, the majority of patients with these pituitary adenomas have a long anamnesis of thyrotoxicosis which requires a differential diagnosis with thyroid pathology (Graves disease, toxic adenoma, autonomously functioning thyroid nodules). The diagnosis of the thyrotropinoma is quite challenging for clinicians. This article describes the case of a combination of the thyrotropinoma with primary hypothyroidism as a result of the Hashimotos disease. A feature of this article is the absence of a typical clinical picture of thyrotoxicosis in combination with an evaluated level of TSH on the background of constantly increasing substitution therapy for primary hypothyroidism. The picture of space-occupying lesion according to MRI of the brain allowed to suspect hormone-active pituitary adenoma (macroadenoma). As a result of surgical treatment (endonasal transsphenoidal adenomectomy), the level of TSH and free thyroid hormone levels were normalized in the postoperative period. The diagnosis of TSH-secreting pituitary adenoma was confirmed by histological and immunohistochemical analysis of postoperative material.

In most cases, estimation of tests of the functional state of the thyroid gland does not cause difficulties. Often, according to results of hormonal research, we can conclude that there is a manifest (subclinical) hypothyroidism or a manifest (subclinical) hyperthyroidism. In these situations, difficulties in diagnosing and prescribing treatment to the patient usually do not arise. However, in clinical practice, can be a situation when the results of laboratory researches either do not correspond to clinical picture or do not correspond to the normal functioning of the pituitary-thyroid axis by the principle of log negative relationship. In such situations, quite often inadequate results can be explained by laboratory errors, effect of drugs or existence of genetic disease. Also, recently more and more questions arise in the diagnosis of the syndrome of euthyroid pathology. The correct algorithm of action in this situation will play a key role in diagnosing and identification of further tactics of patient management. In this study will be considered factors that lead to changes in laboratory researches and structured approach to assess functional state of the thyroid gland, which has big importance in clinical practice.

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Objective: Data regarding rare FPAs from India, a resource limited setting, are limited. We describe a case series of rare FPAs from a single center in western India., Materials and Methods: This was a retrospective case record review of patients diagnosed between January 2010 and July 2022. The diagnosis was based on biochemical(inappropriately elevated serum FSH/LH) and pathologic (positive immunostaining for FSH/LH) features in patients with FGA, and elevated serum thyroid hormones and normal/elevated TSH in patients with TSHomas., Results: We identified 11 patients with a total of six FGAs (median age 43.5 years, five men, one FGA cosecreting TSH, median largest dimension 40 mm, range 33-60 mm) and six TSHomas (median age 34.5 years, four women, two TSHomas cosecreting GH, median largest dimension 42.5 mm, range 13-60 mm). Symptoms of sellar mass effects led to pituitary imaging in most patients with FGA. Patients with TSHomas had symptoms of excess hormone secretion (GH/TSH) or sellar mass effects. The TSHomas that cosecreted GH/FSH were larger than those secreting only TSH. Transsphenoidal resection was the most common first-line therapy but significant residual disease was frequent (3 out of 6 FGAs and 4 out of 5 TSHomas)., Conclusion: This is the first and second case series of FGAs and TSHomas, respectively, from India. In this study, TSHomas presented at younger age, were larger andhad low surgical cure rates.

Introduction: Pituitary disorders during pregnancy are uncommon. The approach should include a close follow-up in order to reduce maternal and fetal risks associated with physiological changes during pregnancy or treatment side effects. Materials and methods: We report a 21-year-old woman with a thyroid-stimulating hormone-secreting pituitary macroadenoma and positive antithyroid antibodies. She was initially treated using transsphenoidal pituitary surgery. The patient relapsed 17-month post-surgery. Somatostatin analog therapy was started which rapidly controlled the hyperthyroidism. Eleven months later, while receiving octreotide, the patient reported to be pregnant and the medication was stopped. Gestation and delivery went well with a healthy full-term newborn. The patient developed a postpartum thyroiditis 15 weeks after giving birth. Twenty-eight months postpartum the patient remains euthyroid without medication. Conclusions: The overall positive outcomes of the four cases reported in literature, including this new case, suggest that pregnancy should not be absolutely contraindicated in women with thyrotropinomas. We emphasize the effectiveness of octreotide to control hyperthyroidism, as well as stopping medication when a patient is found to be pregnant. In our case, close observation following octreotide cessation had a positive outcome. [ABSTRACT FROM AUTHOR]

Background: Thyrotropinomas (TSHoma) are rare pituitary adenomas. Case Description: A 34-year-old female presented with mild bitemporal field defect in third trimester with intact pituitary function. MRI demonstrated an enhancing lesion from the posterior planum to suprasellar, interpeduncular and prepontine cisterns with chiasmal compression and right fetal posterior communicating artery encasement. With no sellar expansion, the differentials included meningioma or craniopharyngioma. She underwent a postpartum expanded endoscopic endonasal transtuberculum transchiasmatic sulcus approach [Video 1]. The lesion was debulked in the chiasmatic cistern to decompress the chiasm with preservation of superior hypophyseal perforators. Pituitary transposition and midclival approach to access the retrosellar component was not undertaken pending formal histology as the lesion encased the perforators and was atypical for the outlined differentials. In addition, the diaphragm was intact. Postoperatively, visual field normalized and the patient developed mild diabetes insipidus. Following the diagnosis of TSHoma (with an abnormal thyroid function test [TFT]) and due to patient preference and slightly increased risk of CSF leak with revisional endoscopic procedure, she underwent an orbitozygomatic craniotomy (pretemporal and transsylvian approach) without tentorial division to resect the disease in the interpeduncular and prepontine cisterns [Video 1]. The anatomical triangles and tumor characteristics facilitated this. A residual cuff was left along the base of the stalk and the floor of the third ventricle to preserve the superior hypophyseal and thalamoperforators. Postoperatively, the patient had normal TFT without any neurological deficit. Conclusion: Operative treatment strategy is presented for a rare large challenging multicompartmental extrasellar TSHoma using endoscopic endonasal and open skull base approaches. [ABSTRACT FROM AUTHOR]

Thyrotropinomas are rare pituitary tumors that are difficult to diagnose due to their variety of clinical presentations. We describe three patients with thyrotropinoma and review the management of this disease based on current literature. Patient 1 had a typical acromegalic phenotype with a pituitary adenoma that secreted both growth hormone (GH) and thyroid-stimulating hormone (TSH). Patient 2 underwent pituitary surgery without antithyroid treatment; this tumor was also positive for both TSH and GH on pathological examination, and the patient experienced transient central hypothyroidism postoperatively. Patient 3, who had both a thyrotropinoma and autoimmune thyroiditis, underwent surgery after one year of treatment with lanreotide; the removed pituitary adenoma was positive only for TSH. To our knowledge, concomitant Graves' disease and thyrotropinoma is very rare, with only two patients previously reported. In conclusion, thyrotropinomas are increasingly being encountered when they are smaller, which has improved patient prognosis. Nevertheless, the diagnosis and perioperative management of thyrotropinomas remain challenging, which suggests that patients with such tumors should be managed by endocrinologists. [ABSTRACT FROM AUTHOR]

Thyrotropinomas (TSHomas) are rare pituitary adenomas, particularly in childhood. We present here the case of an 11-year-old boy with type 1 autoimmune polyglandular syndrome (APS1) and TSHoma which was diagnosed by elevated thyroid - stimulating hormone and thyroid hormones levels without evident clinical signs of hyperthyroidism. He was underwent partial resection of the tumor via transsphenoidal approach and subsequently radiation therapy. Consequently, 1 year after radiotherapy, the patient developed growth hormone deficiency, three and half years after radiation became euthyroid, and five and half years after treatment - hypothyroid. This is the first case of the coexistence of these two rare endocrine diseases in one patient. [ABSTRACT FROM AUTHOR]

Hormone secretion by somatotropinomas, corticotropinomas and prolactinomas exhibits increased pulse frequency, basal and pulsatile secretion, accompanied by greater disorderliness. Increased concentrations of growth hormone (GH) or prolactin (PRL) are observed in about 30% of thyrotropinomas leading to acromegaly or disturbed sexual functions beyond thyrotropin (TSH)-induced hyperthyroidism. Regulation of non-TSH pituitary hormones in this context is not well understood. We there therefore evaluated TSH, GH and PRL secretion in 6 patients with up-to-date analytical and mathematical tools by 24-h blood sampling at 10-min intervals in a clinical research laboratory. The profiles were analyzed with a new deconvolution method, approximate entropy, cross-approximate entropy, cross-correlation and cosinor regression. TSH burst frequency and basal and pulsatile secretion were increased in patients compared with controls. TSH secretion patterns in patients were more irregular, but the diurnal rhythm was preserved at a higher mean with a 2.5 h phase delay. Although only one patient had clinical acromegaly, GH secretion and IGF-I levels were increased in two other patients and all three had a significant cross-correlation between the GH and TSH. PRL secretion was increased in one patient, but all patients had a significant cross-correlation with TSH and showed decreased PRL regularity. Cross-ApEn synchrony between TSH and GH did not differ between patients and controls, but TSH and PRL synchrony was reduced in patients. We conclude that TSH secretion by thyrotropinomas shares many characteristics of other pituitary hormone-secreting adenomas. In addition, abnormalities in GH and PRL secretion exist ranging from decreased (joint) regularity to overt hypersecretion, although not always clinically obvious, suggesting tumoral transformation of thyrotrope lineage cells. [ABSTRACT FROM AUTHOR]

Abstract: We report a 33-year-old woman with invasive thyrotropin-secreting pituitary adenoma after previous thyroid ablation with radioiodine who was successfully treated with transsphenoidal surgery after pre-treatment with octreotide-LAR for 10months. Since invasive and aggressive thyrotropin-secreting macroadenomas are more frequently observed in patients who have undergone thyroid ablation, we suggest preoperative treatment with somatostatin analogs should be considered in these patients to reduce serum thyrotropin and stabilize or reduce tumor size. [Copyright &y& Elsevier]

Introduction: Thyrotropinoma (TSH-secreting pituitary adenomas, TSH-oma) are rare tumor of the pituitary (0.5-2% of all pituitary adenomas). Localization of the tumor with the appropriate neuroendocrine disorders has features. This is due to the direct damage to the nucleus and structures of the pituitary with hypersecretion of TSH, which leads to overstimulation of the thyroid gland and the emergence of the clinical picture of "central" hyperthyroidism. Objectives: To study the emotional disturbances in the structure of the clinical picture of thyrotropinoma. Methods: 26 patients with TSH-oma, normal or elevated TSH levels in combination with elevated levels of St.4, St.3 (2002-2018). 14 women (54%) and 12 men (46%), 15-67 years old (median 38.5). All tumors belonged to macroadenomas (by MRI), the diameter is 14-64 mm (median 26 mm). The criteria for inclusion in the study were high levels of free fractions of thyroid hormones and elevated levels of TSH. Results: The clinical picture was presented with symptoms of hyperthyroidism in 21 (80.7%) patients, anamnesis from 1 to 13 years (median 3). Emotional pathology was detected in 57.6% (n=15): anxiety-phobic disorders 50%, panic attacks-46.1%, depression-11.5%. Patients complained of: increased fatigue and weakness - 8 patients, heartbeat - 13, anxiety - 9, mood lability - 14, sleep disorders - 5, sweating - 5, hand tremor - 3, subfebrile temperature - 2, weight loss - 2. These symptoms were combined with each other. Conclusions: Emotional disturbances occur in 57.6% of patients with TSH-oma. The interdisciplinary approach will allow: 1) to carry out an early diagnosis of these tumors; 2) to conduct adequate medical treatment. [ABSTRACT FROM AUTHOR]

Ectopic thyrotropin-secreting pituitary adenomas are rare, with only 10 published cases. We report the case of a 52-year-old woman who was referred for primary hypothyroidism, who showed clinical signs of hyperthyroidism and had been under treatment with levothyroxine. Her exams revealed high levels of thyroid stimulating hormone (TSH), at odds with free thyroxin (FT4) and raised triiodothyronine (T3), which remained elevated after medication suspension, suggesting possible central hyperthyroidism. Sellar MRI showed normal pituitary gland, with a mass in the sphenoid sinus of 24 mm. A possible ectopic TSH secreting pituitary tumor of sphenoid sinus was hypothesized. After a intramuscularly (IM) single dose of a sustained-relase of a somatostatin analog (octreotide) 20 mg, plasma levels of thyroid hormones were normalized and a significant tumor reduction was demonstrated in MRI control at 7-weeks' follow-up. The tumor was removed by transsphenoidal endoscopy, and the biopsy confirmed an adenoma with positive immunostaining for TSH and GH. Hyperthyroidism recurrence was observed in hormonal controls 4 weeks after surgery. Treatment with sustained-release octreotide was reinitiated, every 60-days for two years, with normalization of the thyroid hormone profile, but with a residual lesion with the appearance of a tumor in the MRI. A second tumor resection was performed, achieving sustained hormonal cure and no residual tumor lesion at 2-years' follow-up. To our knowledge, this is the first report of an ectopic thyrotropin-secreting pituitary adenoma of the sphenoid sinus. Clinical and laboratory aspects relevant to this entity are reviewed, emphasizing the usefulness of octreotide in the management of the reported case. [ABSTRACT FROM AUTHOR]

An abstract of the article "Thyrotropin-Secreting Pituitary Adenoma in an Adolescent Boy: Challenges in Management," by Marion Kessler and colleagues is presented.