bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pituitary Hyperplasia Secondary to Primary Hypothyroidism Mimicking a Macroadenoma With Optical Chiasm Compression
Authors: Fujiwara Mariana Tazima , Daniel Karla Borges , Trentin Marília Bortolotto Felippe , Cerci Murilo Oliveira , Garmes Heraldo Mendes 
Published: May 2021

Abstract
Background: A long term untreated primary hypothyroidism can stimulate thyrotropes proliferation, leading to pituitary hyperplasia. This condition is known as pituitary hyperplasia secondary to primary hypothyroidism (PHPH). It is a rare condition that mimics pituitary adenoma and can achieve large proportions with optic chiasm compression. A misdiagnosis may be catastrophic, and a pituitary resection wrongly performed. Clinical Case: A 25-year-old woman with a medical history of delayed neuro psychomotor development and epilepsy due to congenital CNS malformation had a Brain MRI performed for epilepsy follow-up three years earlier. At that time, neuroimaging had shown a pituitary enlargement, and laboratory data were diagnostic of severe primary hypothyroidism with TSH of 290.6 uUI/mL (normal range 0.4 to 4.5 uUI/mL) and T4L &lt;0.23 ng/dL (normal range: 0.9 to 1.8 ng/dL). She then had received a 75 mcg levothyroxine prescription. However, the patient missed medical follow-up and returned three years later, when sella turcica MRI showed a 0.9x1.0x1,4 cm pituitary lesion, consistent with a macroadenoma with suprasellar extent near the optic chiasm. Because of the cognitive impairment, the patient was not able to complete the visual field test. Neurology service referred the patient to endocrinology evaluation for surgical treatment. Hypothyroidism was still uncontrolled with TSH 157.1 uUI/mL and T4L 0.28 ng/dL. We had adjusted the levothyroxine dose to 125 mcg and advised adherence. Subsequent thyroid function tests had shown TSH 6.91 uUI/mL and T4L 1.15 ng/dL. After thyroid function stabilization, the patient performed a new sella turcica MRI, which had not evidenced pituitary lesion. Pituitary hyperplasia secondary to primary hypothyroidism was her final diagnosis. Conclusion: This case report illustrates the importance of the correct diagnosis and treatment of PHPH. Levothyroxine replacement, with TSH normalization, reverses the gland hyperplasia within 2 to 4 months.
References: 1. Cao J, Lei T, Chen F, Zhang C, Ma C, Huang H. Primary hypothyroidism in a child leads to pituitary hyperplasia: A case report and literature review. Medicine (Baltimore). 2018 Oct;97(42):e12703.2. Shivaprasad KS, Siddardha K. Pituitary Hyperplasia from Primary Hypothyroidism. N Engl J Med. 2019 Feb 21;380(8):e9.
Presyncope in a Woman With Incidental Bladder Mass: A Case of De Novo SDHB Mutation Bladder Paraganglioma
Authors: Grover Ashna , Rodriguez Ana Del Carmen Rivadeneira , Pandit Zuha , McLaughlin Christine D 
Published: May 2021

Abstract
Background: Paragangliomas (PGLs) are catecholamine secreting neuroendocrine tumors originating from extra-adrenal neural crest cells. Urinary bladder paragangliomas (UBPGLs) are extremely rare, accounting for only 0.06% of all bladder tumors. Sporadic UBPGLs associated with Succinate Dehydrogenase B (SDHB) mutation have a higher malignant potential versus patients with a positive family history due to a lack of screening. We present a case of sporadic UBPGL detected early due to follow-up of an incidental bladder mass.
Clinical Case: A 31 year-old female presented to the ER with presyncope and lower abdominal pain while running. On arrival she was mildly hypertensive with BP 140/88 mmHg, HR was 98 beats/min, other vital signs were stable. Denied headaches, diaphoresis, or weight loss. No known personal or family history of any endocrine disorders or cancer. CT abdomen pelvis performed for the evaluation of her abdominal pain revealed a large fibroid along with an unexpected finding of a 3 cm enhancing lesion in the right bladder wall, suggestive of a PGL. 24 hr urine total catecholamines and plasma total metanephrines were elevated at 284 mcg (normal range 26-121 mcg/24h), and 386 pg/mL (normal &lt;205 pg/ml) respectively, confirming the diagnosis of UBPGL. Chromogranin A was within the normal range. Metastasis was ruled out with metaiodobenzylguanidine (MIBG) whole-body scan and octreotide scan which showed isolated uptake in the bladder. Pan CT scan was negative for other SBHB mutation associated tumors such as RCC (14% incidence), GIST, and thyroid carcinoma. Genetic testing with PGLNest revealed a heterozygous SDHB mutation (5’-3’ UTR_5’-3’ UTR deletion). Her parents were found to be negative for the mutation. During follow up she developed episodic palpitations. She was treated with alpha followed by beta blocker and underwent successful resection of the tumor with partial cystectomy, and of the fibroid with myomectomy on the same day (TAH was avoided to minimize manipulation of the uterus hence decreasing the risk of intra-abdominal HTN crisis). She has been tumor-free in the three years of follow up post-surgery with imaging and biochemical surveillance.
Conclusion: This case illustrates the importance of following up on incidental radiographic findings and adds to the limited literature on sporadic UBPGLs, an uncommon, yet potentially life-threatening tumor. The diagnosis of these tumors is especially challenging when patients present with non-specific symptoms. Fortunately, in our case, the imaging study clued us into the possibility of a PGL. SDHB mutation associated PGLs, in particular, are notorious for their late presentation with metastasis and associated tumors. Hence, timely detection with close follow-up of these patients will likely have an impact on their mortality.
Secondary Adrenal Insufficiency and Primary Hypothyroidism: A Complication of Cancer Immunotherapy
Authors: Manrique Helard Andres 
Published: May 2021

Abstract
Background: Immunotherapy has a fundamental role in cancer treatment. However, there have been found secondary effects in different organs due to its use, such as thyroid gland, pituitary gland, and adrenal glands,. This case report describes a case in relation with nivolumab, an antibody that blocks PD1 protein activity, promoting apoptosis Clinical Case: 67 years-old woman, with history of vulvar melanoma since 2016, complicated by pulmonary metastases. She was started with Nivolumab on 2018, every three weeks. After the her last immunotherapy session, she had less appetite, confusion, and incoherent speaking, and was admitted to the hospital In the Emergency, her blood pressure was 70/40mmHg, heart reate was 120 beats per minute. She was treated with fluid replacement. Her initial lab tests showed sodium 115.0 mmol/l(135.0 - 145.0), Chloride 85.9 mmol/l(98.0 - 109.0), Potassium 4.61 mmol/l (3.5 - 5.1), Hemoglobin 12.0, Leucocytes 6500, Platelets 282000, Total Proteins 7.0 (Albumin 4.0 Globulin 3.1), Alanine aminotransferase 10.0U/L (7-56), Aspartate aminotransferase 33.0 U/L (4-50) Creatinine 0.53. Hormonal Profile: T3 (triiodothyronine) 1.33 ng/ml(0.8 - 2.0), T4 (tetraiodothyronine) 7.27 ug/dl (5.1 - 14.1), TSH (thyroid stimulating hormone) ultrasensitive 12.87 uU/mL(0.27 - 4.2), FSH (Follicle stimulating hormone) 26.6mUI/ml, LH (Hormone)10.86 mIU/ml, Prolactin 60.96 ng/ml (6.0 - 29.9), Cortisol (am)0.22 ug/dl (4.2 - 38.4), ACTH (Corticotrophin-releasing hormone) &lt;1.00, Anti TPO (Anti-Thyroid Peroxidase) negative, Glucose: 145 mg /dl, C Peptide: 0.4 ng/ml (0.9 - 7.1). Her brain MRI did not showcerebral edema or metastases. Conclusion: This case concerns metabolic encephalopathy due to severe hyponatremia caused by a secondary adrenal insufficiency (partial hypopituitarism), subclinical hypothyroidism and hyperprolactinemia, related to Nivolumab immunotherapy. Endocrinologists should be aware PD-1 inhibitor’s side effects, of its immunologic modulation mechanisms as they can cause hypophysitis, pituitary pituitary and thyroid dysfunction
Severe Hyperprolactinemia in a Patient With Concurrent ESRD and Medication Use
Authors: Johns Allison , Gorris Matthew 
Published: May 2021

Abstract
Background: A mild increase in prolactin has been previously reported in patients with pathological conditions, such as ESRD, as well as with several medications. Typically this is a mild elevation (&lt;100 ng/mL), though certain medications have been shown to increase prolactin up to 200 ng/mL. However, we report a case of severe hyperprolactinemia in a patient with ESRD and on medications known to cause mild prolactin elevations. Clinical Case: Our patient is a 35-year-old female presenting for hyperprolactinemia. She has a history of uncontrolled diabetes with complications of ESRD on iHD, gastroparesis, and neuropathy. Further history revealed galactorrhea for 15 years following the birth of her second child as well as amenorrhea for the last 1.5 years. At the time of evaluation, she was not breastfeeding and did not have any reported life stressors. Medications were unchanged and included metoclopramide 10 mg daily and promethazine 25 mg PRN. Initial prolactin level one year prior to visit was 618 ng/mL. TSH 1.33. Brain MRI at that time did not reveal a pituitary mass, though pituitary protocol was not performed. Prolactin repeated on our initial visit had increased to 1352 ng/mL. Pituitary MRI was without evidence of pituitary mass. Despite discussing the importance of estrogen replacement, the patient was not interested in OCP therapy due to prior intolerance. Clinical Lesson(s): Hyperprolactinemia can be seen in patients on hemodialysis as a result of increased secretion in the uremic state and decreased clearance. Dialysis does not significantly improve the prolactin level. A recent study revealed a median prolactin of 65.2 ng/mL in dialysis patients. Our case emphasizes that the degree of hyperprolactinemia as a result of ESRD and medications can vary widely. It remains important to continue to consider the diagnosis of pituitary pathology in patients with a prolactin level that is higher than expected. However, if imaging is unrevealing, as in our patient, hyperprolactinemia may be attributed to medications and pathological disease states. Elevated serum prolactin has been shown to increase cardiovascular and all-cause mortality in a level-dependent effect. Hyperprolactinemia has also been associated with insulin resistance and worsened diabetes control. As we continue to learn more regarding the negative side effects of prolactin, it may become increasingly important to monitor and reduce prolactin levels in our patients, especially those on dialysis already with an elevated risk of cardiovascular disease.
Silent Pituitary Adenoma in Nasal Cavity
Authors: Bashir Bilal , Adam Safwaan , Cains Fiona , Higham Claire Emily , Pathmanaban Omar , Dhage Shaishav 
Published: May 2021

Abstract
Introduction: Silent corticotroph adenomas (SCA) represent 3-6% of all pituitary adenomas. SCA are confirmed on post-surgical histological tumour staining and can either have no prior clinical or biochemical evidence of excess ACTH or alternatively only biochemical (not clinical) evidence of Cushing’s disease. SCA are potentially large and aggressive tumours with a greater tendency to post-surgical recurrence. We present a case of a giant SCA which pre-operatively didn’t exhibit any clinical signs of cortisol excess but showed signs of early recurrence on follow up imaging. Case: A 40-year-old man presented with frontal and maxillary sinus pain and nasal stuffiness. Nasal endoscopy revealed a mass in the nasal cavity and sphenoid sinus. Subsequent CT scan of his sinuses and MRI Pituitary reported a 4 x 3.5 x 2.5 cm sellar mass extending through the floor of sella into the sphenoid sinus and nasal cavity. Despite no clinical features of any pituitary hormone excess (including Cushing’s) or deficiency, his biochemical parameters were consistent with hypercortisolaemia. Post-low dose dexamethasone (2 mg over 48 hours) suppression cortisol was inadequate: 106 nmol/L (&lt;50nmol/L). His corresponding serum dexamethasone level was 8.6 nmol/L (&gt;3nmol/L), ACTH 46 ng/L (0-46 ng/L) and 24-hour urinary free cortisol levels were 502 and 260 nmol/24 hours (&lt;165 nmol/24 hours)) on 2 separate occasions. The remainder of his pituitary profile was normal. He therefore underwent expanded endonasal endoscopic resection of tumour without significant complication. His post-operative cortisol was &lt;50nmol/L (other pituitary hormone measurements normal) so hydrocortisone replacement therapy was commenced. A 3-month post-operative MRI pituitary scan revealed near total resection of the tumour with peripheral enhancement within the tumour resection site representing post-surgical changes. Histopathology showed sparsely granulated, diffuse cytokeratin immunostaining and immunopositivity for ACTH and T-pit consistent with a corticotroph PitNet of the sparsely granulated variety with low Ki 67 index. His six month post-operative scan showed recurrent sellar mass of 2.2 x 0.9 x 1.4 cm. Again there was an absence of clinical features of Cushing’s syndrome. After multidisciplinary discussion clinical, biochemical and radiological surveillance was continued with a view to further transsphenoidal surgery and/or radiotherapy depending on the progress of the tumour.
Conclusion: Our patient solely had biochemical (not clinical) evidence of Cushing’s disease despite a large pituitary tumour in keeping with an SCA, which are aggressive, have high recurrence rate and shorter time to recurrence after surgery. Close and frequent post-operative clinical, biochemical and radiological surveillance is critical to detect and manage early recurrence that may require multimodal therapy.
Stuck at the Checkpoint: Pembrolizumab Induced Hypophysitis With ‘Normal’ Cosyntropin Response
Authors: Kasiri Elnaz , Pinsker Richard W , Ravich Marina , Patel Bijal 
Published: May 2021

Abstract
Introduction: Pembrolizumab is an immune checkpoint inhibitor (ICI) that blocks programmed cell death receptor 1 (PD-1) and is indicated in treatment of malignancies including lung adenocarcinoma and melanoma. Anti-PD-1 therapies are responsible for immune related adverse events including endocrine dysfunction. Here we present a case of Pembrolizumab induced hypophysitis causing central adrenal insufficiency apparently missed by a 250 mcg cosyntropin test.
Clinical Case: A 68 year old female with history of COPD and stage 4 lung adenocarcinoma with bone metastases presented to the ED with hypotension during her 14th cycle of treatment with Pembrolizumab. Other symptoms included fatigue and anorexia for about 4 weeks. She denied vomiting, diarrhea, bleeding episodes or chronic steroid use. On exam, patient was obese and pale appearing, with decreased bilateral breath sounds without edema. Random cortisol on admission at 4:44 pm was 6.9 ug/dl, ACTH - was in process, Na was 135 (137 - 145 mEq/L) and K was 3.7 (3.5 - 5.1 mEq/L). A 250 mcg cosyntropin stimulation test resulted in 30 min cortisol level of 22.5 µg/dL and 60 min cortisol level of 34 µg/dL. Patient was treated with fluids, salt tabs and eventually midodrine, however BP remained borderline low. On day 5, the patient was started on IV methylprednisolone 40 mg Q8 hrs for COPD exacerbation. A few days later pre-steroid ACTH was reported as &lt;5 pg/ml (6-50 pg/ml) which prompted further workup for hypopituitarism. LH and FSH levels were inappropriately low for a postmenopausal female at 0.2 and 2.6 mIU/ml respectively, Free T3 was 2.02 pg/ ml (2.77-5.27 pg/ml), free T4 was 1.07 ng/dl (0.80-2.20 ng/dl), and TSH was 0.67 uIU/ml (0.47-4.70 uIU/ml). MRI brain showed partially empty sella. Patient was diagnosed clinically with Pembrolizumab induced hypophysitis causing central adrenal insufficiency. Eventually, steroids were tapered to prednisone 5 mg daily maintenance dose and patient was discharged with stress dosing instructions.
Conclusion: Diagnosis of adrenal insufficiency is challenging as advanced malignancy and adrenal insufficiency can cause similar symptoms. The finding of an empty sella here is a confounding factor. Checkpoint inhibitors are known to cause hypophysitis, thyroiditis and primary adrenal insufficiency, however incidence is &lt;1%. We report a case of missed adrenal insufficiency by a falsely assuring cosyntropin test. Based on our experience, we conclude that when suspecting a diagnosis of checkpoint inhibitor induced adrenal insufficiency, we should start by checking random cortisol and ACTH value. A standard 250 mcg cosyntropin test should not be used solely to completely rule out this diagnosis.
Successful Multimodal Treatment of a TSH-Secreting Pituitary Adenoma (TSH-oma)
Authors: Lee Donna , Mahali Lakshmi , Tabatabaie Vafa 
Published: May 2021

Abstract
Background: TSH-omas are rare tumors accounting for 0.5-2% of all pituitary adenomas. Due to their indolent nature, most TSH-omas are diagnosed at the stage of invasive macroadenomas. Over the past several decades, the management of TSH-omas has evolved substantially. While surgery remains first-line therapy, somatostatin analogs have emerged as important therapeutic agents as a result of their effectiveness in normalizing thyroid hormone levels in ~95% of patients with severe hyperthyroidism and reducing TSH-oma size in ~50% of patients.
Clinical Case: A 52-year-old woman with a history of multinodular goiter was incidentally found to have a 2.2 x 1.8 x 2.1 cm pituitary macroadenoma with suprasellar extension, mass effect on the optic chiasm, and left cavernous sinus involvement when she presented with chest pain, palpitations, headache, and left-sided numbness and weakness. Laboratory results showed high FT4/T4/T3 with inappropriately high TSH, elevated α-subunit, and low cortisol with low-normal ACTH highly suggestive of TSH-oma with concurrent secondary adrenal insufficiency. An ophthalmology exam revealed a left superior temporal defect. The patient was treated with atenolol, prednisone, and octreotide two weeks before surgery with symptomatic improvement and near-normalization of FT4. Following an uncomplicated transsphenoidal resection, FT4 normalized within one week. At her one-month follow-up, both TSH and FT4 were normal, and her secondary adrenal insufficiency had resolved. Her visual field defect also recovered.
Laboratory Results: TSH 5.35 (normal range 0.40-4.60 μU/mL), FT4 3.0 (0.8-1.7 ng/dL), T4 18.1 (5.0-12.0 μg/dL), T3 235 (80-200 ng/dL), ACTH 10 (6-50 pg/mL), cortisol 4.5 (5.0-25.0 μg/dL), α-subunit 8.0 (0.1-1.5 ng/mL); after 2 weeks on SQ octreotide 50mg q12h: TSH 1.93 (0.30-4.20 μU/mL), FT4 1.7 (0.6-1.5 ng/dL); 1 month post-op: TSH 1.53 (0.30-4.20 μU/mL), FT4 0.8 (0.6-1.5 ng/dL), ACTH 12 (7.2-63 pg/mL), cortisol 6.9 (4.0-20.0 μg/dL)
Conclusion: Since the first reported case of TSH-oma in 1960, the diagnostic and therapeutic management of these rare pituitary adenomas have evolved due to the emergence of ultrasensitive TSH assays, advanced imaging and surgical techniques, and somatostatin analogs. However, to this day, most TSH-omas are still diagnosed at the stage of invasive macroadenomas, when successful surgical resection becomes more difficult. Hence, up to two-thirds of patients may require adjuvant therapy with medication or radiation. As evidenced in our patient, who achieved a near-euthyroid state within just two weeks of starting low dose octreotide, somatostatin analogs are highly effective in controlling hyperthyroidism and have solidified their place in the therapeutic management of TSH-omas. This case highlights the success of a multimodal approach to the treatment of TSH-omas.
The Sodium See-Saw: Prolonged Second Phase of Diabetes Insipidus and Complete Resolution Following Surgical Resection of a Supra-Sellar Meningioma
Authors: Pradeep Pallavi , Gandhi Shriya , Amin-Hanjani Sepideh , Kukreja Subhash C 
Published: May 2021

Abstract
Background: Diabetes insipidus (DI) is a common occurrence after sellar/suprasellar surgery. However, signs of recovery after a few days should be treated with caution as this may precede a “triple response”, seen in 3.4% of the patients. In this response, initial postoperative DI gives way to a period of remission or clinically inappropriate antidiuresis before eventual recurrence of DI.
Clinical Case: A 38-year-old Hispanic female was admitted to the hospital for elective transcranial resection of a suprasellar meningioma extending along the planus sphenoidale, measuring 23x19x17mm. Preoperatively, all pituitary hormones were in the normal range. On POD1, she was noted to have polyuria with urine output of &gt;400ml/h, uptrending sodium levels (148, RR: 135-145mmol/L), low urine specific gravity (&lt;1.005, RR:1.005-1.030) and low urine osmolality (82, RR:300-900mOsm/kg). She was diagnosed with DI and was treated with DDAVP over the following three days. Follow-up MRI was done. In addition to post-surgical changes, it showed acute ischemia in the region of the anterior commissure.
After short-term improvement in her clinical status, she was noted to have decreased thirst and decreased urine output on POD6. Sodium was low at 132mmol/L. Urine specific gravity was 1.017. Serum osmolality was 258 mOsm/kg (RR:279-300 mOsm/kg). There was concern for developing SIADH vs concurrent adrenal insufficiency. Patient had received methylprednisolone perioperatively, hence AM cortisol testing would have been inaccurate. Fluid restriction, along with empiric glucocorticoid replacement, was initiated. Sodium continued to worsen with nadir of 122mmol/L and patient reported new onset headache. She was started on 3% (hypertonic) saline continuous infusion and salt tablets. Hyponatremia gradually resolved by POD13, 7 days after onset of SIADH. Salt tablets and fluid restriction were discontinued. Patient was seen in clinic on POD17 and reported normal thirst and urine output. Sodium was noted to be 143mmol/L.
Conclusion: This case highlights the rare presentation of a partial triphasic sodium imbalance after resection of a suprasellar mass, with a prolonged second phase lasting for 7 days. A few studies have reported that it may last longer when pituitary gland is not damaged preoperatively, as was the case in our patient. Similarly, complete recovery may also occur if the hypothalamus and pituitary are normal preoperatively. Postoperatively, patient was noted to have an anterior commissural ischemia. This could have contributed to the fluctuations in ADH secretion, due to the rostral proximity of the hypothalamus to the anterior commissure.
Despite adequate measures, sodium may continue to drop dramatically in the second phase. Patients should be monitored for changes in thirst and normalization of urine output, as these may indicate early symptoms of impending SIADH before hyponatremia develops.