bluuebunny/crossref_metadata_2025_split huggingface dataset:
Co Existence of a Corticotroph Adenoma and a Multifunctional Pituitary Cyst
Authors: McDonnell David John , Kennedy Carmel , Qaoud Yazan , Heverin Karen , Smith Diarmuid , Javadpour Mohsen , Agha Amar 
Published: May 2021

Abstract
The co-existence of a corticotroph adenoma and a pituitary cyst is very unusual. We present the case of a 50-year-old female who presented with a Cushingoid phenotype, severe hypokalaemia, hyperglycaemia and hypertension. Urinary free cortisol was markedly elevated at 50 fold. ACTH levels were elevated at 121.4 pg/ml. She failed both the low and high dose dexamethasone suppression tests. The CRF test did not show a satisfactory rise in ACTH levels but the Inferior petrosal sinus sampling revealed a central to peripheral ACTH gradient highly suggestive of Pituitary dependant Cushing’s. MRI pituitary revealed a large cystic lesion with a small solid component. Computed Tomography (CT) of thorax abdomen and pelvis was normal. A trans-sphenoidal hypophysectomy was performed, during which a fine needle aspiration of the intra-cystic fluid was obtained. This showed markedly elevated pituitary hormone levels of ACTH (1399pg/ml), prolactin (353,084mIU/L), TSH (217IU/L) FSH (205mIU/ml) and GH (519 ng/ml) consistent with a multifunctional pituitary cyst. Neuropathology of the solid component confirmed a corticotroph adenoma. Post-operative am cortisol levels were persistently suppressed to less than 50 nmol/l with marked improvement in clinical features. This case highlights the challenges in the work up of Cushing’s syndrome and the limitations of diagnostic tests. The coexistence of a corticotroph adenoma and a multifunctional pituitary cyst is very unusual and to our knowledge has not been reported before.
Neurosarcoidosis and the Pituitary
Authors: Afreen Samina , Mittal Ashima , Hasan Farhad 
Published: May 2021

Abstract
Background: Sarcoidosis involves the nervous system in 3-10% of cases. Neurosarcoidosis (NS) can mimic other diseases, leading to delay in diagnosis and management. We present 2 cases of hypothalmo-pituitary (HP) NS with widely differing initial presentations.
Case Presentations: Case 1: A 58 year old African American (AA) male presented with decreased libido and hot flashes of 8-10 months duration. He also reported headaches, pain in the face and behind the eyes and dyspnea on exertion. On evaluation, he had central hypogonadism. MRI brain showed thickening and enhancement of pituitary infundibulum (PI). CT chest showed lung lesions and bilateral hilar adenopathies. EBUS with biopsies of lung and hilar lymph node showed non caseating granulomas (NCG). He was started on Prednisone and testosterone leading to symptomatic relief. However, he stopped prednisone after 3 months due to weight gain. He was then started on azathioprine. 9 months later, he presented with recurrence of neurologic symptoms and weight loss of 30 lbs. Labs showed new findings of low prolactin, low IGF-1, central hypothyroidism and central adrenal insufficiency (AI). MRI pituitary showed diffuse thickening of 3rd and 5th cranial nerves and of PI. He was re-started on Prednisone and levothyroxine (LT4) was started with improvement of symptoms. Plan was to start him on Infliximab instead of Azathioprine. Case 2: A 26 year old AA female presented with near syncope and visual impairment in the left eye that started a few weeks prior. Testing showed superior visual field loss and evidence of optic neuropathy, disc edema with mild dyschromatopsia in the left eye. She had had polyuria and polydipsia for the preceding 2-3 months and amenorrhea for 2 years. Lab testing revealed hyperprolactinemia and central DI, hypothyroidism, AI and hypogonadism. Pituitary MRI revealed a suprasellar mass measuring 20 x 19 x 11 mm, displacing the optic chiasm. CT scan showed bilateral hilar lymphadenopathy and lesions in liver and spleen. EBUS and biopsy of mediastinal lymph nodes showed NCG. Patient was treated with IV steroids leading to improved vision. She was started on DDAVP and LT4 and initiated on methotrexate and prednisone on discharge.
Discussion: Pituitary hormone abnormalities due to HP NS include anterior hypopituitarism, hyperprolactinemia and DI. The most common (MC) presentation is of DI. Among anterior pituitary hormone deficits, central hypogonadism is the MC. Systemic high dose glucocorticoids (GC) are the mainstay of initial treatment. Optic atrophy is the MC neuro-ophthalmologic manifestation of NS. Only 10 - 20 % of patients have been found to have biochemical improvement of HP axis in response to high-dose GC treatment. Since pituitary involvement commonly leads to permanent endocrine deficits, it is essential to recognize it early and to treat it aggressively to prevent further permanent hormonal and neurologic deficits.
New Treatment Options for Pituitary Granulomatosis With Polyangiitis
Authors: Boharoon Hessa , AlAmeri Majid , Alnuaimi Abdulla Mohamed , Mendoza Nigel , McAdoo Stephen , Tomlinson James , Levy Jeremy , Wernig Florian 
Published: May 2021

Abstract
Background: Granulomatosis with polyangiitis (GPA) rarely involves the pituitary gland and has been reported in 1% of all cases of GPA. Most frequently, it presents with pituitary mass effect and diabetes insipidus (DI). To date, there are no treatment guidelines for this rare condition. Case Presentation: Case 1: A 55 year old female with a history of ANCA-positive pulmonary GPA, previously treated with glucocorticoids and immunosuppressants, presented two years later with cranial DI and bitemporal hemianopia. MRI showed a large sellar mass with suprasellar extension. High dose glucocorticoids resulted in good clinical and radiological response. Further treatment consisted in a combination of Cyclophosphamide and Rituximab (RTX). Further doses of RTX are planned aiming for a period of B-cell depletion. Case 2: A 38 year old female, presented with polyuria, recurrent nosebleeds, headaches and a left visual field defect. Pituitary profile revealed ACTH deficiency and MRI showed a heterogenous cystic lesion with peripheral enhancement and stalk thickening. Steroid replacement led to immediate improvement in her symptoms. Cranial DI was confirmed and raised Proteinase 3 (PR3) antibody suggested GPA. A combination of prednisolone and Methotrexate led to significant improvement of MRI appearances and declining PR3 antibody levels. For remission maintenance, two cycles of RTX were given with further radiological and biochemical improvement, and, following dynamic assessment of her HPA axis, she could be fully weaned off steroids. Case 3 is a 47 year old female with a history of childhood asthma. She was found to have cavitating lung lesions. ANCA positivity confirmed GPA and she was commenced on high-dose steroids. During follow-up, she developed headache, polyuria and polydipsia. MRI pituitary showed a suprasellar lesion and pituitary biopsy revealed inflammatory hypophysitis. Cranial DI was confirmed by water deprivation testing. Previous allergic reactions to both RTX and Ofatumumab precluded anti-CD40 monoclonal antibodies and she was commenced on Azathioprine. A further recurrence of pituitary GPA necessitated escalation of the steroid dose and switch of azathioprine to mycophenolate mofetil. She remains in remission and her steroids reduced to a maintenance dose. Conclusion: GPA pituitary has been observed to occur at variable time after diagnosis often in the absence of any other systemic features. A combination of glucocorticoids and RTX has been approved for severe relapsing pulmonary GPA, however, limited data is available for pituitary GPA. In this case series, the response to high dose steroids and RTX for remission maintenance has been encouraging. Experience with ‘conventional’ immunosuppresants remains limited and therapeutic responses remain variable. Further clinical studies are required to establish effective treatment for pituitary GPA.
Antiandrogens Target TMPRSS2 and Reduce SARS-CoV-2 Virus Entry in Lung Cells
Authors: Leach Damien A , Andrea Mohr , Zwacka Ralf , Giottis Stathis , Yates Laura , Lloyd Clare , Brooke Greg N , Bevan Charlotte Lynne 
Published: May 2021

Abstract
The SARS-CoV-2 coronavirus is the cause of the COVID-19 pandemic. Entry of the virus into host cells, most destructively lung cells, requires two host cell surface proteins, ACE2 and TMPRSS2, downregulation of which is thus a potential therapeutic approach for COVID-19. Both of these cell surface proteins are steroid regulated: TMPRSS2 is a well-characterised androgen-regulated target in prostate cancer. Analysis of sequencing data shows co-expression of the androgen receptor (AR) and TMPRSS2 in key human lung cell types that are targeted by SARS- CoV-2. We show that treatment with antiandrogens such as enzalutamide (a well-tolerated drug widely used in advanced prostate cancer) significantly reduces TMPRSS2 levels in human lung cells and in vivo in mouse lung. We demonstrate that AR binding in the region of the TMPRSS2 gene differs between lung and prostate, identifying distinct regulatory regions. Together, the data and evidence presented supports clinical trials to assess the efficacy of antiandrogens as a treatment option for COVID-19.
Sellar Plasmacytoma: An Unusual Presentation of Anaplastic Multiple Myeloma
Authors: Motlaghzadeh Yasaman , Sellmeyer Deborah 
Published: May 2021

Abstract
Background: Anaplastic multiple myeloma (AMM) is an extremely rare morphological subtype of multiple myeloma that has been reported only sporadically. Furthermore, pituitary involvement by multiple myeloma is rare and can be misdiagnosed as a nonfunctioning pituitary adenoma, due to its radiological and clinical similarities. Clinical Case: A 46-year-old man with no past medical history presented with a 3-month history of left shoulder pain. Initial physical exam revealed a large left neck mass, which prompted a left shoulder x-ray remarkable for left supraclavicular mass. Chest CT scan showed a bony mass (7 x 4 x 4 cm) in the left anterior second rib with extra osseous soft tissue involvement. CT also noted left supraclavicular/infraclavicular and left axillary adenopathy. Patient also reported lightheadedness and double vision for a few months and found to have diplopia with upward gaze on exam. Brain MRI revealed uniformly enhancing mass in right sphenoid sinus involving the sella measuring 2.3x2.6x3.2cm. With exception of low Hemoglobin (10.5, 13.5 - 17.7 g/dL) and mildly elevated prolactin (29.9, 2.0 - 20.0 ng/mL), laboratory testing was within normal limits including complete blood count, metabolic panel and pituitary hormone levels. Patient underwent fine needle aspiration of the supraclavicular node, which showed anaplastic plasma cell neoplasm. Further work up noted elevated M-spike, Kappa light chain 92.7 (0.3-2.0 mg/dl), kappa/lamda ratio 23 (0.3-1.6) and beta-2 microglobulin 4324 (600-2,200 ng/mL). Bone marrow biopsy confirmed 70% myeloma involvement with kappa monotypic plasma cell population expressing CD20, CD138, CD38, and CD117. Patient underwent endoscopic endonasal trans-sphenoidal biopsy of the mass. Frozen section pathology revealed a plasma cell neoplasm, and a subtotal resection was performed. Final surgical pathology confirmed anaplastic plasma cell neoplasm. He was started on systemic chemotherapy and external beam radiation. Conclusion: Sellar involvement by myeloma is very rare with less than 50 cases reported in the literature. While rare, these tumors should be considered in the differential diagnosis for lesions involving the sella as the therapeutic approach is different from other sellar masses. While the radiographic appearance can be similar, pituitary plasmacytomas typically present with headache and neurological signs such as cranial nerve palsies and visual changes, with preserved pituitary function. Close attention to physical exam and lab parameters can provide clues to this rare diagnosis.
She Has a Big and Weak Heart
Authors: Vallepu Shirisha R , Bantu Sravani , Gunda Mouna , Tadisina Shourya , Thudi Vaishali 
Published: May 2021

Abstract
Background: Acromegaly is a rare clinical syndrome caused by excessive production of growth hormone. The incidence rate is 3 to 4 cases per million people per year. It is a sporadic disease seen in middle-aged adults. Anterior pituitary adenoma secreting the growth hormone (GH) is the most common etiology. Other rare causes include hypothalamic and neuroendocrine tumors. Treatment modalities include surgical, radiotherapeutic and pharmacological with a goal to reduce or remove the tumor, prevent recurrences, and improve comorbidities. The persistent hypersecretion of GH and insulin-like growth factor-1 (IGF-1) leads to an overgrowth in many tissues, including connective tissue, cartilage, bone, skin, and visceral organs. Cardiovascular disease is one of the critical systemic complications resulting in about 60% of the deaths. The cardiovascular manifestations include hypertension, cardiomyopathy (CMP), congestive heart failure, and arrhythmias. Our patient presented with congestive heart failure and dilated CMP in the setting of undiagnosed Acromegaly. Clinical Case: 45 -year old Caucasian lady presented with complaints of worsening shortness of breath, chest tightness, and palpitations for one month. Past medical history includes hypertension, type 2 diabetes mellitus, obesity, and hyperlipidemia. She was diagnosed with dilated CMP in her early 30s, AICD placement seven years ago and normal cardiac catheterization three years ago. On physical exam, she was found to have an enlarged nose and bibasilar crackles. Vitals were stable and proBNP: 4,699 (1-150 pg/ml). ECHO revealed EF of 30-35%, severely dilated left ventricle. Cardiac catheterization showed severely decreased left ventricular systolic dysfunction and elevated LVEDP. Further workup - GH: 16 (&lt;10 ng/ml) and IGF-1: 637 (62 - 205 ng/ml), A1C 13.2%. Pituitary MRI showed a 1.6 cm macroadenoma, extending to the right cavernous sinus border with a deviation of the pituitary stalk to the left. On neurosurgical evaluation, she was considered to be a poor surgical candidate due to her underlying CMP. Currently, she is in the process of getting started on oral octreotide treatment. Conclusion: Cardiovascular disease is the most captious complication and a leading cause of mortality in Acromegaly patients. The subtle clinical presentation results in a diagnostic delay for 5-10 years. A careful review of history and physical examination is vital to identify acromegalic features in non-ischemic DCM. Early diagnosis with prompt treatment has shown improvement in cardiac function and reduced mortality. This case underscores the importance of considering Acromegaly as one of the differentials in patients with dilated cardiomyopathy.
The Biggest Man in the Room
Authors: Tariq Sarah 
Published: May 2021

Abstract
A 42 year old gentleman who had been healthy all his life, began to develop new clinical symptoms including acid reflux. He was tested for H. Pylori by his PCP, and treatment was initiated. During this visit he was also noted to have elevated BP and was ultimately diagnosed with HTN, and started on anti-hypertensives. During these revelations he brought up the fact that he has also developed excessive joint pains over the years, and his shoe size has increased from the age of 30 to now by 3 sizes. When asked, he admitted to noticing an increase in his hands size, and also noted that his rings do not fit anymore. His fiancée works in medicine and requested he be screened for acromegaly, thus PCP checked IGF-1 and GH levels, which were noted to be significantly elevated at 887 and 20.9, respectively, leading to a referral to Endocrinology. Under Endocrinology care he underwent a GH suppression test resulting in a non-suppressed GH at 17.8. This was followed by a Pituitary MRI revealing an 18 x 19 x 18 mm solid/partially cystic hypoenhancing lesion consistent with a pituitary macroadenoma, with leftward deviations of the infundibulum, without involvement of the optic chiasm. He was treated with transsphenoidal resection of the pituitary tumor. On post-op day 4, the IGF level had fallen to 456. His thyroid and gonadal axes were assessed and did not show any evidence of deficiency post-operatively. He was however started on Prednisone post-operatively, and was switched to Hydrocortisone in the outpatient setting with plans to taper and re-assess his HPA axis. Overall he is doing well, and there are plans for repeat labs and imaging about 6 weeks post-operatively. When evaluating patients with a variety of newly developing symptoms, remember to consider a unified diagnosis that could explain the cluster of symptoms that they are experiencing. Although rare, pituitary disorders such as Acromegaly should be explored in patients with clinical features of growth hormone excess, such as macrognathia, enlargement of the hands and feet, and in addition development of other clinical symptoms such OSA, CVD, Type 2 DM, and arthropathies.
TSH, GH, and Prolactin Producing Pituitary Adenoma: An Unusual Case Presentation
Authors: Manrique Helard Andres , Pinto Miguel Eduardo 
Published: May 2021

Abstract
Background: Pituitary adenomas are benign and frequent tumors, some of them with tendency to be recurrent or invasive e. several pituitary tumors are positive for secretion of one or more hormones of the same adenohypophysial cell lineage. However, some cases have a subclinical course and are underestimated because they have plurihormonal secretion of different adenohypophyseal cells lineage. Clinical Case: A 38 years old women with chronic headaches, who is in fertility treatment for 12 month ago presents to the clinic with unremarkable physical examination. Her laboratory studies include: Normal campimetry. T3 (triiodothyronine) 2.15 ng/m L (NR:0.8 - 2.0), TSH (thyroid stimulating hormone) 4.4 uU/mL (NR:0.27 - 4.2), T4 (tetraiodothyronine) 13.54 ug/dl, (NR:5.1 - 14.1), Free T4 2.44 ng/d (NR:0.93 - 1.7), Estradiol 60.2 pg/ml, FSH (Follicle stimulating hormone) 4.3 mUI/ml (NR:1,7-12), Cortisol (?8 am) 9.29 ug/dl (NR:4.2 - 38.4), ACTH (Corticotrophin-releasing hormone) 13.1 pg/ml (NR: 0.0 - 46.0), Somatomedin C (IGF-1) 231 .0 ng/ml (NR for age?: 57.0-241.0). Brain MRI with pituitary protocol showed in T1 an isointense lesion and slightly heterogeneous in T2 in the anterior and left aspect of the turkish saddle, of 1 x 1.1 x 1.1 cm of anteroposterior, transverse and cephalocaudal diameter, suggestive of a macroadenoma. The tumor pathology showed, possitive for a pituitary adenoma. Immunohistochemistry results were negative for: ACTH:, positive for GH, TSH: (+++) and for Prolactin:. Her post-operative findings showed a residual pituitary gland without significant findings. Her post operative pituitary axis include: Sodium 139.2 mmol/l (NR”135.0 - 145.0), Chloride 100.8 mmol/l (NR95.0 - 115.0), Potassium 3.71 mmol/l (NR3.5 - 5.1), ACTH 4.1 pg/ml(NR0.0 - 46.0), Cortisol (am) 11.11 ug/dl (NR4.2 - 38.4), Estradiol 106.5 pg/ml, FSH 5.1 mUI/ml, LH (Luteinizing Hormone) 13.53 mIU/ml, Somatomedin C (IGF-1) 134 .0 ng/m, Free T32.4 pg/ml, TSH 0.9 uU/mL Free T4 0.99 ng/dl, and Prolactin 23.4ng/ml. Patient had a satisfactory clinical course after surgery, she maintained normal pituitary function without requirement of hormonal replacement therapy. Conclusion: We present a a prolactin, TSH and GH secreting pituitary adenoma case, which is very unusual. It i is important to make a proper pituitary evaluation, and offer effective treatment to prevent complications
When Your Pituitary Fails - A Rare Case of Pituitary Dysfunction Due to Nivolumab and Ipilimumab
Authors: Khan Areej , Khan Muhammad Ahmed , Phillips Brian 
Published: May 2021

Abstract
Introduction: Immunomodulators like Nivolumab and Ipilimumab are important regimens in many malignancies. However, their use is linked with the rare side effect of pituitary dysfunction that if screened and treated can prevent misdiagnosis.
Case Description: A 56-year-old male with a history of renal cell carcinoma status post bilateral partial nephrectomy with metastasis to the lung on Nivolumab/Ipilimumab presented with dizziness. He endorsed difficulty with balance, nausea, decreased appetite, feeling dehydrated and recent blood pressure readings in the 90s. Vitals on presentation were stable. An MRI, from 2 weeks ago, showed a slightly enlarged sella but no metastatic disease. The next day, he became hypotensive with minimal response to fluids and was started on Midodrine. However, considering his persistent hypotension, his cortisol level was checked and found to be profoundly low (&lt;1). Literature review of his immunomodulator revealed the possibility of central adrenal insufficiency. In addition, he was noted to have a low TSH (0.02) with normal FT4 (1.08). Subsequently, his ACTH level was also found to be low (&lt;1.5) which further elucidated a central cause for his adrenal insufficiency. Therefore, he was ultimately treated with PO hydrocortisone with plans to taper off in the next few weeks.
Conclusion: This case demonstrates a rare yet significant side effect of Nivolumab/Ipilimumab therapy. Timely diagnosis and therapy can alleviate symptoms due to associated hormonal deficiencies. Moving forward it will be interesting to see if starting prophylactic steroids or routine screening will allow us to diagnose pituitary dysfunction due to Nivolumab/Ipilimumab earlier.
Abnormal Pituitary Imaging and Associated Endocrine Dysfunctions in Erdheim-Chester Disease
Authors: Shekhar Skand , Irizarry-Caro Jorge A , Sinaii Ninet , Gahl William , Estrada-Veras Juvianee I , Dave Rahul , Papadakis Georgios Z , Patronas Nicholas , Stratakis Constantine A , O’Brien Kevin , Hannah-Shmouni Fady 
Published: May 2021

Abstract
Background: Erdheim Chester disease (ECD) is a rare histiocytic neoplasm associated with hypothalamic and pituitary infiltration and dysfunction. We determined the abnormal pituitary imaging (API) phenotypes in subjects with ECD and analyzed their associated endocrine dysfunctions. Methods: This was a cross-sectional examination of a natural history cohort study of 61 subjects with ECD performed at a tertiary care clinical research center. The diagnosis of ECD was based on clinical, molecular, and histopathological features. Enrolled subjects underwent baseline endocrine tests of anterior and posterior pituitary function in addition to pituitary imaging. The following variables were analyzed- age, sex, body mass index (BMI), BRAF V600E, hsCRP, ESR, pituitary hormone deficit number, diabetes insipidus (DI), and panhypopituitarism. Fisher’s exact test or t-test/Wilcoxon tests compared patients with and without API. Results: Sixty-one subjects with ECD (age ±SD: 54.3 ±10.9, 46 (75.4%) males) were studied. The prevalence of API was 32.8% (n=20), who were younger than those with normal imaging (50.3 ±10.5 vs 56.3 ±10.7 yrs, p=0.042). The most common pituitary imaging abnormalities included thickened pituitary stalk (18.03%, n=11/61), followed by pituitary encasement, small pituitary and abnormal morphology (6.55%, n= 4/61 for each). A higher prevalence of DI (45.0% vs 9.8%, p=0.003) and panhypopituitarism (45.0% vs 4.9%, p&lt;0.001), and a higher number of pituitary deficits (median (IQR): 2.0 (0-2.5) vs 0 (0-1.0), p=0.007) were noted in patients with API compared to those with normal imaging. Other biochemical markers were similar between both groups. Conclusion: Abnormal pituitary imaging was commonly seen in ECD and was also associated with a younger age and hormone deficits suggesting associations with pituitary structure-function.