bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pseudopheochromocytoma as a Presentation of Amyotrophic Lateral Sclerosis
Authors: Castellanos-Diaz Jessica , Edwards Catherine M , Ghayee Hans Kumar 
Published: May 2021

Abstract
Introduction: Pseudopheochromocytoma presents as paroxysmal events of hypertension and other noradrenergic symptoms similar to pheochromocytoma (PCC) but do not meet tumoral diagnosis. Case Report: A 70-year-old Caucasian male with history of gastroesophageal reflux disease (GERD) and Barrett’s esophagus presented to the Emergency Department with fatigue, shortness of breath, palpitations, and fasciculations in the anterior chest wall. He reported recent diagnosis of PCC. Patient had episodes of non-exertional palpitations, diaphoresis, dizziness, facial flushing, and facial involuntary movement along with self-monitored average blood pressure (ABP) of 160/100 mmHg and heart rate (HR) of 90 beats per minute (bpm). Spells occurred 2 to 3 times per month at first, later increased. Between episodes self-monitored ABP was 120/70 mmHg and HR of 50 bpm and asymptomatic. He reported unintentional weight loss of 40 pounds in the same interval and newly muscle weakness that he attributed to weight loss and malnutrition. Patient had no remarkable past medical history. Medications included coq10 enzyme, glucosamine, flax seed oil, primrose oil and recently added doxazosin 1 mg tab per day. Evaluation revealed a cachectic patient (body mass index 17.46 k/m2) with normal vital signs, otherwise unremarkable physical exam except for diffuse fasciculations in chest and upper extremities. Outpatient work up included normal complete blood count, comprehensive metabolic panel, drug screening, thyroid function test. He had elevated free plasma metanephrine (FPM) of 73 pg/mL (&lt; 57 pg/mL), free plasma normetanephrine (FPN) of 487 pg/mL (&lt;148 pg/mL), before doxazosin. Repeated FPM of 55 pg/mL (&lt; 57 pg/mL), FPN of 347 pg/mL (&lt;148 pg/mL). The 24-hour urine for metanephrine was normal with total metanephrine of 157 ug/d (55-320 ug/d) and normetanephrine 432 ug/d (114-865 ug/d) with a creatinine of 900 mg/dL (800-2100 mg/d). He also had outpatient computed tomography (CT) scan of thorax, abdomen, and pelvis that was normal. Positron emission tomography CT scan did not reveal any tumors. Our initial suspicion, later confirmed with electromyography, was a neurodegenerative disease with motor neuron dysfunction. Patient was diagnosed with Amyotrophic Lateral Sclerosis (ALS) and started in riluzole with improvement of spells. Since, his muscle weakness progressed, he now requires noninvasive ventilation. Discussion: Pseudopheochromocytoma is an idiopathic disease that mimics catecholamine-secreting PCC and needs adequate and complete evaluation. Catecholamine levels can be affected by illness, stress, medications, and sample collection. In the case presented, the patient’s muscular weakness and fasciculations was caused by ALS. Neurodegenerative diseases such as ALS can cause a central nervous system stress response, consequently causing elevated norepinephrine levels.
Recurrent Head and Neck Adenocarcinoma Presenting With a Pituitary Mass
Authors: Alpertugna Ipek , Safain Mina , Tracy Jeremiah , Tischler Arthur , Lechan Ronald M 
Published: May 2021

Abstract
Background: Metastasis to the pituitary gland is a rare condition. Only 1.8% of all surgically resected pituitary masses are metastases with the majority originating in the breast and lung (1). Salivary gland tumors rarely metastasize to the brain and only a few cases have been reported in the literature. Clinical Case: A 61 year-old woman presented to an outside emergency department with horizontal diplopia, blurry vision and left-sided sharp frontal headaches. MRI of the head showed a large, 2.0 x 4.6 x 1.8 cm sellar/suprasellar mass involving both cavernous sinuses, encasing the right internal carotid artery which was narrowed, and compressing the optic chiasm. Her past medical history was significant for adenocarcinoma of the left buccal mucosa and masseteric space, treated with resection in 2016, and a second resection in 2018 for recurrence followed by radiation therapy. Areas were identified where focal perineural invasion was present. She was referred to our institution for treatment recommendations for the sellar/suprasellar lesion. Laboratory testing revealed secondary hypothyroidism, adrenal insufficiency and partial diabetes insipidus, and she was started on replacement hormone therapy with levothyroxine and hydrocortisone. Neurosurgery and ENT evaluated patient and a surgical biopsy and subtotal resection via an endoscopic endonasal transsphenoidal approach was recommended for diagnosis and possible decompression of the optic chiasm. The final pathology was consistent with metastatic, high grade adenocarcinoma, supported by positive mucicarmine stain for mucin, positive immunohistochemical stain for CAM 5.2 and CK7 and negative immunohistochemical stains for the pituitary transcription factors, PIT-1 and SF-1. Conclusion: We report the second case in the literature of adenocarcinoma of the head and neck to the pituitary gland. In our case, radiological appearance of the tumor together with patient’s malignancy history led to the consideration of a pituitary metastasis. Tumors invading the cavernous sinus and causing internal carotid artery compression are rarely pituitary adenomas (2). Presence of these imaging features in patients with known risk factors for malignancy should raise suspicion for pituitary metastasis. The route of spread from the masseteric space to the sellar region remains unclear. Tumor metastasis can occur through direct invasion, hematogenous spread and perineural spread. Hematogenous spread and/or perineural spread along the trigeminal nerve are the most likely routes of metastasis in this case.
Reference: (1)Javanbakht A, D’Apuzzo M, Badie B, Salehian B. Pituitary metastasis: A rare condition. Endocr Connect 2018; 7(10):1049-1057. PMID: 30139817.
(2)Molitch ME et al (2012) Tumors invading the cavernous sinus that cause internal carotid artery compression are rarely pituitary adenomas. Pituitary 15(4):598–600
Early Follow-up of Atypical Thyroiditis Induced by SARS-CoV-2
Authors: Muller Ilaria , Cannavaro Daniele , Dazzi Davide , Mantovani Giovanna , Longari Virgilio , Cuzzocrea Marco , Re Tiziana E , Gori Andrea , Arosio Maura , Salvi Mario Giovanni 
Published: May 2021

Abstract
Background: In Spring 2020 the severe acute respiratory syndrome coronavirus 2 pandemic disease (Covid-19) badly affected Northern Italy. We have described for the first time the occurrence of thyrotoxicosis due to atypical subacute thyroiditis in 15% of patients hospitalised for Covid-19 pneumonia, compared with only 1% among patients hospitalised in the same wards during Spring 2019, thus before the Covid-19 pandemic. The whole group of Covid-19 patients also had median serum TSH concentrations significantly lower compared with the control group. The atypical thyroiditis induced by Covid-19 is not associated with neck pain, affects more men than women and especially those severely ill, thus coexists with non-thyroidal illness syndrome. Subacute thyroiditis is classically followed by subsequent occurrence of permanent thyroid dysfunction and autoimmunity, thus we have started a systematic follow-up program of these patients.
Methods: Longitudinal follow-up study of survived Covid-19 patients without previous known history of thyroid disorders and/or medications, assessing serum thyroid function and autoantibodies, C reactive protein (CRP), full blood count (FBC) and thyroid ultrasound (US) every 3 months. Patients showing baseline (at hospitalisation for Covid-19) thyroid dysfunction and/or focal hypoechoic areas suggestive for subacute thyroiditis at US performed 3 months post-infection, also underwent thyroid 99mTc or I123uptake.
Results: To date, 53 patients have been included in the follow-up study. At 3 months post-infection, all of them presented with increased median (IQR) serum TSH concentrations compared with baseline: 1.3 (0.9–2.0) mIU/L versus 0.9 (0.5–1.8) mIU/L (p=0.0001). Similarly, serum concentrations of free-thyroxine, free-triiodothyronine, CRP and FBC had normalised compared with baseline. All patients had negative autoantibodies to TSH receptor; autoantibodies to thyroglobulin and to thyroid peroxidase were positive in 6/53 (11%) and 5/53 (9%) of patients, respectively. The thyroid US showed the presence of focal hypoechoic areas of thyroiditis in 16/51 (32%) patients, with thyroid uptake normal in 6/16 (37%), focally reduced in 8/16 (50%) and diffusely reduced in 2/16 (12%).
Conclusions: At 3 months after Covid-19 disease all patients had a normalised thyroid function, however imaging findings suggestive for subacute thyroiditis were still present in about one third of cases. The thyroid dysfunction induced by Covid-19 seems not mediated by autoimmunity. It is important to continue to follow these patients since they might develop thyroid dysfunction during the following months.
Severe Symptomatic SIADH as a Sign of Unknow Hypopituitarism: A Case Report
Authors: Marques-Sá Juliana , Araújo Catarina Silva , Santos Maria Joana 
Published: May 2021

Abstract
Background: SIADH due to secondary adrenal insufficiency is a rare condition that can be life-threatening. Treatment with glucocorticoids is highly effective, but, as symptomatology of hypopituitarism can be mild and unspecific, a high level of clinical suspicion is necessary.
Clinical Case: A 64-year-old postmenopausal woman, with a previously diagnosed and treated hypothyroidism and depressive syndrome, presented at the emergency room with nausea, vomits, asthenia and recurrent syncopes over the past three weeks and weight loss in the last months. One week before, she had been admitted at other hospital due to the same complaints and a serum Na+ of 112mmol/L (N 135-145 mmol/L) was detected. One year earlier, she had reported the same symptomology and hyponatremia had also been found, but no etiologic study was performed. At admission, she was conscient, hydrated, pale, hemodynamically stable, with unremarkable cardiopulmonary auscultation. A severe hyponatremia was found (Na+ 111mmol/L), with low serum osmolarity (241mOsm/Kg, N 280-301mOsm/Kg), low urine osmolality (125mOsm/kg N 300-900 mOsm/Kg) and low urinary sodium (33mmol/24h, N 40-220mmol/24h). Treatment with saline fluids was initiated and the patient was admitted for etiological investigation, which showed a low morning serum cortisol (3.7 µg/dl)) with inappropriately low-normal ACTH level (12.2 pg/mL), low FSH (15.07 mUI/mL, N 23.0-116.3 mUI/mL) and LH (4.28 mUI/mL, N 15.9-54 mUI/mL), estradiol of 78.81 pmol/L (N &lt;118.2 pmol/L), undetectable IGF-1 (&lt;25 ng/mL, N 36-244 ng/mL) and PRL of 21.98 ng/mL (N 1.8-20.3 ng/mL). Under 50µg of levothyroxine, TSH was 2.5 µUI/mL (N 0.358-3.74 µUI/ml), and FT4 was 1.1 ng/dL (N 0.76-1.46 ng/dL). Anti-thyroglobulin and anti-TPO antibodies were negative. A pituitary MRI revealed an empty sella with arachnoidocele. Other differential diagnosis of SIADH were excluded. The patient was started on oral hydrocortisone 30 mg/day, with a remarkable recovery, with better mood, more energy and appetite, resolution of all symptoms and normalization of the natremia.
Conclusion: Hypopituitarism due to an empty sella is a relative rare disorder, whose etiology may be difficult to prove. It usually develops insidiously, with unspecific symptoms, which often delays the diagnosis. In our patient, this severe hyponatremia likely developed over a long time, allowing adaptation to such low levels of Na+. Moreover, the presence of a previously diagnosed hypothyroidism raises the suspicion if this wasn’t the first manifestation of the pituitary disease and if treatment of levothyroxine did not worsen an undetected secondary adrenal insufficiency.
Successful Pregnancies in an Acromegalic Woman After Non-Radical Pituitary Adenomectomy for Somatoprolactinoma
Authors: Gatzk Arruda Anna Catarina , Outuki Giovana , Dias Marcos Antonio , Carrilho Alexandre Jose Faria , Mazzuco Tania Longo 
Published: May 2021

Abstract
Background: Pregnancy is unusual in patients with acromegaly due to somatotropinomas or somatoprolactinomas. Fertility is impaired because of hormonal hypersecretion, pituitary damage by tumor compression or both. Managing somatoprolactinomas and fertility issues are often challenging.
Clinical Case: A 20-year woman with primary amenorrhea and headache was diagnosed with hypogonadotrophic hypogonadism secondary to hyperprolactinemia (2500 µg/L, n&lt;23 µg/L). No other abnormalities were found on the pituitary function screening tests. MRI revealed an intra and suprasellar adenoma (2.5x1.8x1.8 cm) with optic chiasm compression. The onset of menses occurred after 11 months under dopaminergic treatment, and tumor size diminished (1.9x1.5x1.5 cm), bringing on optic chiasm decompression. She remained under dopamine agonist treatment for 6 years, when she realized extremities enlargement and height increase by 3 cm. Acromegaly was confirmed by blood levels of IGF-1 (3.37xULN), GH (8 µg/L, n&lt;8 µg/L), and GH nadir (4.3 µg/L, n&lt;1 µg/L) during OGTT. Then, octreotide LAR was added to cabergoline treatment while waiting for elective surgical treatment. She underwent to transsphenoidal endonasal neurosurgical microscopy approach guided by neuronavigation, with the removal of a large portion of tumor. However, it was not possible to extract the part of invasive adenoma close to right carotid artery due to the risk of vascular and intracavernous cranial nerves injury. Immunohistochemistry analysis of the adenoma was positive only for GH cells with low Ki67 index (&lt;1%). Due to the poor biochemical control (unsuppressed post-OGTT GH, IGF-1 1.66xULN and PRL 301 µg/L) and the presence of a small stable tumor residue, treatment with cabergoline and somatostatin analogues was maintained (3-year octreotide LAR, transitioned to lanreotide in an attempt to achieve a better biochemical response). After 14 years of the initial diagnosis and 5 years post-surgery, the patient expressed the desire to get pregnant and all medications in use were suspended. In the following 3 years, she had two uneventful gestation without complications or worsen of acromegaly; she only breastfed for few months after her first pregnancy. The second one was a twin pregnancy. After one year, the MRI revealed no increase of tumor mass (1.0x0.3x1.0 cm), and PRL levels withing normal range, IGF-1 slightly elevated, but GH not suppressed by OGTT. Cabergoline was reintroduced and the biochemical control of acromegaly was achieved.
Conclusion: We reported the very unusual spontaneous conception and normal course of pregnancies in a woman with acromegaly, who was submitted to successful transsphenoidal neurosurgical microscopy approach in which large part of the tumor was removed and the normal pituitary tissue was preserved, allowing fertility restoration.
Surgical Remission of Acromegaly Resolves Neuroglycopenia and Paradoxical Rise in GH after OGTT
Authors: Magar Maria , Carmichael John 
Published: May 2021

Abstract
Background: Acromegaly is known to cause insulin resistance through increased gluconeogenesis and reduction in peripheral glucose use; however, hypoglycemia related to acromegaly has not been reported.
Clinical Case: A 58-year old man presented for evaluation of several elevated serum IGF1 levels. The patient had reported years of increased body heat but no changes in his hands or feet and no voice deepening. He recently needed 15 dental crowns due to gaps in his teeth. He also had difficult to manage OSA and weight gain. The patient reported neuroglycopenia after a high glycemic meal or drink, although he was never able to objectively measure any low blood glucoses when they occurred; these symptoms improved but did not resolve despite adhering to a low carbohydrate diet. He also had decreased libido and erectile dysfunction. Exam was significant for coarse facial features. Prior testing revealed several elevated IGF1 serum levels, the last one being 227 ng/mL (54-194). One year prior, OGTT resulted in an initial GH level of 0.1 ng/mL with a decrease to &lt;0.1 ng/mL after two hours. Repeat OGTT had an initial GH of 2.98 ng/mL which paradoxically rose to 12 ng/mL. Fasting BG was 90 mg/dL and peaked at 171 mg/dL. Pituitary MRI showed a 5 mm microadenoma, consistent with acromegaly from a GH secreting adenoma. He underwent a TSSC, and his heat intolerance, low libido, and symptom of hypoglycemia resolved completely. Subsequent IGF1 levels and MRI imaging normalized. Postoperatively OGTT showed a peak GH of 0.23 ng/mL with a peak glucose of 134 mg/dL. There was no paradoxical rise in GH.
Discussion: Acromegaly is commonly associated with insulin resistance in ~30% of cases; however, there are no reports of associated neuroglycopenia after a carbohydrate-rich meal or OGTT, which in our patient resolved after successful removal of the pituitary microadenoma. His low glucose symptoms could have been a result of reactive hypoglycemia, which is often seen in patients with diabetes or even prediabetes. However this patient had no history of either. He did not have evidence of any tumors causing hypoglycemia and no gastric surgery to suggest a related etiology (e.g, dumping syndrome or nesidioblastosis). Conversely since GH is normally anabolic and stimulates insulin release, the patient’s elevated GH may have caused an abnormal increase in insulin, leading to his hypoglycemia symptoms. Indeed GIP, which stimulates insulin, is thought to be the cause of the paradoxical rise in GH seen in 30% of acromegaly cases. Remarkably, the patient’s hypoglycemia symptoms disappeared after treatment of the acromegaly, which leads us to consider that excess GH was the culprit.
Characteristics of Pituitary Tumors Including Non-Functioning Tumors - a Tertiary Care Center Experience
Authors: Kumbar Lohit , Dharmalingam Mala 
Published: May 2021

Abstract
Introduction: Pituitary tumors compromise 10-20% of intracranial tumors. Non-functioning pituitary adenomas (NFPAs) are benign neoplasms which constitutes about 1/3rd of the pituitary tumors. There is few data on the characteristics of non-functioning tumors comparing with functioning tumors. Wide spread availability of MRI has eased the diagnosis of non-functioning tumors and early diagnosis. Objectives: To study the characteristics of pituitary tumors and the incidence of non-functioning tumors and compare with functioning tumors presenting to a tertiary care center. MethodsA retrospective study including 146 individuals with pituitary tumor were included from 2014 to 2020. Results: The study included 62(42.5%) male and 84(57.5%) female. The mean age was 41.97 + 13.93 years in males and 39.88 + 16.12 years in females. The mean duration of symptoms prior to presentation was 7.52 + 7.12 months and 10.37 + 11.60 months in males and females respectively. Macroadenoma (size &gt;1cm) incidence was more with 55(88.7%) in males and 58(69%) in females. More number of males (87.1%) underwent surgery when compared to females (64.3%)(p – 0.002). There was more number of suprasellar and invasion of pituitary tumors seen in males compared to females(P- &lt;0.01). There was no significant difference between various parameters like TSH, T4, LH, FSH, prolactin, Electrolytes, cortisol, ACTH, GH and IGF1 between males and females(p- NS). Seventy percent (103) of them were non-functioning tumors. Functioning tumors included 7(4.8%) acromegaly, 8(5.5%) cushings disease, 2(1.4%) gonadotropinoma, 24(16.4%) prolactinoma and 2(1.4%) posterior fossa tumor. Mean age in non- functioning tumors was 43.74 + 15.5 years. Mean diameter of tumor in non-functioning tumors was 21.04 + 11.54 cm when compared to functioning tumors (17.03 + 10.33 cm)(p- 0.61). Most of the patients with non-functioning tumors who underwent surgery received post operatively hydrocortisone and levothyroxine when compared to patients with functioning tumors(p &lt; 0.01). There was no significant difference in terms of gender, suprasellar extension, invasion and pituitary hormones(p-NS). ConclusionMost of the pituitary tumors in our study were macroadenomas with more number of males undergoing surgery as most of them had suprasellar extension and invasion. Non- functioning tumors should be followed up regularly as most of them required hydrocortisone and levothyroxine post-operatively.
Immunohistochemical Profile of Nonfunctioning Pituitary Adenomas
Authors: Solovey Mariana , Guk Mykola , Danevych Olena 
Published: May 2021

Abstract
Background: Nonfunctioning pituitary adenomas (NFPAs) are neuro-endocrine tumors without clinical and laboratory signs of anterior pituitary hormonal hypersecretion. The recent World Health Organization classification is based on the adenohypophyseal cell lineages and requires immunohistochemical evaluation of adenohypophyseal hormones and pituitary transcription factors. There are few data regarding the age and sex prevalence of different cell-types nonfunctioning adenomas and clinical data correlations. Objective: To discover the immunohistochemical profile of large cohort of NFPAs. Materials and Methods: The study includes 100 consecutive cases of endoscopically transsphenoidally removed nonfunctional pituitary adenomas, immunohistochemically assessed for anterior pituitary hormones and transcription factors. Clinical presentation, imaging, laboratory hormonal data and immunohistochemical staining features have been analyzed. All patients (64 women and 36 men) have been divided into four age groups: 20-34 (A) years old, 35-44 (B) years old, 45-59 (C) years old, 60-70 (D) years old. Peculiarities of immunohistochemical profile have been statistically analyzed in those age groups. Results: Most tumors (97%) were macroadenomas with mass effect symptoms. In the groups of silent corticotroph and Pit-1 adenomas most of the patients had subclinical symptoms of hormonal hypersecretion. The proportions of silent gonadotroph adenomas have appeared to be increased with age with predominant prevalence in group D (60%) in women and group C (78, 6%) in men. The proportions of silent Pit-1 adenomas decreased with age with maximum rate in group A (77,8%) in women and in group A (50%) in men. The incidence of silent corticotroph adenomas was different: increasing with age in women with maximum (36,8%) in group C and decreasing from young age (30%-0%) in men age groups B-D respectively. Plurihormonal pituitary adenomas from different cell lineages were found only in women, with maximum incidence rate (17,6%) in group B. The incidence of “null cell” adenomas didn’t differ in men and women in group B and C but was much more higher in men in groups A and D (16,7% vs 0% and 33% vs 6,6% respectively). Conclusions: The different age and sex prevalence of NFPAs, revealed in our study, may be helpful in diagnosing and optimal treatment of NFPAs.