Clinical Overview > Hormone > TSH

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Milena S Pandrc Department of Internal Medicine, Militar Medical Academy, Crnotravska 1711000, Belgrade, Serbia

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Stanko Petrović Department of Gastroenterology, Militar Medical Academy, Crnotravska 1711000, Belgrade, Serbia

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Vanja Kostovski Clinic for Thoracic Surgery, Militar Medical Academy, Crnotravska 1711000, Belgrade, Serbia

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Marijana Petrović Department of Nephrology, Militar Medical Academy, Crnotravska 1711000, Belgrade, Serbia

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Miloš Zarić Department of Pathology, Militar Medical Academy, Crnotravska 1711000, Belgrade, Serbia

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Summary

Immunoglobulin (Ig)G4-related sclerosing disease (IgG4-RSD) is a new disease entity first proposed with regard to autoimmune pancreatitis. A 67-year-old male patient was examined because of weight loss and an abdominal pain. Based on the clinical characteristics, laboratory parameters and ultrasound features, we identified the diagnosis of the IgG4-related systemic disease (IgG4-RSD), that was confirmed by the histopathological analysis after the biopsy of the head of pancreas. After confirmation, we started with the corticosteroid therapy with a good clinical, biochemical and morphological response. During the previous therapy, the disturbance of glucoregulation appeared, so we had to change the modality of treatment. We decided to add Azathioprine to the therapy in a dose of 150 mg/day. We achieved a stable phase of the disease with IgG 4.37 g/l and IgG4 0.179 g/l, and with no side effects from the therapy.

Learning points

  • There are potential clinical applications of identifying subsets of patients with IgG4 thyroiditis (FVHT and Riedel thyroiditis).

  • A trial of immunosuppressive therapy should be included if a resection is deemed inadvisable.

  • In particular, cases of FVHT that mimic malignancy, tissue and serum IgG4 may provide supportive diagnostic information.

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C P Neves Section of Endocrinology, Department of Internal Medicine, Erasmus MC, 's Gravendijkwal 230NL-3015, CE Rotterdam, The Netherlands

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E T Massolt Section of Endocrinology, Department of Internal Medicine, Erasmus MC, 's Gravendijkwal 230NL-3015, CE Rotterdam, The Netherlands

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R P Peeters Section of Endocrinology, Department of Internal Medicine, Erasmus MC, 's Gravendijkwal 230NL-3015, CE Rotterdam, The Netherlands

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S J Neggers Section of Endocrinology, Department of Internal Medicine, Erasmus MC, 's Gravendijkwal 230NL-3015, CE Rotterdam, The Netherlands

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W W de Herder
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Summary

A 21-year-old woman presented with amenorrhea, bilateral galactorrhea and fatigue. Visual acuity and visual fields were normal. Laboratory examination demonstrated hyperprolactinemia. Magnetic resonance imaging (MRI) of the pituitary showed a 19×17×12-mm sellar mass with supra- and parasellar extension, causing compression of the pituitary stalk and optic chiasm. Further examinations confirmed mild hyperprolactinemia, strongly elevated TSH (>500 mU/l), low free thyroxine (FT4), hypogonadotropic hypogonadism and secondary adrenal insufficiency. Hydrocortisone and l-T4 replacement therapy was started. Three months later, the galactorrhea had disappeared, thyroid function was normalized and MRI revealed regression of the pituitary enlargement, confirming the diagnosis of pituitary hyperplasia (PH) due to primary hypothyroidism. Subsequently, the menstrual cycle returned and the hypocortisolism normalized. This case demonstrates that severe primary hypothyroidism may have an unusual presentation and should be considered in the differential diagnosis of pituitary enlargement associated with moderate hyperprolactinemia.

Learning points

  • One should always try to find one etiology as the common cause of all the clinical findings in a pathologic process.

  • Amenorrhea, galactorrhea and fatigue may be the only presenting clinical manifestations of primary hypothyroidism.

  • Not every patient with galactorrhea, hyperprolactinemia and a pituitary mass has a prolactinoma.

  • Primary hypothyroidism should always be considered in the differential diagnosis of hyperprolactinemia associated with pituitary enlargement and pituitary hormone(s) deficiency(ies).

  • When PH due to primary hypothyroidism is suspected, thyroid hormone replacement should be started and only regression of pituitary enlargement on MRI follow-up can confirm the diagnosis.

  • Examination of thyroid function in patients with a pituitary mass may avoid unnecessary surgery.

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W C Candy Sze Department of Endocrinology, St Bartholomew's Hospital, London, EC1A 7BE, UK

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Joe McQuillan Department of Ophthalmology, St Bartholomew's Hospital, London, EC1A 7BE, UK

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P Nicholas Plowman Department of Clinical Oncology, St Bartholomew's Hospital, London, EC1A 7BE, UK

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Niall MacDougall Department of Clinical Oncology, St Bartholomew's Hospital, London, EC1A 7BE, UK

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Philip Blackburn Gamma Knife Unit, St Bartholomew's Hospital, London, EC1A 7BE, UK

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H Ian Sabin Gamma Knife Unit, St Bartholomew's Hospital, London, EC1A 7BE, UK

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Nadeem Ali Department of Ophthalmology, St Bartholomew's Hospital, London, EC1A 7BE, UK

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William M Drake Department of Endocrinology, St Bartholomew's Hospital, London, EC1A 7BE, UK

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Summary

We report three patients who developed symptoms and signs of ocular neuromyotonia (ONM) 3–6 months after receiving gamma knife radiosurgery (GKS) for functioning pituitary tumours. All three patients were complex, requiring multi-modality therapy and all had received prior external irradiation to the sellar region. Although direct causality cannot be attributed, the timing of the development of the symptoms would suggest that the GKS played a contributory role in the development of this rare problem, which we suggest clinicians should be aware of as a potential complication.

Learning points

  • GKS can cause ONM, presenting as intermittent diplopia.

  • ONM can occur quite rapidly after treatment with GKS.

  • Treatment with carbamazepine is effective and improve patient's quality of life.

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Anastasia Dimakopoulou
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Karunakaran Vithian Colchester Hospital University NHS Foundation Trust, Colchester, UK

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David Gannon Colchester Hospital University NHS Foundation Trust, Colchester, UK

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Allan Harkness Colchester Hospital University NHS Foundation Trust, Colchester, UK

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Summary

A 55-year-old female patient presented to the endocrine clinic with Grave's disease. She was initially treated with carbimazole. After an early relapse, a decision was made to proceed with radioactive iodine therapy. Four days after radioiodine administration, she presented to the emergency department with chest tightness and dyspnea due to heart failure. Biochemistry revealed thyrotoxicosis and significantly elevated Troponin-T. There was ST segment elevation on electrocardiography. However, coronary angiography was normal. Ventricular function was fully restored after 6 weeks of supportive medical management. A diagnosis of stress cardiomyopathy following radioactive iodine therapy was made. This is the second case reported in the literature so far to the best of our knowledge.

Learning points

  • Stress cardiomyopathy in the context of radiation thyroiditis is a rare complication following radioiodine therapy.

  • A degree of awareness is essential because the approach is multidisciplinary. Management is mainly supportive and cardiac dysfunction is completely reversible in most cases.

  • The pathogenesis of this condition remains unclear. Post-menopausal women and susceptible individuals appear to be pre-disposed.

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Sunita M C De Sousa Department of Endocrinology, St Vincent's Hospital, Sydney, New South Wales, Australia
Hormones and Cancer Group, Garvan Institute of Medical Research, 384 Victoria Street, Sydney, New South Wales, 2010, Australia

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Peter Earls Department of Anatomical Pathology, St Vincent's Hospital, Sydney, New South Wales, Australia

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Ann I McCormack Department of Endocrinology, St Vincent's Hospital, Sydney, New South Wales, Australia
Hormones and Cancer Group, Garvan Institute of Medical Research, 384 Victoria Street, Sydney, New South Wales, 2010, Australia

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Summary

Pituitary hyperplasia (PH) occurs in heterogeneous settings and remains under-recognised. Increased awareness of this condition and its natural history should circumvent unnecessary trans-sphenoidal surgery. We performed an observational case series of patients referred to a single endocrinologist over a 3-year period. Four young women were identified with PH manifesting as diffuse, symmetrical pituitary enlargement near or touching the optic apparatus on MRI. The first woman presented with primary hypothyroidism and likely had thyrotroph hyperplasia given prompt resolution with thyroxine. The second and third women were diagnosed with pathological gonadotroph hyperplasia due to primary gonadal insufficiency, with histopathological confirmation including gonadal-deficiency cells in the third case where surgery could have been avoided. The fourth woman likely had idiopathic PH, though she had concomitant polycystic ovary syndrome which is a debated cause of PH. Patients suspected of PH should undergo comprehensive hormonal, radiological and sometimes ophthalmological evaluation. This is best conducted by a specialised multidisciplinary team with preference for treatment of underlying conditions and close monitoring over surgical intervention.

Learning points

  • Normal pituitary dimensions are influenced by age and gender with the greatest pituitary heights seen in young adults and perimenopausal women.

  • Pituitary enlargement may be seen in the settings of pregnancy, end-organ insufficiency with loss of negative feedback, and excess trophic hormone from the hypothalamus or neuroendocrine tumours.

  • PH may be caused or exacerbated by medications including oestrogen, GNRH analogues and antipsychotics.

  • Management involves identification of cases of idiopathic PH suitable for simple surveillance and reversal of pathological or iatrogenic causes where they exist.

  • Surgery should be avoided in PH as it rarely progresses.

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I R Wallace Regional Centre for Endocrinology and Diabetes, Royal Victoria Hospital, Grosvenor Road, Belfast, BT12 6BA, UK

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E Healy Department of Neuropathology, Royal Victoria Hospital, Grosvenor Road, Belfast, BT12 6BA, UK

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R S Cooke Department of Neurosurgery, Royal Victoria Hospital, Grosvenor Road, Belfast, BT12 6BA, UK

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P K Ellis Department of Radiology, Royal Victoria Hospital, Grosvenor Road, Belfast, BT12 6BA, UK

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R Harper Department of Endocrinology and Diabetes, Ulster Hospital, Dundonald, BT16 1RH, UK

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S J Hunter Regional Centre for Endocrinology and Diabetes, Royal Victoria Hospital, Grosvenor Road, Belfast, BT12 6BA, UK

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Summary

TSH-secreting pituitary adenomas are rare and the optimal investigation and management is uncertain. We describe a case of a 43 year-old woman with a TSH-secreting pituitary adenoma, highlighting diagnostic testing and our use, pre-operatively of somatostatin analogue therapy, which induced biochemical euthyroidism and a reduction in tumour size.

Learning points

  • The differential diagnosis of the syndrome of inappropriate TSH secretion is non-thyroidal illness, medications, assay interference due to heterophilic antibodies, thyroid hormone resistance and TSH-secreting pituitary adenoma.

  • TRH stimulation test and triiodothyronine suppression test assist in differentiating thyroid hormone resistance and TSH-secreting pituitary adenoma.

  • Somatostatin analogue therapy can induce biochemical euthyroidism and reduce tumour size.

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Mohammed Al-Sofiani
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Dhimitri Nikolla Lake Erie College of Osteopathic Medicine, Erie, Pennsylvania, USA

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V V S Ramesh Metta
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Summary

We report the case of a 42-year-old female with a history of hypothyroidism and asthma presenting with progressive dyspnea and orthopnea after 2 days of an upper respiratory tract infection (URTI). Based on the clinical and radiological findings, the patient was admitted as a case of cardiogenic pulmonary edema secondary to possible viral myocarditis. However, a normal brain natriuretic peptide (BNP) level with a normal ejection fraction (EF) on echocardiogram changed our working diagnosis from cardiogenic to non-cardiogenic pulmonary edema. Further questioning revealed a history of nocturnal snoring, frequent awakening, and daytime fatigue, suggesting a possible sleep apnea syndrome (SAS). In conclusion, we believe that SAS was the missing link between our patient's hypothyroidism and non-cardiogenic pulmonary edema.

Learning points

  • Always keep an open mind and look for a pathology that would explain the whole clinical scenario.

  • The involvement of the respiratory system in hypothyroidism can range from SAS, pulmonary hypertension, hypoventilation, and severe respiratory failure.

  • Hypothyroidism and SAS should be considered in the differential diagnosis of non-cardiogenic pulmonary edema.

  • Patients should be instructed to take levothyroxine on an empty stomach 30–60 min before food to avoid erratic absorption of the hormone.

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Wann Jia Loh Osteoporosis and Bone Metabolism Unit, Department of Endocrinology, Singapore General Hospital, Academia, 20, College Road, Singapore, 169856, Singapore

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Kesavan Sittampalam Department of Pathology, Singapore General Hospital, Academia, 20, College Road, Singapore, 169856, Singapore

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Suan Cheng Tan Department of Radiology, Singapore General Hospital, Academia, 20, College Road, Singapore, 169856, Singapore

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Manju Chandran Osteoporosis and Bone Metabolism Unit, Department of Endocrinology, Singapore General Hospital, Academia, 20, College Road, Singapore, 169856, Singapore

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Summary

Erdheim–Chester disease (ECD) is a potentially fatal condition characterized by infiltration of multiple organs by non-Langerhans histiocytes. Although endocrine dysfunction has been reported in association with ECD, to date, there have been no previous reports of empty sella syndrome (ESS) associated with it. We report the case of a patient with ECD who had symptomatic ESS. A 55-year-old man of Chinese ethnicity initially presented with symptoms of heart failure, fatigue and knee joint pain. Physical examination revealed xanthelasma, gynaecomastia, lung crepitations, hepatomegaly and diminished testicular volumes. He had laboratory evidence of hypogonadotrophic hypogonadism, secondary hypoadrenalism and GH deficiency. Imaging studies showed diffuse osteosclerosis of the long bones on X-ray, a mass in the right atrium and thickening of the pleura and of the thoracic aorta on fusion positron emission tomography–computed tomography. Magnetic resonance imaging (MRI) of the brain showed an empty sella. The diagnosis of ECD was confirmed by bone biopsy.

Learning points

  • ECD is a multisystemic disease that can affect the pituitary and other organs. The diagnosis of ECD is based on clinical and radiological features and histology, showing lipid-laden CD68+ CD1a S100 histiocytes surrounded by fibrosis.

  • The finding of xanthelasmas especially in the presence of normal lipid levels in the presence of a multisystem infiltrative disorder should raise the suspicion of ECD.

  • Systemic perturbation of autoimmunity may play a role in the pathogenesis of ECD and is an area that merits further research.

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Stephanie Teasdale Department of Diabetes and Endocrinology, Princess Alexandra Hospital, Brisbane, Queensland, Australia

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Fahid Hashem Department of Diabetes and Endocrinology, Princess Alexandra Hospital, Brisbane, Queensland, Australia

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Sarah Olson Department of Neurosurgery, Princess Alexandra Hospital, Brisbane, Queensland, Australia

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Benjamin Ong Department of Radiology, Princess Alexandra Hospital, Brisbane, Queensland, Australia

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Warrick J Inder Department of Diabetes and Endocrinology, Princess Alexandra Hospital, Brisbane, Queensland, Australia
School of Medicine, The University of Queensland, Brisbane, Queensland, Australia

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Summary

A case of recurrent pituitary apoplexy is described in a 72-year-old man who initially presented with haemorrhage in a non-functioning pituitary adenoma. Five years later, he re-presented with a severe pituitary haemorrhage in an enlarging sellar mass invading both cavernous sinuses causing epistaxis and bilateral ocular paresis. Subsequent histology was consistent with a sellar malignant spindle and round cell neoplasm. Multiple pituitary tumours have previously been reported to coexist in the same individual, but to our knowledge this is the only case where two pathologically distinct pituitary neoplasms have sequentially arisen in a single patient. This case is also notable with respect to the progressive ocular paresis, including bilateral abducens nerve palsies, and the presentation with epistaxis.

Learning points

  • Ocular paresis in pituitary apoplexy can result from tumour infiltration of nerves, or by indirect compression via increased intrasellar pressure.

  • Epistaxis is a very rare presentation of a pituitary lesion.

  • Epistaxis more commonly occurs following trans-sphenoidal surgery, and can be delayed.

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Philip C Johnston Department of Endocrinology, Diabetes and Metabolism, Cleveland Clinic Foundation, 9500 Euclid Avenue Desk F20, Cleveland, Ohio 44195, USA

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Amir H Hamrahian Department of Endocrinology, Diabetes and Metabolism, Cleveland Clinic Foundation, 9500 Euclid Avenue Desk F20, Cleveland, Ohio 44195, USA

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Richard A Prayson Patholgy and Laboratory Medicine Institute, Rose Ella Burkhardt Brain Tumor and Neuro-Oncology Center, Cleveland Clinic, Cleveland, Ohio 44195, USA

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Laurence Kennedy Department of Endocrinology, Diabetes and Metabolism, Cleveland Clinic Foundation, 9500 Euclid Avenue Desk F20, Cleveland, Ohio 44195, USA

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Robert J Weil Department of Neurosurgery and the Neurological Institute, Rose Ella Burkhardt Brain Tumor and Neuro-Oncology Center, Cleveland Clinic, Cleveland, Ohio 44195, USA

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Summary

A 54-year-old woman presented with bi-temporal hemianopia, palpitations, and diaphoresis. An invasive pituitary macroadenoma was discovered. The patient had biochemical evidence of secondary hyperthyroidism and GH excess; however, she did not appear to be acromegalic. Surgical removal of the pituitary mass revealed a plurihormonal TSH/GH co-secreting pituitary adenoma. TSH-secreting adenomas can co-secrete other hormones including GH, prolactin, and gonadotropins; conversely, co-secretion of TSH from a pituitary adenoma in acromegaly is infrequent.

Learning points

  • This case highlights an unusual patient with a rare TSH/GH co-secreting pituitary adenoma with absence of the clinical features of acromegaly.

  • Plurihormonality does not always translate into the clinical features of hormonal excess.

  • There appears to be a clinical and immunohistochemical spectrum present in plurihormonal tumors.

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