Clinical Overview > Topic > Pituitary
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Search for other papers by Yuji Kadowaki in
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Health Care Center, Kochi University, Kochi City, Kochi, Japan
Search for other papers by Mitsuru Nishiyama in
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Summary
Langerhans cell histiocytosis (LCH) is a rare disease characterized by the proliferation of abnormal Langerhans cells in various tissues and organs, including bone, skin, the lungs, and the pituitary gland. Hypothalamic–pituitary lesions in LCH often cause central diabetes insipidus (CDI), but the natural course of LCH in the CNS remains to be elucidated. In this study, we report an interesting case of altered LCH lesions in the CNS from the pituitary to the hypothalamus in a 45-year-old woman. She developed symptoms of polyuria and was diagnosed with CDI with lymphocytic hypophysitis due to an enlarged pituitary gland with stalk thickening shown on MRI. Short-term glucocorticoid therapy cured pituitary enlargement, but serum prolactin levels gradually increased. Six years later, the immunohistological findings of a skin biopsy revealed positive for leukocyte common antigen, S-100, and CD1a expression, indicating a diagnosis of LCH. MRI revealed a new lesion in the hypothalamus without pituitary involvement, likely due to LCH. Chemotherapy improved LCH lesions both in the skin and hypothalamus, but therapy was stopped on the patient’s request. Although adult-onset LCH is rare, it should be considered as a differential diagnosis in cases of CDI as the primary disease. The clinical course in the present case indicated that LCH lesion was altered from pituitary to suprasellar extension; where such changes were observed, the possibility of LCH should be considered.
Learning points
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Diagnosing the primary disease of CDI is challenging; therefore, careful observation is necessary in pathologically unknown cases.
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Enhanced MRI should be performed in cases with suspected hypothalamic lesions, such as elevated serum prolactin.
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Although adult-onset LCH is rare, it should be considered a differential diagnosis in cases of CDI as the primary disease.
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The direction of changing CNS lesion from pituitary to suprasellar extension might be a unique MRI finding in LCH.
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Summary
Pituitary apoplexy (PA) is a medical emergency with complex diagnosis and management. In this study, we describe a case of PA in a 63-year-old male treated with oral anticoagulant therapy for atrial fibrillation. In the patient, PA manifested itself with asthenia and severe headache not responsive to common analgesics. Despite the finding of a pituitary mass through CT, and in anticipation of the endocrinological evaluation and pituitary MRI, the patient’s clinical condition worsened with an escalation of headache and asthenia associated with deterioration of the visual field and impairment of consciousness level. The emergency assessments revealed an adrenal failure, whereas MRI showed a haemorrhagic pituitary macroadenoma with compression of the optic chiasm. Intravenous fluids repletion and high-dose hydrocortisone were started with a rapid improvement of the patient’s health and visual field abnormalities. Hydrocortisone was gradually reduced to a replacement dose. During the follow-up, panhypopituitarism was documented, and replacement therapies with l-thyroxine and testosterone were introduced. Three months later, a pituitary MRI showed a 50% reduction in the pituitary adenoma volume.
Learning points
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Pituitary apoplexy (PA) is a medical emergency that can result in haemodynamic instability and abnormalities in the level of consciousness.
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The management of PA requires a multidisciplinary team that includes endocrinologists, ophthalmologists, neuro-radiologists, and neuro-surgeons.
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Pituitary MRI with gadolinium is the diagnostic gold standard for PA.
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PA therapy aims to improve general conditions and treat compression symptoms, especially visual field abnormalities.
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Adrenocorticotrophic hormone deficiency is a common and severe complication of PA. Thus, all patients with PA must be promptly treated with injective synthetic glucocorticoids (e.g. hydrocortisone 100 mg) and i.v. saline.
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PA must be taken into consideration in case of sudden headache in patients with a pituitary macroadenoma, especially if other risk factors are recognized.
Search for other papers by Hidekuni Takahashi in
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Search for other papers by Shigeo Nishimata in
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Search for other papers by Atsushi Kumada in
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Search for other papers by Yasuyo Kashiwagi in
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Search for other papers by Hisashi Kawashima in
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Summary
We encountered a case of childhood-onset lymphocytic infundibuloneurohypophysitis, based on the MRI and endocrinological findings, with decreased function of the anterior and posterior lobes of the pituitary. Three years after the diagnosis, the patient developed non-alcoholic steatohepatitis (NASH), which was effectively treated by growth hormone (GH) supplementation. The present case demonstrated that NASH can be effectively treated by short-term GH supplementation, even in late childhood.
Learning points
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In recent years, the efficacy of growth hormone replacement therapy in normalizing the liver function of adult-onset growth hormone deficiency patients with non-alcoholic steatohepatitis (NASH) has been reported.
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Lymphocytic infundibuloneurohypophysitis is a very rare disease, particularly in childhood.
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We here presented a rare case of a child with lymphocytic infundibuloneurohypophysitis who developed NASH and showed substantial improvement in liver function after growth hormone treatment.
Search for other papers by Eugénie Van Mieghem in
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Search for other papers by Valent Intan-Goey in
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Department of Pathological Anatomy, ZNA Hospitals, Antwerpen, Belgium
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Search for other papers by Pieter Van Loo in
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Search for other papers by Pascale Abrams in
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Summary
Pituitary carcinoma is a rare type of malignancy and only accounts for 0.1–0.2% of all pituitary tumours. Most pituitary carcinomas are hormonally active and they are mostly represented by corticotroph and lactotroph carcinomas. Corticotroph carcinoma can present as symptomatic Cushing’s disease or can evolve from silent corticotroph adenoma which is not associated with clinical or biochemical evidence of hypercortisolism. We hereby present a case of a bone-metastasized corticotroph pituitary carcinoma masquerading as an ectopic adrenocorticotropic hormone (ACTH) syndrome in a patient with a history of a non-functioning pituitary macro-adenoma. Our patient underwent two transsphenoidal resections of the primary pituitary tumour followed by external beam radiation therapy. Under hydrocortisone substitution therapy she developed ACTH-dependent hypercortisolism without arguments for recurrence on pituitary MRI and without central-to-peripheral ACTH-gradient on inferior petrosal sinus sampling, both suggesting ectopic production. Ultimately, she was diagnosed with an ACTH-secreting vertebral metastasis originating from the primary pituitary tumour. This case report demonstrates the complex pathophysiology of pituitary carcinoma and the long diagnostic work-up. Certain features in pituitary adenoma should raise the suspicion of malignancy.
Learning points
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The diagnosis of pituitary carcinoma can only be made based on documented metastasis, therefore, due to the often long latency period between the detection of the primary tumour and the occurrence of metastasis, the diagnostic work-up most often spans over multiple years.
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Pituitary carcinoma including corticotroph carcinoma is very rare in contrast to pituitary adenoma and only accounts for 0.1–0.2% of all pituitary tumours.
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Histopathology in pituitary adenoma should certainly accomplish the following goals: accurate tumour subtyping and assessment of tumoural proliferative potential.
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Repeated recurrence of pituitary adenoma after surgical resection, a discrepancy between biochemical and radiological findings, resistance to medical and radiation therapy, and silent tumours becoming functional are all hallmarks of pituitary carcinoma.
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Silent corticotroph adenomas are non-functioning pituitary adenomas that arise from T-PIT lineage adenohypophyseal cells and that can express adrenocorticotropic hormone on immunohistochemistry, but are not associated with biochemical or clinical evidence of hypercortisolism. Silent corticotroph adenomas exhibit a more aggressive clinical behaviour than other non-functioning adenomas.
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Treatment options for corticotroph carcinoma include primary tumour resection, radiation therapy, medical therapy, and chemotherapy. Sometimes bilateral adrenalectomy is necessary to achieve sufficient control of the cortisol excess.
Search for other papers by Wouter W. de Herder in
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Summary
The iconic photograph ‘A Jewish giant at home with his parents in the Bronx, N.Y. 1970’ by the famous American photographer Diane Arbus (1923–1971) shows the 2.34 m (7 ft. 8¼ in.) acromegalic giant Eddie Carmel (1936–1972) and his parents in the living room of their New York home. The picture is a typical example of Arbus’ style. The relationship between the artist and the tall subject is described. A growth hormone-secreting pituitary macroadenoma was unsuccessfully treated with two cycles of pituitary radiotherapy achieving a 7000 rad cumulative dose and by incomplete pituitary surgery. Hypopituitarism was treated according to medical standards in the 1960s and 1970s. The giant patient died of increased intracranial pressure and at autopsy a residual acidophil pituitary macroadenoma was found, but also a perisellar meningioma which was most probably induced by the high dose of pituitary radiotherapy. The case report illustrates the possibilities and impossibilities of treating acromegaly 50 years ago and demonstrates the potential risks of high dose pituitary radiotherapy (in acromegaly).
Learning points
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Acromegaly is a very old disease.
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Therapy for acromegaly has evolved over the decades.
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In art museums one can come across artistic impressions of endocrine disorders.
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People suffering from disfiguring endocrine disorders like acromegaly were pre-WW2 ‘exposed’ in theaters and circuses.
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High dose pituitary radiotherapy can be associated with secondary brain tumor formation.
Search for other papers by Nur Aisyah Zainordin in
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Search for other papers by Fatimah Zaherah Mohd Shah in
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Search for other papers by Nur Aini Eddy Warman in
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Search for other papers by Rohana Abdul Ghani in
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Summary
A 17-year-old lady presented with primary amenorrhoea, headache, nausea and lethargy. She had delayed pubertal development that also includes under-developed breast (Tanner Stage 2). Hormonal investigations showed a high serum prolactin level of 1 680 000 mIU/L (normal value: 45–375 mIU/L), with low oestradiol, progesterone, follicular-stimulating hormone and luteinizing hormone. Early morning cortisol level was 206 nmol/L (normal value: >450 nmol/L), thyroxine was 7.5 pmol/L (normal value: 9.0–24.0 pmol/L) with TSH 5.091 mIU/L (normal value: 0.4–4.5 mlU/L). A pituitary MRI showed a 2.7 (AP) × 3.7 (W) × 4.6 cm (CC) macroadenoma, with invasion into the left cavernous sinus and encasement of cavernous portion of the left internal carotid artery. MRI pelvis showed absent uterus, cervix and 2/3 upper vagina confirming Mullerian hypoplasia. Cytogenetics showed 46XX. These findings were suggestive of Mayer–Rokitansky–Kauser–Hauser (MRKH) syndrome with the presence of a pituitary macroprolactinoma and panhypopituitarism. She was treated with hydrocortisone, levothyroxine and cabergoline. Repeated MRI showed a reduction in tumour size by approximately 50%. This case illustrated a rare coexistence of these two conditions, being only the third reported case in the world. In addition, this would be the first case of a functioning pituitary adenoma in a patient with MRKH syndrome.
Learning points
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Comprehensive hormonal and radiological investigations are important in the management of a young patient with primary amenorrhoea.
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Coexistence pathology of two separate pathologies should be considered in patient presenting with primary amenorrhoea.
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Early diagnosis of MRKH or any disorders of sex development should be treated early, providing pharmacological, surgical, psychological and emotional support to the patient and reducing risk of associated complications.
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Abnormal pituitary hormones, particularly panhypopituitarism, would impose greater impact not only psychologically but also metabolically leading to cardiovascular, morbidity and mortality risks in this patient if not treated early.
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A multidisciplinary approach is necessary for patients presenting with MRKH to ensure appropriate treatments and follow-up across the lifespan of the patient.
Search for other papers by Mark R Postma in
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Summary
In July 2017, a 35-year-old woman was referred to our care for treatment of a large pituitary mass with an unusually high growth rate. She presented with right-sided ptosis and diplopia (n. III palsy), increasing retrobulbar pain and vertigo. Although laboratory investigations were consistent with acromegaly, she exhibited no clear phenotypic traits. During transsphenoidal surgery aimed at biopsy, typical adenomatous tissue was encountered, upon which it was decided to proceed to debulking. Histopathological analysis demonstrated a poorly differentiated plurihormonal Pit-1-positive adenoma with focal growth hormone (GH) and prolactin positivity, positive SSTR2 staining and a Ki-67 of 20–30%. Postoperative magnetic resonance imaging (MRI) examination revealed a large tumour remnant within the sella invading the right cavernous sinus with total encasement of the internal carotid artery and displacement of the right temporal lobe. As a consequence, she was treated additionally with radiotherapy, and a long-acting first-generation somatostatin analogue was prescribed. Subsequently, the patient developed secondary hypocortisolism and diabetes mellitus despite adequate suppression of GH levels. In September 2019, her symptoms recurred. Laboratory evaluations indicated a notable loss of biochemical control, and MRI revealed tumour progression. Lanreotide was switched to pasireotide, and successful removal of the tumour remnant and decompression of the right optic nerve was performed. She received adjuvant treatment with temozolomide resulting in excellent biochemical and radiological response after three and six courses. Symptoms of right-sided ptosis and diplopia remained. Evidence for systemic therapy in case of tumour progression after temozolomide is currently limited, although various potential targets can be identified in tumour tissue.
Learning points
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Poorly differentiated plurihormonal Pit-1-positive adenoma is a potentially aggressive subtype of pituitary tumours.
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This subtype can express somatostatin receptors, allowing treatment with somatostatin analogues.
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A multidisciplinary approach involving an endocrinologist, neurosurgeon, pituitary pathologist, neuroradiologist, radiation oncologist and medical oncologist is key for the management of patients with aggressive pituitary tumours, allowing the successful application of multimodality treatment.
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Temozolomide is first-line chemotherapy for aggressive pituitary tumours and carcinomas.
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Further development of novel targeted therapies, such as peptide receptor radionuclide therapy (PRRT), vascular endothelial growth factor (VEGF) receptor-targeted therapy, tyrosine kinase inhibitors, mammalian target of rapamycin (mTOR) inhibitors and immune checkpoint inhibitors, is needed.
Search for other papers by Darija Tudor in
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Department of Pediatrics, University of Split School of Medicine, Split, Croatia
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Department of Pediatrics, University of Split School of Medicine, Split, Croatia
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Summary
This is a case report of a child with chronic hyponatremia due to the syndrome of inappropriate antidiuretic hormone secretion (SIADH) as a paraneoplastic manifestation of olfactory neuroblastoma (OFN). We hereby report a clinical presentation as well as a pragmatic approach to one of the most common electrolytic disorders in the pediatric population and have emphasized the necessity of involving the sinonasal area in the diagnostic procedure while evaluating possible causes of SIADH. This report indicates that the chronicity of the process along with the gradual onset of hyponatremia occurrence is responsible for the lack of neurological symptoms at the moment of disease presentation.
Learning points
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Hyponatremia is not infrequently attributed to SIADH.
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Paraneoplastic syndromes are uncommon but they should be considered in the differential diagnosis of pediatric SIADH.
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Chronic insidious hyponatremia may not be associated with clear neurological symptoms despite its severity.
Search for other papers by Priya Darshani Chhiba in
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Search for other papers by David Segal in
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Summary
Recombinant human growth hormone therapy (rhGH) has been available since 1985 for a variety of conditions and has expanded the indications for rhGH therapy and the number of patients receiving therapy. The very nature of the therapy exposes individuals to years of injections. There are a number of well-known adverse events, however, a lesser-known and rarely reported adverse event of rhGH therapy is localized lipoatrophy. We report nine cases of localized lipoatrophy during rhGH therapy accounting for 14.5% of patients taking rhGH presenting to a single centre for routine follow-up over just a 2-month period. The development of localized lipoatrophy does not appear to be age, indication or dose-related but rather related to repeated administration of rhGH into a limited number of sites. The most likely putative mechanism is the local lipolytic action of growth hormone (GH) itself, although the possibility of an excipient-based interaction cannot be excluded. Given the high prevalence of this adverse event and the potential to prevent it with adequate site rotation, we can recommend that patients be informed of the possible development of localized lipoatrophy. Doctors and nurses should closely examine injection sites at each visit, and site rotation should be emphasized during injection technique education.
Learning points
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There are a number of well-known adverse events, however, a lesser-known and rarely reported adverse event of rhGH therapy is localized lipoatrophy.
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Examination of the injection sites at each visit by the treating healthcare practitioner.
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To advise the parents/caregivers/patients to change their injection site with each injection.
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To advise the parents/caregivers/patients to change the needles after every use.
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For parents, caregivers and patients to self-inspect their injection sites and have a high alert for the development of lipoatrophy and to then immediately report it to their doctor.
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Summary
A 38-year-old female was initially seen in the intensive care unit after severe postpartum hemorrhage. She was initially diagnosed to have Sheehan’s syndrome and after discharge, she was diagnosed to have a vesicovaginal fistula which initially caused a delay in diagnosing diabetes insipidus in the patient because she was having urinary incontinence. The patient had the vesicovaginal fistula repaired and was on replacement with levothyroxine, prednisone, and desmopressin. Years after her diagnosis, the patient experienced recurrent episodes of hyponatremia in the setting of desmopressin therapy. This case highlights the challenges of diagnosing diabetes insipidus in a patient with Sheehan’s syndrome and a vesicovaginal fistula, as well as the long-term management of central diabetes insipidus in a resource-limited setting.
Learning points
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Sheehan’s syndrome is rarely associated with diabetes insipidus, and in our patient, it was initially missed due to a vesicovaginal fistula which caused urinary incontinence.
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Water intoxication is more common in young children and older adults but can occur years after initiating treatment with desmopressin in adults and should be kept in mind when treating patients with hyponatremia who have hypopituitarism associated with diabetes insipidus.
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Water intoxication is much more common in patients with diabetes insipidus being treated with intranasal desmopressin than in those using the oral preparations.