GCT-75. ISOLATED PITUITARY STALK THICKENING

Abstract OBJECTIVES Only few studies have examined the predictive factors and outcome of isolated pituitary stalk thickening (PST) in children. We aim to describe our institutional cohort to determine predictors of future malignancy. METHODS A search of the radiology, endocrinology and neuro-oncology databases was performed to identify patients with isolated PST diagnosed between January 2000 and June 2019. Clinical data was collected. A detailed radiology review of baseline and follow up magnetic resonance imaging (MRI) was undertaken in a blinded fashion by two examiners. RESULTS Forty-four patients were identified, with 37 meeting criteria for isolated PST and adequate imaging. Median age of baseline MRI was 9.9 years (range 0.9–17.5). Twenty-three were female (62%). Median follow up time was 5 (0.31–18.6) years. Indication for MRI was symptoms of diabetes insipidus (DI) in 28 patients with the remainder having other concerns for endocrine disturbance (7), headache (1) or visual impairment (1). Thirty-five subjects had pituitary dysfunction (95%), including 30 with diabetes insipidus (81%). Nine patients developed a malignancy (24%), with germinoma (5), Langerhans cell histiocytosis (3) and lymphoma (1) at a median of 0.36 years, 0.63 years and 1.1 years respectively. Elevated white blood cell count (>5 x 106/L) in initial cerebrospinal fluid analysis was predictive of future diagnosis of germinoma or lymphoma (p=0.027). CONCLUSION In this cohort 24% of children with PST were eventually diagnosed with a neoplasia after a median of 0.63 years. Pleocytosis in initial CSF samples was predictive for future development of germinoma or lymphoma.

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Central Diabetes Insipidus as the Inaugural Manifestation of Langerhans Cell Histiocytosis: Natural History and Medical Evaluation of 26 Children and Adolescents

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/jc.2011-0513 ◽

2011 ◽

Vol 96 (9) ◽

pp. E1352-E1360 ◽

Cited By ~ 35

Author(s):

Isis Marchand ◽

Mohamed Aziz Barkaoui ◽

Catherine Garel ◽

Michel Polak ◽

Jean Donadieu ◽

...

Keyword(s):

Diabetes Insipidus ◽

Langerhans Cell Histiocytosis ◽

Third Ventricle ◽

Langerhans Cell ◽

Central Diabetes Insipidus ◽

Pituitary Stalk ◽

The Third ◽

Mri Features ◽

Pituitary Stalk Thickening

Abstract Context: Isolated central diabetes insipidus (CDI) can be the first manifestation of Langerhans cell histiocytosis (LCH), creating diagnostic dilemmas such as dysgerminoma and other inflammatory lesions. Method: In 2010, the French national LCH registry had enrolled 1236 LCH patients under 18 yr of age. Isolated CDI was the initial presentation of LCH in 26 patients. We reviewed their clinical and magnetic resonance imaging (MRI) features. Results: Median age at the diagnosis of CDI was 9.6 yr (1.8–16.3), and median follow-up after CDI diagnosis was 9.9 yr (3.5–26.6). In addition to CDI, two patients had visual field defects, four had secondary amenorrhea, and 11 had anterior pituitary deficiency. Cerebral imaging (including computed tomography in two cases), performed in 22 patients within 3 months of CDI diagnosis, showed pituitary stalk thickening in 14 patients, which was moderate (3.0–7 mm) in nine cases and marked (>7 mm) in five cases. In eight cases, the lesion extended to the floor of the third ventricle. One child with LCH presented with a mild enlarged sellar content. During follow-up, 22 patients developed extrapituitary involvement, mainly of bone (n = 15), lung (n = 9), and skin (n = 9). Pituitary biopsy was performed in eight cases and was conclusive in six cases. Conclusions: Pituitary stalk thickening can be observed in LCH as well as lesions extending to the floor of the third ventricle. In all cases but one, the intrasellar content was not enlarged. Long-term follow-up with close attention to bone, skin, and lung disorders may lead to the diagnosis of LCH.

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Idiopathic Hypothalamic Diabetes Insipidus, Pituitary Stalk Thickening, and the Occult Intracranial Germinoma in Children and Adolescents*

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/jcem.82.5.3955 ◽

1997 ◽

Vol 82 (5) ◽

pp. 1362-1367

Author(s):

Sudha L. Mootha ◽

Anthony J. Barkovich ◽

Melvin M. Grumbach ◽

Michael S. Edwards ◽

Stephen E. Gitelman ◽

...

Keyword(s):

Diabetes Insipidus ◽

Brain Mri ◽

Inflammatory Cells ◽

Pituitary Stalk ◽

Pituitary Hormone ◽

Resonance Imaging ◽

Anterior Pituitary Hormone ◽

Mri Scans ◽

Pituitary Stalk Thickening

Abstract We report nine consecutive children and adolescents [five females and four males; aged 2 yr 8 months (m) to 18 yr 1 m] studied over the last 5 yr with idiopathic central diabetes insipidus. In addition to vasopressin deficiency, anterior pituitary hormone deficiencies were detected, either on evaluation at presentation or during follow-up studies over the following 3 yr. Four patients had an increased concentration of plasma PRL. One patient had multiple pituitary hormone deficiencies at diagnosis, and two others developed the same by 21 m of follow-up. Brain magnestic resonance imaging scans, performed at presentation, were originally interpreted as normal in four of nine patients, except for absence of the bright posterior pituitary signal; after retrospective review, two of nine were considered normal. All of the brain magnetic resonance imaging (MRI) scans showed positive findings by 14 m of follow-up. The first abnormal finding in all patients was isolated pituitary stalk thickening. Evaluation of cerebrospinal fluid (CSF) for hCG was positive in three of eight evaluated patients; the three positive CSF values were found at presentation and 3 and 9 m after presentation. All eight patients assessed were negative for CSF α-fetoprotein and cytology, and no patient had serum tumor markers. Transsphenoidal biopsy of the lesion in seven of nine patients showed a germinoma in six patients and inflammatory cells in one. The six patients with documented germinoma comprise 31% of the intracranial germinomas diagnosed in this age group at the University of California-San Francisco during the last 5 yr. The patient with mononuclear inflammatory cells on biopsy along with one other patient have had spontaneous resolution of their stalk thickening. So-called “idiopathic” central diabetes insipidus warrants close follow-up to determine the etiology, especially if anterior pituitary hormone deficiencies are detected. Normal brain MRI scans or scans that show isolated pituitary stalk thickening merit follow-up with serial contrast enhanced brain MRI for the early detection of an evolving occult hypothalamic-stalk lesion. CSF evaluation is recommended at presentation because elevated CSF hCG may precede MRI abnormalities.

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Approach To The Pediatric Patient: Central Diabetes Insipidus

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/clinem/dgab930 ◽

2022 ◽

Author(s):

Giuseppa Patti ◽

Flavia Napoli ◽

Daniela Fava ◽

Emilio Casalini ◽

Natascia Di Iorgi ◽

...

Keyword(s):

Differential Diagnosis ◽

Diabetes Insipidus ◽

Central Diabetes Insipidus ◽

Pituitary Stalk ◽

Central Nervous System Damage ◽

Gold Standard Method ◽

Underlying Condition ◽

Detailed Medical History ◽

Over Time

Abstract Central diabetes insipidus (CDI) is a complex disorder in which large volumes of dilute urine are excreted due to arginine-vasopressin deficiency, and it is caused by a variety of disorders affecting the hypothalamic-posterior pituitary network. The differential diagnosis is challenging and requires a detailed medical history, physical examination, biochemical approach, imaging studies and, in some cases, histological confirmation. Magnetic resonance imaging is the gold standard method for evaluating congenital or acquired cerebral and pituitary stalk lesions. Pituitary stalk size at presentation could be normal, but it may change over time, depending on the underlying condition, while other brain areas or organs may become involved during follow up. Early diagnosis and treatment are crucial in order to avoid central nervous system damage, germ cell tumor dissemination, and to minimize complications of multiple pituitary hormone defects. We provide a practical update on the diagnosis and management of patients with CDI and highlight several pitfalls that may complicate the differential diagnosis of conditions presenting with polyuria and polydipsia. The need for a careful and close follow-up of patients with “apparently” idiopathic CDI is particularly emphasized, because the underlying condition may be recognized over time. The clinical scenario that we outline at the beginning of this article represents the basis for the discussion about how the etiological diagnosis of CDI can be overlooked, and demonstrates how a water intake and urine output improvement can be a sign of progressive damage of both hypothalamus and anterior pituitary gland with associated pituitary hormonal deficiencies.

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Pituitary Morphology and Function in 43 Children with Central Diabetes Insipidus

International Journal of Endocrinology ◽

10.1155/2016/6365830 ◽

2016 ◽

Vol 2016 ◽

pp. 1-7 ◽

Cited By ~ 8

Author(s):

Wendong Liu ◽

Limin Wang ◽

Minghua Liu ◽

Guimei Li

Keyword(s):

Diabetes Insipidus ◽

Central Diabetes Insipidus ◽

Pituitary Function ◽

Pituitary Stalk ◽

Hormone Deficiency ◽

Pituitary Hormone ◽

Initial Manifestation ◽

Stalk Diameter ◽

And Function

Objective. In pediatric central diabetes insipidus (CDI), etiology diagnosis and pituitary function monitoring are usually delayed. This study aimed to illustrate the importance of regular follow-up and pituitary function monitoring in pediatric CDI.Methods. The clinical, hormonal, and neuroradiological characteristics of children with CDI at diagnosis and during 1.5–2-year follow-up were collected and analyzed.Results. The study included 43 CDI patients. The mean interval between initial manifestation and diagnosis was 22.29 ± 3.67 months (range: 2–108 months). The most common complaint was polyuria/polydipsia. Causes included Langerhans cell histiocytosis, germinoma, and craniopharyngioma in 2, 5, and 4 patients; the remaining were idiopathic. No significant changes were found during the 1.5–2 years after CDI diagnosis. Twenty-three of the 43 cases (53.5%) had ≥1 anterior pituitary hormone deficiency. Isolated growth hormone deficiency was the most frequent abnormality (37.5%) and was not associated with pituitary stalk diameter. Multiple pituitary hormone deficiencies were found in 8 cases with pituitary stalk diameter > 4.5 mm.Conclusion. Diagnosis of CDI is usually delayed. CDI with a pituitary stalk diameter > 4.5 mm carries a higher risk of multiple pituitary hormone deficiencies. Long-term MRI and pituitary function follow-ups are necessary for children with idiopathic CDI.

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GERM-22. DECISION-MAKING MANAGEMENT GUIDELINE FOR CHILDREN AND YOUNG PEOPLE UP TO 19 YEARS (CYP) PRESENTING WITH IDIOPATHIC PITUITARY STALK THICKENING (iTPS) AND/OR IDIOPATHIC CENTRAL DIABETES INSIPIDUS (iCDI)

Neuro-Oncology ◽

10.1093/neuonc/noy059.269 ◽

2018 ◽

Vol 20 (suppl_2) ◽

pp. i88-i88

Author(s):

Manuela Cerbone ◽

Johannes Visser ◽

Chloe Bulwer ◽

Ashraf Ederies ◽

Kirtana Vallabhaneni ◽

...

Keyword(s):

Decision Making ◽

Young People ◽

Diabetes Insipidus ◽

Central Diabetes Insipidus ◽

Pituitary Stalk ◽

Management Guideline ◽

Children And Young People ◽

Pituitary Stalk Thickening

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Importance of Follow-Up Cerebrospinal Fluid Analysis in Cryptococcal Meningoencephalitis

Disease Markers ◽

10.1155/2014/162576 ◽

2014 ◽

Vol 2014 ◽

pp. 1-10 ◽

Cited By ~ 17

Author(s):

Thomas Skripuletz ◽

Philipp Schwenkenbecher ◽

Kaweh Pars ◽

Matthias Stoll ◽

Josef Conzen ◽

...

Keyword(s):

Cell Count ◽

Antifungal Therapy ◽

Fatal Outcome ◽

University Hospital ◽

Cerebrospinal Fluid Analysis ◽

Cryptococcal Meningoencephalitis ◽

Fluid Analysis ◽

Serious Infection ◽

The Central Nervous System

Cryptococcal meningoencephalitis represents a serious infection of the central nervous system, where reliable prognostic factors during the disease course are needed. Twenty-one patients diagnosed with cryptococcal meningoencephalitis in a German university hospital from 1999 to 2013 were analysed retrospectively. CSF parameters were analysed prior to therapy and during antifungal treatment and were compared between patients who survived or deceased. Fifteen patients clinically improved after antifungal therapy, while six patients died. No differences were observed between the outcome groups for the CSF parameters cell count, lactate, total protein, and CSF-serum albumin quotients (QAlb). Follow-up examinations of serum cryptococcal antigen titer and CSF cell count have shown that these parameters cannot be used to monitor the efficacy of antifungal therapy as well. In contrast, the course of QAlb during therapy was indicative for the outcome as a possible prognostic marker. In patients with clinical improvement QAlb values were falling under therapy, while rising QAlb values were found in patients with fatal outcome indicating a continuing dysfunction of the blood-CSF barrier. In conclusion, our results indicate that, among the various CSF parameters, the course of QAlb presents a promising marker that might be used to monitor the efficacy of antifungal therapy.

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Acute and Chronic Posterior Pituitary Insufficiency Among Traumatic Brain Injury Patients at a Tertiary Care Center

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.1270 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A623-A623

Author(s):

Imtiyaz Ahmad Bhat ◽

Moomin Hussain Bhat ◽

Shariq Rashid Masoodi ◽

Javid Ahmad Bhat ◽

Zafar A Shah ◽

...

Keyword(s):

Traumatic Brain Injury ◽

Brain Injury ◽

Head Injury ◽

Diabetes Insipidus ◽

Hospital Stay ◽

Tertiary Care ◽

Posterior Pituitary ◽

Urinary Osmolality ◽

Pituitary Dysfunction

Abstract Background: Traumatic brain injury (TBI) is the leading cause of death and disability in young adults. Disorders of salt and water balance are the most commonly recognized medical complications in the immediate post-TBI period and contribute to early morbidity and mortality. Objective: We aimed to evaluate the prevalence of acute (during hospital stay) and chronic posterior pituitary dysfunction in patients of head injury admitted at our tertiary care hospital. Study Design: Prospective, Observational study. Participants: 136 patients, attending tertiary care in North India with TBI with radiological evidence of head injury. Methodology: The severity of brain injury was assessed by the Glasgow Coma Scale (GCS), and Modified Rankin Scale (MRS) score at the time of admission. Lab measurements, apart from routine CBC and biochemical tests, included tests of serum and urinary osmolality, serum sodium, cortisol, and thyroid function test during the hospital stay. All patients were monitored closely during the hospital stay. Surviving patients were evaluated at 3, 6, and 12 months of follow-up. Urinary output and water deprivation tests were done to determine chronic posterior pituitary dysfunction. The results were compared against normative data obtained from 25 matched, healthy controls. Serum & urinary osmolality was measure by the freezing point method. Diabetes insipidus (DI) and Syndrome of inappropriate ADH secretion (SIADH) were diagnosed according to standard criteria. Results: Of 136 patients admitted, 61 (44.85%) had a mild head injury (GCS, ≤8), 47 (35.55%) had a moderate injury (GCS, 9-12), and 27 (19.85%) had a severe injury (GCS, 13-15). DI occurred in 10 patients (7.4%), while SIADH was observed in 4 patients in the immediate TBI period. Risk factors for diabetes insipidus were GCS of ≤ 8 at admission, midline shift, and surgical intervention. DI was an independent risk factor for death. There was a negative correlation between the presence of DI and GCS score (r, -0.367). Most of the patients with DI (8 out of 10) died during the hospital stay. One patient persisted to have partial diabetes insipidus and another one SIADH at three months post-TBI; both patients had recovered at six months of follow-up. No new case of DI or SIADH occurred on the follow up to 12 months. Conclusion: The incidence of acute DI in severe head injury (GCS ≤ 8) could be an indicator of the severity of TBI, and associated with increased mortality as most of our patients died during the hospital stay.

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An unusual headache: CSF negative APML relapse in the brain

Oxford Medical Case Reports ◽

10.1093/omcr/omaa075 ◽

2020 ◽

Vol 2020 (9) ◽

Author(s):

Thomas Quinn ◽

Manish Jain ◽

Ming-Te Lee

Keyword(s):

Diagnostic Delay ◽

Diagnostic Challenge ◽

Cerebrospinal Fluid Analysis ◽

Fluid Analysis ◽

Diagnostic Difficulties ◽

The Central Nervous System ◽

High Degree ◽

The Brain ◽

Post Therapy

ABSTRACT Acute Promyelocytic Leukaemia (APML) is a subtype of Acute Myeloid Leukaemia (AML), responsible for around 10% of cases of the disease in adults. Extra medullary disease (EMD) occurs infrequently in APML, but where EMD does occur, the central nervous system is one of the most commonly infiltrated sites. Our case describes a man in his 40s undergoing post-therapy surveillance for APML who presented to follow-up clinic with a headache, which was ultimately found to be caused by a tumour comprised of APML cells. His case presented a diagnostic challenge due to the benign appearances of the lesion on initial computed tomography brain imaging and the non-diagnostic cerebrospinal fluid analysis. The diagnostic difficulties described in our case emphasizes that clinicians working with APML patients must approach new neurological symptoms with a high degree of suspicion to prevent diagnostic delay.

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