Hypothalamic-Pituitary Axis Dysfunction in Survivors of Childhood CNS Tumors: Importance of Systematic Follow-Up and Early Endocrine Consultation

2016 ◽  
Vol 34 (36) ◽  
pp. 4315-4319 ◽  
Author(s):  
Wassim Chemaitilly ◽  
Gregory T. Armstrong ◽  
Amar Gajjar ◽  
Melissa M. Hudson
Keyword(s):  
Relevant Literature ◽  
Pubic Hair ◽  
Hormone Deficiency ◽  
Shunt Revision ◽  
Plasma Testosterone ◽  
Low Grade ◽  
Pubertal Stage ◽  
Suprasellar Region ◽  
Hypothalamic Pituitary Axis

The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors’ suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice. An 11-year-old male with a history of metastatic tectal plate low-grade glioma who was diagnosed at age 2.8 years transferred his care to the long-term follow-up clinic. He completed treatment with multiagent chemotherapy—carboplatin, vincristine, temozolomide, procarbazine, lomustine, and thioguanine—at age 4.5 years and did not require radiotherapy. At primary diagnosis, he presented with hydrocephalus that required ventriculoperitoneal shunt placement, with a subsequent shunt revision at age 6 years. Residual metastatic tumors in the third and fourth ventricles and in the suprasellar region remained stable for more than 5 years. The patient achieved normal developmental milestones and was not taking medications. He was offered screening for hypothalamic-pituitary axis (HPA) dysfunction because of his suprasellar lesion. His height was at the 25th percentile for chronological age, with decline from the 50th percentile noted during the preceding 18 months ( Fig 1 , point c). Pubertal stage was Tanner 4 for pubic hair and penile size, which contrasted with small testes (4.5 mL). Pubic hair and voice changes were noticed 2 to 3 years before this visit. Plasma testosterone level was consistent with Tanner 4 (255 ng/dL = 8.9 nmol/L). An x-ray of the left hand revealed a notably advanced bone age of 15.5 years. Plasma free T4, thyroid-stimulating hormone (TSH), and 8 am cortisol levels were normal. The patient was referred to the endocrinology clinic where he was diagnosed with growth hormone deficiency and was started on replacement therapy. He reached his final adult height of 144.1 cm at age 13.3 years ( Fig 1 , point d).

PLASMA LH AND FSH RESPONSE TO LRH AND PLASMA TESTOSTERONE LEVELS IN BOYS WITH IRREGULAR PUBERTY

1977 ◽  
Vol 85 (3) ◽  
pp. 456-464 ◽  
Author(s):  
Zvi Dickerman ◽  
Yehuda Bar-Haim ◽  
Ruth Prager-Lewin ◽  
Haiuta Kaufman ◽  
Zvi Laron
Keyword(s):  
Bone Age ◽  
Pubic Hair ◽  
Growth Spurt ◽  
Plasma Testosterone ◽  
Control Group ◽  
Testicular Development ◽  
Pubertal Stage ◽  
Testosterone Levels ◽  
Basal Plasma ◽  
Pubertal Stages

ABSTRACT Nineteen boys with irregular puberty (IP), defined as a discrepancy of two or more pubertal stages between the criteria for genitalia and that for pubic hair, were subjected to a standard LRH test (50 μg/m2, iv) and the response of gonadotrophins as well as the basal levels of plasma testosterone, LH and FSH were compared to those of boys with normal, regular puberty. When the results were plotted against the pubertal stage for genitalia (Pg), it was found that in the boys with IP the basal plasma testosterone levels were lower and the response of plasma LH to LRH stimulation lesser than in the controls. However, when these parameters were plotted against the pubertal stage for pubic hair (Ph) it was found, that in the boys with IP the plasma testosterone levels were significantly higher and the response of both LH and FSH stimulation greater than in the control group. It was concluded that irregular puberty in boys may be regarded as a normal variation. The delayed development of sexual hair and penile length, and retarded pubertal growth spurt and bone age maturation seen in these boys, with normal testicular development, may be explained by a temporary reduced peripheral sensitivity to androgens and a compensatory effort by the pituitary, manifested in increased secretion of LH and testosterone, relatively to their pubertal stage for pubic hair.

scholarly journals Phenotypic Variability in Familial Combined Pituitary Hormone Deficiency Caused by a PROP1 Gene Mutation Resulting in the Substitution of Arg→Cys at Codon 120 (R120C)1

1998 ◽  
Vol 83 (10) ◽  
pp. 3727-3734 ◽  
Author(s):  
Christa Flück ◽  
Johnny Deladoey ◽  
Kuno Rutishauser ◽  
Andrée Eblé ◽  
ULRICH Marti ◽  
...  
Keyword(s):  
Phenotypic Variability ◽  
Failure To Thrive ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Pituitary Hormone Deficiency ◽  
Combined Pituitary Hormone Deficiency ◽  
Ames Dwarf ◽  
Hypothalamic Pituitary Axis ◽  
Prop1 Gene

As pituitary function depends on the integrity of the hypothalamic-pituitary axis, any defect in the development and organogenesis of this gland may account for a form of combined pituitary hormone deficiency (CPHD). A mutation in a novel, tissue-specific, paired-like homeodomain transcription factor, termed Prophet of Pit-1 (PROP1), has been identified as causing the Ames dwarf (df) mouse phenotype, and thereafter, different PROP1 gene alterations have been found in humans with CPHD. We report on the follow-up of two consanguineous families (n = 12), with five subjects affected with CPHD (three males and two females) caused by the same nucleotide C to T transition, resulting in the substitution of Arg→Cys in PROP1 at codon 120. Importantly, there is a variability of phenotype, even among patients with the same mutation. The age at diagnosis was dependent on the severity of symptoms, ranging from 9 months to 8 yr. Although in one patient TSH deficiency was the first symptom of the disorder, all patients became symptomatic by exhibiting severe growth retardation and failure to thrive, which was mainly caused by GH deficiency (n = 4). The secretion of the pituitary-derived hormones (GH, PRL, TSH, LH, and FSH) declined gradually with age, following a different pattern in each individual; therefore, the deficiencies developed over a variable period of time. All of the subjects entered puberty spontaneously, and the two females also experienced menarche and periods before a replacement therapy was necessary.

Il craniofaringioma

2021 ◽  
Vol 24 (7) ◽  
pp. 210-216
Author(s):  
Nicola Salce ◽  
Elena Giovannelli ◽  
Vanna Graziani ◽  
Mariarosaria Cozzolino ◽  
Diego Mazzatenta ◽  
...  
Keyword(s):  
Bimodal Distribution ◽  
Surgical Removal ◽  
Low Grade ◽  
Suprasellar Region ◽  
Paediatric Age ◽  
Hypothalamic Pituitary Axis ◽  
Subtype A ◽  
Grade Tumour ◽  
Visual Disturbances

Craniopharyngioma (CP) is a rare epithelial low-grade tumour that develops in the sellar/suprasellar region of the brain, along the craniopharyngeal duct. It has a bimodal distribution and the first peak occurs in paediatric age almost exclusively consisting of the adamantinomatous subtype. A second peak occurs in adulthood after the fifth decade of life and is more likely to be of the papillary subtype. Therapy is based on surgical removal, so the best approach is the endonasal approach, which is sometimes associated with radiation therapy. Molecular target drugs are a promising novelty, indeed they are already in use in adults and are being tested in children. Although CP is considered a low malignancy tumour, its localization and close relationships with important structures such as the optical pathways, the hypothalamic-pituitary axis and the thalamus burden it with important complications (visual disturbances, central obesity, dysendocrinopathies) that can interfere with the patient’s quality of life.

scholarly journals Serum soluble urokinase plasminogen activator receptor (suPAR) in adults with growth hormone deficiency

2019 ◽  
Vol 8 (6) ◽  
pp. 772-779 ◽  
Author(s):  
Charlotte Höybye ◽  
Laia Faseh ◽  
Christos Himonakos ◽  
Tomasz Pielak ◽  
Jesper Eugen-Olsen
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Plasminogen Activator ◽  
Hormone Deficiency ◽  
Low Grade ◽  
Urokinase Plasminogen Activator Receptor ◽  
Gh Treatment ◽  
Plasminogen Activator Receptor ◽  
Low Grade Inflammation

Growth hormone deficiency (GHD) syndrome is associated with adverse levels of several risk factors for cardiovascular diseases (CVD), including metabolic inflammation. However, the impact of GHD and GH treatment on low-grade inflammation is unknown. The aim of the study was to establish the level of the low-grade inflammation biomarker soluble urokinase plasminogen activator receptor (suPAR) in adults with GHD and the response to long-term GH treatment. Measurements of suPAR and CRP were performed in bio-bank serum samples from 72 adults, 34 males and 38 females, with GHD before and during at least 5 years of GH treatment. Mean age was 52.5 ± 15.5 years, BMI 27.3 ± 5 kg/m2. Clinical evaluations and blood sampling were performed at routine visits. Data on demography, anthropometry, lab results and clinical events were retrieved from post-marketing surveillance study databases and medical records. suPAR and high-sensitive (hs) CRP were analysed using ELISA and immunochemistry, respectively. At baseline blood pressure, lipid profile and fasting glucose were within the normal reference range. Baseline geometric mean and 95% CI of suPAR was 2.9 (2.7–3.3) ng/mL and of CRP 2.3 (0.6–4.0) mg/L. Mean follow-up was 8 ± 2 years. The suPAR levels remained stable during follow-up, although individual increases were seen on occurrence or presence of co-morbidities. In contrast, levels of CRP decreased. In conclusion, the decrease in CRP and indirectly the absence of an expected increase in suPAR over time indicates a favourable effect of GH on low-grade inflammation.

scholarly journals A Rare Case of Keratoacanthoma of the Palm: Special Considerations when Treating Keratoacanthoma of the Hand

2021 ◽  
pp. 1-3
Author(s):  
Ofelia Leroux ◽  
Ofelia Leroux ◽  
Y Vu Robert Van ◽  
Ryan Engdahl
Keyword(s):  
Rare Case ◽  
Relevant Literature ◽  
Surgical Excision ◽  
Complete Removal ◽  
Skin Tumor ◽  
Low Grade ◽  
Mucous Membranes ◽  
Soles Of The Feet ◽  
One Year

Keratoacanthoma (KA) is a low grade, rapidly growing skin tumor which is thought to originate from the pilosebaceous unit from hyperkeratosis of the infundibulum and are often thought to originate on hair bearing skin or sun exposed surfaces. There are very scarce reports demonstrating they may occur in other areas such as mucous membranes or soles of the feet. We present a rare case of palmar KA in a 65-year-old female with no known antecedent history. Surgical excision was performed with complete removal of the tumor. Following surgical excision, the pathology of the irregularly elevated 0.7 x 0.2 cm lesion revealed a keratoacanthoma. The patient remained without evidence of recurrence at one year follow-up. We believe there is only one other report of isolated palmar KA to date in the literature. We review relevant literature on hand KA.

Long-term stability of fusiform dilatation of the internal carotid artery following surgery adjacent to the circle of Willis: report of 2 cases

2020 ◽  
pp. 1-4
Author(s):  
Madeline B. Karsten ◽  
R. Michael Scott
Keyword(s):  
Carotid Artery ◽  
Internal Carotid Artery ◽  
Arachnoid Cyst ◽  
Pediatric Patients ◽  
Internal Carotid ◽  
Imaging Finding ◽  
Neurological Status ◽  
Suprasellar Region

Fusiform dilatation of the internal carotid artery (FDCA) is a known postoperative imaging finding after craniopharyngioma resection. FDCA has also been reported following surgery for other lesions in the suprasellar region in pediatric patients and is thought to be due to trauma to the internal carotid artery (ICA) wall during tumor dissection. Here, the authors report 2 cases of pediatric patients with FDCA. Case 1 is a patient in whom FDCA was visualized on follow-up scans after total resection of a craniopharyngioma; this patient’s subsequent scans and neurological status remained stable throughout a 20-year follow-up period. In case 2, FDCA appeared after resection and fenestration of a giant arachnoid cyst in a 3-year-old child, with 6 years of stable subsequent follow-up, an imaging finding that to the authors’ knowledge has not previously been reported following surgery for arachnoid cyst fenestration. These cases demonstrate that surgery involving dissection adjacent to the carotid artery wall in pediatric patients may lead to the development of FDCA. On very long-term follow-up, this imaging finding rarely changes and virtually all patients remain asymptomatic. Neurointerventional treatment of FDCA in the absence of symptoms or significant late enlargement of the arterial ectasia does not appear to be indicated.

Biomarkers of Acromegaly and Growth Hormone Action

2020 ◽  
Vol 27 (12) ◽  
pp. 1231-1245
Author(s):  
Filippo Maffezzoni ◽  
Teresa Porcelli ◽  
Andrea Delbarba ◽  
Letizia Pezzaioli ◽  
Carlo Cappelli ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Biological Markers ◽  
Clinical Care ◽  
Hormone Deficiency ◽  
Surrogate Outcomes ◽  
Current State ◽  
Growth Hormone Action ◽  
Igf I ◽  
Definition Of

: Biological markers (biomarkers) play a key role in drug development, regulatory approval and clinical care of patients and are linked to clinical and surrogate outcomes. : Both acromegaly and Growth Hormone Deficiency (GHD) are pathological conditions related to important comorbidities that, in addition to having stringent diagnostic criteria, require valid markers for the definition of treatment, treatment monitoring and follow-up. GH and insulin-like growth factor-I (IGF-I) are the main biomarkers of GH action in children and adults while, in acromegaly, both GH and IGF-I are established biomarkers of disease activity. : However, although GH and IGF-I are widely validated biomarkers of GHD and acromegaly, their role is not completely exhaustive or suitable for clinical classification and follow-up. Therefore, new biological markers for acromegaly and GH replacement therapy are strongly needed. : The aim of this paper is to review and summarize the current state in the field pointing out new potential biomarkers for acromegaly and GH use/abuse.

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