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Persistent neonatal hypoglycaemia as a result of hypoplastic pituitary gland

Yong SC, Boo NY, Wu LL

Br J Hosp Med (Lond), 2006 Jun;67(6):326.
PMID: 16821748 DOI: 10.12968/hmed.2006.67.6.21296
Matched MeSH terms: Hypopituitarism/complications*; Hypopituitarism/diagnosis; Hypopituitarism/drug therapy
Multiple system Erdheim-Chester disease with massive hypothalamic-sellar involvement and hypopituitarism

Oweity T, Scheithauer BW, Ching HS, Lei C, Wong KP

J. Neurosurg., 2002 Feb;96(2):344-51.
PMID: 11838810

Erdheim-Chester disease (ECD) is a rare multiple system histiocytosis that is characterized pathologically by xanthogranulomatous infiltrates and radiologically by symmetrical sclerosis of long bones. The diagnosis is often confirmed by biopsy of bone or of orbital or retroperitoneal soft tissue. Intracranial involvement is rare. The authors report a case of ECD in which the diagnosis was made after biopsy of a hypothalamic mass. The mass had been discovered during a workup for panhypopituitarism in a 55-year-old man with urological and bone disease. Aside from diabetes insipidus, other features of pituitary insufficiency have seldom been reported and no patients have presented with a hypothalamic tumor. The endocrinological and neurological aspects of ECD are discussed, as is its differential diagnosis. Reported cases of the disorder associated with hypopituitarism or found during biopsy of central nervous system structures are also reviewed.

Matched MeSH terms: Hypopituitarism/complications*; Hypopituitarism/pathology*; Hypopituitarism/physiopathology
Fulltext Addisonian-like crisis in congenital hypopituitarism and cholestatic jaundice

Lee WS, Lum LCS, Harun F

Med J Malaysia, 2003 Jun;58(2):279-81.
PMID: 14569750

A six-week-old male infant was admitted for investigation of cholestasis and pale stools. He became lethargic and apnoeic with prolonged seizures after a percutaneous liver biopsy. Subsequent investigations showed conjugated hyperbilirubinaemia, elevated liver enzymes, and hypoglycaemia. The radinuclide hepatobiliary scintigraphy was non-excretory. After an operative cholangiogram, the infant developed Addisonian-like crisis with bradycardia, hypotension, respiratory distress, metabolic acidosis, hypoglycaemia, hyponatraemia, and hyperkalaemia. Blood investigations confirmed congenital hypopituitarism. Hormone replacement therapy with L-thyroxine and cortisone acetate resulted in dissolution of jaundice and the reduction of the liver size.

Matched MeSH terms: Hypopituitarism/complications*; Hypopituitarism/diagnosis; Hypopituitarism/therapy
Fulltext Masking of central diabetes insipidus and hypogonadotrophic hypogonadism by germ cell tumour in suprasellar--pineal region

Isa SH, Wong M, Khalid BA

Med J Malaysia, 2006 Dec;61(5):630-2.
PMID: 17623967

A patient with beta hCG-secreting germ cell carcinoma of the pineal and suprasellar regions presented with hydrocephalus, Parinaud's syndrome, hypopituitarism and polyuria. Central diabetes insipidus was strongly suspected although the water deprivation test was not diagnostic. The polyuria however, responded to ADH analogue when the hypothyroidism and hypocortisolism were treated. Pubertal development was evident and serum testosterone was normal despite the low FSH/LH, suggesting hCG stimulation of Leydig cells. This case illustrates that a beta hCG-germ cell tumour of the suprasellar region causing hypopituitarism can mask the presence of central diabetes insipidus and hypogonadotrophic hypogonadism.

Matched MeSH terms: Hypopituitarism/diagnosis*; Hypopituitarism/etiology
Now You See, Now You Don't: A Case Of Spontaneous Regression of Pituitary Tumour

Jaafar AS, Mohd Shokri SS, Paramasvaran S, Palaniandy K, Fadzil F

Cureus, 2020 Jul 14;12(7):e9174.
PMID: 32802610 DOI: 10.7759/cureus.9174

Spontaneous regression of pituitary tumours are rare and can be due to tumour ischaemia, pituitary apoplexy, or lymphocytic hypophysitis. We report a case of a 32-year-old female, who presented with symptoms and signs of extrasellar pituitary enlargement and hypothyroidism. MRI revealed a pituitary mass that spontaneously regressed after a month, with complete resolution of symptoms. Not all pituitary tumours require surgical intervention especially in the case of autoimmune lymphocytic hypophysitis.

Matched MeSH terms: Hypopituitarism
Penile length of newborns in Singapore

Lian WB, Lee WR, Ho LY

J Pediatr Endocrinol Metab, 2000 Jan;13(1):55-62.
PMID: 10689638

Micropenis is an important sign in congenital hypopituitarism and various disorders. Documented norms for penile length exist only for babies of Caucasian and Middle-Eastern origin. This study was carried out to establish such norms for Asian newborns. We studied 228 male live births within their first three days of life. Stretched penile lengths were marked off on unmarked wooden spatulas, which were placed vertically along the dorsal aspect of the penis, with one rounded end on the pubic bone. The mean penile length +/- S.D. for the full-term Asian baby was 3.6 +/- 0.4 cm. Race had a significant effect: Chinese 3.5 cm, Malay 3.6 cm and Indian 3.8 cm. Penile length correlated with birth weight and gestational age. Asian babies thus have similar norms to Caucasian babies. An Asian newborn whose penis measures less than 2.6 cm has micropenis and may need prompt investigation for underlying endocrine disorders.

Matched MeSH terms: Hypopituitarism/complications
Fulltext Burkitt's lymphoma presenting with hypopituitarism: a case report and review of literature

Foo SH, Sobah SA

Endocrinol Diabetes Metab Case Rep, 2014;2014:140029.
PMID: 25045522 DOI: 10.1530/EDM-14-0029

Hypopituitarism is a rare presentation of Burkitt's lymphoma (BL). The purpose of this report is to present a case of BL presenting with panhypopituitarism and to review other case reports of lymphoma presenting with pituitary dysfunction to highlight the distinguishing features of these cases from other benign aetiologies of pituitary dysfunction such as non-functioning pituitary adenomas. We reviewed a total of 11 cases of lymphoma presenting with pituitary dysfunction published from 1998 to 2013 including the present case. The demographics, clinical presentations, laboratory features, radiological findings, histological diagnosis, treatment administered and outcomes were described. Of the total number of patients, 45.5% of the cases had diffuse large B-cell lymphoma while 27.3% had BL. Anterior pituitary dysfunction was more common than posterior pituitary dysfunction at presentation. The other common associated presenting symptoms were painful ophthalmoplegia, cranial nerve palsies and constitutional symptoms. Hypothalamic-pituitary abnormalities were often demonstrated radiologically to be associated with cavernous sinus and/or stalk involvement. All patients who completed immunochemotherapy responded haematologically. Pituitary dysfunction also improved in most cases although the recovery tended to be partial. In conclusion, a high index of suspicion of underlying malignancy, such as lymphoma, should be present in patients presenting with acute pituitary dysfunction associated with painful ophthalmoplegia, rapidly evolving neurological features, radiological features atypical of a pituitary adenoma and constitutional symptoms. An early diagnosis is essential as prompt initiation of definitive therapy will induce disease remission and recovery of pituitary dysfunction.

Matched MeSH terms: Hypopituitarism
Fulltext Granulomatous hypophysitis: a case of severe headache

Wan Muhamad Hatta SF, Hamdan MF, Md Ali SA, Abdul Ghani R

BMJ Case Rep, 2016 Sep 09;2016.
PMID: 27613264 DOI: 10.1136/bcr-2016-216395

Idiopathic granulomatous hypophysitis (GH) is an uncommon inflammatory disease of the pituitary with impairment of pituitary gland function due to infiltration by lymphocytes, plasma cells and macrophages. We report the case of a 39-year-old woman who presented with worsening of headaches for 1 month and blurring of vision over 5 days. An MRI revealed a homogeneous supra-sellar mass evoking a pituitary tumour with bulky pituitary stalk extending into the left and right cavernous sinuses. Hormonal investigations showed anterior pituitary hormone deficiencies; meanwhile histopathological examination revealed an aspect of hypophysitis. Clinical and radiological remission occurred immediately postglucocorticoid therapy with the addition of a steroid-sparing agent later in view of recurrence of symptoms on glucocorticoid dose reduction. GH has important diagnostic and therapeutic implications, as clinical and radiological features ameliorate via medical treatment. With further understanding and recognition of the disease, we hope to highlight a case of GH, in which signs and symptoms improved after initiation of corticosteroids.

Matched MeSH terms: Hypopituitarism/diagnosis
Hypothalamic pituitary dysfunction amongst nasopharyngeal cancer survivors

Ratnasingam J, Karim N, Paramasivam SS, Ibrahim L, Lim LL, Tan AT, et al.

Pituitary, 2015 Aug;18(4):448-55.
PMID: 25134488 DOI: 10.1007/s11102-014-0593-6

PURPOSE: Radiation fields for nasopharyngeal cancer (NPC) include the base of skull, which places the hypothalamus and pituitary at risk of damage. We aimed to establish the prevalence, pattern and severity of hypothalamic pituitary (HP) dysfunction amongst NPC survivors.
METHODS: We studied 50 patients (31 males) with mean age 57 ± 12.2 years who had treatment for NPC between 3 and 21 years (median 8 years) without pre-existing HP disorder from other causes. All patients had a baseline cortisol, fT4, TSH, LH, FSH, oestradiol/testosterone, prolactin and renal function. All patients underwent dynamic testing with insulin tolerance test to assess the somatotroph and corticotroph axes. Baseline blood measurements were used to assess thyrotroph, gonadotroph and lactotroph function.
RESULTS: Hypopituitarism was present in 82% of patients, 30% single axis, 28% two axes, 18% three axes and 6% four axes deficiencies. Somatotroph deficiency was most common (78%) while corticotroph, gonadotroph and thyrotroph deficiencies were noted in 40% (4 complete/16 partial), 22 and 4% of the patients respectively. Hyperprolactinaemia was present in 30% of patients. The development of HP dysfunction was significantly associated with the time elapsed from irradiation, OR 2.5 (1.2, 5.3), p = 0.02, for every 2 years post treatment. The use of concurrent chemo-irradiation (CCRT) compared to those who had radiotherapy alone was also significantly associated with HP dysfunction, OR 14.5 (2.4, 87.7), p < 0.01.
CONCLUSION: Despite low awareness and detection rates, HP dysfunction post-NPC irradiation is common. Use of CCRT may augment time related pituitary damage. As these endocrinopathies result in significant morbidity and mortality we recommend periodic assessment of pituitary function amongst NPC survivors.

Matched MeSH terms: Hypopituitarism/blood; Hypopituitarism/epidemiology*