Hypophysial tuberculoma with hypopituitarism

In this patient with a caseating pituitary tuberculoma the diagnosis of hypopituitarism was established antemortem by modern tests of pituitary function. Pituitary tuberculoma is rare and may resemble pituitary adenoma. Tuberculoma should be considered in patients with hypopituitarism or enlargement of the sella turcica, or both, who have or have had tuberculosis of any site. Antituberculous therapy should be instituted in such patients, especially when surgery is contemplated.     

Hyperlipidaemia in patients with hypopituitarism

Five patients with hypopituitarism due to Sheehan's syndrome showed hyperlipidaemia of various lipoprotein phenotypes. Postheparin plasma lipoprotein lipase activity was subnormal in 4 of the 5 patients and hepatic triglyceride lipase was markedly decreased in all patients studied. After supplementation of both corticosteroid and thyroid hormones, lipoprotein lipase activity was restored to normal within 2 months, while it took longer for hepatic triglyceride lipase to return to normal. Together with the normalization of the two lipase activities, hyperlipidaemia subsided. The findings suggest that reduced activities of the two lipases may, at least in part, account for the development of hyperlipidaemia in hypopituitarism. The study identifies a new group of patients with hyperlipidaemia secondary to a disorder in endocrine function.     

Chondroid chordoma presenting with hypopituitarism.

A 28-year-old man with chondroid chordoma, an uncommon variant of chordoma, is reported. The patient presented with visual disturbance and hypopituitarism. The latter is a rare complication of intracranial chordoma. The preoperative diagnosis of chondroid chordoma of the skull base was based on unique findings on computed tomography and magnetic resonance imaging studies. The development of the tumor over six years suggests that the prognosis of chondroid chordoma may be poor in younger patients, as recently reported. Although chondroid chordoma is very rare, it should be included in the differential diagnosis of hypopituitarism.     

Fertility outcomes in women with hypopituitarism

BACKGROUND: Women with hypopituitarism are known to have a poor outcome once pregnancy has been achieved by ovulation induction. There are no data, however, recording the efficacy of ovulation induction and pregnancy rates in this group of subfertile women. METHODS: The outcome of fertility treatments in all 19 women with hypopituitarism attending the fertility clinics of University College London Hospitals over the past 20 years was audited. RESULTS: Ovulation was achieved in almost all women (95%) but occurred in only 60% of treatment cycles. Pregnancy was achieved in 47% of women or 11% of cycles resulting in a live birth rate of 6.7% per cycle. Seven of the 18 pregnancies (39%) miscarried. Only 42% of women treated achieved a live birth. CONCLUSION: Ovulation induction in women with hypopituitarism yields relatively low pregnancy rates in comparison to other causes of anovulation and a high miscarriage rate. Pituitary hormone deficiency beyond gonadotrophins has a major adverse effect on achieving pregnancy.     

Pediatric nonalcoholic steatohepatitis associated with hypopituitarism

We experienced two cases of pediatric nonalcoholic steatohepatitis (NASH) associated with hypopituitarism. The first patient was diagnosed with a craniopharyngioma at 5 years of age. After an operation to treat the condition, the patient gradually became obese, and an elevation of transaminases was observed. At 16 years of age, the patient was diagnosed as having NASH with liver cirrhosis. He was started on hormone replacement therapy; however, his insulin resistance and liver fibrosis, as evaluated by hyaluronic acid and platelet count, progressed. In addition, his hyperleptinemia continued. The second patient was diagnosed, at 10 years of age, as having pituitary dysfunction due to fetal asphyxia, and he was started on hormone replacement therapy. This patient was noted to have been obese throughout his life. He was diagnosed as having NASH with advanced fibrosis at 18 years of age. It is important for both hepatologists and endocrinologists to be aware of the association between pituitary dysfunction and NASH.     

Androgen deficiency in women with hypopituitarism

Androgen deficiency in men is associated with severe osteopenia, alterations in body composition including an increase in fat mass, and decreased libido. Little is known about the pathophysiology, metabolic consequences, or gender-specific effects of androgen deficiency in women. Acquired hypopituitarism in women is characterized by central hypogonadism and/or hypoadrenalism and therefore may affect critical sources of androgen production in women. We hypothesized that serum androgen levels would be decreased in women with hypopituitarism. We therefore determined serum androgen levels in 55 women with hypopituitarism and 92 controls. This included 4 subsets of hypopituitary women: 1) women less than 50 yr old not receiving estrogen, 2) women less than 50 yr old receiving estrogen, 3) women more than 50 yr old not receiving estrogen, and 4) women more than 50 yr old receiving estrogen. Premenopausal controls with regular menstrual cycles were studied in the early follicular phase, midcycle, and luteal phase during one cycle. All other subjects were studied 3 times during the month at comparable intervals to mimic these 3 time points of the normal menstrual cycle. Serum testosterone, free testosterone, androstenedione, and dehydroepiandrosterone sulfate levels were markedly reduced in all 4 groups of women with hypopituitarism compared with controls (P < 0.0001). Moreover, serum testosterone, free testosterone, and androstenedione levels were lower in women with central hypoadrenalism and hypogonadism than in subjects with hypoadrenalism or hypogonadism alone (P < 0.025). Mean DHEAS levels were decreased in hypopituitary women with both hypogonadism and hypoadrenalism compared with those in women with hypogonadism alone (P < 0.0001). These data demonstrate hypoandrogenemia in women with hypopituitarism. The physiological consequences of low androgen levels in this population remain to be determined.     

An Unusual Case of Local Gigantism

A 38-year-old female with an unusual degree of gigantism of the left leg is described. There was no other known association. Angiogram did not reveal any abnormality. The patient refused surgical intervention.     

Relapsing Whipple's disease presenting with hypopituitarism.

A 44-year-old man with a history of Whipple's disease 8 years ago presented with recurrent grand mal seizures and signs of hypopituitarism on physical examination. Magnetic resonance imaging of the brain revealed a hypothalamic lesion of 1 cm diameter in the region of the rostral infundibulum. Hypopituitarism was confirmed by low levels of serum cortisol, free testosterone and free thyroxine without an elevated TSH. Whipple encephalitis with hypothalamic involvement was suggested and verified by positive polymerase chain reaction (PCR) for Tropheryma whippelii in the cerebrospinal fluid. PCR for T. whippelii has become an important diagnostic tool for establishing the diagnosis of Whipple's disease especially in patients with unusual presentations and if the diagnosis cannot be confirmed histologically. Whipple's disease should be included in the differential diagnosis in hypopituitarism caused by infectious disease.     

Increased cerebrovascular mortality in patients with hypopituitarism

OBJECTIVE  An increased prevalence of atherosclerosis has been shown among patients with hypopituitarism. The aim of the present study was to assess whether patients with hypopituitarism experience increased cardiovascular, in particular cerebrovascular, mortality.
DESIGN AND PATIENTS  Retrospective cohort study of mortality, 1952–1992, in 344 patients, of whom 130 were female, receiving conventional hormone replacement for hypopituitarism following neurosurgery for pituitary tumours. The general population in the catchment area of southern Sweden from which the patients were recruited constituted the reference population. Expected mortality was obtained from cause, sex, calendar year, and 5-year age-specific death rates for the area.
RESULTS  Increased mortality from cerebrovascular disease (standardized mortality ratio (SMR) 3.39; 95% CI 2.27–4.99) was the main contributor to the increased overall cardiovascular mortality (SMR 1.75; 95% CI 1.40–2.19). The increase in mortality from cardiac diseases was much smaller (SMR 1.41; 95% CI 1.04–1.88). The risk for cerebrovascular death was higher in women (SMR 4.91) than in men (SMR 2.64). The relative risk for cerebrovascular death was independent of the time interval since diagnosis of pituitary insufficiency, but was greater in subjects diagnosed at an earlier age (<55 years). No increased mortality in malignant tumours was observed (SMR 0.95; 95% CI 0.60–1.48).
CONCLUSION  The increased cerebrovascular mortality may be due to GH deficiency, or to long-term lack or inadequacy of substitution for other pituitary hormones. The observations that an early onset of pituitary insufficiency and female sex are predictors for a high risk for cerebrovascular mortality merit particular attention when treating this group of patients.     

Granulomatous hypophysitis with meningitis and hypopituitarism.

We report an unusual case of granulomatous hypophysitis in which visual impairment, meningitis and hypopituitarism in a 76-year-old female were associated with radiological evidence of a pituitary mass. The sellar lesion was indistinguishable from pituitary tumor on neuroimaging studies, but the recovery of visual acuity and visual field abnormalities together with the improvement of pituitary function after steroid administration indicated that the mass lesion was due to an inflammatory disease of the pituitary gland. The pituitary tissue obtained by transsphenoidal hypophysectomy revealed granulomatous inflammatory cell infiltration with epithelioid cells and scattered multinucleated giant cells. Although a causal relationship with meningitis was not ascertained, possible exposure of the CSF space to the autoimmune inflammatory process of the pituitary gland was likely in view of the positive pituitary antibody reaction and radiological evidence of suprasellar extension. This entity should be considered when evaluating patients with a pituitary mass, hypopituitarism and meningitis.     

Gigantism due to growth hormone excess in a boy with optic glioma

True gigantism is rare in early childhood and is usually due to excess GH secretion from a pituitary adenoma. We report a case in which the endocrine abnormality is secondary to an optic glioma. Careful endocrine evaluation has shown that GH peak amplitude was not increased but rather there was failure of GH levels to suppress to baseline and a lack of pulsatility. There is no evidence of a direct secretory role for the tumour and we postulate that the tumour is affecting GH secretion through an effect on somatostatin tone. Specific tumour therapy is not indicated for this patient in the absence of mass effect or visual disturbance. The GH excess is being treated with somatostatin analogue (Octreotide) and as he has developed precocious puberty he is also receiving long acting GnRH analogue (Zoladex). This boy appears likely to have neurofibromatosis type 1 (NF1) which raises the question of subtle GH excess in NF1 patients with tall stature.     

Coronary artery bypass grafting in patients with hypopituitarism

Three patients with angina pectoris and hypopituitarism underwent coronary artery bypass grafting. The patients received perioperative replacement steroid and thyroid hormone therapy, and there were no complications. Careful perioperative hormonal management is necessary for patients with hypopituitarism.