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Rare case with metastatic involvement of hypothalamo-pituitary and pineal body presenting as hypopituitarism and diabetes insipidus.

An unusual case with metastatic spread of a small oat cell lung carcinoma to the hypothalamo-pituitary region and pineal body presenting as pituitary insufficiency is reported. Computed tomographic scan revealed an isodense mass in the hypothalamo-pituitary region and pineal body that was strongly enhanced by contrast medium. Magnetic resonance imaging demonstrated a downward displacement of the posterior lobe of the pituitary by the hypothalamo-pituitary mass. Dynamic assessment of hypothalamo-pituitary function showed hypopituitarism and diabetes insipidus; partial recovery was observed with the improvement in the carcinoma by chemotherapy. The brain was subsequently irradiated. The patient survived 10 months.

Aged↗

Growth and neuroendocrine dysfunction following therapy for childhood cancer.

Poor linear growth and short adult stature are common complications following successful treatment of childhood cancer. Although several factors contribute to the impaired growth of these patients, growth potential is most reduced following radiotherapy to the head or spine. Younger age at treatment and female sex seem to be significant and independent risk factors for short adult height. Early diagnosis and timely therapy of the endocrine sequelae of cancer treatment (i.e., GH deficiency, hypothyroidism, and precocious puberty) ensure that these individuals will reach their optimum growth potential. For patients exposed to high-dose radiotherapy (> 35-40 Gy) to the region of the hypothalamus and pituitary gland, a variety of neuroendocrine abnormalities in addition to GH deficiency and early sexual development may occur, including deficiencies of LH/FSH, TSH, and ACTH as well as hypersecretion of prolactin. Because these problems may develop many years after irradiation, patients at risk for neuroendocrine disturbances require long-term endocrine follow-up.

Adult↗

Long-term effect of 90Y pituitary implantation in acromegaly.

This report examines the long-term trends in GH levels and pituitary function in a group of 38 acromegalic patients who were selected insofar as we were able to follow them up for more than 10 years after a single dose of 90Y interstitial pituitary irradiation as the sole treatment. Mean serum GH had fallen from 106 to 24 mIU/l within 3-6 months and then slowly declined to 4 mIU/l after 10 years. GH levels of less than or equal to 5 mIU/l during a 50 g oral glucose tolerance test were obtained in 8% of patients at 3-6 months and in 18% at 1 year, the cumulative percentage increasing to 53% at 10, and 76% at 14 years. The percentage of patients requiring hormone replacement therapy rose from nil pre-implant to 16% by 3-6 months, and then slowly increased to 39% by 14 years. Serial coned radiographs of the pituitary fossa were available for 32 patients. By 10 years, 16 showed thickening of the dorsum sellae and/or reduction of at least one diameter by 3 mm. Concerning symptoms, all 29 patients whose GH level fell to less than or equal to 5 mIU/l showed improvements, 22 becoming asymptomatic. Seven patients with lesser falls in GH levels (from a mean of 193 to a mean of 15 mIU/l) all improved, one becoming asymptomatic. Two showed no variation. These results show that 90Y pituitary implants have a cumulative effect over the years in inducing remission and hypopituitarism in acromegalic patients, the early decline in GH levels being swifter than from other forms of irradiation.

Acromegaly↗

Amenorrhea, hyperprolactinemia and pituitary enlargement secondary to primary hypothyroidism. Successful treatment with thyroid replacement.

A 22-year-old nulliparous woman presented with primary amenorrhea, primary hypothyroidism, hyperprolactinemia, and radiologic evidence of pituitary enlargement. Investigation demonstrated limited reserves of ACTH and growth hormone. Circulating concentrations of TSH and prolactin were elevated; they increased in response to thyrotropin releasing hormone and decreased following L-dopa administration. After treatment with L-tri-iodothyronine, serum TSH and prolactin levels fell markedly, reserves of growth hormone and ACTH returned to normal, menstrual periods began, and the patient conceived. She experienced an uncomplicated prenatal, intrapartum, and postpartum course. It is thought that this patient represents a distinct clinical entity: a syndrome of amenorrhea, hyperprolactinemia, and pituitary enlargement, all secondary to primary thyroid failure. This syndrome should be distinguished from the Forbes-Albright syndrome, as it is reversible with thyroid replacement therapy. Recognition of this syndrome may thus spare the patient unnecessary, and potentially dangerous, pituitary surgery or irradiation.

Adult↗

[Endocrine function in the late period after radiation therapy of acromegaly].

The results of treatment of pituitary adenomas manifested by the acromegaly syndrome with catamnestic follow-up of up to 10 years are discussed. The dynamics of changes in acromegaly and various endocrine functions with the use of distance gamma therapy and proton beam irradiation were studied in the comparative aspect, on the basis of which the indications for these methods of radiotherapy in acromegaly were specified.

Acromegaly↗

Endocrine and reproductive dysfunction following fractionated total body irradiation in adults.

The endocrine and reproductive sequelae of total body irradiation for haematological malignancy have been studied in 21 patients (11 male) who were treated with 10 Gy in five fractions or 12 or 13.2 Gy in six fractions over 3 days. Eighteen patients (eight male) aged 16-49 years underwent dynamic tests of the hypothalamic-pituitary axis with insulin hypoglycaemia, thyrotrophin releasing hormone (TRH) and gonadotrophin releasing hormone stimulation and basal measurement of prolactin, sex steroids and thyroid hormones. Growth hormone responses (mean peak 64 +/- 36 mU/l, range 21-146 mU/l) and cortisol responses (mean peak 831 +/- 122 nmol/l, range 626-1105 nmol/l) were all within the normal range. Two patients had minimally elevated serum prolactin levels (445 and 588 mU/l, normal less than 350 mU/l). Serum thyroxine levels (57-133 nmol/l) were normal but six patients had elevated basal thyrotrophin (TSH) levels (6-9 mU/l) and seven had an exaggerated TSH response to thyrotrophin releasing hormone, indicating radiation-induced damage to the thyroid. Amenorrhea developed within 3 months of irradiation in all females and oestradiol levels were low, at 37-108 pmol/l (mean 58 +/- 22 pmol/l). Severe oligospermia or azoospermia was noted in men tested 5-70 months after irradiation and testicular volume was below the normal adult range in five of seven men assessed. Serum testosterone levels (12.4-35 nmol/l) were normal. Gonadotrophin-releasing hormone-stimulated gonadotrophin levels were elevated in all patients. However, two men have fathered two children each; one has refused semen analysis, but the other has a sperm count of 7 x 10(6)/ml (60 per cent motile, 20 per cent abnormal forms) 70 months after irradiation. When given by the above fractionated regimens, the endocrine sequelae of total body irradiation are limited to gonadal failure requiring oestrogen replacement in women and severe impairment of fertility in men. Subclinical thyroid dysfunction has been seen in 39 per cent of patients there is no evidence of direct damage to the hypothalamic pituitary axis.

Adolescent↗