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[Clinical thinking and decision making in practice. A patient with postpartum malaise, anorexia, and ventricular tachycardia].

A 34-year-old woman was hospitalised with hypotension, lethargy and ventricular tachycardia, five months after an uneventful pregnancy and delivery. Laboratory values were compatible with anterior (abnormal concentrations of adrenocorticotropin hormone, thyroid stimulating hormone, prolactin) and posterior (diabetes insipidus) pituitary gland insufficiency. An MRI scan revealed that there was no pituitary enlargement and that the pituitary stalk was normal. The prompt recognition of hypophysitis as a potentially fatal condition is important, as a life-saving treatment is available.

Acute Disease↗

[Cushing's disease and corticotroph adenoma: anterior pituitary function before and after trans-sphenoidal microsurgery].

BACKGROUND AND PURPOSE: Cushing's disease is characterized by an overproduction of ACTH inducing an hypersecretion of corticosteroids. We assessed the non-corticotrophic pituitary functions before and after microsurgical resection of the corticotrophic adenoma. METHODS: We reviewed retrospectively a series of 98 patients (19 males, 79 females) undergoing transsphenoidal surgery between 1974 and 2001. Prolactin levels, somatrotrophic, gonadotrophic and thyroidotrophic functions were assessed and compared before and after resection of the adenoma. RESULTS: Before surgery, we observed an elevation of prolactin and a reduction of the GH, TSH, LH and FSH levels. These modifications were not linked to the volume of the adenoma but depended on the systemic effect of the hypercorticism or on local paracrine mechanisms. Selective adenomectomy usually restored altered corticotrophic and also, unlike (partial or total) hypophysectomy, the non-corticotrophic functions. CONCLUSIONS: Non-corticotrophic functions are often altered in Cushing's disease but can be restored after selective adenomectomy.

Adenoma↗

[57-year-old patient with diplopia and headache].

A 57-year old patient presented with an 8 week history of headache and diplopia. Left-sided incomplete oculomotor nerve paresis was found, whereas further clinically relevant visual impairment was not seen. Magnetic resonance imaging of the head showed an extensive intra-, para- and suprasellar tumor which in combination with a markedly elevated serum prolactin level was identified as a macroprolactinoma. The symptoms began 7 years ago with a loss of libido and virility. The application of a dopamine agonist lead to a reduction of serum prolactin level to 0.5% of the initial value and a considerable tumor regression within three months. The development of oculomotor nerve paresis is an unusual manifestation of prolactinoma. By contrast, long-term medical history with slowly developing signs and symptoms of hypogonadotropic hypogonadism are often to be found in men. The delayed diagnosis is responsible for the high percentage of macroprolactinomas in male subjects.

Diagnosis, Differential↗

[Hormonally inactive pituitary adenomas].

BACKGROUND: This paper surveys hormonally inactive pituitary tumours on the basis of the current international literature; it also reflects the experience of the authors. INTERPRETATION: Pituitary tumours are frequently diagnosed and usually show a low potential for growth. Although benign they may invade adjacent structures such as the cavernous and sphenoid sinuses. The diagnosis is usually made on the basis of pituitary failure or visual problems, or incidentally. Tumour debulking or removal, usually by transsphenoidal surgery, is indicated if there is a compression of the optic chiasm or if the tumour shows signs of growth on consecutive MRI scans. Surgery is an effective treatment of these tumours, with little risk of complications. Repeated surgery, either transsphenoidally or by craniotomy, is indicated if chiasmal decompression is not achieved initially. Fractionated single beam irradiation or stereotactic radiosurgery is indicated in cases where there is a residual of growing tumours that may not be removed surgically.

Acromegaly↗

[A case of Wegener's granulomatosis complicated by hypopituitarism].

A 71-year-old male complaining of chest pain was admitted to our hospital. A single cavitary mass shadow was observed on chest X-ray films. Urinalysis revealed microscopic hematuria. CT examination demonstrated a tumorous shadow in the maxillary sinus. The diagnosis of Wegener's granulomatosis was histologically established by biopsy specimens from the nasal mucosa which showed necrotizing vasculitis and granuloma with fibrinoid degeneration. He was treated with combination therapy of prednisolone and cyclophosphamide. The abnormal shadows on chest X-ray and in the maxillary sinus on CT improved rapidly, but the patient developed progressive weight loss and complained of cold intolerance, weakness and dysphagia. Serum T3, T4 and TSH were found to be reduced. Anterior pituitary function tests showed reduction of TSH, GH and ACTH responses, which was probably due to irreversible vasculitis.

Aged↗

Twelve cases of pituitary apoplexy.

In 12 cases of pituitary apoplexy, a preexisting unsuspected adenoma was found. The initial manifestations were sudden onset of headache (12 patients), signs of meningeal irritation (10) with fever (four), altered consciousness (12), and ophthalmologic disturbances (eight). The diagnosis was retrospective in three cases. Radiologic investigations were always suggestive if carefully considered. The plain skull roentgenograms, in particular, showed an enlarged sella turcica in 11 cases. Three patients had prolactin adenomas, and nine had nonfunctional adenomas. Medical treatment was successful in only three patients; surgery was performed in 10 cases by means of a sublabial transseptal microsurgical approach. Postoperative neurologic complications were serious in two cases. Endocrine insufficiencies were common: eight cases of permanent panhypopituitarism, two cases of pluritropic anterior pituitary dysfunction, and three cases of persistent hyperprolactinemia.

Adenoma↗

Endocrine-inactive pituitary adenomas.

I have described some of the currently known characteristics of a group of pituitary adenomas that produce no syndrome of hormone secretion. These tumors, termed endocrine-inactive, are engaged in low levels of endocrine activity and under certain circumstances can secrete hormones, primarily glycoprotein hormones and their subunits. I have advocated a more vigorous attempt to achieve total removal of these usually large tumors because of my conviction that many, although certainly not all, can be removed completely with preservation of anterior pituitary function and, possibly, recovery of lost anterior pituitary function. Transsphenoidal removal of endocrine-inactive tumors is safe as well as effective and, in the foreseeable future, will continue to be the treatment of first choice.

Adenoma↗

[Hypophyseal dysfunction and tumors].

Some pituitary hormones secrete hormones while others do not. Nonsecreting tumors can interfere with normal pituitary hormone secretion and produce tumor symptoms and signs like headaches and visual field defects. The most frequent hormone-secreting tumors are prolactinomas. Growth hormone or ACTH or gonadotropin or gonadotropin-alpha and beta chain-producing tumors are less frequent, TSH producing tumors are extremely rare. The most important elements of the diagnostic work-up are clinical signs and symptoms, assessment of pituitary function (measurement of TSH, free T4, LH, FSH, oestradiol/free testosteron, growth hormone, IGF-1, prolactin, ACTH, Cortisol, serum and urine osmolality), CT and/or MRI and, in patients with large tumors, a visual field exam. The treatment of choice of pituitary tumors is often surgery. Alternative therapies are radiation treatment (in nonoperable patients or when hormone levels are persistently elevated after pituitary surgery) and drug treatment (dopamine agonists in hyperprolactinemia, somatostatin analogues in acromegaly). Pituitary hormone deficiencies are treated depending on the specific deficiency with thyroxine, cortisone, oestrogen/gestagen/testosterone gonadotropines or ADH analogues.

Adenoma↗