A remarkable case of thyrotoxicosis initially caused by graves' disease followed by a probable TSHoma - a case report.

BACKGROUND: Graves' disease is the commonest cause of thyrotoxicosis whilst thyrotropin (TSH)-producing pituitary adenomas (thyrotropinomas, TSHomas) are very rare and account for just 1-2% of all pituitary adenomas. Coexistence of a TSHoma and Graves' disease has been very rarely reported. Here, we report a case of a patient whose initial presentation with primary thyrotoxicosis due to Graves' disease, was subsequently followed by a relapse of thyrotoxicosis due to a probable TSHoma. CASE: A sixty-eight year old woman was referred to our department with classical features of thyrotoxicosis. Initial biochemistry confirmed hyperthyroxinaemia [free thyroxine (fT4) 20.4 pmol/L (reference range 7.0-16.0)] and a suppressed TSH [< 0.02mIU/L (0.50-4.20)]. A technetium pertechnetate uptake scan was consistent with Graves' Disease. She was treated with carbimazole for 18 months and remained clinically and biochemically euthyroid. After stopping carbimazole her fT4 started to rise but TSH remained normal. Laboratory assay interference was excluded. A TRH stimulation test demonstrated a flat TSH response and pituitary MRI revealed a microadenoma. Remaining pituitary hormones were in the normal range other than a slightly raised IGF-1. An 11C-methionine PET/CT scan coregistered with volumetric MRI (Met-PET-MRICR) demonstrated high tracer uptake in the left lateral sella region suggestive of a functioning adenoma. The patient declined surgery and was unable to tolerate cabergoline or octreotide. Thereafter, she has elected to pursue a conservative approach with periodic surveillance. CONCLUSION: This is a very unusual case of thyrotoxicosis caused by two different processes occurring in the same patient. It highlights the importance of considering dual pathology when previously concordant thyroid function tests become discordant. It also highlights a potential role of Met-PET-MRICR in the localisation of functioning pituitary tumours.

[Therapeutic possibilities in patients with selective pituitary resistance to thyroid hormones]. / Posibilidades terapéuticas en la resistencia hipofisaria selectiva a las hormonas tiroideas.

Selective pituitary resistance to thyroid hormones (SPRTH) is a non-neoplastic form of inappropriate secretion of thyrotropin (TSH). The etiology of this hormonal resistance is linked to inactivating mutations in the thyroid hormone receptor beta (TR-beta) gene. These mutations affect critical portions of the receptor's triiodothyronine (T3)-binding domain. Clinically, SPRTH is characterized by hyperthyroidism with goiter and absence of pituitary mass in the morphologic study. Laboratory data show an elevation of free T3 and free thyroxine concentrations without suppression of TSH, with normal molar subunit alpha/TSH ratio. At this time, there is no specific therapy for SPRHT. Beta blockers, such as atenolol, and benzodiazepines have been used as a symptomatic therapy. Among the drugs with the capacity for reducing TSH secretion are TR agonists, such as triiodothyroacetic acid, D-thyroxine, triiodothyropropionic acid, and L-T3.

[Neuroendocrine dysfunction in female patients with systemic dysplasia of the connective tissue]. / Neiroéndokrinnaia difunktsiia u zhenshchin s sistemnoi displaziei soedinitel'noi tkani.

The study covered 119 women with systemic undifferentiated dysplasia of the connective tissue (DCT) aged 14-39 years. All the examinees had vegetative disorders, 26.1% had a neuroendocrine-metabolic form of hypothalamic syndrome (HS), 51.3%--premenopausal syndrome, 65.5%--dysmenorrhea. It was found that women with DCT and HS develop symptoms of neuroendocrine disorders at earlier age than those with HS but free of DCT. Arterial hypertension was registered in 96.8% of patients with DCT and HS, 80.3% of DCT patients with premenopausal syndrome. Central hemodynamics was different in DCT patients without neuroendocrine disorders and with neuroendocrine dysfunction evidencing the role of the latter in development of arterial hypertension in DCT.

Laparoscopic adrenalectomy for adrenocorticotropin-dependent Cushing's syndrome.

Bilateral adrenalectomy is indicated for the treatment of ACTH-dependent Cushing's syndrome when the tumorous source of ACTH hypersecretion cannot be identified or removed. Potential advantages of laparoscopic over open adrenalectomy include shorter hospitalization, decreased requirement for postoperative analgesia, and decreased postoperative morbidity due to incisional complications. Bilateral laparoscopic adrenalectomy performed for the treatment of ACTH-dependent Cushing's syndrome was attempted in 19 patients at our institution between 1995 and 1998. Conversion to an open procedure was required in three patients. All patients who underwent bilateral laparoscopic adrenalectomy were subsequently followed to assess the outcome of this intervention. Twelve patients with pituitary-dependent Cushing's syndrome and four with ectopic ACTH syndrome underwent successful bilateral laparoscopic adrenalectomy. All patients experienced resolution of the signs and symptoms (e.g. proximal myopathy, hirsutism, and emotional lability) of Cushing's syndrome as well as weight loss, improved glucose tolerance, and improved control of blood pressure. No residual cortisol secretion was detected in the patients. Bilateral laparoscopic adrenalectomy is a safe and effective treatment for Cushing's syndrome when the ACTH-secreting neoplasm cannot be removed.

Primary empty sella: differences and similarities between children and adults.

To identify possible differences between empty sella in children and adults we studied 43 subjects (age 13.6 +/- 5.4 years, range 4.1-27 years) with hypothalamic-pituitary disorders and empty sella at magnetic resonance imaging. Pituitary function, presence of non-endocrine symptoms, perinatal history, sellar volume, pituitary height, midline or intrasellar anatomical abnormalities were evaluated. Twenty subjects had isolated growth hormone deficiency, 17 multiple pituitary hormone deficiency and 6 puberty disorders (3 precocious puberty, 2 idiopathic delayed puberty, 1 Kallmann syndrome). The group with multiple pituitary hormone deficiency had a higher percentage of subjects with complete empty sella, i.e. pituitary height < 2 mm (p = 0.016), or intrasellar anatomical abnormalities (p = 0.0002) than the other groups. The subjects with puberty disorders had a mean sellar volume higher than the other groups (p < 0.05). Apart from pituitary dysfunction, symptoms of the empty sella syndrome were infrequent (9.3% of cases) in our subjects. The age of our subjects, the frequent association between empty sella and pituitary dwarfism and the non-enlarged sellae suggest a different aetiology, perhaps congenital, for empty sella in our subjects. As in adults, empty sella may be associated with both pituitary hypo- and hyperfunction.

[The role of endoscopic methods in the diagnosis of endocrine infertility in women with persistent anovulation]. / Ro'l éndoskopicheskikh metodov v diagnostike éndokrinnogo besplodiia u zhenshchin so stoikoi anovuliatsiei.

The incidence of stubborn anovulation in patients ineffectively treated with hormones has made up. 22.7%. Laparoscopy and biopsy of the ovaries followed by histologic and morphometric examinations of ovarian tissue have detected three characteristic types of the ovaries in the cases with stubborn anovulation in the presence of LH and FSH dissociation and elevated gonadotropin content. Laparoscopic biopsy of the ovaries in not only a diagnostic, but a therapeutic method as well.

Articular manifestations of acromegaly.

Forty-five patients with acromegaly or gigantism were reviewed for musculoskeletal abnormalities. Abnormalities of peripheral joints occurred in 74% of the patients and spinal involvement in 47%, leading to significant morbidity. Joint abnormalities most frequently affected the large joints (hips, knees and shoulders) but the wrist and hand were also involved. The radiological features of acromegalic arthropathy are described, including vertical widening of the hip joint, enthesopathy and osteophytosis. A favourable response to treatment is associated with a less severe arthropathy and a good functional outcome.

[Surgery of hypophyseal tumors by the trans=sphenoid approach. apropos of 164 cases]. / La chirurgie des tumeurs hypophysaires par voie trans-sphénoïdale. A propos de 164 observations

PIP: This review touches on differential diagnosis of pituitary tumors, their histologic types, prognosis, X-ray appearance, the technique of transsphenoid ablation of pituitary tumors, indications of the transsphenoid vs. the upper approach and the results of 164 cases operated on since 1966. Diagnosis relies clinically on evidence of panhypopituitarism or hypersecretion of growth hormone of ACTH and ophthalmologic or neurologic signs. In their histologic cell types, somatotrophs correspond to eosinophils, nonfunctional adenomas to chromophobes, and hypercorticism to basophils. Various types of tumors can be distinguished by tomography, gas pneumoencephalography and carotid angiography. The technical details of transsphenoid surgery under a microscope of 350 mm focal length and televised gas encephalography are described. Macroscopically, the tumors may be invasive adenomas, soft friable, or cystic. Possible immediate complications, besides bleeding, may be collapse of the roof of the sella, or rupture of the sellar diaphragm causing leakage of cephalorachidian fluid. the transsphenoid route is indicated in 60% of cases with rounded tumors, in tumors evolving downward into the floor of the sella, in patients over 65 or blind because of optic nerve compression. The upper route is advised in tumors growing upward, restrained by the optic nerve or in patients with anatomic defects preventing the lower approach. In 164 operations the authors had 5 deaths, 6 fistulas of cephalorachidian fluid, 2 patients temporarily blinded, 2 with loss of visual acuity, and only 4 relapses.^ieng