Patient Demographics > Country of Treatment > Argentina
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Search for other papers by Teresa M Canteros in
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Search for other papers by Valeria De Miguel in
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Search for other papers by Patricia Fainstein-Day in
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Summary
Severe Cushing syndrome (SCS) is considered an emergency that requires immediate treatment to lower serum cortisol levels. Fluconazole may be considered an alternative treatment in Cushing syndrome when ketoconazole is not tolerated or unavailable. We report a 39-year-old woman with a history of partial pancreaticoduodenectomy due to a periampullary neuroendocrine tumor with locoregional extension. Three years after surgery, she developed liver metastases and was started on 120 mg of lanreotide/month, despite which, liver metastases progressed in the following 6 months. The patient showed extreme fatigue, muscle weakness, delirium, moon face, hirsutism and severe proximal weakness. Laboratory tests showed anemia, hyperglycemia and severe hypokalemia. 24-h urinary free cortisol: 2152 nmol/day (reference range (RR): <276), morning serum cortisol 4883.4 nmol/L (RR: 138–690), ACTH 127.3 pmol/L (RR: 2.2–10). She was diagnosed with ectopic ACTH syndrome (EAS). On admission, she presented with acute upper gastrointestinal tract bleeding and hemodynamic instability. Intravenous fluconazole 400 mg/day was started. After 48 h, her mental state improved and morning cortisol decreased by 25%. The dose was titrated to 600 mg/day which resulted in a 55% decrease in cortisol levels in 1 week, but then had to be decreased to 400 mg/day because transaminase levels increased over 3 times the upper normal level. After 18 days of treatment, hemodynamic stability, lower cortisol levels and better overall clinical status enabled successful bilateral adrenalectomy. This case report shows that intravenous fluconazole effectively decreased cortisol levels in SCS due to EAS.
Learning points:
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Severe Cushing syndrome can be effectively treated with fluconazole to achieve a significant improvement of hypercortisolism prior to bilateral adrenalectomy.
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Intravenous fluconazole is an alternative treatment when ketoconazole is not tolerated and etomidate is not available.
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Fluconazole is well tolerated with mild side effects. Hepatotoxicity is usually mild and resolves after drug discontinuation.
Search for other papers by Valeria de Miguel in
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Search for other papers by Andrea Paissan in
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Search for other papers by Patricio García Marchiñena in
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Search for other papers by Alberto Jurado in
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Search for other papers by Mariana Isola in
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Search for other papers by José Alfie in
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Search for other papers by Patricia Fainstein-Day in
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Summary
We present the case of a 25-year-old male with a history of neurofibromatosis type 1 and bilateral pheochromocytoma 4 years after kidney transplantation that was successfully treated with simultaneous bilateral posterior retroperitoneoscopic adrenalectomy.
Learning points:
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Hypertensive patients with NF1 should always be screened for pheochromocytoma.
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Pheochromocytoma is rarely associated with transplantation, but it must be ruled out in patients with genetic susceptibility.
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Posterior retroperitoneoscopic adrenalectomy (PRA) allows more direct access to the adrenal glands, especially in patients with previous abdominal surgeries.
Fundacion de Endocrinologia
Search for other papers by Oscar D Bruno in
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Search for other papers by Ricardo Fernández Pisani in
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Search for other papers by Gabriel Isaac in
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Search for other papers by Armando Basso in
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Summary
The role of mechanical forces influencing the growth of a pituitary adenoma is poorly understood. In this paper we report the case of a young man with hyperprolactinaemia and an empty sella secondary to hydrocephalia, who developed a macroprolactinoma following the relief of high intraventricular pressure.
Learning points:
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The volume of a pituitary tumour may be influenced not only by molecular but also by local mechanical factors.
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Intratumoural pressure, resistance of the sellar diaphragm and intracranial liquid pressure may play a role in the final size of a pituitary adenoma.
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The presence of hydrocephalus may hide a pituitary macroadenoma.
Search for other papers by Soledad Bell in
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Search for other papers by Gabriela Alejandra Sosa in
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Search for other papers by Ana del Valle Jaen in
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Search for other papers by María Fabiana Russo Picasso in
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Summary
Thyroid lipomatosis is a rare disease, as a total of 20 cases have been described in the literature. It is characterized by diffuse infiltration of the stroma by mature adipose tissue and by progressive growth that produces different degrees of compressive symptoms. Our aim is to present the case of a 36-year-old woman who consulted because of dyspnea caused by a multinodular goiter. She underwent surgery with the presumptive diagnosis of a malignant neoplasia, but the pathological examination of the surgical specimen established the diagnosis of thyroid lipomatosis.
Learning points
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Thyroid lipomatosis is a rare, benign disease characterized by diffuse infiltration of the stroma by mature adipose tissue.
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The pathophysiology of diffuse proliferation of adipose tissue in the thyroid gland is unclear.
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Thyroid lipomatosis is clinically manifested by a progressive enlargement of the thyroid that can involve the airway and/or upper gastrointestinal tract, producing dyspnea, dysphagia, and changes in the voice.
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Given the rapid growth of the lesion, the two main differential diagnoses are anaplastic carcinoma and thyroid lymphoma.
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Imaging studies may suggest a differential diagnosis, but a definitive diagnosis generally requires histopathological confirmation after a thyroidectomy.
Search for other papers by Gabriela Alejandra Sosa in
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Search for other papers by Soledad Bell in
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Search for other papers by Silvia Beatriz Christiansen in
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Search for other papers by Mariela Glerean in
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Search for other papers by Soledad Lovazzano in
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Search for other papers by Pablo Ajler in
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Search for other papers by Patricia Fainstein Day in
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Summary
IgG4-related hypophysitis is a recently described entity belonging to the group of IgG4-related diseases. Many other organs can also be affected, and it is more common in older men. To date, 32 cases of IgG4-related hypophysitis have been reported in the literature, 11 of which included confirmatory tissue biopsy and the majority affecting multiple organs. The aim of this report is to present two cases of biopsy-proven IgG4-related hypophysitis occurring in two young female patients with no evidence of involvement of other organs at the time of diagnosis.
Learning points
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IgG4-related hypophysitis belongs to the group of IgG4-related diseases, and is a fibro-inflammatory condition characterized by dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells and storiform fibrosis.
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It is more common in older men, but young women may also present this type of hypophysitis.
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Although involvement of other organs is frequent, isolated pituitary disease is possible.
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Frequent clinical manifestations include anterior hypopituitarism and/or diabetes insipidus.
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The diagnosis may be confirmed with any of the following criteria: a pituitary biopsy with lymphoplasmacytic infiltrates, with more than ten IgG4-positive cells; a sellar mass and/or thickened pituitary stalk and a biopsy-proven involvement of another organ; a sellar mass and/or thickened pituitary stalk and IgG4 serum levels >140 mg/dl and sellar mass reduction and symptom improvement after corticosteroid treatment.
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Glucocorticoids are recommended as first-line therapy.
