Department of Clinical Science and K.G. Jebsen-Center for Autoimmune Diseases, University of Bergen, Bergen, Norway
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Department of Clinical Science and K.G. Jebsen-Center for Autoimmune Diseases, University of Bergen, Bergen, Norway
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Department of Clinical Science and K.G. Jebsen-Center for Autoimmune Diseases, University of Bergen, Bergen, Norway
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Summary
Feminizing estrogen-secreting adrenocortical carcinomas (ACCs) are exceedingly rare and carry a poor prognosis. The most common presenting trait is gynecomastia, but enlarged breasts are also a frequent clinical finding in healthy men. Biochemical evaluation may be challenging. As such, there is a high risk of delayed diagnosis and treatment opportunity. Here, we present a case with an estrogen-producing ACC where the abnormal steroid profile obtained at the time of initial workup was essential for the prompt diagnosis. Wider adoption of liquid chromatography mass spectrometry-based steroid assays has potential to improve early diagnosis of feminizing estrogen-secreting ACC.
Learning points
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Feminizing estrogen-secreting adrenocortical carcinomas (ACCs) are a rare, but an important differential diagnosis in men with rapidly developing gynecomastia.
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Biochemical evaluation is essential for a prompt diagnosis.
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Steroid hormone profiling using liquid chromatography mass spectrometry technology has the potential to improve early diagnosis of feminizing estrogen-secreting ACC.
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Department of Clinical Science, University of Bergen, Bergen, Norway
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Department of Clinical Science, University of Bergen, Bergen, Norway
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Summary
Myxedema coma is an important differential diagnosis in critically ill patients. Early diagnosis and treatment are paramount but challenging due to a lack of diagnostic criteria. We report a case about a patient who suffered from untreated hypothyroidism for several years. Before the correct diagnosis was made, he was admitted three times due to severe constipation. Eventually, he developed myxedema coma in connection with a urinary tract infection. The course was complicated by recurrent seizures, and neuroimaging showed bilateral hygromas. Hormone replacement therapy resulted in complete recovery and regression of hygromas. To the best of our knowledge, this is the first time hygroma is reported in association with myxedema coma.
Learning points
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Myxedema coma is a difficult diagnosis to make due to a lack of diagnostic criteria.
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Cardinal features include hypothermia, bradycardia, gastrointestinal symptoms, pericardial/pleural effusions and affection of CNS. Anemia and hyponatremia are common.
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In case of suspected myxedema coma, neuroimaging should be a part of the evaluation in most cases.
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There is a possible association between longstanding/severe hypothyroidism and hygroma.
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Department of Medicine, Haukeland University Hospital, Bergen, Norway
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Department of Medicine, Haukeland University Hospital, Bergen, Norway
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Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway
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Summary
Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency. More than 90% is of B-cell origin. The condition is bilateral in up to 75% of cases, with adrenal insufficiency in two of three patients. We report two cases of adrenal insufficiency presenting at the age of 70 and 79 years, respectively. Both patients had negative 21-hydroxylase antibodies with bilateral adrenal lesions on CT. Biopsy showed B-cell lymphoma. One of the patients experienced intermittent disease regression on replacement dosage of glucocorticoids.
Learning points:
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Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency.
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Bilateral adrenal masses of unknown origin or in individuals with suspected extra-adrenal malignancy should be biopsied quickly when pheochromocytoma is excluded biochemically.
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Steroid treatment before biopsy may affect diagnosis.
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Adrenal insufficiency with negative 21-hydroxylase antibodies should be evaluated radiologically.
