Endocrine Abstracts

Introduction: Hyperkalemia due to zona glomerulosa (ZG) insufficiency is generally transient and mild for patients with aldosterone-producing adenoma (APA) after adrenalectomy. We report here a case with prolonged ZG insuffiency requiring long-term mineralocorticoid replacement (MR) therapy.Case report: A 45-years-old female with hypertension and hypokalemia admitted to outpatient clinic with incidentally detected right adrenal adenoma (3 cm) showing sig...

Introduction: Ganglioneuroma is an extremely rarely seen tumour in the adrenal gland, which originates from neural crest cells. Clinically, they generally have a silent course and in laboratory tests, the hormone levels expressed from the adrenal gland are within the normal range.Case: A 46-year old male patient, for whom a mass in the adrenal gland was diagnosed incidentally on abdominal ultrasonography and MRI. The physical examination, laboratory test...

Primary adrenal insufficiency (PAI) is uncommon in pregnancy. Women with PAI have reduced fertility and parity. If unrecognized or untreated during pregnancy PAI increases risk for maternal and foetal morbiditiy and mortality. Majority of women diagnosed before conception and appropriately treated, have uneventful pregnancies, with increased risk of cesarean section (CS) and preterm delivery.We report a 38-year-old woman with PAI during first spontaneus ...

Introduction: CCHD comprises a number of different congenital heart defects associated with elevated pulmonary artery pressure and pulmonary vascular resistance, resulting in a reversed or bidirectional shunt (Eisenmenger syndrome). These entities develop systemic hypoxia. Pheochromocytoma and paraganglioma (PHEO/PG) are neuroendocrine tumours. Several inheritance genetic alterations have been reported in PHEO/PG syndromes. A pathogenic association between these entities is pr...

Von Hippel-Lindau (VHL) disease is an inherited, autosomal dominant syndrome manifested by a variety of benign and malignant tumors. Herein we describe, two families who had VHL. A 21-year-old man (index case 1) presented with bilateral adrenal mass, serendipitously discovered by radiologic examination. He had newly diagnosed arterial hypertension. His family history was significant for his mother who had operated for pheochromocytoma, his two uncles who had experienced cerebr...

Adrenal incidentalomas (prevalence between 0.4 and 7%) have become a very common clinical problem, the major concern being the risk of malignancy and hormone overproduction.A 57-year-old woman was admitted for asthenia and uncontrolled hypertension. The only relevant clinical signs were abdominal obesity (BMI=31.1 kg/m2) and hypertension. Abdominal ultrasonography showed a voluminous left adrenal solid mass of 10/9 cm, with well-defined sharp ...

Adrenal Lymphoma is extremely rare with fewer than 200 cases reported. The most common subtype is the diffuse large B cell lymphoma. Malignant lymphoma arising in the endocrine glands represents only 3% of extranodal malignant lymphomas and is usually confined to the thyroid gland. NK/T-cell lymphoma presents primarily in the nasopharynx and sinuses. Although involvement of the adrenal glands can be seen in disseminated disease, primary presentation with adrenal masses and adr...

Introduction: 30% of the adult population has high blood pressure. About 6% of the hypertension may be caused by primary aldosteronism and very rare by primary hyperparathyroidism. The combination of these two causes is very rare.Case report: Our patient is women (age 50) with a 21 year long history of uncontrolled hypertension. Treatment of hypertension consist of a beta-blocker, calcium channel blockers, angiotensin converting enzyme inhibitor, loop di...

Introduction: Menopausal adrenal tumours may be elements of a complex panel of co-morbidities. Some of these are represented by the presence of a second pathology requiring surgery at the kidney level.Aim: We aim to introduce a series of two cases involving menopausal women who were referred for specific endocrine assays after they had a unilateral nephrectomy (UN).Method: This is a cases series observational study. The patients ga...

Adrenal metastasis is common (35%) among the patients with lung cancer, while less than 3% of the patients develop bilateral adrenal metastasis. Adrenal metastases are generally small and clinically asymptomatic. Adrenal insufficiency is rare despite the presence of adrenal metastasis. Lam et al. reported this ratio as 0.7%. We aimed to present a case with lung cancer and bilateral adrenal metastasis who developed adrenal insufficiency.A 63-year old male...

Background: Bilateral adrenal haemorrhage (Waterhouse–Friderichsen syndrome) is a rare consequence of sepsis (usually a result of meningococcal infection), with an estimated 15% mortality. Despite the predominant association with meningococcal infection, there are other recognised aetiologies: sepsis resulting from other organisms, and non-infectious causes, such as anticoagulant treatment, trauma and postoperative adrenal haemorrhage.Case report: A...

Introduction: Dramatically with increased use of computed tomography (CT) and magnetic resonance imaging (MRI), more and more uncommon adrenal masses have been detected at abdominal examinations. Adrenal cavernous hemangiomas (ACH) are rare vascular tumor. The first case report was published in 195 by Johnson and Jeppesen. Approximately less than 70 cases were reported in the literature to date.ACH are nonfunctioning benign tumors that is often discovere...

Adrenal lymphangioma is a rare benign tumors of the adrenal gland. Because of the increased use of imaging methods in recent times, the diagnosis is usually made incidentally. It is usually due to malformation between the lymphatic and the venous system. Those with small indiameters are generally asymptomatic and can be evacuated by needlea spiration. However, surgery is recommended for cysts over 6 cm in diameter due to risk of infection, bleeding, compression of neighboring ...

Introduction: MEN4 syndrome is a recently described form of MEN in patients with parathyroid and anterior pituitary tumors, which may also develop bronchial, gastric and pancreatic neuroendocrine tumors. In general, the patients present with clinical signs of primary hyperparathyroidism and simptoms caused by pituitary hormones hypersecretion or due to the tumor mass. However, clinical cases with the coexistence of pituitary tumors and pheochromocytoma are very rare described....

Phaeochromocytoma and paragangliomas (PH/PG) are rare neuroendocrine tumours. Prediction of aggressive tumour behaviour remains a major challenge. The Phaeochromocytoma of the Adrenal gland Scaled Score (PASS) is used to separate benign from malignant lesions with a score > 4 showing potential for biologically aggressive behaviour. Pre-operatively, MIBG together with CT/MRI remain the diagnostic radiological gold standard.We report a 68-year-old fema...

A 24 year old man was admitted by the Surgeons with left sided abdominal pain. He was febrile and tachycardic and found to have raised inflammatory markers. Abdominal CT scan revealed a 6 cm left adrenal haemorrhage with no evidence of an underlying lesion. Initial endocrine investigations were unremarkable for Cushings, Conn’s and adrenal insufficiency. Norepinephrine levels were high but settled subsequently. He had a coagulopathy and evidence of reactivation of EB viru...

Introduction: Incidentally discovered adrenal masses (incidentalomas) are common and in some cases differential diagnosis could be a challenge.Case report: A 55-years old woman presented with an incidental left adrenal mass of 36mm discovered on an abdominal ultrasound. As sole background she underwent resective surgery of an ovarian serous cyst. She denied symptoms like flushing, headache, diaphoresis or palpitations. Physical examination was irrelevant...

Aldosterone-secreting adrenocortical carcinoma (ACC) is rare neoplasm, which detected in 2.5% of active ACCs.Clinical case: A 58 years old female, was admitted to our clinic with complaints of high blood pressure, back pain, weakness, convulsions. Biochemical evaluation confirmed primary hyperaldosteronism (serum aldosterone 1012 pg\ml, serum renin concentration 0.5 mkME\ml, aldosterone-renin ratio 202.4, hypokalemia 1.2 mmol\l). There was no evidence of...

Introduction: A rare cause of adrenal incidentaloma is congenital adrenal hyperplasia (CAH). Nonclassic CAH is one of the most frequent autosomal recessive disorders. Most cases of nonclassic CAH are never diagnosed due to very mild symptoms.Case report: A 62-year-old woman admitted at our Department for right adrenal incidentaloma sized 39×34×38 mm confirmed by MRI. She was asymptomatic. Her past medical history included stabile hypertension, ...

Secondary endocrine hypertension affects around 10% of all hypertensive population, most frequently primary aldosteronism (PA). Less frequent forms of adrenal hypertension include pheochromocytomas and other causes of mineralocorticoid excess than PA, featuring suppressed renin without excess of aldosterone. Featuring. We present a 29-year-old woman with difficult to control hypertension diagnosed two years before and poor response to ACEI and ARA II treatment. She was referre...

Introduction: Adrenocortical carcinoma (ACC) is a rare neoplasm with a variable but overall poor prognosis. The presentation is heterogeneous usually with mass effect and less often with evidence of hormonal secretion. About half of cases the tumors are hormonally active most commonly with cortisol secretion followed by sexual hormones production, being rare the aldosterone secretion.Case report: A 41-year-old male presented with hypertensive crisis and ...

A 30-year-old male presented to emergency with history of palpitations for 6 months. At birth he was diagnosed with salt-losing congenital adrenal hyperplasia (female pseudohermaphrodite) and commenced on steroid therapy. He also underwent corrective surgery. He needed both fludrocortisone and hydrocortisone. At age 17, he visited urology because of bilateral testicular enlargement. On examination he had normal secondary sexual features, but his testicles felt hard and four ti...

Background: Antiphospholipid syndrome (APS) is characterized by venous or arterial thrombosis and/or an adverse pregnancy outcome in the presence of persistent laboratory evidence of antiphospholipid antibodies. Loss of adrenal function due to bilateral adrenal vein thrombosis, resulting in hemorrhagic infarction, may occur in association with APS.Case presentation: We present the case of a 45-year-old woman with APS diagnosis since she was 37, with hist...

A 27-year-old woman with had sweating flushing attacks for three years prior to pregnancy, but did not follow with any medical examination. At 12th gestational week, hypertension was detected and alpha-methyldopa was started, no abnormal findings were found in the routine examinations, in the 24-h holter study, blood pressure values (using alpha-methyldopa) were found to be higher than 144/99 (general), 147/101 (awake) and 134/92 mmHg (sleep). 24-hour urinary levels of catecho...

POEMS syndrome characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes is a rare disease that usually presents in the 6th decade. We report a case of a young male in whom the presenting manifestations were mandibular mass, adrenal insufficiency and peripheral polyneuropathy. Clinical case: A thirty-three year old man from Guinea-Bissau was admitted to our hospital with asthenia, weight loss, decreased tactile sensibility with pain ...

Phaeochromocytoma is a common endocrinological diagnosis with an incidence of 1–2 per 10 000 people. Prompt evaluation and treatment leads to an excellent prognosis. Recurrence has been documented rarely (with literature reporting possibility of up to 8 years delay) and causes are generally attributed to inherited mutations, seeding during surgery or malignancy. Malignancy rates have been variable from 10% depending on site and mutations. In addition, the definition of ma...

Introduction: Ganglioneuroma is a rare benign tumour arising from neural crest sympathogonia. It is usually asymptomatic and non functional. An association between ganglioeneuroma and genetic or autoimmune diseases such as Addison’s disease or Mediterranean fever has never been described.Case description: We report the case of a 24-year-old woman presenting with paroxysmal abdominal pain with loss of weight and no fever. Physical examination along w...

Adrenocortical tumorigenesis has been observed in mice. It is speculated to be gonadotropin dependent. In particular LH receptors appear to be found within such adrenal tumors in high density and elevated LH levels may be related to adrenal tumorigenesis. The aim was to describe the case of a patient who after ovariectomy developed a large malignant adrenal tumor. A patient, female, aged 42 presented with uterine leiomyomas. She was treated by surgical excision of the uterus a...

Introduction: The incidence of parathyroid carcinoma in primary hyperparathyroidism is less than 1% and the association with ACTH-independent Cushing syndrome is very rare.Case report: A 40-year-old female was admitted for weight gain and reddish-purple striae. One month earlier she had a ¾ parathyroidectomy for primary hyperparathyroidism with histopathological examination showing a parathyroid carcinoma and two parathyroid adenomas. She also had a...

Introduction: Cystic adrenal lesions (CAL) are a rare diseases representing 5.4–6.0% of all pathological changes affecting adrenal glands. In about one-third of the cases, lesions are detected incidentally; in the remaining two-thirds cysts lesions are symptomatic, which is typically related to their large size or rapid growth. Although CAL are usually benign, since malignancy is reported in up to 7% of all CAL. Management algorithms for CAL vary and are controversial bec...

Estrogen-producing adrenal gland tumors (EPAGT) are extremely rare, accounting for only 1–2% of all adrenal tumors. They are most commonly observed in men or in children, and are unusual in women. They are almost always malignant even if they seem benign at presentation, and most of them have a poor prognosis. We present a case of a 71-year-old man with painful bilateral gynecomastia, without galactorrhea, over 4 months. Markedly elevated plasma estradiol levels were foun...

Introduction: Adrenocortical carcinoma (ACC) is a rare and aggressive endocrine malignancy with a poor prognosis. Hormone-secreting ACC appear with manifestations of virilization, feminization or Cushing syndrome.Case Report: A 63-year-old woman with no pathological history of relief was referred to Endocrinology evaluation with an history of deepening of the voice since 1-year ago, as well as hair loss and facial and abdominal hirsutism with 6-month evo...