Ectopic secretion of ACTH from non-pituitary tumors, referred to as ectopic ACTH syndrome (EAS), accounts for about 10–20% of Cushing’s syndrome (CS). Ectopic hormone-secreting pheochromocytomas (Pheo) are rare. The first publication of association between pheochromocytoma and Cushing’s syndrome by Roux is dated 1955. Pheochromocytoma represents a rare cause of hypercortisolism, accounting for less than 5 % of ectopic Cushing’s syndrome while less than 1 % of pheochromocytomas is accompanied by Cushing’s syndrome.We are reporting 4 cases of ACTH-secreting pheochromocytoma presenting as Cushing’s syndrome. Data from 4 patients were analysed. There were 4 women from 50 to 63 years old. All patients had a clinical presentation of hypercorticoidism. Their levels of adrenocorticotropic hormone in plasma, 24-hour urinary free cortisol and urinary catecholamine were high. Computed tomography scan of the abdomen in all cases revealed a mass in the left adrenal gland. Left sided adrenalectomy was performed under treatment with a-blocker doxazosin and b-blocker atenolol. Histological examination revealed in 3 cases – pheocromocytoma and in 1 case corticomedullary mixed tumor of the adrenal gland. Additional immunostaining (IHC)of these tumors showed positive immunostaining for chromogranin and ACTH. The IHC search for somatostatin receptors of subtype 2 and 5 (SSTR2, SSTR5) was performed in 3 cases and showed predominately expression SSTR2. The case index of Ki-67 ranged, from 0,5 to 4%. Biochemical signs of hypercortisolism rapidly began to disappear after surgery. Follow up of the patients during the next 2 years on average was with disease remission.
BACKGROUND: Primary hyperparathyroidism (PHPT) is a widespread endocrine disease characterized by excessive production of parathyroid hormone (PTH) due to parathyroid gland hyperplasia (PGH) or tumor lesions (adenoma or cancer of the parathyroid gland (PG) in 80% and 15% of cases respectively). Choline kinase alpha (XK) overexpression is described in tumors of different localization, but there is no data on its expression in PG tumors. AIMS: To study the character of XK expression in PG neoplasms and its relationship with clinical, laboratory, and visualization characteristics (positron emission tomography combined with computed tomography (PET/CT) with 18Ffluorocholine (18FFC)). MATERIALS AND METHODS: The material for the study was based on tissue samples from 10 patients of 3470 years old (Me = 61.5; [48; 66]), with a laboratoryconfirmed diagnosis of PHT. An immunohistochemical study (IHC) was carried out on materials from 2 patients with hyperplasia of the main cells, from 5 patients with adenoma of PG, from 1 patient with atypical adenoma and 1 with carcinoma of PG; in 1 case the metastasis of cancer of the neck with lymph node was examined. RESULTS: The expression of XK is spotted in all types of PG cells (chief cells: active and inactive forms), transitional forms between the chief cells and oxyphil; oxyphil cells, but it was most intense in active chief cells. The expression of XK was observed in neoplasms of PG of various degrees of malignancy. In the most numerous group of PG formations with a favorable prognosis (11 samples from 7 patients), no statistically significant correlation (p 0.05) was obtained between the intensity expression of the XK, of the PTH and the proliferative activity index Ki67, the level of radiopharmaceutical accumulation in PET/CT with 18FFC (SUVmax) and laboratory data (PTH, Ca, Ca++). CONCLUSIONS: In the majority of investigated cases, moderate and intensive expression of the XK was detected in PG cells. A small amount of studied cases does not allow us to identify the connection between the intensity of XK expression and the malignant potential for the formation of PG.
Background. Adrenocortical carcinoma (ACC) is a rare malignant tumor characterized by an annual incidence of 0.5—2 cases per million population. Surgery is the-first line treatment for ACC. When total tumor resection is not possible due to its proliferation or progression, mitotane (o,p’DDD) is used. In this case, stabilization and partial response (as assessed by RECIST criteria) was observed only in 48.7% of cases, necessitating the search for new therapeutic targets. Objective — the study was aimed at assessing the somatostatin receptor expression in adrenocortical carcinomas and adrenal cortex tumors with uncertain malignant potential. Material and methods. Surgical material from adrenocortical tumors of 13 patients (4 males and 9 females aged from 28 to 68 years) was used. In all cases, the diagnosis was verified by morphological and immunohistochemical (IHC) studies: ACC was detected in 10 cases (including 1 case of ACC liver metastasis), oncocytic carcinoma — 1 case, oncocytoma with uncertain malignant potential — 2 cases. Morphological assessment of ACC was carried out according to Weiss criteria (for ACC tumors) and Lin—Weiss—Bisceglia criteria (for oncocytic neoplasms of the adrenal cortex). IHC study was carried out with antibodies to the spectrum of adrenal cortex-specific markers, as well as Ki-67 and somatostatin receptors 2 and 5 (SSTR2 and SSTR5). Results. The expression of SSTR2 and/or 5 was detected in 8 (61.5%) of 13 cases of ACC. Isolated SSTR2 expression was observed in 4 cases (4/13), while SSTR5 expression was observed in 6 cases (6/10). In 2 cases (2/10), co-expression of both receptor types was observed. SSTR expression was observed both in ACC and ACC liver metastasis, as well as in oncocytic ACC. Conclusion. SSTR2 and/or 5 expression in ACC tissue expands diagnostic and prognostic capabilities for this pathology.
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