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NCBI: db=pubmed; Term=adrenal tumor
Updated: 1 day 11 hours ago

Pleural dedifferentiated liposarcoma: A case report.

Wed, 01/23/2019 - 11:26
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Pleural dedifferentiated liposarcoma: A case report.

Mol Clin Oncol. 2019 Jan;10(1):132-136

Authors: Matsukuma S, Oshika Y, Utsumi Y, Obara K, Tanimoto T, Katsurada Y, Takeo H

Abstract
The present case report describes a rare case of pleural liposarcoma. A 45-year-old Japanese man was hospitalized for increasing left chest pain. Imaging revealed a 10-cm pleural tumor and a 1.7-cm contralateral right pulmonary nodule. Biopsy specimens of the pleural tumor showed undifferentiated spindle-shaped and/or rounded sarcomatous features with myxoid stroma. The patient underwent embolization of the arteries feeding the left pleural tumor and palliative partial resection of the pleural tumor. The surgically removed specimens exhibited similar undifferentiated sarcomatous features. The left pleural tumor regrew aggressively, and the patient succumbed to mortality ~4.2 months following hospitalization. Autopsy demonstrated a 35-cm left pleural tumor, metastasizing to both adrenal glands and lumbar vertebral bones, and a 2.2-cm primary adenocarcinoma of the right lung. The majority of the left pleural tumor and its metastases consisted of undifferentiated sarcomatous elements, however, scattered or aggregated lipoblasts were identified in localized areas adjacent to the diaphragm. Immunohistochemically, these lipoblasts were diffusely positive for MDM2 and focally positive for S-100 protein. Undifferentiated sarcomatous tumor cells were focally positive for MDM2 but negative for S-100 protein. This case was diagnosed as pleural dedifferentiated liposarcoma. The local aggressiveness of the pleural liposarcoma directly contributed to the patient's mortality. A review of the literature indicated that the dedifferentiated subtype may serve as a factor that is indicative of a poor prognosis for pleural liposarcoma.

PMID: 30655988 [PubMed]

Aldosterone-producing adrenocortical carcinoma with myxoid differentiation in a cat.

Wed, 01/23/2019 - 11:26
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Aldosterone-producing adrenocortical carcinoma with myxoid differentiation in a cat.

Vet Clin Pathol. 2018 Dec;47(4):660-664

Authors: Attipa C, Beck S, Lipscomb V, English K, Carvalho S, Kiupel M, Szladovits B, Peters LM

Abstract
A 10-year-old male neutered Persian cat was presented with an abdominal mass and history of weakness. Blood smear examination found marked elliptocytosis, and serum biochemical analysis revealed hypokalemia, hypochloremia, increased creatine kinase activity, and a high aldosterone concentration. Cytologic examination of the mass revealed neoplastic endocrine cells with moderate criteria of malignancy, favoring adrenocortical neoplasia. The adrenal mass was surgically excised and histologically characterized by lobules of mildly pleomorphic, polygonal neoplastic cells with moderate to abundant, occasionally granular, eosinophilic cytoplasm. Lobules were separated by fine fibrovascular trabeculae, and numerous cystic cavities containing amorphous eosinophilic material that stained positive with Alcian blue and periodic acid-Schiff were seen. Neoplastic cells were multifocally positive for cytochrome P450 aldosterone synthase. Based on clinicopathologic and immunohistochemical findings the present case was diagnosed as an aldosterone-producing adrenocortical carcinoma with myxoid differentiation. While this entity has not been reported in cats, myxoid differentiation of adrenocortical carcinomas has been found in other species and can pose a major diagnostic challenge on microscopic examination.

PMID: 30240029 [PubMed - indexed for MEDLINE]

[Pheochromocytoma and pregnancy: About one case].

Wed, 01/23/2019 - 11:26
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[Pheochromocytoma and pregnancy: About one case].

Gynecol Obstet Fertil Senol. 2018 09;46(9):667-668

Authors: Valdeyron C, Chartier C, Accoceberry M, Chadeyras JB, Gallot D

PMID: 30120067 [PubMed - indexed for MEDLINE]

Over-diagnosis of potential malignant behavior in MEN 2A-associated pheochromocytomas using the PASS and GAPP algorithms.

Wed, 01/23/2019 - 11:26
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Over-diagnosis of potential malignant behavior in MEN 2A-associated pheochromocytomas using the PASS and GAPP algorithms.

Langenbecks Arch Surg. 2018 Sep;403(6):785-790

Authors: Stenman A, Zedenius J, Juhlin CC

Abstract
PURPOSE: Pheochromocytomas (PCCs) exhibit malignant potential, but current histological modalities for the proper detection of aggressive behavior are debated. The two most widespread algorithms are the "Pheochromocytoma of the Adrenal Gland Scaled Score" (PASS) and the "Grading System for Adrenal Pheochromocytoma and Paraganglioma" (GAPP), both which mostly rely on histological parameters to identify PCC patients at risk of disseminated disease. Since the algorithms are derived from studies using predominantly sporadic PCCs, little is known whether the PASS or GAPP scores can predict malignant potential in hereditary cases.
METHODS: PASS and GAPP were applied on 41 PCCs; 13 PCCs were diagnosed in ten multiple endocrine neoplasia type 2A (MEN 2A) patients carrying established germline RET proto-oncogene mutations, as well as 28 assumed sporadic PCCs.
RESULTS: Six out of thirteen MEN 2A tumors (46%) exhibited PASS scores ≥ 4, indicative of a potential for aggressive behavior. In addition, 7/13 tumors (54%) exhibited GAPP scores ≥ 3, indicative of a "moderately differentiated type" with risk of future recurrence. All MEN 2A PCCs with an elevated PASS score also displayed an elevated GAPP score. In contrast, 4/28 (14%) sporadic PCCs demonstrated PASS scores ≥ 4, and 9/28 (32%) displayed GAPP scores ≥ 3. Follow-up found all cases in the study are free of metastatic or recurrent disease.
CONCLUSIONS: We conclude that the PASS and GAPP scoring systems might be suboptimal for determining true malignant potential in PCCs with constitutional RET mutations and advocate restrictive use of these scores in MEN 2A cases until the results are reproduced in larger numbers.

PMID: 29779047 [PubMed - indexed for MEDLINE]

Adrenocortical Carcinoma with Hypercortisolism.

Wed, 01/23/2019 - 11:26
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Adrenocortical Carcinoma with Hypercortisolism.

Endocrinol Metab Clin North Am. 2018 06;47(2):395-407

Authors: Puglisi S, Perotti P, Pia A, Reimondo G, Terzolo M

Abstract
Adrenocortical carcinoma (ACC) is a rare and aggressive tumor. ACC may be associated with different syndromes of hormone excess, most frequently Cushing's syndrome with or without hypersecretion of androgens. Recent data suggest that cortisol excess is a negative prognostic factor in advanced and localized ACC. Surgery with radical intent, when feasible, is the most effective treatment for ACC with hypercortisolism. Mitotane is the medical treatment of choice, both postoperatively and in inoperable or metastatic cases. Because of its slow onset of action, combination with other antisecretory agents (ie, metyrapone) is helpful to achieve more rapid and effective control of hypercortisolism.

PMID: 29754640 [PubMed - indexed for MEDLINE]

Mortality in Patients with Endogenous Cushing's Syndrome.

Wed, 01/23/2019 - 11:26
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Mortality in Patients with Endogenous Cushing's Syndrome.

Endocrinol Metab Clin North Am. 2018 06;47(2):313-333

Authors: Javanmard P, Duan D, Geer EB

Abstract
Cushing's syndrome is associated with increased morbidity and mortality. Cardiovascular events, sepsis, and thromboembolism are the leading causes of mortality. Patient's with Cushing's due to a pituitary adenoma and those with Cushing's due to benign adrenal adenoma have relatively good survival outcomes often mirroring that of the general population. Persistent or recurrent disease is associated with high mortality risk. Ectopic Cushing's syndrome and Cushing's due to adrenocortical carcinoma confer the highest mortality risk among Cushing's etiologies. Prompt diagnosis and treatment, and specific monitoring for and treatment of associated comorbidities are essential to decrease the burden of mortality from Cushing's.

PMID: 29754634 [PubMed - indexed for MEDLINE]

Genetics of Cushing's Syndrome.

Wed, 01/23/2019 - 11:26
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Genetics of Cushing's Syndrome.

Endocrinol Metab Clin North Am. 2018 06;47(2):275-297

Authors: Hernández-Ramírez LC, Stratakis CA

Abstract
The knowledge on the molecular and genetic causes of Cushing's syndrome (CS) has greatly increased in the recent years. Somatic mutations leading to overactive 3',5'-cyclic adenosine monophosphate/protein kinase A and wingless-type MMTV integration site family/beta-catenin pathways are the main molecular mechanisms underlying adrenocortical tumorigenesis. Corticotropinomas are characterized by resistance to glucocorticoid negative feedback, impaired cell cycle control and overexpression of pathways sustaining ACTH secretion. Recognizing the genetic defects behind corticotroph and adrenocortical tumorigenesis proves crucial for tailoring the clinical management of CS patients and for designing strategies for genetic counseling and clinical screening to be applied in routine medical practice.

PMID: 29754632 [PubMed - indexed for MEDLINE]

Human DHEA sulfation requires direct interaction between PAPS synthase 2 and DHEA sulfotransferase SULT2A1.

Wed, 01/23/2019 - 11:26
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Human DHEA sulfation requires direct interaction between PAPS synthase 2 and DHEA sulfotransferase SULT2A1.

J Biol Chem. 2018 06 22;293(25):9724-9735

Authors: Mueller JW, Idkowiak J, Gesteira TF, Vallet C, Hardman R, van den Boom J, Dhir V, Knauer SK, Rosta E, Arlt W

Abstract
The high-energy sulfate donor 3'-phosphoadenosine-5'-phosphosulfate (PAPS), generated by human PAPS synthase isoforms PAPSS1 and PAPSS2, is required for all human sulfation pathways. Sulfotransferase SULT2A1 uses PAPS for sulfation of the androgen precursor dehydroepiandrosterone (DHEA), thereby reducing downstream activation of DHEA to active androgens. Human PAPSS2 mutations manifest with undetectable DHEA sulfate, androgen excess, and metabolic disease, suggesting that ubiquitous PAPSS1 cannot compensate for deficient PAPSS2 in supporting DHEA sulfation. In knockdown studies in human adrenocortical NCI-H295R1 cells, we found that PAPSS2, but not PAPSS1, is required for efficient DHEA sulfation. Specific APS kinase activity, the rate-limiting step in PAPS biosynthesis, did not differ between PAPSS1 and PAPSS2. Co-expression of cytoplasmic SULT2A1 with a cytoplasmic PAPSS2 variant supported DHEA sulfation more efficiently than co-expression with nuclear PAPSS2 or nuclear/cytosolic PAPSS1. Proximity ligation assays revealed protein-protein interactions between SULT2A1 and PAPSS2 and, to a lesser extent, PAPSS1. Molecular docking studies showed a putative binding site for SULT2A1 within the PAPSS2 APS kinase domain. Energy-dependent scoring of docking solutions identified the interaction as specific for the PAPSS2 and SULT2A1 isoforms. These findings elucidate the mechanistic basis for the selective requirement for PAPSS2 in human DHEA sulfation.

PMID: 29743239 [PubMed - indexed for MEDLINE]

Alteration in skeletal muscle mass in women with subclinical hypercortisolism.

Wed, 01/23/2019 - 11:26
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Alteration in skeletal muscle mass in women with subclinical hypercortisolism.

Endocrine. 2018 07;61(1):134-143

Authors: Kim JH, Kwak MK, Ahn SH, Kim H, Cho YY, Suh S, Kim BJ, Song KH, Lee SH, Koh JM

Abstract
PURPOSE: Despite the well-known deleterious effects of cortisol on skeletal muscle, whether subtle cortisol excess in subclinical hypercortisolism (SH) affects skeletal muscle mass is unknown. Our objective was to understand the effects of the cortisol level on skeletal muscle mass in patients with SH.
METHODS: We compared skeletal muscle mass and fat mass (FM) between 21 patients with SH (12 women and 9 men) and 224 controls (67 women and 157 men) with nonfunctioning adrenal incidentaloma (NFAI). Medical records were reviewed, and we measured body composition parameters using bioelectrical impedance analysis and serum cortisol levels after the overnight 1-mg dexamethasone suppression test (DST).
RESULTS: After adjusting for confounding factors, 1-mg DST levels were inversely correlated with appendicular skeletal muscle mass (ASM) (γ = -0.245, P = 0.040), lower limb ASM (γ = -0.244, P = 0.040), and appendicular skeletal muscle index (ASMI; height-adjusted ASM) (γ = -0.229, P = 0.048) in all women, but not men. ASM and ASMI were significantly lower by 6.2% (P = 0.033) and 5.9% (P = 0.046), respectively, in women with SH compared with those with NFAI, but not men. Conversely, FM and percent fat mass were similar between the two groups. Compared with women with NFAI, among those with SH, lower limb, but not upper limb, ASM was lower by 6.8% (P = 0.020).
CONCLUSIONS: This study showed that women with SH had lower skeletal muscle mass, especially of the lower limb, and suggested that subtle cortisol excess also has adverse effects on skeletal muscle metabolism.

PMID: 29717464 [PubMed - indexed for MEDLINE]

Does IGF-1 play a role in the etiopathogenesis of non-functioning adrenocortical adenoma?

Wed, 01/23/2019 - 11:26
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Does IGF-1 play a role in the etiopathogenesis of non-functioning adrenocortical adenoma?

J Endocrinol Invest. 2018 Nov;41(11):1317-1323

Authors: Bahadir CT, Ecemis GC, Atmaca H

Abstract
PURPOSE: The aim of this study was to investigate the possible association of insulin-like growth factor-1 (IGF-1) with the pathogenesis of non-functioning adrenocortical adenomas (NFAs).
METHODS: This study included 50 female patients (mean age 54 years) with NFAs, 55 patients (mean age 48 years; 20 male, 35 female) with acromegaly and 38 female control subjects (mean age 58 years). Body mass index (BMI) and waist circumference (WC) of the subjects were recorded and blood samples for IGF-1 were taken. Insulin resistance was calculated using the homeostatic model assessment (HOMA) score. Since most of the acromegaly patients had been using medicine that could have effected insulin resistance, HOMA scores were calculated only in patients with NFAs and the controls. Computerized tomography or magnetic resonance imaging was taken of the acromegalics and controls to detect adrenal mass frequency.
RESULTS: The mean age was similar among the groups. As expected, the serum IGF-1 levels were significantly higher in patients with acromegaly than in patients with NFAs and the controls (p < 0.001). Although BMI, WC, and serum IGF-1 levels were significantly higher (p < 0.001) in patients with NFAs, the HOMA scores were similar between patients with NFAs and control groups. Although none of the control subjects had adrenal masses, NFAs were detected in 14 (25%) out of 55 acromegalic patients.
CONCLUSIONS: Higher serum IGF-1 levels in patients with NFAs compared to the control group and an increased prevalence of NFAs in acromegaly patients compared to control subjects and the general population suggest an association of IGF-1 with the etiopathogenesis of NFA.

PMID: 29542030 [PubMed - indexed for MEDLINE]

Germinal Centers Determine the Prognostic Relevance of Tertiary Lymphoid Structures and Are Impaired by Corticosteroids in Lung Squamous Cell Carcinoma.

Wed, 01/23/2019 - 11:26
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Germinal Centers Determine the Prognostic Relevance of Tertiary Lymphoid Structures and Are Impaired by Corticosteroids in Lung Squamous Cell Carcinoma.

Cancer Res. 2018 03 01;78(5):1308-1320

Authors: Siliņa K, Soltermann A, Attar FM, Casanova R, Uckeley ZM, Thut H, Wandres M, Isajevs S, Cheng P, Curioni-Fontecedro A, Foukas P, Levesque MP, Moch H, Linē A, van den Broek M

Abstract
In solid tumors, the presence of lymph node-like structures called tertiary lymphoid structures (TLS) is associated with improved patient survival. However, little is known about how TLS develop in cancer, how their function affects survival, and whether they are affected by cancer therapy. In this study, we used multispectral microscopy, quantitative pathology, and gene expression profiling to analyze TLS formation in human lung squamous cell carcinoma (LSCC) and in an experimental model of lung TLS induction. We identified a niche of CXCL13+ perivascular and CXCL12+LTB+ and PD-L1+ epithelial cells supporting TLS formation. We also characterized sequential stages of TLS maturation in LSCC culminating in the formation of germinal centers (GC). In untreated patients, TLS density was the strongest independent prognostic marker. Furthermore, TLS density correlated with GC formation and expression of adaptive immune response-related genes. In patients treated with neoadjuvant chemotherapy, TLS density was similar, but GC formation was impaired and the prognostic value of TLS density was lost. Corticosteroids are coadministered with chemotherapy to manage side effects in LSCC patients, so we evaluated whether they impaired TLS development independently of chemotherapy. TLS density and GC formation were each reduced in chemotherapy-naïve LSCC patients treated with corticosteroids before surgery, compared with untreated patients, a finding that we confirmed in the experimental model of lung TLS induction. Overall, our results highlight the importance of GC formation in TLS during tumor development and treatment.Significance: Corticosteroid treatment during chemotherapy negatively affects the development of tertiary lymphoid structures and abrogates their prognostic value in patients with lung cancer. Cancer Res; 78(5); 1308-20. ©2018 AACR.

PMID: 29279354 [PubMed - indexed for MEDLINE]

Adding metyrapone to chemotherapy plus mitotane for Cushing's syndrome due to advanced adrenocortical carcinoma.

Wed, 01/23/2019 - 11:26
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Adding metyrapone to chemotherapy plus mitotane for Cushing's syndrome due to advanced adrenocortical carcinoma.

Endocrine. 2018 07;61(1):169-172

Authors: Claps M, Cerri S, Grisanti S, Lazzari B, Ferrari V, Roca E, Perotti P, Terzolo M, Sigala S, Berruti A

PMID: 29019062 [PubMed - indexed for MEDLINE]

Iodine and Fat Quantification for Differentiation of Adrenal Gland Adenomas From Metastases Using Third-Generation Dual-Source Dual-Energy Computed Tomography.

Wed, 01/23/2019 - 11:26
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Iodine and Fat Quantification for Differentiation of Adrenal Gland Adenomas From Metastases Using Third-Generation Dual-Source Dual-Energy Computed Tomography.

Invest Radiol. 2018 03;53(3):173-178

Authors: Martin SS, Weidinger S, Czwikla R, Kaltenbach B, Albrecht MH, Lenga L, Vogl TJ, Wichmann JL

Abstract
OBJECTIVES: The aim of this study was to investigate the value of third-generation dual-source dual-energy computed tomography (DECT) iodine and fat quantification in differentiating adrenal gland adenomas from metastases.
MATERIALS AND METHODS: Sixty-two patients (38 men and 24 women; mean age, 69.1 years) underwent clinically indicated DECT of the abdomen on a third-generation dual-source scanner. Examinations were retrospectively included due to detected adrenal masses. For each adrenal lesion, unenhanced and contrast-enhanced attenuation values, as well as dual-energy iodine density and fat fraction, were recorded. Additional magnetic resonance imaging data, positron emission tomography/computed tomography scans, interval imaging follow-up, and histopathological analysis were used as the reference standard for all adrenal lesions. Mean values of unenhanced and contrast-enhanced attenuation, as well as material densities, were compared between adenomas, metastases, and normal adrenal glands. Furthermore, the diagnostic accuracy of unenhanced, contrast-enhanced, and material density analysis was assessed between adrenal adenomas and metastases.
RESULTS: Adrenal adenomas showed significant differences regarding iodine density and fat fraction values (1.3 ± 0.4 mg/mL and 34.2% ± 12.6%) in comparison with adrenal metastases (3.2 ± 1.4 mg/mL and 10.7% ± 7.8%) and normal adrenal glands (1.7 ± 0.6 mg/mL and 18.7% ± 12.0%) (all P ≤ 0.004). Analysis of unenhanced attenuation values revealed no significant differences between healthy adrenal parenchyma (19.1 ± 15.6 HU) and adrenal metastases (26.9 ± 16.2 HU) (P = 0.135). Iodine density and fat fraction analysis showed significantly higher diagnostic accuracy for the diagnosis of adenomas (sensitivity, 97% and 89%; specificity, 96% and 89%, respectively) compared with unenhanced and contrast-enhanced evaluation (sensitivity, 65% and 58%; specificity, 73% and 85%, respectively) (P ≤ 0.023). The combined diagnostic value of iodine density and fat fraction analysis revealed a sensitivity of 97% and a specificity of 100%.
CONCLUSIONS: Third-generation dual-source DECT iodine and fat quantification allow for differentiation between adrenal adenomas and metastases with high diagnostic accuracy.

PMID: 28990974 [PubMed - indexed for MEDLINE]

Evaluation and diagnostic potential of circulating extracellular vesicle-associated microRNAs in adrenocortical tumors.

Wed, 01/23/2019 - 11:26
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Evaluation and diagnostic potential of circulating extracellular vesicle-associated microRNAs in adrenocortical tumors.

Sci Rep. 2017 07 14;7(1):5474

Authors: Perge P, Butz H, Pezzani R, Bancos I, Nagy Z, Pálóczi K, Nyírő G, Decmann Á, Pap E, Luconi M, Mannelli M, Buzás EI, Tóth M, Boscaro M, Patócs A, Igaz P

Abstract
There is no available blood marker for the preoperative diagnosis of adrenocortical malignancy. The objective of this study was to investigate the expression of extracellular vesicle-associated microRNAs and their diagnostic potential in plasma samples of patients suffering from adrenocortical tumors. Extracellular vesicles were isolated either by using Total Exosome Isolation Kit or by differential centrifugation/ultracentrifugation. Preoperative plasma extracellular vesicle samples of 6 adrenocortical adenomas (ACA) and 6 histologically verified adrenocortical cancer (ACC) were first screened by Taqman Human Microarray A-cards. Based on the results of screening, two miRNAs were selected and validated by targeted quantitative real-time PCR. The validation cohort included 18 ACAs and 16 ACCs. Beside RNA analysis, extracellular vesicle preparations were also assessed by transmission electron microscopy, flow cytometry and dynamic light scattering. Significant overexpression of hsa-miR-101 and hsa-miR-483-5p in ACC relative to ACA samples has been validated. Receiver operator characteristics of data revealed dCT hsa-miR-483-5p normalized to cel-miR-39 to have the highest diagnostic accuracy (area under curve 0.965), the sensitivity and the specifity were 87.5 and 94.44, respectively. Extracellular vesicle-associated hsa-miR-483-5p thus appears to be a promising minimally invasive biomarker in the preoperative diagnosis of ACC but needs further validation in larger cohorts of patients.

PMID: 28710381 [PubMed - indexed for MEDLINE]

Nonparathyroid Hypercalcemia.

Wed, 01/16/2019 - 11:16
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Nonparathyroid Hypercalcemia.

Front Horm Res. 2019;51:77-90

Authors: Goltzman D

Abstract
Primary hyperparathyroidism is among the most common causes of hypercalcemia. However, ingestion of medication, including hydrochlorathiazide, lithium, and foscarnet, excessive vitamin A ingestion, endocrinopathies such as hyperthyroidism, adrenal insufficiency, and acromegaly, abnormal nutrient intake such as parenteral nutrition in preterm infants and milk-alkali syndrome, and prolonged immobilization have all been associated with hypercalcemia. The most common cause of nonparathyroid hypercalcemia is neoplasia. Hypercalcemia is generally due to the secretion of parathyroid hormone (PTH)-related peptide (PTHrP) by a wide variety of nonmetastatic solid tumors, including squamous cell tumors but also hematologic tumors. PTHrP, although encoded by a distinct gene, shares amino acid sequence homology with PTH in the amino-terminal domain, which allows it to cross-react at a common G protein receptor, the type 1 PTH/PTHrP receptor (PTHR1), resulting in similar skeletal effects and effects on calcium and phosphorus metabolism. Increased PTHrP action with hypercalcemia may be seen in the benign disease Jansen's metaphyseal chondrodysplasia due to a gain-of-function mutation in PTHR1. Another humoral factor, 1,25-dihyroxyvitamin D [1,25(OH)2D] may be produced by lymphomas, but also by benign granulomatous disorders and may also cause hypercalcemia when its metabolism is genetically impaired. Vitamin D intoxication may cause hypercalcemia due to overproduction of the metabolite, 25 hydroxyvitamin D, apparently in the absence of conversion to 1,25(OH)2D. Malignancies metastatic to bone or arising in bone (such as multiple myeloma) may produce a variety of growth factors and cytokines, in addition to PTHrP, which can contribute to tumor growth as well as osteolysis and hypercalcemia.

PMID: 30641526 [PubMed - in process]

Familial and Hereditary Forms of Primary Hyperparathyroidism.

Wed, 01/16/2019 - 11:16
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Familial and Hereditary Forms of Primary Hyperparathyroidism.

Front Horm Res. 2019;51:40-51

Authors: Cetani F, Saponaro F, Borsari S, Marcocci C

Abstract
Individuals with a familial predisposition to the development of parathyroid tumors constitute a small minority of all patients with primary hyperparathyroidism (PHPT). These familial syndromes exhibit Mendelian inheritance patterns and the main causative genes in most families have been identified. They include multiple endocrine neoplasia (MEN; types 1, 2A, and 4), hyperparathyroidism-jaw tumor (HPT-JT) syndrome, familial isolated hyperparathyroidism, familial hypocalciuric hypercalcemia (FHH), and neonatal severe PHPT. Each MEN type is associated with the various combinations of specific tumors. MEN1 is characterized by the occurrence of parathyroid, enteropancreatic, and pituitary tumors; MEN2A is characterized by medullary thyroid carcinoma and pheochromocytoma, and MEN4 is characterized by a pathological spectrum similar to that of MEN1 in association with tumors of the adrenal, kidney, and reproductive organs. HPT-JT is characterized by PHPT, ossifying fibromas of maxillary bones, kidney disease, and uterine neoplasias. The prompt diagnosis of these diseases is of great importance for planning appropriate surveillance of the mutant carriers and correct surgical management. The search for mutation is also useful for the identification of the family members who do not carry the mutation and can avoid unnecessary biochemical and instrumental evaluations. Surgery remains the treatment of choice in all familial forms except FHH.

PMID: 30641519 [PubMed - in process]

Pheochromocytoma-related cardiomyopathy presenting as broken heart syndrome: Case report and literature review.

Wed, 01/16/2019 - 11:16
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Pheochromocytoma-related cardiomyopathy presenting as broken heart syndrome: Case report and literature review.

Int J Surg Case Rep. 2019 Jan 09;55:7-10

Authors: Diaz B, Elkbuli A, Ehrhardt JD, McKenney M, Boneva D, Hai S

Abstract
INTRODUCTION: Pheochromocytoma are neuroendocrine tumors that arise from sympathetic chromaffin cells within the adrenal medulla. They principally secrete catecholamines, potentially causing life-threatening cardiovascular complications. A myriad of symptomatology and clinical findings are associated with pheochromocytoma, including a catecholamine-induced dilated cardiomyopathy.
PRESENTATION OF CASE: A 50-year-old woman presented with retrosternal chest pain and underwent diagnostic evaluation for acute coronary syndrome. Cardiac catheterization demonstrated patent coronary arteries and a pattern of ventricular hypokinesis consistent with takotsubo cardiomyopathy, also known as broken heart syndrome. Further imaging with abdominal CT revealed an adrenal mass. Laboratory markers supported the clinical picture of pheochromocytoma. Right adrenalectomy was performed and our patient was symptom-free at discharge on post-operative day three.
DISCUSSION: Alpha and beta adrenergic blockade are used in a critical care setting to prevent perioperative hemodynamic instability as well as catecholamine-induced heart failure in the setting of pheochromocytoma. Patients commonly require vasopressors in the postoperative period due to the rapid reduction in circulating catecholamines following resection. Discharge planning should include recommendations for genetic counseling to screen for syndromic causes of pheochromocytoma that increase the risk for other neoplasms.
CONCLUSION: We present a case report of a rare adrenal tumor in a middle-aged woman that manifested as acute coronary syndrome. A presumptive diagnosis of takotsubo cardiomyopathy on cardiac catheterization led to further investigation. Abdominal imaging located an adrenal mass that correlated with laboratory studies positive for high levels of catecholamines and their metabolites. The tumor was excised and the patient recovered without complications.

PMID: 30641325 [PubMed - as supplied by publisher]

A Case of Stage IV Chromophobe Renal Cell Carcinoma Treated with the Oncolytic ECHO-7 Virus, Rigvir®.

Wed, 01/16/2019 - 11:16
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A Case of Stage IV Chromophobe Renal Cell Carcinoma Treated with the Oncolytic ECHO-7 Virus, Rigvir®.

Am J Case Rep. 2019 Jan 12;20:48-52

Authors: Ismailov Z, Rasa A, Bandere K, Brokāne L, Tilgase A, Olmane E, Nazarovs J, Alberts P

Abstract
BACKGROUND Renal cell carcinoma is the most commonly diagnosed primary malignant tumor of the kidney in adults, and includes the variant of chromophobe renal cell carcinoma. Despite new targeted therapies that improve progression-free survival (PFS) and overall survival (OS) for early-stage renal cell carcinoma, the 5-year survival for patients with stage IV renal cell carcinoma remains below 10%, and the 50% OS is less than one year. Metastatic renal cell carcinoma can be resistant to cytotoxic chemotherapy. This report is of a case of stage IV chromophobe renal cell carcinoma that responded well to treatment with the oncolytic ECHO-7 virus, Rigvir®. CASE REPORT In December 2015, a 59-year-old man presented with a right-sided chromophobe renal cell carcinoma stage IV (pT₁N₀M₁) with adrenal gland metastasis. He underwent right nephro-adrenalectomy followed by treatments with Rigvir® (≥10⁶ TCID₅₀/ml) by intramuscular (i.m.) injection on three consecutive days. Treatment with Rigvir® continued once per week for three months, and from March 2016, once per month, with continued treatment until computed tomography (CT) scans confirmed that the tumor metastases had stabilized. CONCLUSIONS This case report has demonstrated that the oncolytic ECHO-7 virus, Rigvir® should be evaluated further as a potential treatment for advanced renal carcinoma.

PMID: 30635548 [PubMed - in process]

Suprasellar chordoid glioma: a report of two cases.

Wed, 01/16/2019 - 11:16
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Suprasellar chordoid glioma: a report of two cases.

Arch Endocrinol Metab. 2018;62(6):648-654

Authors: Danilowicz K, Abbati SG, Sosa S, Witis FL, Sevlever G

Abstract
Chordoid glioma (CG) is considered a slow growing glial neoplasm. We report two new cases with endocrinological presentation, management and outcome. Case reports: 1) An 18 year-old female patient was admitted due to headaches, nausea and vomiting and visual abnormalities. She was in amenorrhea. A brain magnetic resonance imaging (MRI) demonstrated a 35 mm-diameter sellar and suprasellar mass. An emergency ventricular peritoneal valve was placed due to obstructive hydrocephalus. Transcraneal surgery was performed. The patient developed central hypothyroidism, adrenal insufficiency and transient diabetes insipidus; she never recovered spontaneous menstrual cycles. Histopathologic study showed cells in cords, inside a mucinous stroma, positive for glial fibrillary acidic protein (GFAP). Due to residual tumor gamma knife radiosurgery was performed. Three years after surgery, the patient is lucid, with hypopituitarism under replacement. 2) A 46 year-old woman complained about a three year-history of amenorrhea, galactorrhea and headache. An MRI showed a solid-cystic sellar mass 40 mm-diameter that extended to the suprasellar cistern. She had hypogonatropic hypogonadism and mild hyperprolactinemia. The tumor mass was removed via nasal endoscopic approach. Histopathological study reported cellular proliferation of glial lineage positive for GFAP. The patient evolved with central hypothyroidism and diabetes insipidus. She was re-operated for fistula and again under the diagnosis of extradural abscess. She evolved with cardiorespiratory descompensation and death, suspected to be due to a thromboembolism. In conclusion, the first case confirms that best treatment for CG is surgery considering radiotherapy as an adjuvant therapy. The other case, on the contrary, illustrates the potentially fatal evolution due to surgical complications.

PMID: 30624507 [PubMed - in process]

Postmenopausal androgen-secreting ovarian tumors: challenging differential diagnosis in two cases.

Wed, 01/16/2019 - 11:16
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Postmenopausal androgen-secreting ovarian tumors: challenging differential diagnosis in two cases.

Climacteric. 2019 Jan 09;:1-5

Authors: Arteaga E, Martinez A, Jaramilo J, Villaseca P, Cuello M, Valenzuela P, Gejman R, Blumel JE

Abstract
Postmenopausal hyperandrogenism constitutes a very rare condition of tumoral or non-tumoral origin primarily residing either in the ovary or in the adrenal glands. We present herein two cases with this condition; one with abnormal postmenopausal genital bleeding and mild increase in facial hair, and the second with slow-developing hirsutism and virilization. Both cases shared a notorious increase in libido. The laboratory tests showed high levels of testosterone (>100 ng/ml). A normal value of dehydroepiandrosterone sulfate and a normal cortisol level at 9 am after 1 mg of dexamethasone administered at midnight (Nugent test) made an adrenal etiology very unlikely. On the other hand, a high level of inhibine B oriented to an ovarian source. Transvaginal sonography failed to demonstrate an ovarian tumor, but an abdominal and pelvic computed tomography scan or magnetic resonance imaging detected an ovarian tumor and normal adrenal glands. A laparoscopic oophorectomy was performed, and the histological study demonstrated a steroidal cell tumor in the first case and a Leydig cell tumor in the second.

PMID: 30623686 [PubMed - as supplied by publisher]

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