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Pheochromocytoma Secreting Large Quantities of Both Epinephrine and Norepinephrine Presenting with Episodes of Hypotension and Severe Electrolyte Imbalance.

Tue, 10/02/2018 - 15:14
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Pheochromocytoma Secreting Large Quantities of Both Epinephrine and Norepinephrine Presenting with Episodes of Hypotension and Severe Electrolyte Imbalance.

Cureus. 2018 Jul 25;10(7):e3050

Authors: Shahbaz A, Aziz K, Fransawy Alkomos M, Nabi U, Zarghamravanbakhsh P, Sachmechi I

Abstract
Pheochromocytoma is a rare tumor usually arising from the adrenal medulla (strictly speaking, those arising outside the adrenal gland are called paragangliomas). We report a case of pheochromocytoma presenting as orthostatic hypotension and electrolyte imbalance. A 51-year-old woman was admitted because of vomiting and chest pain. She had fluctuating blood pressure (BP) with episodes of orthostatic hypotension. Computed tomography pulmonary angiogram was performed to rule out pulmonary embolism; it showed a clear chest, but an incidental right suprarenal mass. The biochemical analysis supports the diagnosis of pheochromocytoma. Her electrolyte panel revealed persistently low potassium, calcium, and magnesium levels despite aggressive replacement. We speculated that hypotension was mainly due to vasodilatation caused by excess plasma epinephrine and prescribed doxazosin and a nonselective beta-adrenergic blocker which stabilized BP. The right adrenal tumor excised, and postoperatively she remained hemodynamically stable with no hypotensive episode. Laboratory data taken six weeks after surgery show normal 24-hour urine metanephrine and normetanephrine and normal serum magnesium and calcium levels. This case report highlights the variable presentation of pheochromocytoma. We also discuss the probable mechanisms of electrolyte imbalance in our case.

PMID: 30263879 [PubMed]

[Minimally invasive surgery of neural tumors in childhood].

Tue, 10/02/2018 - 15:14
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[Minimally invasive surgery of neural tumors in childhood].

Cir Pediatr. 2018 Aug 03;31(3):140-145

Authors: Crehuet Gramatyka D, Gómez-Chacón Villalba J, Cortés Sáez J, Marco Macián A, Vila Carbó JJ

Abstract
PURPOUSE: The aim of the paper is to describe the experience in our center with the use of minimally invasive surgery (MIS) of neural tumors in childhood.
METHODS: Descriptive and retrospective study of patients diagnosed with neural neoplasia (neuroblastoma and neuroganglioma) on whom MIS technique surgery has been performed between October 2012 and December 2017. The inclusion criteria were patients with a neural tumor diagnosis who, at the time of the intervention, did not have imaging-defined risk factors (IDRFs). Patients with a different diagnosis than neural tumor or with IDRFs were excluded from the study.
RESULTS: The study comprises 19 cases (6 female and 13 male) with a median age of 47 months. According to the International Neuroblastoma Risk Group Staging System (INRGSS) classification, nine cases were in L1 stage, six in L2, two in M and two in MS. Laparoscopy was used in 14 patients (12 adrenal and 2 abdominal tumors) and thoracoscopy was used in the other 5. In 4 of the 19 cases (21%), conversion to open surgery was needed due to fibrosis in 2 cases and vascular structures entrapment in another 2 (3 in laparoscopy and 1 in thoracoscopy). There were no surgical complications, achieving complete resection in all cases. Three cases showed postsurgical adverse effects grade I and II, according to Clavien-Dindo classification. After a median of 27 months of follow up, two patients showed disease progression without local recurrence.
CONCLUSIONS: In conclusion, MIS are useful techniques in the surgical exeresis of non-disseminated neural tumors without IDRFs.

PMID: 30260107 [PubMed - in process]

Multiple Endocrine Neoplasia Type 1: A Case Report With Review of Imaging Findings.

Tue, 10/02/2018 - 15:14
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Multiple Endocrine Neoplasia Type 1: A Case Report With Review of Imaging Findings.

Ochsner J. 2018;18(2):170-175

Authors: Keller HR, Record JL, Lall NU

Abstract
Background: Multiple endocrine neoplasia type 1 (MEN1) is a rare, autosomal dominant inherited syndrome caused by mutations in the MEN1 tumor suppressor gene. The diagnosis is defined clinically by the presence of 2 or more primary MEN1 tumors (parathyroid, anterior pituitary, and pancreatic islet). We describe the case of a patient who presented with classic history and imaging findings for MEN1.
Case Report: A male in his early thirties with a history of hyperparathyroidism and a transsphenoidal prolactinoma resection presented years later with abdominal symptoms concerning for Zollinger-Ellison syndrome: worsening epigastric abdominal pain, nausea, vomiting, and diarrhea. Contrast-enhanced computed tomography (CT) of the abdomen revealed hyperenhancing pancreatic lesions and duodenal inflammation, suggesting pancreatic neuroendocrine tumor (gastrinoma) with secondary duodenitis. Bilateral indeterminate hypoattenuating adrenal nodules were also seen on contrast-enhanced CT, and follow-up magnetic resonance imaging confirmed benign adrenal adenomas. Furthermore, thyroid ultrasound and sestamibi scintigraphy revealed a parathyroid adenoma. With confirmatory imaging findings, history, and presenting symptoms, the patient was clinically diagnosed with MEN1 syndrome and underwent surgical and medical management.
Conclusion: This case exhibits the classic history with corresponding imaging findings of MEN1 syndrome, including pancreatic neuroendocrine tumors, parathyroid adenoma, and adrenal adenomas. High clinical suspicion for MEN1 should lead to endocrinology evaluation with appropriate laboratory workup and targeted imaging evaluation of the typical endocrine organs as described for this patient.

PMID: 30258300 [PubMed]

MAPK/ERK pathway inhibition is a promising treatment target for adrenocortical tumors.

Tue, 10/02/2018 - 15:14
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MAPK/ERK pathway inhibition is a promising treatment target for adrenocortical tumors.

J Cell Biochem. 2018 Sep 06;:

Authors: Pereira SS, Monteiro MP, Costa MM, Ferreira J, Alves MG, Oliveira PF, Jarak I, Pignatelli D

Abstract
Unraveling molecular mechanisms that regulate tumor development and proliferation is of the utmost importance in the quest to decrease the high mortality rate of adrenocortical carcinomas (ACC). Our aim was to evaluate the role of two of the mitogen-activated protein kinase (MAPK) signaling pathways (extracellular signal-regulated protein kinases [ERKs 1/2] and p38) in the adrenocortical tumorigenesis, as well as the therapeutic potential of MAPK/ERK inhibition. ERKs 1/2 and p38 activation were evaluated in incidentalomas (INC; n = 10), benign Cushing's syndrome (BCS; n = 12), malignant Cushing's syndrome (MCS; n = 6) and normal adrenal glands (NAG; 8). ACC cell line (H295R) was used to evaluate the ability of PD184352 (0.1, 1, and 10 µM), a specific MEK-MAPK-ERK pathway inhibitor, to modulate cell proliferation, viability, metabolism, and steroidogenesis. ERKs 1/2 activation was significantly higher in MCS (2.83 ± 0.17) compared with NAG (1.00 ± 0.19 "arbitrary units"), INC (1.20 ± 0.13) and BCS (2.09 ± 0.09). Phospho-p38 expression was absent in all the MCS analyzed. MAPK/ERK kinase (MEK) inhibition with PD184352 significantly decreased proliferation as well as steroidogenesis and also increased the redox state of the H295R cells. This data suggests that MEK-MAPK-ERK signaling has a role in adrenocortical tumorigenesis that could be potentially used as a diagnostic marker for malignancy and targeted treatment in ACC.

PMID: 30256438 [PubMed - as supplied by publisher]

Multiple endocrine neoplasia-like syndrome in 24 baboons (Papio spp.).

Tue, 10/02/2018 - 15:14
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Multiple endocrine neoplasia-like syndrome in 24 baboons (Papio spp.).

J Med Primatol. 2018 Sep 06;:

Authors: Confer A, Owston MA, Kumar S, Dick EJ

Abstract
Multiple endocrine neoplasia (MEN) has not been reported in baboons, but this condition is well described in humans. An internal database was searched for all cases of concurrent endocrine hyperplasia and neoplasia in baboons. Twenty-four baboons (Papio spp.) with concurrent endocrine hyperplasia and neoplasia were identified. Twenty-one baboons had lesions in two endocrine organs, two baboons had lesions in three organs, and one baboon had lesions in four organs. Ten baboons aligned with the MEN1 classification; 14 baboons did not match any current human MEN classification. We report 24 cases of MEN-like syndrome in baboons. MEN1-like lesions accounted for nearly half (41%) of the affected animals. Genetic analysis of baboons with MEN-like syndrome could further elucidate the mechanisms of MEN and support the use of baboons as animal models for human MEN.

PMID: 30256416 [PubMed - as supplied by publisher]

Does Urinary Tract Ultrasound Have Its Place in the Treatment of Early Neonatal Jaundice? Neonatal Bilateral Adrenal Hemorrhage: Case Report.

Tue, 10/02/2018 - 15:14
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Does Urinary Tract Ultrasound Have Its Place in the Treatment of Early Neonatal Jaundice? Neonatal Bilateral Adrenal Hemorrhage: Case Report.

Acta Clin Croat. 2018 Mar;57(1):161-165

Authors: Trutin I, Valent Morić B, Borošak J, Stipančić G

Abstract
Adrenal hemorrhage is a rare clinical entity in the neonatal period, with an incidence of 1.7-2.1/1000 births. It is more often diagnosed on the right side, whilst bilateral hemorrhage occurs in 10%-15% of cases. Clinical presentation shows a wide range of symptoms, from the signs of adrenal insufficiency to asymptomatic course of illness with incidental finding of changes on testing. Neonatal jaundice due to hemolysis of hemorrhagic content often is an accompanying sign. We present a male neonate born at term, with early neonatal jaundice of unknown cause and without evi-dence of perinatal infection. Ultrasound of the urinary tract revealed hypoechoic formations in the upper poles of both kidneys, confirmed by magnetic resonance imaging of the abdomen. Clinical and laboratory test results showed no signs of adrenal insufficiency. There was no confirmation of em-bryonic tumor or neuroblastoma. Ultrasound of the urinary tract as an available and noninvasive test has its place in the treatment of early neonatal jaundice of unknown cause. Additional invasive treat-ment and unnecessary laparotomy can be avoided with ultrasound monitoring of the formation re-gression.

PMID: 30256026 [PubMed - in process]

Circulating epinephrine is not required for chronic stress to enhance metastasis.

Tue, 10/02/2018 - 15:14
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Circulating epinephrine is not required for chronic stress to enhance metastasis.

Psychoneuroendocrinology. 2018 Sep 15;99:191-195

Authors: Walker AK, Martelli D, Ziegler AI, Lambert GW, Phillips SE, Hill SJ, McAllen RM, Sloan EK

Abstract
Signaling through β-adrenergic receptors drives cancer progression and β-blockers are being evaluated as a novel therapeutic strategy to prevent metastasis. Orthotopic mouse models of breast cancer show that β-adrenergic signaling induced by chronic stress accelerates metastasis, and that β2-adrenergic receptors on tumor cells are critical for this. Endogenous catecholamines are released during chronic stress: norepinephrine from the adrenal medulla and sympathetic nerves, and epinephrine from the adrenal medulla. β2-adrenergic receptors are much more sensitive to epinephrine than to norepinephrine. To determine if epinephrine is necessary in the effects of stress on cancer progression, we used a denervation strategy to eliminate circulating epinephrine, and quantified the effect on metastasis. Using both human xenograft and immune-intact murine models of breast cancer, we show that circulating epinephrine is dispensable for the effects of chronic stress on cancer progression. Measured levels of circulating norepinephrine were sufficiently low that they were unlikely to influence β2-adrenergic signaling, suggesting a possible role for norepinephrine release from sympathetic nerve terminals.

PMID: 30253326 [PubMed - as supplied by publisher]

Current Standards and Recent Advances in Biomarkers of Major Endocrine Tumors.

Tue, 10/02/2018 - 15:14
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Current Standards and Recent Advances in Biomarkers of Major Endocrine Tumors.

Front Pharmacol. 2018;9:963

Authors: Luo Y, Zhu H, Tan T, He J

Abstract
The complexity of endocrine tumor diagnosis stems from its variable symptoms and presentation that may mimic many other disease states, or display asymptomatic properties for a prolonged amount of time. Early and accurate disease identification is needed for better patient prognosis. The key to this may be in using validated biomarkers with enhanced sensitivity and specificity. Several biomarkers are consistently used across various endocrine tumor types, possibly indicating a deeper pathophysiological mechanism behind endocrine cancer genesis and development. For example, carbohydrate antigen (CA) is measured in both pancreatic adenocarcinoma as well as ovarian cancer for diagnosis, surveillance, and risk stratification. The discovery of measuring miRNAs that are highly expressed in malignant tumors is also a novel strategy across multiple endocrine tumor types, and is propelling the future advancement of biomarker development. This review introduces currently utilized biomarkers in some of the commonly known endocrine tumors, including thyroid, adrenal, pituitary, pancreatic, and gonadal carcinoma, as well as future research directions.

PMID: 30250431 [PubMed]

Genomic insights into Cushing syndrome.

Tue, 10/02/2018 - 15:14
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Genomic insights into Cushing syndrome.

Ann Endocrinol (Paris). 2018 Jun;79(3):119-122

Authors: Assié G

Abstract
In the setting of Cushing syndrome, genomic analyses can be performed either in tumors responsible for endogenous Cushing, or in patients exposed to glucocorticoid excess. Genomics of tumors identified several new genes - including ZNRF3 in adrenocortical carcinomas, PRKACA in cortisol-producing adrenal adenomas, ARMC5 in primary macronodular adrenal hyperplasia and USP8 in pituitary corticotroph adenomas. These genes shed new lights on the mechanisms responsible for these tumors. Integrated genomic studies of adrenal carcinomas identified distinct molecular classes, with remarkably different prognostic outcome. Beyond the mechanistic novelties, a new generation of prognostic markers emerges, with potentially important impact on patients care. For the future, genomic efforts should be pursued, focusing on poorly characterized tumors responsible for Cushing syndrome - including endocrine tumors secreting ACTH. In addition, epigenomics is emerging as an outstanding set of tools for characterizing tumors, unraveling unprecedented aspects of tumorigenesis. Applying these tools to endocrine tumors responsible for Cushing syndrome may also lead to important discoveries. Genomics of patients exposed to glucocorticoid excess is an emerging research field. Proof of principle studies have been performed, identifying molecular markers of glucocorticoid excess in blood. Research efforts should now concentrate on markers of mild glucocorticoid excesses - endogenous or exogenous -, owing to their high prevalence in general population. In addition, markers of individual susceptibility to each type of glucocorticoid complication are needed. It remains to be determined whether genomics can identify such markers.

PMID: 29735160 [PubMed - indexed for MEDLINE]

Cardiovascular mortality in patients with subclinical Cushing.

Tue, 10/02/2018 - 15:14
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Cardiovascular mortality in patients with subclinical Cushing.

Ann Endocrinol (Paris). 2018 Jun;79(3):149-152

Authors: Morelli V, Arosio M, Chiodini I

Abstract
Patients with adrenal incidentaloma (AI) and subclinical hypercortisolism (SH) show a high prevalence of cardiovascular risk factors and an increased prevalence and incidence of cardiovascular events. Furthermore, some recent data suggest that in these patients, the cardiovascular mortality is also increased. Unfortunately, to date, the diagnosis of SH is still a matter of debate, and, therefore, it is still not possible to address the treatment of choice (i.e. surgical or conservative approach) in many AI patients. Overall, the available data show that in AI patients with established SH the surgical removal of the adrenal mass causing SH can lead to the improvement of hypertension and diabetes, but in many patients with possible SH the effect of surgery is still largely unknown. Finally, no data are available on the effect of the recovery from SH on the cardiovascular events. Therefore, randomized studies are needed to investigate the possibility of predicting the usefulness of surgery by using the available indexes of cortisol secretion in the individual AI patient. Finally, the development of safe and well-tolerated drugs aimed to control cortisol secretion will be among the goals of the future research.

PMID: 29606280 [PubMed - indexed for MEDLINE]

Metastatic pheochromocytoma in MEN 2A: A rare association.

Tue, 10/02/2018 - 15:14
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Metastatic pheochromocytoma in MEN 2A: A rare association.

BMJ Case Rep. 2018 Mar 28;2018:

Authors: Pal R, Rastogi A, Kumar S, Bhansali A

Abstract
A 45-year-old woman was diagnosed as having multiple endocrine neoplasia type 2A in 2014. She had bilateral pheochromocytoma, medullary thyroid carcinoma and biopsy-proven cutaneous lichen amyloidosis in the interscapular area. She underwent bilateral adrenalectomy; following which, she achieved clinical and biochemical remission. She was planned for total thyroidectomy at a later date; however, she was lost to follow-up. She presented to us again in December 2016 with abdominal pain. Examination revealed hypertension with postural drop. Positron emission tomography scan showed Ga68 and fluorodeoxyglucose (FDG)-avid suprarenal, hepatic, peritoneal and mesenteric masses with abdominal lymph nodes. Twenty-four-hour urinary metanephrines/normetanephrines were elevated. Serum calcitonin was as high as it was 2-1/2 years ago. Ultrasonography-guided fine-needle aspiration cytology (FNAC) from the liver mass revealed neuroendocrine cells that did not stain for calcitonin. Hence, a diagnosis of metastatic pheochromocytoma was made. She underwent total thyroidectomy and was started on cyclophosphamide, vincristine, dacarbazine-based chemotherapy regimen.

PMID: 29592980 [PubMed - indexed for MEDLINE]

Efficacy and safety of metyrosine in pheochromocytoma/paraganglioma: a multi-center trial in Japan.

Tue, 10/02/2018 - 15:14
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Efficacy and safety of metyrosine in pheochromocytoma/paraganglioma: a multi-center trial in Japan.

Endocr J. 2018 Mar 28;65(3):359-371

Authors: Naruse M, Satoh F, Tanabe A, Okamoto T, Ichihara A, Tsuiki M, Katabami T, Nomura M, Tanaka T, Matsuda T, Imai T, Yamada M, Harada T, Kawata N, Takekoshi K

Abstract
To assess the efficacy, safety, and pharmacokinetics of metyrosine (an inhibitor of catecholamine synthesis) in patients with pheochromocytoma/paraganglioma (PPGL), we conducted a prospective, multi-center, open-label study at 11 sites in Japan. We recruited PPGL patients aged ≥12 years requiring preoperative or chronic treatment, receiving α-blocker treatment, having baseline urinary metanephrine (uMN) or normetanephrine (uNMN) levels ≥3 times the upper limit of normal values, and having symptoms associated with excess catecholamine. Metyrosine treatment was started at 500 mg/day and modified according to dose-adjustment criteria up to 4,000 mg/day. The main outcome measure was the proportion of patients who achieved at least 50% reduction in uMN or uNMN levels from baseline. Sixteen patients (11 males/5 females) aged 12-86 years participated. After 12 weeks of treatment and at the last evaluation of efficacy, the primary endpoint was achieved in 31.3% of all patients, including 66.7% of those under preoperative treatment and 23.1% of those under chronic treatment. Sedation, anemia, and death were reported in 1 patient each as serious adverse drug reactions during the 24-week treatment. Metyrosine was shown to be tolerated and to relieve symptoms by reducing excess catecholamine in PPGL patients under both preoperative and chronic treatment.

PMID: 29353821 [PubMed - indexed for MEDLINE]

Paraganglioma as a risk factor for bone metastasis.

Tue, 10/02/2018 - 15:14
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Paraganglioma as a risk factor for bone metastasis.

Endocr J. 2018 Mar 28;65(3):253-260

Authors: Yokomoto-Umakoshi M, Umakoshi H, Tsuiki M, Ogasawara T, Hamanaka-Hiroshima K, Tanabe A, Tagami T, Shimatsu A, Naruse M

Abstract
Malignant pheochromocytoma (PHEO) and paraganglioma (PGL) (PHEO and PGL: PPGL) are frequently associated with bone metastasis. Bone metastasis requires long-term management and may lead to skeletal-related events (SREs) that remarkably reduce patients' quality of life (QOL). The aim of this study was to elucidate the risk factors for developing bone metastasis in patients with PPGL. The medical records of 40 consecutive adult patients with malignant PPGL at the National Hospital Organization Kyoto Medical Center between 2006 and 2016 were reviewed. SREs were defined as pathologic fracture, spinal cord compression, and the need for bone irradiation and/or surgery. PHEO (20/40) and PGL (20/40) were each present in 50% of the patients. Bone was the most frequent site of metastasis, detected in 60% (24/40). Bone metastasis was more frequent in patients with PGL (16/20, 80%) than in patients with PHEO (8/20, 40%) (p = 0.02). Half (12/24) of the patients with bone metastasis had at least one SRE. Extra-skeletal invasion of the spine, defined as local infiltration to the surrounding tissue beyond the cortical bone, was more frequently observed in patients with bone metastasis associated with SREs than without them (p = 0.001). Careful follow-up and management are warranted especially in patients with PGL as a risk factor for bone metastasis and with extra-skeletal invasion of the spine as risk factor of SREs.

PMID: 29187703 [PubMed - indexed for MEDLINE]

Tumor necrosis factor-associated periodic syndrome in adults.

Tue, 10/02/2018 - 15:14
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Tumor necrosis factor-associated periodic syndrome in adults.

Rheumatol Int. 2018 Jan;38(1):3-11

Authors: Menon SG, Efthimiou P

Abstract
Tumor necrosis factor-associated periodic syndrome is an autoinflammatory disorder classified under hereditary periodic fever syndromes. Mutations in the tumor necrosis factor receptor contribute to tumor necrosis factor-associated periodic syndrome. Decreased shedding of receptors and increased mitochondrial reactive oxygen species production leading to elevated proinflammatory cytokines are documented. Inflammation in various organs is hallmark of tumor necrosis factor-associated periodic syndrome and manifests as spiking fever, abdominal pain, conjunctivitis and polyserositis in adults. The ongoing challenge is to diagnose the disease early in its course to prevent amyloidosis. The treatment options have evolved from use of nonsteroidal anti-inflammatory drugs and corticosteroids to targeted therapy like tumor necrosis factor receptor inhibitors and interleukin-1 blockers. The aim of this review is to give an overview of the pathogenesis, clinical features and the various treatment modalities available for tumor necrosis factor-associated periodic syndrome and aid physicians in recognizing the signs of the disease earlier.

PMID: 28942479 [PubMed - indexed for MEDLINE]

adrenal tumor; +24 new citations

Tue, 09/25/2018 - 14:50

24 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

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Composite Paraganglioma: Pioneering in the Head and Neck.

Tue, 09/18/2018 - 12:31
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Composite Paraganglioma: Pioneering in the Head and Neck.

Int J Surg Pathol. 2018 Sep 16;:1066896918799940

Authors: Delgado S, Smith SM, Mehra S, Prasad ML

Abstract
Composite paragangliomas are rare with less than 20 cases documented in the abdomen, retroperitoneum, and urinary bladder. In this article, we report the first case of composite paraganglioma in the head and neck presenting as a soft tissue mass in the neck adjacent to the carotid artery in a 50-year-old woman. We discuss the clinicopathologic findings and genetic implications, and we review the literature of this rare entity.

PMID: 30222023 [PubMed - as supplied by publisher]

Clinical and molecular prognostic factors in adrenocortical carcinomas.

Tue, 09/18/2018 - 12:31
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Clinical and molecular prognostic factors in adrenocortical carcinomas.

Minerva Endocrinol. 2018 Sep 12;:

Authors: Libé R

Abstract
INTRODUCTION: Adrenocortical carcinoma (ACC) is a rare cancer, with an incidence less than 0.7-1.5 per 1 million people per year, with a poor prognosis. The overall survival (OS) depens on the ENSAT stage: in particular in metastatic ACC the OS varies between 10 and 20 months, with a 5-yrs survival around 10%. ACC present a different behaviour, probably due to a different biology. For these reason a careful prognostic classification is mandatory, in order to stratify the patients and purpose a specific management.
EVIDENCE ACQUISITION: Prognostic factors can be divides in three groups: clinical factors (tumor stage, Age, hormone- related symptoms), pathological factor (Weiss score, mitotic count, Ki67,SF-1 and AVA2, P53, beta-catenin immunohistochemistry, resection status), molecular factor (chromosomal aberrations, methylation profile, altered gene expression and miRNA expression, gene mutations).
EVIDENCE SYNTHESIS: The best way in order to stratify ACC patients and purpose the best therapeutic option is to combine clinical, pathological and molecular factors.
CONCLUSIONS: Individualizing patients' prognosis and tumor biology appears as a necessary step for personalized medicine. In addition to tumor stage and tumor grade, the genomic classification may precise the risk stratification and thus help defining therapeutic strategy.

PMID: 30221891 [PubMed - as supplied by publisher]

Autogenous transplants of adrenal fragments in an animal model.

Tue, 09/18/2018 - 12:31
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Autogenous transplants of adrenal fragments in an animal model.

Pol Przegl Chir. 2018 Jun 13;90(4):22-28

Authors: Dworzyńska A, Paduszyńska K, Pomorski L

Abstract
Introduction Adrenal insufficiency is a typical complication after surgical treatment of adrenal tumors, especially after the removal of both adrenal glands. Human beings are not able to survive without adrenal glands and without proper hormonal substitution. Autotransplantation of a fragment of the adrenal gland may prevent this complication. This can be done by transplanting the entire adrenal glands or its fragment, such as the adrenal cortex cells. In the case of adrenal tumors, the entire adrenal gland can not be transplanted. However, it is possible to transplant cells from the tumor-free part. Succesful adrenal autografts may result in a new treatment of adrenal insufficiency.
MATERIALS AND METHODS: Autograft transplantation was performed on 3 groups of Sprague Dawley rats. In the first group, physiological corticosterone concentrations were determined. These animals were not operated. In the second group, both adrenal glands were removed. Corticosterone concentrations were determined after bilateral adrenalectomy. The third group was divided into two parts. In the first subgroup, bilateral adrenalectomy was performed simultaneosly with adrenal transplant into the omentum. In the second subgroup, right adrenalectomy was performed simultaneosly with and adrenal transplant into the omentum followed a month later by left adrenalectomy. During the experiment, corticosterone concentrations were measured at 4 time points.
RESULTS: The statistical difference between corticosterone concentrations in rats after two timed adrenalectomies and rats after bilateral adrenalectomy was statistically different, but these results were far from physiological concentrations.

PMID: 30220673 [PubMed - in process]

[Clinical characteristics of silent pheochromocytoma].

Tue, 09/18/2018 - 12:31
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[Clinical characteristics of silent pheochromocytoma].

Zhonghua Yi Xue Za Zhi. 2018 Sep 11;98(34):2727-2731

Authors: Yang H, Wu HH, Bu N, Zhou L, Zhang JQ, Zhou LQ, Guo XH

Abstract
Objective: To summarize the clinical characteristics of silent paraganglioma. Methods: A total of 247 pheochromocytoma cases in Peking University First Hospital between January 1993 and December 2015 were analyzed retrospectively.The cases were divided into two groups according to whether they had hypertension: non-silent group (193 cases) and silent group (53 cases), then the clinical characteristics between the groups were compared, and the clinical features of silent pheochromocytoma were reviewed. Results: There were 53 silent pheochromocytoma cases in this study, which accounted for 21.5% (53/247), and imaging was the main way to find the tumor. Forty-one in 53 cases (77.4%) located in adrenal gland, in which 31 cases (75.6%) were benign and 2.3-8.0 cm in diameter, while 10 cases (24.4%) were malignant and 3.5-12.0 cm in diameter. Twelve in 53 cases (22.6%) located in extra-adrenal tissue, in which 4 cases were benign and 2.0-5.5 cm in diameter, while 8 cases were malignant and 5.0-10.5 cm in diameter. With the tumor diameter increased, the malignant rate increased: 1 in 8 cases in diameter<3 cm, 2 in 12 cases in diameter 3-5 cm, 15 in 33 cases in diameter>5 cm were malignant. There was no significant difference in age, sex, tumor location, benign or malignant rate between the two groups (all P>0.05). Compared with the non-silent group, the proportion of tumor size≥5 cm in silent group was significantly higher (62.3% vs 45.9%, P=0.034), and the incidence of hyperglycemia and the concentrations of norepinephrine and epinephrine were lower (all P<0.05) in silent group.Misdiagnosis was common in silent group, and up to 35.8% (19/53) had not been diagnosed correctly before operation. Twenty-one in 53 (39.6%) silent pheochromocytoma cases occured severe intra-operative blood pressure fluctuation. Conclusions: Silent pheochromocytoma was not uncommon and imaging was the main way to find it. The tumor size was always big and misdiagnosis was common, especially extra-adrenal tumors. Therefore, regardless of the adrenal or extra-adrenal tumors, especially in diameter>3.0 cm but with normal blood pressure, the possibility of silent pheochromocytoma should be considered. In order to reduce misdiagnosis and intra-operative blood pressure fluctuations, preoperative diagnosis and preparation, as well as intra-operative monitoring should be fully made.

PMID: 30220169 [PubMed - in process]

Laparoscopic Trans-Abdominal Right Adrenalectomy for a Large Primitive Adrenal Oncocytic Carcinoma: A Case Report and Review of Literature.

Tue, 09/18/2018 - 12:31
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Laparoscopic Trans-Abdominal Right Adrenalectomy for a Large Primitive Adrenal Oncocytic Carcinoma: A Case Report and Review of Literature.

Am J Case Rep. 2018 Sep 15;19:1096-1102

Authors: Panizzo V, Rubino B, Piozzi GN, Ubiali P, Morandi A, Nencioni M, Micheletto G

Abstract
BACKGROUND Adrenocortical oncocytic neoplasms (AONs) are extremely rare tumors. AONs are classified as: oncocytoma (AO), oncocytic neoplasm of uncertain malignant potential (AONUMP), and oncocytic carcinoma (AOC). Among the 162 reported cases of AONs in the literature, 30 cases were classified as malignant. Adrenalectomy is the treatment of choice for AON. CASE REPORT We report the case of a 48-year-old man with a primitive 12-cm mass affecting the right adrenal gland, detected by ultrasonography during follow-up for alcoholic liver cirrhosis. Computed tomography (CT) scan and magnetic resonance imaging (MRI) showed a mass of the right adrenal gland compressing the inferior vena cava (IVC) and dislocating the right lobe of the liver, with no invasion of kidney, liver, or IVC. Preoperative blood tests showed mild transaminase increase. Laparoscopic right adrenalectomy with lateral transperitoneal approach was performed. The postoperative course was uneventful. The lesion was diagnosed as a primitive adrenal oncocytic carcinoma (AOC). No recurrence was evidenced during 24-month follow-up. CONCLUSIONS Although AONs are very rare, they must be considered in the differential diagnosis of adrenal masses due to their prognostic difference compared to non-oncocytic tumors. AOCs are a rare presentation of AONs. Only 30 cases are described in the literature. Laparotomic adrenalectomy is the treatment of choice for AOC. We report the first case of laparoscopic lateral trans-abdominal adrenalectomy for a voluminous AOC (120×95×110 mm) and we review the literature regarding AOCs. Laparoscopy in experienced hands is safe and effective for the treatment of AONs. Despite the rarity of AOC, a case series should be performed to confirm the results of our case report.

PMID: 30217968 [PubMed - in process]

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