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NCBI: db=pubmed; Term=adrenal tumor
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Novel insights into the polycythemia-paraganglioma-somatostatinoma syndrome.

Sat, 12/30/2017 - 16:50
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Novel insights into the polycythemia-paraganglioma-somatostatinoma syndrome.

Endocr Relat Cancer. 2016 Dec;23(12):899-908

Authors: Därr R, Nambuba J, Del Rivero J, Janssen I, Merino M, Todorovic M, Balint B, Jochmanova I, Prchal JT, Lechan RM, Tischler AS, Popovic V, Miljic D, Adams KT, Prall FR, Ling A, Golomb MR, Ferguson M, Nilubol N, Chen CC, Chew E, Taïeb D, Stratakis CA, Fojo T, Yang C, Kebebew E, Zhuang Z, Pacak K

Abstract
Worldwide, the syndromes of paraganglioma (PGL), somatostatinoma (SOM) and early childhood polycythemia are described in only a few patients with somatic mutations in the hypoxia-inducible factor 2 alpha (HIF2A). This study provides detailed information about the clinical aspects and course of 7 patients with this syndrome and brings into perspective these experiences with the pertinent literature. Six females and one male presented at a median age of 28 years (range 11-46). Two were found to have HIF2A somatic mosaicism. No relatives were affected. All patients were diagnosed with polycythemia before age 8 and before PGL/SOM developed. PGLs were found at a median age of 17 years (range 8-38) and SOMs at 29 years (range 22-38). PGLs were multiple, recurrent and metastatic in 100, 100 and 29% of all cases, and SOMs in 40, 40 and 60%, respectively. All PGLs were primarily norepinephrine-producing. All patients had abnormal ophthalmologic findings and those with SOMs had gallbladder disease. Computed tomography (CT) and magnetic resonance imaging revealed cystic lesions at multiple sites and hemangiomas in 4 patients (57%), previously thought to be pathognomonic for von Hippel-Lindau disease. The most accurate radiopharmaceutical to detect PGL appeared to be [18F]-fluorodihydroxyphenylalanine ([18F]-FDOPA). Therefore, [18F]-FDOPA PET/CT, not [68Ga]-(DOTA)-[Tyr3]-octreotate ([68Ga]-DOTATATE) PET/CT is recommended for tumor localization and aftercare in this syndrome. The long-term prognosis of the syndrome is unknown. However, to date no deaths occurred after 6 years follow-up. Physicians should be aware of this unique syndrome and its diagnostic and therapeutic challenges.

PMID: 27679736 [PubMed - indexed for MEDLINE]

Preclinical progress and first translational steps for a liposomal chemotherapy protocol against adrenocortical carcinoma.

Sat, 12/30/2017 - 16:50
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Preclinical progress and first translational steps for a liposomal chemotherapy protocol against adrenocortical carcinoma.

Endocr Relat Cancer. 2016 Oct;23(10):825-37

Authors: Jung S, Nagy Z, Fassnacht M, Zambetti G, Weiss M, Reincke M, Igaz P, Beuschlein F, Hantel C

Abstract
Systemic therapy of adrenocortical carcinoma (ACC) is limited by heterogeneous tumor response and adverse effects. Recently, we demonstrated anti-tumor activity of LEDP-M (etoposide, liposomal doxorubicin, liposomal cisplatin, mitotane), a liposomal variant of EDP-M (etoposide, doxorubicin, cisplatin, mitotane). To improve the therapeutic efficacy and off-target profiles of the clinical gold standard EDP-M, we investigated liposomal EDP-M regimens in different preclinical settings and in a small number of ACC patients with very advanced disease. Short- and long-term experiments were performed on two ACC models (SW-13 and SJ-ACC3) in vivo We evaluated the anti-tumoral effects and off-target profiles of EDP-M, LEDP-M and a novel regimen L(l)EDP-M including liposomal etoposide. Furthermore, the role of plasma microRNA-210 as a therapeutic biomarker and first clinical data were assessed. Classical and liposomal protocols revealed anti-proliferative efficacy against SW-13 (EDP-M P < 0.01; LEDP-M: P < 0.001; L(l)EDP-M: P < 0.001 vs controls), whereas in SJ-ACC3, only EDP-M (P < 0.05 vs controls) was slightly effective. Long-term experiments in SW-13 demonstrated anti-tumor efficacy for all treatment schemes (EDP-M: P < 0.01, LEDP-M: P < 0.05, L(l)EDP-M P < 0.001 vs controls). The analysis of pre-defined criteria leading to study termination revealed significant differences for control (P < 0.0001) and EDP-M (P = 0.003) compared to L(l)EDP-M treatment. Raising its potential for therapy monitoring, we detected elevated levels of circulating microRNA-210 in SW-13 after LEDP-M treatment (P < 0.05). In contrast, no comparable effects were detectable for SJ-ACC3. However, overall histological evaluation demonstrated improved off-target profiles following liposomal regimens. The first clinical data indicate improved tolerability of liposomal EDP-M, thus confirming our results. In summary, liposomal EDP-M regimens represent promising treatment options to improve clinical treatment of ACC.

PMID: 27550961 [PubMed - indexed for MEDLINE]

Genetics of primary hyperaldosteronism.

Sat, 12/30/2017 - 16:50
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Genetics of primary hyperaldosteronism.

Endocr Relat Cancer. 2016 Oct;23(10):R437-54

Authors: Dutta RK, Söderkvist P, Gimm O

Abstract
Hypertension is a common medical condition and affects approximately 20% of the population in developed countries. Primary aldosteronism is the most common form of secondary hypertension and affects 8-13% of patients with hypertension. The two most common causes of primary aldosteronism are aldosterone-producing adenoma and bilateral adrenal hyperplasia. Familial hyperaldosteronism types I, II and III are the known genetic syndromes, in which both adrenal glands produce excessive amounts of aldosterone. However, only a minority of patients with primary aldosteronism have one of these syndromes. Several novel susceptibility genes have been found to be mutated in aldosterone-producing adenomas: KCNJ5, ATP1A1, ATP2B3, CTNNB1, CACNA1D, CACNA1H and ARMC5 This review describes the genes currently known to be responsible for primary aldosteronism, discusses the origin of aldosterone-producing adenomas and considers the future clinical implications based on these novel insights.

PMID: 27485459 [PubMed - indexed for MEDLINE]

Laparoscopic Surgery for Pheochromocytoma and Paraganglioma Removal: A Retrospective Analysis of Anaesthetic Management.

Sat, 12/30/2017 - 16:50
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Laparoscopic Surgery for Pheochromocytoma and Paraganglioma Removal: A Retrospective Analysis of Anaesthetic Management.

Curr Hypertens Rev. 2016;12(3):222-227

Authors: Ramachandran R, Rewari V, Sharma A, Kumar R, Trikha A

Abstract
INTRODUCTION: Minimal invasive approaches to pheochromocytoma (PCC) and paraganglioma (PGL) removal may be complicated by the hemodynamic disturbances that are associated with the catecholamine secretion from the tumour. The anaesthetic and perioperative monitoring techniques need to be customized to handle these complications effectively. This retrospective analysis was undertaken to review the perioperative management of these patients handled by the same anaesthetic and surgical team.
METHODS: Case details were collected and data analysed for the perioperative management of 29 patients who underwent laparoscopic removal of PCC and PGL. Parameters collected included details of preoperative alpha-and beta blockade, tumour size, number of hypertensive surges, dose of sodium nitroprusside (SNP) and other vasodilators used and incidence of postoperative hypotension and other complications.
RESULTS: All patients received prazosin for pre-operative optimization. Hypertensive emergencies were seen in 4 patients during induction and endotracheal intubation and in 1 patient during pneumoperitoneum insufflation. Overall mean number of hypertensive emergencies was 3.41 (SD-2.45). The patients undergoing PGL removal had significantly more crisis compared to those undergoing unilateral PCC removal. The dose of SNP used correlated significantly with tumour size.
CONCLUSION: Laparoscopic surgery for PCC and PGL removal is associated with hypertensive emergencies which are amenable to usual doses of antihypertensives used intraoperatively. Surgical factors like tumour size and location affect the number of crisis and the dose of anti-hypertensives used more than the anaesthetic drugs and procedures.

PMID: 27160411 [PubMed - indexed for MEDLINE]

What is the current role of biologics in the management of patients with severe refractory asthma?

Sat, 12/30/2017 - 16:50
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What is the current role of biologics in the management of patients with severe refractory asthma?

Ann Allergy Asthma Immunol. 2016 May;116(5):383-7

Authors: Draikiwicz S, Oppenheimer J

PMID: 27153737 [PubMed - indexed for MEDLINE]

Co-inhibition of EGFR and IGF1R synergistically impacts therapeutically on adrenocortical carcinoma.

Sat, 12/30/2017 - 16:50
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Co-inhibition of EGFR and IGF1R synergistically impacts therapeutically on adrenocortical carcinoma.

Oncotarget. 2016 Jun 14;7(24):36235-36246

Authors: Xu L, Qi Y, Xu Y, Lian J, Wang X, Ning G, Wang W, Zhu Y

Abstract
PURPOSE: Adrenocortical carcinoma (ACC) is a rare tumor with very poor prognosis and no effective treatment. The aim of this study was to explore a novel therapy co-targeting EGFR and IGF1R in vitro and vivo.
METHODS: The expression of EGFR and IGF1R were evaluated in a series of adrenocortical tumors by immunohistochemistry. Cell viability of ACC cell lines H295R and SW13 were determined by MTT assay after treatment with the combination of EGFR inhibitor Erlotinib and IGF1R inhibitor NVP-AEW541. Apoptosis was assessed by flow cytometry. The mechanism within intracellular signaling pathways was analyzed by Western blot. Mice bearing human ACC xenografts were treated with Erlotinib and NVP-AEW541, and the effects on tumour growth were assessed.
RESULTS: Our results show a significant over-expression of EGFR (66.67%) and IGF1R (80.0%) in ACC. Besides, the co-overexpression of EGFR and IGF1R was seen in 8/15 ACCs, as compared with ACAs (P<0.05). Erlotinib and NVP-AEW541 significantly inhibited cell viability and induced apoptosis by blocking phosphorylation of MEK/ERK and AKT, respectively. Meanwhile, we found that single inhibition of IGF1R induced compensatory activation of MEK/ERK, leading to sustained activation of mTOR, which represent as aggregation of EGFR and IGF1R downstream components. More importantly, the combination of Erlotinib and NVP-AEW541 enhances anti-tumour efficacy compared to treatment with either agent alone or to untreated control in vitro and vivo.
CONCLUSIONS: In conclusion, coinhibition therapy targeting EGFR and IGF1R may be considerable for treatment of ACC in the future.

PMID: 27105537 [PubMed - indexed for MEDLINE]

Lymph Node Collision Tumor Consisting of Metastatic Pulmonary Adenocarcinoma and Diffuse Large B-cell Lymphoma: A Case Report.

Sat, 12/23/2017 - 14:21
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Lymph Node Collision Tumor Consisting of Metastatic Pulmonary Adenocarcinoma and Diffuse Large B-cell Lymphoma: A Case Report.

Intern Med. 2017 Dec 21;:

Authors: Ikemura S, Moriyama M, Matsumoto K, Nakayama S, Matsuzaki T, Nakajima T, Sasaki A, Miyauchi J, Terashima T

Abstract
A 67-year-old man presented with a fever and general malaise. Computed tomography showed multiple nodules in the lungs and liver, associated with mediastinal and para-aortic lymphadenopathy. Bone marrow aspiration revealed diffuse large B-cell lymphoma (DLBCL). Renal and liver dysfunction and pancytopenia inhibited chemotherapy administration; the patient subsequently died of multiorgan failure. An autopsy revealed pulmonary adenocarcinoma with metastases to the lungs, liver, and adrenal glands; the DLBCL spread to the liver, spleen, and bone marrow. Adenocarcinoma and DLBCL collision was observed in the mediastinal and para-aortic lymph nodes. This was a rare case of collision metastasis occurring in the lymph node.

PMID: 29269648 [PubMed - as supplied by publisher]

A Novel Case of Somatic KCNJ5 Mutation in Pediatric-Onset Aldosterone-Producing Adenoma.

Sat, 12/23/2017 - 14:21
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A Novel Case of Somatic KCNJ5 Mutation in Pediatric-Onset Aldosterone-Producing Adenoma.

J Endocr Soc. 2017 Aug 01;1(8):1056-1061

Authors: Uchida N, Amano N, Yamaoka Y, Uematsu A, Sekine Y, Suzuki M, Watanabe J, Nishimoto K, Mukai K, Fukuzawa R, Hasegawa T, Ishii T

Abstract
Aldosterone-producing adenoma (APA), a subtype of primary aldosteronism, is a common cause of secondary hypertension in adults. Somatic KCNJ5 mutations have been identified in about 12%-80% of adult-onset APA. In contrast, there has been no previous reported case of pediatric-onset APA in whom a somatic KCNJ5 mutation was confirmed. We report an 11-year-old Japanese girl who had experienced recurrent headaches and nausea for more than 2 years before hypertension was observed (blood pressure, 150/82 mm Hg). Plasma renin activity was <0.1 ng/mL per hour even after a captopril-challenge or upright furosemide-loading test. Plasma aldosterone concentrations (PACs) before and after saline-infusion test were 28.0 and 40.6 ng/dL, respectively. Plasma adrenocorticotropic hormone (ACTH) and serum cortisol levels were 16.5 pg/mL and 16.7 µg/dL, respectively. The patient was diagnosed with APA in the left adrenal gland on the basis of selective adrenal venous sampling after ACTH stimulation (PAC in the left adrenal vein, 3630 ng/dL) and histopathologic findings of the tumor obtained by laparoscopic left adrenalectomy. Sanger sequencing of KCNJ5 using genomic DNA from peripheral lymphocytes and laser-captured microdissected APA tissues demonstrated the presence of a somatic KCNJ5 mutation p.L168R, previously reported only in adult-onset APA. Immunohistochemistry detected strong immunoreactivity for CYP11B2, but not for CYP11B1 in the APA, consistent with the endocrinologic findings in this patient. Somatic KCNJ5 mutations are also identified in pediatric-onset APA. Further cases are needed to elucidate functional characteristics of pediatric-onset APA with a somatic KCNJ5 mutation.

PMID: 29264557 [PubMed]

EGFR Induces E2F1-Mediated Corticotroph Tumorigenesis.

Sat, 12/23/2017 - 14:21
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EGFR Induces E2F1-Mediated Corticotroph Tumorigenesis.

J Endocr Soc. 2017 Feb 01;1(2):127-143

Authors: Araki T, Liu X, Kameda H, Tone Y, Fukuoka H, Tone M, Melmed S

Abstract
The epidermal growth factor receptor (EGFR), expressed in adrenocorticotrophic hormone (ACTH)-secreting pituitary adenomas causing Cushing disease, regulates ACTH production and corticotroph proliferation. To elucidate the utility of EGFR as a therapeutic target for Cushing disease, we generated transgenic (Tg) mice with corticotroph-specific human EGFR expression (corti-EGFR-Tg) using a newly constructed corticotroph-specific promoter. Pituitary-specific EGFR expression was observed by 2.5 months, and aggressive ACTH-secreting pituitary adenomas with features of Crooke's cells developed by 8 months with 65% penetrance observed. Features consistent with the Cushing phenotype included elevated plasma ACTH and corticosterone levels, increased body weight, glucose intolerance, and enlarged adrenal cortex. Gefitinib, an EGFR tyrosine kinase inhibitor, suppressed tumor POMC expression and downstream EGFR tumor signaling, and ACTH and corticosterone levels were attenuated by 80% and 78%, respectively. Both E2F1 and phosphorylated Ser-337 E2F1 were increased in corti-EGFR-Tg mice and also colocalized with human POMC (hPOMC) in human pituitary corticotroph tumor samples. EGFR inhibition reversed E2F1 activity in vivo, whereas E2F1 inhibition suppressed POMC and ACTH in cultured human pituitary tumor cells. The corti-EGFR-Tg phenotype recapitulates ACTH-secreting pituitary adenomas and Cushing disease, validating the relevance of EGFR to corticotroph tumorigenesis. E2F1 is identified as a promising corticotroph-specific target for ACTH-dependent Cushing disease.

PMID: 29264472 [PubMed]

Reversal of a Blunted Follicle-Stimulating Hormone by Chemotherapy in an Inhibin B-Secreting Adrenocortical Carcinoma.

Sat, 12/23/2017 - 14:21
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Reversal of a Blunted Follicle-Stimulating Hormone by Chemotherapy in an Inhibin B-Secreting Adrenocortical Carcinoma.

J Endocr Soc. 2017 Jan 01;1(1):46-50

Authors: Espiard S, Lahlou N, Sibony M, Louiset E, Bienvenu M, Bertherat J, Dousset B, Groussin L, Libé R

Abstract
Context: Adrenocortical carcinomas (ACCs) are revealed in 60% of cases by steroid hypersecretion. Alternatively, it is uncommon to observe a paraneoplastic syndrome due to a peptide oversecretion.
Case Description: We describe a 60-year-old man with a right adrenal mass. Hormonal evaluation showed an ACTH-independent Cushing syndrome. Surprisingly, follicle-stimulating hormone (FSH) levels were suppressed and blunted during gonadotropin-releasing hormone stimulation, despite normal luteinizing hormone levels. Levels of inhibin B, which negatively regulates the pituitary FSH, were very high. Given the atypical hormonal findings, an adrenal mass biopsy was performed, which allowed the diagnosis of an adrenocortical tumor (positive for steroidogenic factor-1 immunostaining). Moreover, an intense α-inhibin subunit immunostaining was observed. Because of the presence of metastases, the patient received mitotane and chemotherapy (etoposide and cisplatin). After 2 cycles, the inhibin B dropped. After 5 cycles, tumor size was reduced by 15%. Inhibin B levels remained low, and basal and gonadotropin-releasing hormone-stimulated FSH levels normalized. The patient underwent tumor resection, and pathology confirmed the ACC diagnosis (Weiss score of 9). The intensity of the α-inhibin subunit immunostaining was significantly decreased.
Conclusions: We report the case of an inhibin B-secreting ACC in which the response to chemotherapy and mitotane was associated with a normalization of inhibin B secretion, allowing the reversal of the blunted FSH secretion. Inhibin B should be measured in case of suppressed FSH levels despite normal luteinizing hormone levels and may be considered a tumoral marker in some ACCs, even during treatment follow-up.

PMID: 29264444 [PubMed]

Dynamic Adaptation of Tumor Immune Response With Nivolumab Demonstrated by 18F-FDG PET/CT.

Sat, 12/23/2017 - 14:21
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Dynamic Adaptation of Tumor Immune Response With Nivolumab Demonstrated by 18F-FDG PET/CT.

Clin Nucl Med. 2017 Dec 19;:

Authors: Eshghi N, Lundeen TF, Kuo PH

Abstract
A 61-year-old woman with lung adenocarcinoma failed first-line treatment and was placed on immunotherapy with nivolumab. FDG-PET/CT before immunotherapy showed metastases to thoracic nodes, liver, adrenal gland, and skeleton. Seven weeks after starting nivolumab, FDG-PET/CT showed mild residual activity in thoracic nodes and otherwise complete response. After 15 weeks, enlarged and FDG-avid axillary lymphadenopathy and worsening supraclavicular lymphadenopathy developed. After 20 weeks, FDG-PET/CT demonstrated marked improvement of axillary and supraclavicular lymphadenopathy. This case demonstrates that later progression of disease can still respond to continuing immunotherapy, hypothetically because of dynamic adaptations in the tug-of-war between the immunotherapy-augmented immune system and tumor.

PMID: 29261621 [PubMed - as supplied by publisher]

Leydig cell tumor of the testis, presenting with azoospermia.

Sat, 12/23/2017 - 14:21
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Leydig cell tumor of the testis, presenting with azoospermia.

Reprod Med Biol. 2017 Oct;16(4):392-395

Authors: Hibi H, Yamashita K, Sumitomo M, Asada Y

Abstract
Case: A case of Leydig cell tumor, associated with azoospermia, is presented.
Outcome: The levels of sex hormones obviously were decreased, including luteinizing hormone (LH) and follicle-stimulating hormone (FSH), with elevated testosterone. Computed tomography revealed no adrenal gland tumor, but a significant calcification in the right scrotal content was observed. He received a right radical orchiectomy and then he was unable to ejaculate. An endocrine panel revealed significantly decreased levels of testosterone and the low LH level had remained. Hormone replacement therapy with combined LH and FSH successfully recovered and preserved spermatogenesis.
Conclusions: Although the patient's sexual function deteriorated after surgery, hormone replacement therapy was successful in establishing spermatogenesis.

PMID: 29259494 [PubMed]

Complete remission of metastatic pheochromocytoma in 123I-metaiodobenzylguanidine scintigraphy after a single session of 131I-metaiodobenzylguanidine therapy: a case report.

Sat, 12/23/2017 - 14:21
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Complete remission of metastatic pheochromocytoma in 123I-metaiodobenzylguanidine scintigraphy after a single session of 131I-metaiodobenzylguanidine therapy: a case report.

BMC Res Notes. 2017 Dec 19;10(1):750

Authors: Sugino T, Ando R, Unno R, Iida K, Naiki T, Hamamoto S, Mizuno K, Okada A, Umemoto Y, Kawai N, Tozawa K, Hayashi Y, Inaki A, Kayano D, Kinuya S, Yasui T

Abstract
BACKGROUND: Pheochromocytomas are rare neuroendocrine tumors, with a malignancy frequency of approximately 10%. The treatment of malignant pheochromocytoma is palliative, and the traditional management strategy has limited efficacy. Furthermore, no clear criteria exist for the treatment of metastatic pheochromocytoma, especially for unresectable lesions. We report a case of complete remission of metastatic pheochromocytoma in 123I-metaiodobenzylguanidine (MIBG) scintigraphy after a single session of 131I-MIBG therapy.
CASE PRESENTATION: A 61-year-old woman had a right adrenal grand tumor and lymph node metastasis on the hilum of the right kidney, both of which incorporated MIBG. After surgery, immunostaining of a tumor specimen showed expression of the tumor makers chromogranin and synaptophysin. One year postoperatively, abdominal computed tomography revealed a local recurrence and retroperitoneal lymph node swelling. The local recurrence was positive for MIBG uptake, whereas the swollen retroperitoneal lymph nodes were negative. She underwent surgery again, but the local recurrence was unresectable because of rigid adhesion to the surrounding tissue. Immunostaining of an intraoperatively extracted swollen retroperitoneal lymph node showed expression of tumor markers. The patient then underwent a single session of 131I-MIBG therapy (7.4 GBq, 200 mCi), after which the residual lesions no longer incorporated MIBG, and a complete response in 123I- metaiodobenzylguanidine (MIBG) scintigraphy was achieved. The 131I-MIBG treatment was repeated 6 months later. None of the lesions were positive for MIBG uptake.
CONCLUSIONS: 131I-MIBG therapy efficaciously treats unresectable lesions that are positive for MIBG uptake.

PMID: 29258609 [PubMed - in process]

Functional extra-adrenal paraganglioma of the retroperitoneum giving thoracolumbar spine metastases after a five-year disease-free follow-up: a rare malignant condition with challenging management.

Sat, 12/23/2017 - 14:21
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Functional extra-adrenal paraganglioma of the retroperitoneum giving thoracolumbar spine metastases after a five-year disease-free follow-up: a rare malignant condition with challenging management.

Pan Afr Med J. 2017;28:94

Authors: Kapetanakis S, Chourmouzi D, Gkasdaris G, Katsaridis V, Eleftheriadis E, Givissis P

Abstract
Paragangliomas are benign neoplasms that arise from the autonomic nervous system and the associated paraganglia. Although benign, they have been shown to possess metastatic potential. Extra-adrenal retroperitoneal paraganglioma with vertebral metastasis is considered very uncommon. Here, we present a case of a functional extra-adrenal paraganglioma of the retroperitoneum giving metastasis to T4 vertebra after five years of follow-up in a 48-year-old man who had been initially treated with complete resection of the primary tumor. The condition of the patient improved significantly after radiosurgery and somatostatin analogs treatment, until lumbar spine lesions appeared six months later. Our case demonstrates that retroperitoneal paraganglioma is a rare condition which should be considered in the differential diagnosis of a retroperitoneal mass combined with vertebral lesions. Additionally, increased physician awareness and long-term follow-up is mandatory for all patients with history of retroperitoneal paraganglioma since metastases may occur after long latent intervals from the initial diagnosis.

PMID: 29255564 [PubMed - indexed for MEDLINE]

Innovation in endocrine surgery: robotic versus laparoscopic adrenalectomy. Meta-analysis and systematic literature review.

Sat, 12/23/2017 - 14:21
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Innovation in endocrine surgery: robotic versus laparoscopic adrenalectomy. Meta-analysis and systematic literature review.

Oncotarget. 2017 Nov 24;8(60):102392-102400

Authors: Agrusa A, Romano G, Navarra G, Conzo G, Pantuso G, Buono GD, Citarrella R, Galia M, Monte AL, Cucinella G, Gulotta G

Abstract
Several studies in the last years demonstrated the better surgical outcome of laparoscopic approach to adrenal gland. Laparoscopic surgery is more difficult to learn and requires different psychomotor skills than open surgery, especially with regard to complex maneuvers requiring precision and dexterity. The development of robotic platform with three-dimensional vision and increased degrees of freedom of the surgical instruments has the aim to overcome these problems. We performed a systematic literature review with meta-analysis to evaluate preoperative data and surgical outcomes of robotic adrenalectomy compared with laparoscopic technique. In September 2016 we performed a systematic literature review using the Pubmed, Scopus and ISI web of knowledge database with search term "robotic adrenalectomy". We identified 13 studies with eligible criteria that compared surgical outcomes. This present systematic review with meta-analysis includes 798 patients: 379 underwent to robotic adrenalectomy (cases group) and 419 to laparoscopic adrenalectomy (controls group). There were no significant differences between the two groups of patients respect to age, gender, laterality and tumor size. BMI instead was significant lower in the robotic group. In this group we found also patients with higher incidence of previous abdominal surgery. The results from operative time demonstrated lower operative time for laparoscopic group but there were no significant differences with robotic group. Robotic adrenalectomy showed a significant lower blood loss. Robotic adrenalectomy is a safe and feasible technique with reduced blood loss and shorter hospital stay than laparoscopic adrenalectomy. Laparoscopic approach seems to be a more rapid technique when comparing to robotic technique, although recent studies demonstrate a significant operative time reduction in robotic group with the learning curve improvement and the development of new surgical technology.

PMID: 29254254 [PubMed]

Mass effect: a plethora of symptoms caused by an otherwise benign transitional pituitary meningioma. Case report.

Sat, 12/23/2017 - 14:21
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Mass effect: a plethora of symptoms caused by an otherwise benign transitional pituitary meningioma. Case report.

Rom J Morphol Embryol. 2017;58(3):983-988

Authors: Bădilă E, Weiss AE, Bartoş D, Dumitrache EL, Tătăranu LG, Ciubotaru GV, Neagu TP, Enache V, Popa VB, Japie C

Abstract
Meningiomas are among the most commonly encountered tumors of the central nervous system, being more frequent in females. We present the case of a dyslipidemic male patient, previously diagnosed with coronary artery disease for which he previously underwent percutaneous coronary intervention with the placement of two bare metal stents on the left anterior descending artery. He was presented to the emergency department for atypical angina and a seven-day history of dizziness when switching from clino- to orthostatism, reduced visual acuity, diplopia and vomiting. Electrocardiogram (ECG), both at rest and exercise test were suggestive for myocardial ischemia. Echocardiography revealed myocardial hypokinesia in the territory of the right coronary artery and of the left descending artery, while coronarography showed insignificant intra-stent stenosis. Imaging techniques revealed a frontobasal extraneuraxial mass, creating a compressive effect on both middle cerebral arteries and on the optic chiasm as well as thickening of the dura mater adjacent to the mass. Endocrinology blood tests showed hypocortisolemia, hyperprolactinemia and low levels of free thyroxine (fT4), suggesting secondary combined pituitary hormone deficiency. The patient underwent surgery and total resection of the tumor was performed. Definite diagnosis - transitional meningioma - was obtained through histological examination and immunohistochemistry. The key feature of this case was the extra-cardiac cause of angina accompanied by ECG abnormalities in a patient with stable coronary heart disease, in whom the clinical presentation was secondary to blood pressure variations in the context of pituitary and adrenal deficiency.

PMID: 29250678 [PubMed - in process]

Primary atrial fibromyxosarcoma with multiple-system metastases: A case report.

Sat, 12/23/2017 - 14:21
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Primary atrial fibromyxosarcoma with multiple-system metastases: A case report.

Medicine (Baltimore). 2017 Dec;96(49):e8930

Authors: Qiu J, Tang L, Han Y, Wang C, Gu T, Li G

Abstract
RATIONALE: Fibromyxosarcoma is common in head and neck, vessel, omentum, and reproductive system, with low-grade malignant behavior. However, primary atrial fibromyxosarcoma with highly malignant behavior is extremely rare.
PATIENT CONCERNS: A 34-year-old female presented with oppression in the chest, short breath, and onset of headache as initial symptoms. The preoperative echocardiogram showed a medium-size echogenic mass close to the posterior leaflet of the mitral valve in the left atrium.
DIAGNOSIS: Primary atrial fibromyxosarcoma with multiple-system metastases.
INTERVENTIONS: The patient underwent surgery, and the tumor was removed completely. The diagnosis of left atrium fibromyxosarcoma was confirmed through postoperative histopathological examination. Positron emission tomography/computed tomography scan was performed, which revealed multiple metastases to left adnexa, bilateral adrenal glands, left iliacus, right lateral ventricle, and skeletal system.
OUTCOMES: The patient died of cerebral hernia caused by hemorrhage from the metastatic brain tumor, 30 days after the surgery, without receiving chemotherapy or radiotherapy.
LESSONS: Cardiac fibromyxosarcoma is a rare primary malignant cardiac neoplasm, probably with systemic metastases. The possibility of malignancy should be considered as differential diagnosis for cardiac mass.

PMID: 29245258 [PubMed - in process]

Calneuron 1 Increased Ca2+ in the Endoplasmic Reticulum and Aldosterone Production in Aldosterone-Producing Adenoma.

Sat, 12/23/2017 - 14:21
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Calneuron 1 Increased Ca2+ in the Endoplasmic Reticulum and Aldosterone Production in Aldosterone-Producing Adenoma.

Hypertension. 2018 Jan;71(1):125-133

Authors: Kobuke K, Oki K, Gomez-Sanchez CE, Gomez-Sanchez EP, Ohno H, Itcho K, Yoshii Y, Yoneda M, Hattori N

Abstract
Aldosterone production is initiated by angiotensin II stimulation and activation of intracellular Ca2+ signaling. In aldosterone-producing adenoma (APA) cells, the activation of intracellular Ca2+ signaling is independent of the renin-angiotensin-aldosterone systems. The purpose of our study was to clarify molecular mechanisms of aldosterone production related to Ca2+ signaling. Transcriptome analysis revealed that the CALN1 gene encoding calneuron 1 had the strongest correlation with CYP11B2 (aldosterone synthase) among genes encoding Ca2+-binding proteins in APA. CALN1 modulation and synthetic or fluorescent compounds were used for functional studies in human adrenocortical carcinoma (HAC15) cells. CALN1 expression was 4.4-fold higher in APAs than nonfunctioning adrenocortical adenomas. CALN1 expression colocalized with CYP11B2 expression as investigated using immunohistochemistry in APA and zona glomerulosa of male rats fed by a low-salt diet. CALN1 expression was detected in the endoplasmic reticulum (ER) by using GFP-fused CALN1, CellLight ER-RFP, and the corresponding antibodies. CALN1-overexpressing HAC15 cells showed increased Ca2+ in the ER and cytosol fluorescence-based studies. Aldosterone production was potentiated in HAC15 cells by CALN1 expression, and dose-responsive inhibition with TMB-8 showed that CALN1-mediated Ca2+ storage in ER involved sarcoendoplasmic reticulum calcium transport ATPase. The silencing of CALN1 decreased Ca2+ in ER, and abrogated angiotensin II- or KCNJ5 T158A-mediated aldosterone production in HAC15 cells. Increased CALN1 expression in APA was associated with elevated Ca2+ storage in ER and aldosterone overproduction. Suppression of CALN1 expression prevented angiotensin II- or KCNJ5 T158A-mediated aldosterone production in HAC15 cells, suggesting that CALN1 is a potential therapeutic target for excess aldosterone production.

PMID: 29109191 [PubMed - indexed for MEDLINE]

Äntligen! Nytt program för adrenala incidentalom.

Sat, 12/23/2017 - 14:21
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Äntligen! Nytt program för adrenala incidentalom.

Lakartidningen. 2017 Sep 29;114:

Authors: Zedenius J

PMID: 28972642 [PubMed - indexed for MEDLINE]

Nationellt vårdprogram för adrenala incidentalom - Programmet har harmoniserats med europeiska riktlinjer – ger förenklad handläggning av patienter.

Sat, 12/23/2017 - 14:21
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Nationellt vårdprogram för adrenala incidentalom - Programmet har harmoniserats med europeiska riktlinjer – ger förenklad handläggning av patienter.

Lakartidningen. 2017 Sep 29;114:

Authors: Muth A, Hammarstedt L, Hellström M, Nyman U, Sundin A, Wahrenberg H, Wängberg B

Abstract
Swedish guidelines for the management of adrenal incidentalomas  Adrenal incidentalomas are seen in about five percent of abdominal CT examinations, and in most cases represent non-hormone-producing adrenocortical adenomas, but hormone-producing or malignant lesions occur. Revised Swedish guidelines for the evaluation and management of adrenal incidentalomas based on recently published European guidelines are presented. The importance of a thorough radiological, clinical and biochemical initial evaluation is emphasized. Long-term biochemical follow-up is not recommended and use of CT contrast medium »washout« calculation is omitted. No radiological evaluation or follow-up indicated for adrenal incidentalomas <1 cm size. For patients with diagnosed lipid rich adenomas (≤ 10 HU) 1-4 cm in size no radiological follow-up is suggested after initial evaluation.

PMID: 28972640 [PubMed - indexed for MEDLINE]

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