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Comparison of plasma free metanephrines between healthy dogs and 3 dogs with pheochromocytoma.

Fri, 05/15/2015 - 02:43
Related Articles

Comparison of plasma free metanephrines between healthy dogs and 3 dogs with pheochromocytoma.

Vet Clin Pathol. 2013 Dec;42(4):499-503

Authors: Green BA, Frank EL

Abstract
BACKGROUND: Adrenomegaly and hypertension are common clinical entities in canine medicine for which testing for pheochromocytoma is recommended. Yet, a validated biochemical test for the diagnosis of pheochromocytoma in dogs does not exist. In human medicine, plasma free metanephrine testing is the diagnostic standard for the biochemical diagnosis of pheochromocytoma.
OBJECTIVES: The purpose of this study was to investigate the utility of measurement of plasma free metanephrines in dogs for the diagnosis of pheochromocytoma.
METHODS: Plasma free metanephrines were measured in 11 healthy dogs and in 3 dogs confirmed to have a pheochromocytoma. The metanephrine assays were performed at a reference laboratory using high-performance liquid chromatography with electrochemical detection.
RESULTS: The plasma free metanephrine and normetanephrine concentrations in 11 healthy dogs were normally distributed and were used to create tentative reference intervals. All 3 dogs with histologically confirmed pheochromocytoma clearly had higher concentrations of plasma free metanephrines.
CONCLUSIONS: This pilot study demonstrates the potential utility of plasma free metanephrines levels for the biochemical diagnosis of pheochromocytoma in dogs.

PMID: 24320781 [PubMed - indexed for MEDLINE]

Myxoid variant of adrenocortical carcinoma: a report of two illustrative cases and a brief review of the literature.

Fri, 05/15/2015 - 02:43
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Myxoid variant of adrenocortical carcinoma: a report of two illustrative cases and a brief review of the literature.

Pathology. 2014 Jan;46(1):83-5

Authors: Gurzu S, Szentirmay Z, Bara T, Bara T, Jung I

PMID: 24300732 [PubMed - indexed for MEDLINE]

MAX mutations status in Swedish patients with pheochromocytoma and paraganglioma tumours.

Fri, 05/15/2015 - 02:43
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MAX mutations status in Swedish patients with pheochromocytoma and paraganglioma tumours.

Fam Cancer. 2014 Mar;13(1):121-5

Authors: Crona J, Maharjan R, Delgado Verdugo A, Stålberg P, Granberg D, Hellman P, Björklund P

Abstract
Pheochromocytoma (PCC) and Paraganglioma are rare tumours originating from neuroendocrine cells. Up to 60% of cases have either germline or somatic mutation in one of eleven described susceptibility loci, SDHA, SDHB, SDHC, SDHD, SDHAF2, VHL, EPAS1, RET, NF1, TMEM127 and MYC associated factor-X (MAX). Recently, germline mutations in MAX were found to confer susceptibility to PCC and paraganglioma (PGL). A subsequent multicentre study found about 1% of PCCs and PGLs to have germline or somatic mutations in MAX. However, there has been no study investigating the frequency of MAX mutations in a Scandinavian cohort. We analysed tumour specimens from 63 patients with PCC and PGL treated at Uppsala University hospital, Sweden, for re-sequencing of MAX using automated Sanger sequencing. Our results show that 0% (0/63) of tumours had mutations in MAX. Allele frequencies of known single nucleotide polymorphisms rs4902359, rs45440292, rs1957948 and rs1957949 corresponded to those available in the Single Nucleotide Polymorphism Database. We conclude that MAX mutations remain unusual events and targeted genetic screening should be considered after more common genetic events have been excluded.

PMID: 23743562 [PubMed - indexed for MEDLINE]

Catecholamine Metabolism in Paraganglioma and Pheochromocytoma: Similar Tumors in Different Sites?

Fri, 05/08/2015 - 00:46
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Catecholamine Metabolism in Paraganglioma and Pheochromocytoma: Similar Tumors in Different Sites?

PLoS One. 2015;10(5):e0125426

Authors: Grouzmann E, Tschopp O, Triponez F, Matter M, Bilz S, Brändle M, Drechser T, Sigrist S, Zulewski H, Henzen C, Fischli S, Abid K

Abstract
Pheochromocytoma (PHEO) and paraganglioma (PGL) are catecholamine-producing neuroendocrine tumors that arise respectively inside or outside the adrenal medulla. Several reports have shown that adrenal glucocorticoids (GC) play an important regulatory role on the genes encoding the main enzymes involved in catecholamine (CAT) synthesis i.e. tyrosine hydroxylase (TH), dopamine β-hydroxylase (DBH) and phenylethanolamine N-methyltransferase (PNMT). To assess the influence of tumor location on CAT metabolism, 66 tissue samples (53 PHEO, 13 PGL) and 73 plasma samples (50 PHEO, 23 PGL) were studied. Western blot and qPCR were performed for TH, DBH and PNMT expression. We found a significantly lower intra-tumoral concentration of CAT and metanephrines (MNs) in PGL along with a downregulation of TH and PNMT at both mRNA and protein level compared with PHEO. However, when PHEO were partitioned into noradrenergic (NorAd) and mixed tumors based on an intra-tumoral CAT ratio (NE/E >90%), PGL and NorAd PHEO sustained similar TH, DBH and PNMT gene and protein expression. CAT concentration and composition were also similar between NorAd PHEO and PGL, excluding the use of CAT or MNs to discriminate between PGL and PHEO on the basis of biochemical tests. We observed an increase of TH mRNA concentration without correlation with TH protein expression in primary cell culture of PHEO and PGL incubated with dexamethasone during 24 hours; no changes were monitored for PNMT and DBH at both mRNA and protein level in PHEO and PGL. Altogether, these results indicate that long term CAT synthesis is not driven by the close environment where the tumor develops and suggest that GC alone is not sufficient to regulate CAT synthesis pathway in PHEO/PGL.

PMID: 25946206 [PubMed - as supplied by publisher]

Downregulation of miR-375 in aldosterone-producing adenomas promotes tumor cell growth via MTDH.

Fri, 05/08/2015 - 00:46
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Downregulation of miR-375 in aldosterone-producing adenomas promotes tumor cell growth via MTDH.

Clin Endocrinol (Oxf). 2015 May 6;

Authors: He J, Cao Y, Su T, Jiang Y, Jiang L, Zhou W, Zhang C, Wang W, Ning G

Abstract
OBJECTIVE: Previous studies have investigated the genetic and molecular basis of primary aldosteronism (PA), a common cause of human hypertension, but the effects of microRNAs (miRNAs) on the adrenocortical cell proliferation and aldosterone production are largely obscure. Here, we characterized miRNA expression patterns in the subtypes of PA to gain a better understanding of its pathogenesis.
METHODS: miRNA expression was assessed by microarray profiling analysis in aldosterone-producing adenoma (APA), unilateral adrenal hyperplasia (UAH) and normal adrenal cortex tissues. Selected differentially expressed miRNAs were further validated in a validation cohort by qRT-PCR. A gain-of-function approach was used to explore the functional role of the specific miRNA in vitro.
RESULTS: 31 miRNAs including miR-375, miR-7, miR-29b were found to be significantly differentially expressed among these three groups. miR-375 was the most downregulated one in adrenal cortex tissues from PA patients and its expression level was inversely correlated with the tumor size in APA. Overexpression of miR-375 in a human adrenocortical cell line (H295R) reduced cell proliferation and suppressed the expression of MTDH (metadherin, also known as Astrocyte Elevated Gene-1). Moreover, MTDH was verified as a direct target of miR-375 through luciferase reporter assays. Knockdown of MTDH in H295R cells attenuated Akt-Ser473 phosphorylation and inhibited cell viability.
CONCLUSION: Our findings suggest that miR-375 exerts its tumor-suppressive function via targeting MTDH/Akt pathway and implicate a potential therapeutic target in PA. This article is protected by copyright. All rights reserved.

PMID: 25944465 [PubMed - as supplied by publisher]

False Positive Radioiodinated Metaiodobenzylguanidine ((123)I-MIBG) Uptake in Undifferentiated Adrenal Malignant Tumor.

Thu, 04/30/2015 - 22:43

False Positive Radioiodinated Metaiodobenzylguanidine ((123)I-MIBG) Uptake in Undifferentiated Adrenal Malignant Tumor.

Case Rep Oncol Med. 2015;2015:164280

Authors: Jung HS, Moon SJ, Kim YM, Kang HR, Lee SM, Jung SJ, Choi SJ, Kim TK, Kwon MJ, Park JH, Lee SH

Abstract
(123)I-Metaiodobenzylguanidine ((123)I-MIBG) scintigraphy is a widely used functional imaging tool with a high degree of sensitivity and specificity in diagnosis of pheochromocytoma. However, rare cases of false positive reactions have been reported. A 67-year-old male patient was admitted with epigastric pain. Abdominal computed tomography (CT) revealed a heterogeneous left adrenal mass 6 cm in diameter; following hormone testing, (123)I-MIBG scintigraphy was performed to determine the presence of pheochromocytoma, which confirmed eccentric uptake by a large left adrenal gland mass. Chest CT and PET-CT confirmed metastatic lymphadenopathy; therefore, endobronchial ultrasound transbronchial needle aspiration was performed. Metastatic carcinoma of unknown origin was suspected from a lymph node biopsy, and surgical resection was performed for definitive diagnosis and correction of excess hormonal secretion. A final diagnosis of undifferentiated adrenal malignant tumor was rendered, instead of histologically malignant pheochromocytoma, despite the uptake of (123)I-MIBG demonstrated by scintigraphy.

PMID: 25918656 [PubMed]

Immunohistochemical Expression of Stem Cell Markers in Pheochromocytoma/Paraganglioma is Associated with SDHx-mutations.

Thu, 04/30/2015 - 22:43

Immunohistochemical Expression of Stem Cell Markers in Pheochromocytoma/Paraganglioma is Associated with SDHx-mutations.

Eur J Endocrinol. 2015 Apr 27;

Authors: Oudijk L, Neuhofer CM, Lichtenauer UD, Papathomas TG, Korpershoek E, Stoop H, Oosterhuis JW, Smid M, Restuccia DF, Robledo M, de Cubas AA, Mannelli M, Gimenez-Roqueplo AP, Dinjens WN, Beuschlein F, de Krijger RR

Abstract
OBJECTIVE: Pheochromocytomas (PCC) are neuroendocrine tumors occurring in the adrenal medulla, while paragangliomas (PGL) arise from paraganglia in the head, neck, thorax or abdomen. In a variety of tumors, cancer cells with stem cell-like properties seem to form the basis of tumor initiation by their ability of self-renewal and proliferation. Specifically targeting this small cell population may lay the foundation for more effective therapeutic approaches. In this study we intend to identify stem cells in PCC/PGL.
METHODS: We examined the immunohistochemical expression of 11 stem cell markers (SOX2, LIN28, NGFR, THY1, PREF1, SOX17, NESTIN, CD117, OCT3/4, NANOG, CD133) on tissue microarrays containing 208 PCCs/PGLs of different genetic backgrounds from 5 European centers.
RESULTS: SOX2, LIN28, NGFR, and THY1 were expressed in more than 10% of tumors and PREF1, SOX17, NESTIN, and CD117 were expressed in less than 10% of samples. OCT3/4, NANOG and CD133 were not detectable at all. Double-staining for Chromogranin A/SOX2 and S100/SOX2 demonstrated SOX2 immunopositivity in both tumor and adjacent sustentacular cells. Expression of SOX2, SOX17, NGFR, LIN28, PREF1 and THY1 was significantly associated with mutations in one of the succinate dehydrogenase (SDH) genes. In addition, NGFR expression was significantly correlated with metastatic disease.
CONCLUSIONS: Immunohistochemical expression of stem cell markers was found in a subset of PCC/PGL. Further studies are required to validate if some stem cell-associated markers such as SOX2 could serve as targets for therapeutic approaches and whether NGFR expression could be utilized as a predictor for malignancy.

PMID: 25916394 [PubMed - as supplied by publisher]

Characteristics of clinical N0 metastatic non-small cell lung cancer.

Thu, 04/30/2015 - 22:43
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Characteristics of clinical N0 metastatic non-small cell lung cancer.

Lung Cancer. 2015 Apr 13;

Authors: Tamura T, Kurishima K, Watanabe H, Shiozawa T, Nakazawa K, Ishikawa H, Satoh H, Hizawa N

Abstract
OBJECTIVES: Non-small cell lung cancer (NSCLC) patients who have clinically no mediastinal lymph node metastasis but have distant metastasis are occasionally found in clinical practice. Such clinical N0 metastatic NSCLC may be a different subtype from the clinical N1-3 patients with regional lymph node metastasis. The aim of this study was to evaluate the prognosis, clinical features, and incidence of clinical N0 NSCLC patients with metastasis.
METHODS: All metastatic NSCLC patients (n=761) diagnosed at our hospitals from April 1999 to August 2012 were retrospectively analyzed. They were divided into two groups: N0 and N1-3. Staging was recorded according to the UICC 7th edition of the TNM classification. Differences between the two groups were analyzed using a Chi-square test. Prognostic factors were analyzed by the Kaplan-Meier method and Cox proportional hazards analysis. A probability value less than 0.05 was considered to be significant.
RESULTS: A total of 761 patients with NSCLC were registered. 124 patients (16.3%) were N0 and 637 (83.7%) were N1-3. There were no differences between the two groups in age, sex, smoking history, performance status, and histological type. The ratio of adrenal gland metastasis was low in the N0 group (N0 7.3%, N1-3 13.4%, p=0.002). Median survival time was longer in the N0 group (N0 11.9 months vs N1-3 7.2 months, p<0.001). N0 was an independent favorable prognostic factor.
CONCLUSION: Metastatic NSCLC patients with clinical N0 had a favorable prognosis and a lower ratio of adrenal gland metastasis than those with clinical N1-3. Our results suggest that a certain type of adrenal metastasis may result from direct lymphatic spread from a primary lung tumor. About one sixth of metastatic NSCLC cases are clinical N0. Therefore, clinical evaluations for detecting metastasis are important even in clinical N0 patients.

PMID: 25913230 [PubMed - as supplied by publisher]

Needlescopic-assisted laparoendoscopic single-site adrenalectomy.

Thu, 04/30/2015 - 22:43
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Needlescopic-assisted laparoendoscopic single-site adrenalectomy.

Asian J Surg. 2015 Apr 23;

Authors: Inoue S, Kajiwara M, Teishima J, Matsubara A

Abstract
OBJECTIVE: Our objective was to compare the perioperative parameters of needle-assisted and conventional laparoendoscopic single-site adrenalectomy (LESS-A).
METHODS: We compared 23 patients undergoing needle-assisted LESS-A with 29 patients undergoing conventional LESS-A at Hiroshima University Hospital between November 2009 and February 2014. Needle-assisted LESS-A was performed using a MiniLap instrument (Stryker, San Jose, CA, USA). We used this instrument to protectively retract the liver at the right side of the tumor and the spleen at the left side by grasping with a Securea endoscopic surgical spacer (Hogy Medical Co., Ltd., Tokyo, Japan). Various parameters including insufflation time, estimated blood loss, pain scale, resumption of oral intake, transfusion rate, and complications were analyzed using the Mann-Whitney U test.
RESULTS: In all cases, LESS-A was completed successfully with no major intraoperative complications. Patients in both treatment groups had similar age, body mass index, sex, and laterality. Significantly, needle-assisted LESS-A was performed using the transumbilical approach rather than the subcostal approach. The insufflation time of the needle-assisted LESS-A was shorter than that of the conventional LESS-A (p = 0.0335). No patients required intraoperative or postoperative blood transfusions. Retrospective design and the small sample size are main limitations of this study.
CONCLUSION: Needle-assisted LESS-A was performed safely and in a manner that mitigated many of the difficulties of LESS surgery.

PMID: 25912104 [PubMed - as supplied by publisher]

Characterization of adrenal lesions at chemical-shift MRI: a direct intraindividual comparison of in- and opposed-phase imaging at 1.5 T and 3 T.

Thu, 04/30/2015 - 22:43
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Characterization of adrenal lesions at chemical-shift MRI: a direct intraindividual comparison of in- and opposed-phase imaging at 1.5 T and 3 T.

AJR Am J Roentgenol. 2015 Mar;204(3):536-41

Authors: Ream JM, Gaing B, Mussi TC, Rosenkrantz AB

Abstract
OBJECTIVE. The purpose of this article is to perform an intraindividual comparison between 1.5 T and 3 T chemical-shift MRI in differentiating adrenal adenomas and nonadenomas, including comparison of quantitative thresholds. MATERIALS AND METHODS. In this retrospective study, 37 adrenal lesions in 36 patients (20 men and 16 women; mean [± SD] age, 66.7 ± 12.9 years; 27 benign adenomas in 27 patients; 10 nonadenomas in nine patients) imaged at 1.5 T and 3 T were identified. Two readers qualitatively assessed intralesional signal loss between in- and opposed-phase images. One reader placed ROIs on adrenal lesions, spleen, liver, and muscle. Quantitative measures of signal loss, such as signal intensity (SI) index, adrenal-to-spleen ratio, adrenal-to-liver ratio, and adrenal-to-muscle ratio, were calculated. Qualitative and quantitative measures between field strengths were assessed with McNemar test and ROC analysis, respectively. RESULTS. Accuracy in qualitative adenoma identification (86.5% [32/37] at 1.5 T and 81.1% [30/37] at 3 T for reader 1; 81.1% [30/37] at 1.5 T and 83.8% [31/37] at 3 T for reader 2; both p ≥ 0.180) was equivalent at both field strengths. AUCs were not statistically significantly different between field strengths for quantitative measures: AUCs at 1.5 T versus 3 T were 0.956 versus 0.915 for SI index, 0.963 versus 0.870 for adrenal-to-spleen ratio, 0.935 versus 0.852 for adrenal-to-liver ratio, and 0.948 versus 0.948 for adrenal-to-muscle ratio (all p > 0.11). The optimal threshold for SI index was lower at 3 T (> 7.4%) than at 1.5 T (> 21.6%) but had similar sensitivity (1.5 T, 92.6% [25/27]; 3 T, 88.9% [24/27]) and specificity (1.5 T, 90.0% [9/10]; 3 T, 90.0% [9/10]). CONCLUSION. Chemical-shift imaging has similar diagnostic efficacy for differentiating adrenal adenomas and nonadenomas at 1.5 T and 3 T. However, quantitative measures have different thresholds for this differentiation at 3 T; in particular, the commonly applied SI index is much lower at 3 T.

PMID: 25714282 [PubMed - indexed for MEDLINE]

Sunitinib for refractory malignant pheochromocytoma: two case reports.

Thu, 04/23/2015 - 20:42
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Sunitinib for refractory malignant pheochromocytoma: two case reports.

Int Urol Nephrol. 2014 Jul;46(7):1309-12

Authors: Hata J, Haga N, Ishibashi K, Takahashi N, Ogawa S, Kataoka M, Akaihata H, Satoh Y, Koguchi T, Kojima Y

Abstract
Sunitinib, a molecular-targeted therapy, is a potential new treatment strategy for malignant pheochromocytoma. However, because of the rarity of malignant pheochromocytoma and the consequent limited number of patients available for clinical study, there is no good evidence of the efficacy of sunitinib for malignant pheochromocytoma. The present report describes our experience with sunitinib for refractory malignant pheochromocytoma. Two patients were treated with sunitinib at a standard dose (50 mg daily; 4 weeks on, 2 weeks off) after cyclophosphamide/vinblastine/dacarbazine chemotherapy, because vascular endothelial growth factor (VEGF)-positive cells were partly observed by immunohistochemical staining. Both patients were assessed as having stable disease according to the Response Evaluation Criteria in Solid Tumors 1.1. The duration of overall survival from the time sunitinib was initiated was 13 and 8 months, respectively, and the progression-free survival was 5 and 4 months, respectively. Adverse events were evaluated according to the Common Terminology Criteria for adverse events of the US Department of Health and Human Services version 4.0. One patient experienced hypothyroidism (Grade 2) and thrombocytopenia (Grade 2). The other patient experienced anorexia (Grade 3) and general malaise (Grade 3). In conclusion, sunitinib was effective in the treatment of malignant pheochromocytoma when VEGF-positive cells were observed in the tumor specimens.

PMID: 24510251 [PubMed - indexed for MEDLINE]

Calcific malignant pheochromocytoma.

Thu, 04/23/2015 - 20:42
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Calcific malignant pheochromocytoma.

QJM. 2014 Jul;107(7):577-8

Authors: Chen CT, Chu HC

PMID: 24170888 [PubMed - indexed for MEDLINE]

[Primary neuroendocrine tumor of the kidney : A rarity].

Thu, 04/16/2015 - 18:46
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[Primary neuroendocrine tumor of the kidney : A rarity].

Urologe A. 2015 Apr 16;

Authors: Maletzki P, Diener PA, Bjasch D, Schmid HP, Gramann T

Abstract
This article presents a rare case of a primary neuroendocrine tumor (NET) of the kidney. Abdominal magnetic resonance imaging (MRI) was performed in a 56-year-old man during the follow-up of an adrenal adenoma. Incidentally a renal tumor at the upper pole of the left kidney was diagnosed and was suspected of being a renal cell carcinoma. After partial nephrectomy a NET measuring 4.2 cm in diameter could be diagnosed histologically. An extrarenal primary tumor was ruled out by a postoperative gallium 68-tetraazacyclododecane tetraacetic acid-octreotate (68Ga-DOTATATE) positron emission tomography/computed tomography (PET/CT) scan. A primary NET of the genitourinary tract is rare and less than 100 cases have been reported in the medical literature.

PMID: 25875931 [PubMed - as supplied by publisher]

Diagnosis, treatment and outcome of adrenocortical cancer.

Thu, 04/16/2015 - 18:46
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Diagnosis, treatment and outcome of adrenocortical cancer.

Br J Surg. 2015 Mar;102(4):291-306

Authors: Mihai R

Abstract
BACKGROUND: Adrenocortical cancer (ACC) is a rare disease with a dismal prognosis. The majority of patients are diagnosed with advanced disease and raise difficult management challenges.
METHODS: All references identified in PubMed, published between 2004 and 2014, using the keywords 'adrenocortical cancer' or 'adrenal surgery' or both, were uploaded into a database. The database was interrogated using keywords specific for each field studied.
RESULTS: In all, 2049 publications were identified. There is ongoing debate about the feasibility and oncological outcomes of laparoscopic adrenalectomy for small ACCs, and data derived from institutional case series have failed to provide an evidence level above expert opinion. The use of mitotane (1-(2-chlorophenyl)-1-(4-chlorophenyl)-2,2-dichloroethane) in combination with chemotherapy in the treatment of metastatic disease has been assessed in an international randomized trial (FIRM-ACT trial) involving patients with ACC. Based on this trial, mitotane plus etoposide, doxorubicin and cisplatin is now the established first-line cytotoxic therapy owing to a higher response rate and longer median progression-free survival than achieved with streptozocin-mitotane. For patients with tumours smaller than 5 cm and with no signs of lymph node or distant metastases, survival is favourable with a median exceeding 10 years. However, the overall 5-year survival rate for all patients with ACC is only 30 per cent.
CONCLUSION: Open and potentially laparoscopic adrenalectomy for selected patients is the main treatment for non-metastatic ACC, but the overall 5-year survival rate remains low.

PMID: 25689291 [PubMed - indexed for MEDLINE]

Progress in primary aldosteronism. Mineralocorticoid antagonist treatment for aldosterone-producing adenoma.

Thu, 04/09/2015 - 17:23
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Progress in primary aldosteronism. Mineralocorticoid antagonist treatment for aldosterone-producing adenoma.

Eur J Endocrinol. 2015 Mar;172(3):R125-9

Authors: Amar L, Lorthioir A, Azizi M, Plouin PF

Abstract
Mineralocorticoid receptor antagonists have been used in patients with aldosterone-producing adenomas (APAs) as a test designed to predict the blood pressure (BP) outcome of surgery. They are commonly used in patients undergoing adrenalectomy to reduce BP and increase plasma potassium levels during the preoperative period. A small number of studies have compared the effects of surgery and mineralocorticoid antagonists either on BP, on serum potassium levels, or on the incidence of cardiovascular and renal outcomes in patients with primary aldosteronism with or without an APA; these studies found no difference between the two therapeutic options. Mineralocorticoid receptor antagonists can be used as a maintenance treatment for patients with APAs, who are judged to be poor operative risks or who do not want to undergo surgery.

PMID: 25315875 [PubMed - indexed for MEDLINE]

The combination of insulin-like growth factor receptor 1 (IGF1R) antibody cixutumumab and mitotane as a first-line therapy for patients with recurrent/metastatic adrenocortical carcinoma: a multi-institutional NCI-sponsored trial.

Thu, 04/09/2015 - 17:23
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The combination of insulin-like growth factor receptor 1 (IGF1R) antibody cixutumumab and mitotane as a first-line therapy for patients with recurrent/metastatic adrenocortical carcinoma: a multi-institutional NCI-sponsored trial.

Horm Cancer. 2014 Aug;5(4):232-9

Authors: Lerario AM, Worden FP, Ramm CA, Hesseltine EA, Hasseltine EA, Stadler WM, Else T, Shah MH, Agamah E, Rao K, Hammer GD

Abstract
Adrenocortical carcinoma (ACC) is an aggressive malignancy, which lacks an effective systemic treatment. Abnormal activation of insulin-like growth factor receptor 1 (IGF1R) has been frequently observed. Preclinical studies demonstrated that pharmacological inhibition of IGF1R signaling in ACC has antiproliferative effects. A previous phase I trial with an IGF1R inhibitor has demonstrated biological activity against ACC. The objective of this study is to assess the efficacy of the combination of the IGF1R inhibitor cixutumumab (IMC-A12) in association with mitotane as a first-line treatment for advanced/metastatic ACC. We conducted a multicenter, randomized double-arm phase II trial in patients with irresectable recurrent/metastatic ACC. The original protocol included two treatment groups: IMC-A12 + mitotane and mitotane as a single agent, after an initial single-arm phase for safety evaluation with IMC-A12 + mitotane. IMC-A12 was dosed at 10 mg/kg intravenously every 2 weeks. The starting dose for mitotane was 2 g daily, subsequently adjusted according to serum levels/symptoms. The primary endpoint was progression-free survival (PFS) according to RECIST (Response Evaluation Criteria in Solid Tumors). This study was terminated before the randomization phase due to slow accrual and limited efficacy. Twenty patients (13 males, 7 females) with a median age of 50.2 years (range 21.9-79.6) were enrolled for the single-arm phase. Therapeutic effects were observed in 8/20 patients, including one partial response and seven stable diseases. The median PFS was 6 weeks (range 2.66-48). Toxic events included two grade 4 (hyperglycemia and hyponatremia) and one grade 5 (multiorgan failure). Although the regimen demonstrated activity in some patients, the relatively low therapeutic efficacy precluded further studies with this combination of drugs.

PMID: 24849545 [PubMed - indexed for MEDLINE]

The diagnostic role of (18)F-FDG PET for primary central nervous system lymphoma.

Thu, 04/09/2015 - 17:23
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The diagnostic role of (18)F-FDG PET for primary central nervous system lymphoma.

Ann Nucl Med. 2014 Aug;28(7):603-9

Authors: Yamaguchi S, Hirata K, Kobayashi H, Shiga T, Manabe O, Kobayashi K, Motegi H, Terasaka S, Houkin K

Abstract
OBJECTIVE: (18)F-FDG PET has become one of the most important methods for studying malignant lymphoma, but its diagnostic role for primary central nervous system lymphoma (PCNSL) has not been established. The aim of this study was to determine the appropriate cut-off values of FDG uptake and to investigate how corticosteroid administration influences PCNSL.
METHODS: We retrospectively reviewed 82 patients with contrast-enhanced brain tumors who underwent an FDG PET scan at onset, including 19 PCNSLs. FDG uptake of the lesion was assessed by the maximum standardized uptake value (SUVmax) and the ratio of tumor to normal contralateral cortex activity (T/N ratio). Receiver operating characteristic (ROC) curves were generated from the SUVmax and T/N ratios. To investigate the influence of corticosteroid application before a FDG PET scan, we evaluated the association between the FDG uptake of the lesion and the cumulative dose of corticosteroid administration on 13 PCNSL patients who had received steroid treatment before an FDG PET examination.
RESULTS: The mean FDG SUVmax and T/N ratio of PCNSLs were 22.6 and 2.79, respectively, and these values were significantly higher than those of the other malignant brain tumors. ROC analysis indicated that the evaluation of FDG uptake using the T/N ratio was more reliable than the SUVmax with respect to the differential diagnosis. When PCNSL patients went without steroid application before FDG PET, the accuracy of the T/N ratio with a cut-off point of 2.0 was 91.1%, the sensitivity was 94.7%, and the specificity was 87.3%. Although there are no significant differences in the FDG T/N ratio for PCNSL patients with or without steroid treatment, a negative correlation was found between the T/N ratio and cumulative dose of corticosteroid before PET study (r = -0.71, p = 0.032).
CONCLUSIONS: We concluded that the T/N ratio was superior to SUVmax for FDG uptake assessment as for distinguishing PCNSLs from other malignant brain tumors; the appropriate T/N ratio cut-off point was 2.0. In addition, FDG uptake could be influenced by cumulative doses of corticosteroid before a PET scan, and thus this fact should be taken into consideration when evaluating FDG PET for PCNSL diagnosis.

PMID: 24743915 [PubMed - indexed for MEDLINE]

A pancreatic pseudopapillary tumor enucleated curatively.

Thu, 04/02/2015 - 14:55
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A pancreatic pseudopapillary tumor enucleated curatively.

Int J Surg Case Rep. 2015 Mar 25;10:118-120

Authors: Karakas S, Dirican A, Soyer V, Koç S, Ersan V, Ates M

Abstract
INTRODUCTION: Pseudopapillary tumors (PPT) of the pancreas are very rare, comprising 0.3-2.7% of all pancreatic tumors, and they occur mostly in young women. Generally, they are benign, but in rare cases they can enlarge, invade adjacent organs, and metastasize distantly. Radiological assessments and biochemical markers are important for diagnosing tumor characteristics. The main treatment is tumor resection.
PRESENTATION OF CASE: An 18-year-old female was referred to our department suffering from abdominal discomfort and upper quadrant abdominal pain. Abdominal computed tomography (CT) revealed a 6-×5-cm mass between the pancreatic head and right adrenal gland (Fig. 1). The histological assessment was a solid PPT of the pancreas with intact surgical borders.
DISCUSSION: PPT are very rare, comprising approximately 5% of cystic pancreatic tumors and ∼1% of exocrine pancreatic neoplasms and present mainly during the second and third decades of life. PPTs are usually indolent tumors. As such, they tend to produce vague nonspecific symptoms or may be detected incidentally on imaging. Complete surgical resection (R0) is the most effective therapy for PPT.
CONCLUSION: Although PPT is a very rare, benign tumor, it has the potential to metastasize to adjacent and distant organs. Consequently, they should be detected early, so that they can be treated surgically before malignant conversion.

PMID: 25828476 [PubMed - as supplied by publisher]

Randomized clinical trial of posterior retroperitoneoscopic adrenalectomy versus lateral transperitoneal laparoscopic adrenalectomy with a 5-year follow-up.

Thu, 04/02/2015 - 14:55
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Randomized clinical trial of posterior retroperitoneoscopic adrenalectomy versus lateral transperitoneal laparoscopic adrenalectomy with a 5-year follow-up.

Ann Surg. 2014 Nov;260(5):740-7; discussion 747-8

Authors: Barczyński M, Konturek A, Nowak W

Abstract
OBJECTIVE: To test if posterior retroperitoneoscopic adrenalectomy (PRA) is superior to lateral transperitoneal laparoscopic adrenalectomy (LTLA).
BACKGROUND: Most popular LTLA has been recently challenged by an increasing popularity of PRA, which is believed by many surgeons (not evidence-based) as superior to LTLA in the treatment of small and benign adrenal tumors.
METHODS: Participants were assigned randomly to PRA or LTLA and followed for 5 years after surgery. The primary endpoint was the duration of surgery. Secondary endpoints were blood loss, conversion rate, postoperative recovery, morbidity, and costs.
RESULTS: Sixty-five patients were included, of whom 61 (PRA 30, LTLA 31) completed the 5-year follow-up. The following differences were identified in favor of PRA vs LTLA: shorter duration of surgery (50.8 vs 77.3 minutes), lower intraoperative blood loss (52.7 vs 97.8 mL), diminished pain intensity within 48 hours postoperatively, lower prevalence of shoulder-tip pain (3.0% vs 37.5%), shorter time to oral intake (4.4 vs 7.3 hours), shorter time to ambulation (6.1 vs 11.5 hours), shorter length of hospital stay, and lower cost (1728 € vs 2315 €), respectively (P<0.001 for all). No differences were noted in conversion rate or morbidity except for herniation occurring more often after LTLA than PRA (16.1% vs 0%, P=0.022) and need for hernia repair (12.9% vs 0%, P=0.050), respectively.
CONCLUSIONS: Both approaches were equally safe. However, outcomes of PRA operations were superior to LTLA in terms of shorter surgery duration, lower blood loss, lower postoperative pain, faster recovery, improved cost-effectiveness, and abolished risk of surgical access site herniation.
REGISTRATION NUMBER: NCT01959711 (http://www.clinicaltrials.gov).

PMID: 25243546 [PubMed - indexed for MEDLINE]

Adrenal and extra-adrenal myelolipomas - a comparative case report.

Thu, 04/02/2015 - 14:55
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Adrenal and extra-adrenal myelolipomas - a comparative case report.

J Radiol Case Rep. 2014 Jan;8(1):1-12

Authors: Hakim A, Rozeik C

Abstract
Myelolipoma is an uncommon benign tumour composed of mature fat tissue and haematopoietic elements and is most commonly found in the adrenal gland. We report a case, which was discovered incidentally on chest X-ray, of a rare occurrence of multifocal extra-adrenal myelolipoma in the thoracic paravertebral region. This was further investigated with multi-detector computed tomography and magnetic resonance imaging. The presumed diagnosis, of extra-adrenal myelolipoma, was histologically confirmed via tissue sample obtained by computed tomography guided biopsy. We compare the adrenal and extra-adrenal entities from the perspective of published literature and also review the cases, published in Pubmed, of extra-adrenal myelolipomas in order to summarize the different locations of this lesion.

PMID: 24967008 [PubMed - indexed for MEDLINE]

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