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NCBI: db=pubmed; Term=adrenal tumor
Updated: 2 days 22 min ago

Total Laparoscopic Management for Stage IV Colorectal Cancer Requiring Multivisceral Resection.

Thu, 05/18/2017 - 03:56
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Total Laparoscopic Management for Stage IV Colorectal Cancer Requiring Multivisceral Resection.

Ann Surg Oncol. 2017 May 16;:

Authors: You YN, Shiozaki H, Lee JE, Passot G, Goumard C, Okuno M, Aloia TA, Eng C, Chang G, Vauthey JN, Conrad C

Abstract
BACKGROUND: Surgical resection of all sites of disease, in combination with effective systemic chemotherapy, offers the only potential chance for cure for patients with stage IV colorectal cancer (CRC). Coordinated multistage resection using a minimally invasive approach may provide optimal oncologic outcome while potentially offering the benefit of decreased morbidity.
PATIENT: A 66-year-old women presented with transverse colon cancer and synchronous metastasis (CRLM) in segment IV involving the middle hepatic vein and main left portal pedicle, as well as the left adrenal gland. Due to favorable response to neoadjuvant chemotherapy (FOLFOX/bevacizumab), the patient was considered for resection but developed some obstructive symptoms from the primary tumor, necessitating re-coordination of treatment sequencing from the 'liver-first' approach.
METHODS: The first procedure combined laparoscopic subtotal colectomy (extracorporeal anastomosis) with left adrenalectomy. After restaging, CRLM was removed separately 2 months later via laparoscopic left hepatectomy extending beyond the middle hepatic vein. Successful completion of the two procedures depended on optimal patient/port positioning for the combined colon/adrenal surgery and the second-stage liver resection. Postoperative lengths of stay were 4 and 3 days, respectively, and were without complication. Adjuvant FOLFOX was initiated 21 days following liver surgery, and the patient has been disease-free for 36 months.
CONCLUSION: This case illustrates the feasibility of the total laparoscopic approach to multivisceral resection for synchronous stage IV CRC in the context of a preplanned, staged multidisciplinary strategy. This approach may offer optimal cancer management, including early return to systemic therapy, shortened time intervals between stages, and minimal postoperative morbidity.1 (-) 3.

PMID: 28510798 [PubMed - as supplied by publisher]

Adrenal ganglioneuroma: The Padua Endocrine Surgery Unit experience.

Thu, 05/18/2017 - 03:56
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Adrenal ganglioneuroma: The Padua Endocrine Surgery Unit experience.

Int J Surg. 2017 May;41 Suppl 1:S103-S108

Authors: Iacobone M, Torresan F, Citton M, Schiavone D, Viel G, Favia G

Abstract
BACKGROUND: Adrenal ganglioneuroma (AGN) is a rare tumor that originates from the gangliar cells of the sympathetic nervous system. It represents less than 5% of all adrenal masses. AGN occurs as a large mass, with benign behavior and no relevant symptoms and hormonal secretion, but it is often misdiagnosed because the preoperative radiological diagnosis is generally challenging. The aim of the present paper is to report the experience of a tertiary referral academic center regarding the management of AGN and review the relevant literature.
METHODS: The demographic, diagnostic, surgical, and pathological findings of 666 consecutive patients who were adrenalectomized at the Padua Endocrine Surgery Unit between 1990 and 2015 were retrospectively reviewed in order to focus on AGN.
RESULTS: The pathology confirmed AGN in 10 patients (1.5% of cases; median age 35 years, range 19-73). The diagnosis was incidental and never available before surgery. Eight patients were asymptomatic, two presented lower back and abdominal pain. The mass was non-secreting in all cases. Preoperative imaging techniques were inconclusive about the nature of the mass in all cases. The median size of the tumor was 55 mm (range 30-80). The laparoscopic approach was performed in 60% of cases. No recurrences occurred after surgery at a median follow-up of 10 years (range 1.7-18.2).
CONCLUSION: The diagnosis of AGN may be challenging and most often occurs after surgery, since it is frequently confirmed by histological examination. Radical excision may be achieved by laparoscopic adrenalectomy, with a subsequent definitive cure and excellent prognosis at long term follow up.

PMID: 28506406 [PubMed - in process]

Luteolin-induced apoptosis through activation of endoplasmic reticulum stress sensors in pheochromocytoma cells.

Thu, 05/18/2017 - 03:56
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Luteolin-induced apoptosis through activation of endoplasmic reticulum stress sensors in pheochromocytoma cells.

Mol Med Rep. 2017 May 12;:

Authors: Kwon K, Kwon YS, Kim SW, Yu K, Lee KH, Kwon OY

Abstract
Luteolin [2-(3,4-dihydroxyphenyl)-5,7-dihydroxy-4-chromenone] is an active flavonoid compound from Lonicera japonica (Caprifoliaceae). Luteolin inhibits tumor cell proliferation, inflammatory and oxidative stress better, when compared with other flavonoids. In the present study, it was demonstrated that luteolin induces typical apoptosis in PC12 cells (derived from a pheochromocytoma of the rat adrenal medulla) accompanied by DNA fragmentation and formation of apoptotic bodies. In addition, luteolin regulates expression of the endoplasmic reticulum (ER) chaperone binding immunoglobulin protein, activating ER stress sensors (eukaryotic initiation factor 2α phosphorylation and X‑box binding protein 1 mRNA splicing) and induced autophagy. The results indicated that luteolin induces the upregulation of the unfolded protein response pathway through the ER stress sensors, which helps as an influential regulator for the apoptosis pathway in PC12 cells. The results suggested that the understanding of the molecular mechanisms underlying luteolin‑induced apoptosis may be useful in cancer therapeutics, chemoprevention and neurodegenerative diseases, such as Parkinson's disease and Alzheimer's disease.

PMID: 28498401 [PubMed - as supplied by publisher]

Robot-assisted adrenalectomy: indications and drawbacks.

Thu, 05/18/2017 - 03:56
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Robot-assisted adrenalectomy: indications and drawbacks.

Updates Surg. 2017 May 12;:

Authors: Nomine-Criqui C, Germain A, Ayav A, Bresler L, Brunaud L

Abstract
Adrenal tumors can vary from a benign adrenocortical adenoma with no hormonal secretion to a secretory adrenocortical malignancy (adrenocortical carcinoma) or a hormone-secreting tumor of the adrenal medulla (pheochromocytoma). Currently, laparoscopic adrenalectomy is regarded as the preferred surgical approach for the management of most adrenal surgical disorders, although there are no prospective randomized trials comparing this technique with open adrenalectomy. However, widespread adoption of robotic technology has positioned robotic adrenalectomy as an option in some medical centers. Speculative advantages associated with the use of the robotic system have rarely been evaluated in clinical settings and cost increase remains an important drawback associated with robotic surgery. This review summarizes current available data regarding robotic transperitoneal adrenalectomy including its indications, advantages, limitations, and comparison with conventional laparoscopic adrenalectomy. We believe that the use of a robotic system seems to be useful especially in more difficult patients with larger tumors, truncal paragangliomas, and bilateral and/or partial adrenalectomies. Overall, we believe that overcosts due to robotic system use could be balanced by hospital stay decrease, patients' referral increase, improved postoperative outcomes in more difficult patients and ergonomics for the surgeon. However, we also believe that the current surgical intuitive business model is counterproductive, because there are no available strong clinical data that could balance overcosts associated with the use of the robotic system.

PMID: 28497219 [PubMed - as supplied by publisher]

TAK-264 (MLN0264) in Previously Treated Asian Patients with Advanced Gastrointestinal Carcinoma Expressing Guanylyl Cyclase C: Results from an Open-label, Non-randomized Phase 1 Study.

Thu, 05/18/2017 - 03:56
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TAK-264 (MLN0264) in Previously Treated Asian Patients with Advanced Gastrointestinal Carcinoma Expressing Guanylyl Cyclase C: Results from an Open-label, Non-randomized Phase 1 Study.

Cancer Res Treat. 2017 May 10;:

Authors: Bang YJ, Takano T, Lin CC, Fasanmade A, Yang H, Danaee H, Asato T, Kalebic T, Wang H, Doi T

Abstract
Purpose: This phase 1 dose-escalation portion of the study evaluated the safety, pharmacokinetics (PK), and antitumor activity of TAK-264 in Asian patients with advanced gastrointestinal (GI) carcinoma or metastatic or recurrent gastric or gastroesophageal junction adenocarcinoma expressing guanylyl cyclase C (GCC).
Materials and Methods: Adult patients with advanced GI malignancies expressing GCC (H-score ≥ 10) received TAK-264 on day 1 of 3-week cycles as 30-minute intravenous infusions for up to 1 year or until disease progression or unacceptable toxicity. The primary objectives were to evaluate the safety profile including dose-limiting toxicities (DLTs) during cycle 1, determine the maximum tolerated dose (MTD), and characterize the PK profile of TAK-264 (NCT02391038).
Results: Twelve patients were enrolled and treated with 1.2 mg/kg (n=3), 1.5 mg/kg (n=3), or 1.8 mg/kg TAK-264 (n=6). Median number of treatment cycles received was two (range, 1-10). None of the patients experienced a DLT and the MTD was not determined. Ten patients (83%) experienced adverse events (AEs). The most common were neutropenia, anorexia, and nausea (each reported by four patients). Five patients (42%) experienced grade ≥3 AEs consisting of tumor hemorrhage and hypertension, ascites, adrenal insufficiency, neutropenia and asthenia. Serum exposure toTAK-264 increased proportionally with the dose and the median half-life was approximately 5.5-6.6 days. No patients experienced an objective response.
Conclusions: TAK-264 demonstrated a manageable safety profile with limited antitumor activity consistent with studies conducted in Western patients with advanced GI malignancies. TAK-264 exposure increased proportionally with the dose.

PMID: 28494535 [PubMed - as supplied by publisher]

Primary lung carcinoid metastatic to the breast.

Thu, 05/18/2017 - 03:56
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Primary lung carcinoid metastatic to the breast.

Radiol Case Rep. 2017 Jun;12(2):223-228

Authors: Zagurovskaya M, Tran-Harding K, Gibbs R

Abstract
Lung carcinoid tumors account for approximately 2% of lung cancers, with 10% of the tumors represented by the atypical type. While atypical carcinoids are metastatic to intrathoracic lymph nodes in approximately half of the cases on the initial presentation, distant metastases are seen in only 20% of the patients and are found most frequently in bones, liver, adrenal glands, and brain. We present a case of an unusual metastatic disease to the breast in 51-year-old female who developed a new breast mass 2 years after left lower lobectomy due to atypical carcinoid tumor. Atypical pulmonary carcinoid metastases to the breast are exceptionally uncommon, yet they are important considerations for appropriate management, especially with an anamnesis of this neoplasm.

PMID: 28491156 [PubMed - in process]

Clear cell renal cell carcinoma: a comparative study of histological and chromosomal characteristics between primary tumors and their corresponding metastases.

Thu, 05/18/2017 - 03:56
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Clear cell renal cell carcinoma: a comparative study of histological and chromosomal characteristics between primary tumors and their corresponding metastases.

Virchows Arch. 2017 May 10;:

Authors: Dagher J, Kammerer-Jacquet SF, Dugay F, Beaumont M, Lespagnol A, Cornevin L, Verhoest G, Bensalah K, Rioux-Leclercq N, Belaud-Rotureau MA

Abstract
Clear cell renal cell carcinoma (ccRCC) has a poor prognosis with a 50% risk of metastases. Little is known about the phenotypic and molecular profiles of metastases regarding their corresponding primary tumors. This study aimed to screen phenotypic and genotypic differences between metastases and their corresponding primary tumors. We selected four cases with available frozen material. The histological, immunohistochemical (VEGFA, CD31, SMA, Ki67, p53, PAR-3), FISH (VHL gene), next-generation sequencing (VHL and c-MET genes), multiplex ligation-dependent probe amplification, and array-(comparative genomic hybridization) CGH analyses were realized. Metastases were nodal, hepatic (synchronous), adrenal, and pulmonary (metachronous). High-grade tumor cells were significantly more frequent in metastases (p = 0.019). Metastases and high-grade zones of primary tumors shared similar characteristics compared to low-grade zones: a lower microscopic vascular density (43.5 vs 382.5 vessels/mm(2); p = 0.0027), a higher expression of VEGF (73 vs 10%, p = 0.045), Ki67 (37.6 vs 8.3%; p = 0.011), and p53 (54 vs 10.6%; p = 0.081), and a cytoplasmic and membranous PAR-3 staining. Metastases exhibited more chromosomal imbalances than primary tumors in total (18.75 ± 6.8; p = 0.044) with more genomic gains (13.5 ± 7; p = 0.013). The loss of chromosome 9 and gain of Xq were found in both primary tumors and metastases but gains of loci or chromosomes 2p, 3q, 5, 8q, 12, and 20 were only found in metastases. The VHL gene status was similar in each tumor couple. Although metastases and primary tumors share common histological features, this study highlights chromosomal differences specific to metastases which could be involved in ccRCC metastatic evolution.

PMID: 28488172 [PubMed - as supplied by publisher]

Adrenocortical carcinoma masquerading as Cushing's disease.

Thu, 05/18/2017 - 03:56
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Adrenocortical carcinoma masquerading as Cushing's disease.

BMJ Case Rep. 2017 Mar 29;2017:

Authors: Jarial KD, Walia R, Kumar S, Bhansali A

Abstract
Cushing's syndrome (CS) can be classified as adrenocorticotropic hormone (ACTH)-dependent or ACTH-independent depending on the ACTH levels. However, 30% of the patients with CS have ACTH levels in the 'grey zone' (5-20 pg/mL), thereby posing a challenge in establishing the aetiological diagnosis. In a patient with full-blown features of Cushing's syndrome with equivocal ACTH levels, and a pituitary microadenoma on contrast-enhanced MRI sella, can falsely lead to a diagnosis of Cushing's disease. Pituitary microadenoma, if <6 mm in size, may be an incidental finding (incidentaloma) in this scenario and can be present in ∼3-27% of the healthy population. Therefore, in a patient with CS with equivocal ACTH levels and a pituitary microadenoma, multiple samplings for ACTH and adrenal imaging should be performed to exclude ACTH-independent CS and if required, bilateral inferior petrosal sinus sampling to determine the source of ACTH excess.

PMID: 28356253 [PubMed - indexed for MEDLINE]

Steroid profiling in H295R cells to identify chemicals potentially disrupting the production of adrenal steroids.

Thu, 05/18/2017 - 03:56
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Steroid profiling in H295R cells to identify chemicals potentially disrupting the production of adrenal steroids.

Toxicology. 2017 Apr 15;381:51-63

Authors: Strajhar P, Tonoli D, Jeanneret F, Imhof RM, Malagnino V, Patt M, Kratschmar DV, Boccard J, Rudaz S, Odermatt A

Abstract
The validated OECD test guideline 456 based on human adrenal H295R cells promotes measurement of testosterone and estradiol production as read-out to identify potential endocrine disrupting chemicals. This study aimed to establish optimal conditions for using H295R cells to detect chemicals interfering with the production of key adrenal steroids. H295R cells' supernatants were characterized by liquid chromatography-mass spectrometry (LC-MS)-based steroid profiling, and the influence of experimental conditions including time and serum content was assessed. Steroid profiles were determined before and after incubation with reference compounds and chemicals to be tested for potential disruption of adrenal steroidogenesis. The H295R cells cultivated according to the OECD test guideline produced progestins, glucocorticoids, mineralocorticoids and adrenal androgens but only very low amounts of testosterone. However, testosterone contained in Nu-serum was metabolized during the 48h incubation. Thus, inclusion of positive and negative controls and a steroid profile of the complete medium prior to the experiment (t=0h) was necessary to characterize H295R cells' steroid production and indicate alterations caused by exposure to chemicals. Among the tested chemicals, octyl methoxycinnamate and acetyl tributylcitrate resembled the corticosteroid induction pattern of the positive control torcetrapib. Gene expression analysis revealed that octyl methoxycinnamate and acetyl tributylcitrate enhanced CYP11B2 expression, although less pronounced than torcetrapib. Further experiments need to assess the toxicological relevance of octyl methoxycinnamate- and acetyl tributylcitrate-induced corticosteroid production. In conclusion, the extended profiling and appropriate controls allow detecting chemicals that act on steroidogenesis and provide initial mechanistic evidence for prioritizing chemicals for further investigations.

PMID: 28235592 [PubMed - indexed for MEDLINE]

The Carbohydrate Lectin Receptor Dectin-1 Mediates the Immune Response to Exserohilum rostratum.

Thu, 05/18/2017 - 03:56
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The Carbohydrate Lectin Receptor Dectin-1 Mediates the Immune Response to Exserohilum rostratum.

Infect Immun. 2017 Mar;85(3):

Authors: Reedy JL, Negoro PE, Feliu M, Lord AK, Khan NS, Lukason DP, Wiederhold NP, Tam JM, Mansour MK, Patterson TF, Vyas JM

Abstract
Dematiaceous molds are found ubiquitously in the environment and cause a wide spectrum of human disease, including infections associated with high rates of mortality. Despite this, the mechanism of the innate immune response has been less well studied, although it is key in the clearance of fungal pathogens. Here, we focus on Exserohilum rostratum, a dematiaceous mold that caused 753 infections during a multistate outbreak due to injection of contaminated methylprednisolone. We show that macrophages are incapable of phagocytosing Exserohilum Despite a lack of phagocytosis, macrophage production of tumor necrosis factor alpha is triggered by hyphae but not spores and depends upon Dectin-1, a C-type lectin receptor. Dectin-1 is specifically recruited to the macrophage-hyphal interface but not the macrophage-spore interface due to differences in carbohydrate antigen expression between these two fungal forms. Corticosteroid and antifungal therapy perturb this response, resulting in decreased cytokine production. In vivo soft tissue infection in wild-type mice demonstrated that Exserohilum provokes robust neutrophilic and granulomatous inflammation capable of thwarting fungal growth. However, coadministration of methylprednisolone acetate results in robust hyphal tissue invasion and a significant reduction in immune cell recruitment. Our results suggest that Dectin-1 is crucial for macrophage recognition and the macrophage response to Exserohilum and that corticosteroids potently attenuate the immune response to this pathogen.

PMID: 28031265 [PubMed - indexed for MEDLINE]

Recurrence of phaeochromocytoma in pregnancy in a patient with multiple endocrine neoplasia 2A: a case report and review of literature.

Thu, 05/18/2017 - 03:56
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Recurrence of phaeochromocytoma in pregnancy in a patient with multiple endocrine neoplasia 2A: a case report and review of literature.

Gynecol Endocrinol. 2016 Nov;32(11):875-880

Authors: Tingi E, Kyriacou A, Verghese L

Abstract
Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant inherited condition with a prevalence of one in 40 000 individuals. It causes the development of tumours in endocrine glands, such as medullary thyroid cancer, pheochromocytomas, as well as primary hyperparathyroidism. MEN 2A in pregnancy is very rare with only 29 cases reported in the literature. The presence of pheochromocytoma is a rare cause of hypertension during pregnancy with an incidence of 0.007% of all pregnancies. This has severe implications on both mother and the foetus. This case report describes a 22-year-old nulliparous Caucasian woman with known MEN2A syndrome, who underwent thyroidectomy for medullary thyroid carcinoma in childhood and excision of left sided pheochromocytoma at the age of 19. She was found to have a recurrence of pheochromocytoma in the right adrenal gland during pregnancy at 16 weeks of gestation and was oddly normotensive. Catecholamine effects were blocked with phenoxybenzamine and she delivered by an uneventful elective caesarean section at 36 weeks gestation. She underwent a laparoscopic right adrenalectomy six weeks postpartum, followed by lifelong corticosteroid replacement.

PMID: 27808580 [PubMed - indexed for MEDLINE]

Eccrine squamous syringometaplasia in sites of radiation recall secondary to vemurafenib therapy for BRAF-mutated carcinoma.

Thu, 05/18/2017 - 03:56
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Eccrine squamous syringometaplasia in sites of radiation recall secondary to vemurafenib therapy for BRAF-mutated carcinoma.

J Dermatol. 2016 Oct;43(10):1233-1234

Authors: Moioli EK, Ligtenberg KG, Kenkare S, Shea CR, Soltani K

PMID: 27094584 [PubMed - indexed for MEDLINE]

Genomic and proteomic analysis of the inhibition of synthesis and secretion of aldosterone hormone induced by quinocetone in NCI-H295R cells.

Thu, 05/18/2017 - 03:56
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Genomic and proteomic analysis of the inhibition of synthesis and secretion of aldosterone hormone induced by quinocetone in NCI-H295R cells.

Toxicology. 2016 Mar 28;350-352:1-14

Authors: Wang X, Bai Y, Cheng G, Ihsan A, Zhu F, Wang Y, Tao Y, Chen D, Dai M, Liu Z, Yuan Z

Abstract
Quinoxaline 1,4-dioxides (QdNOs) are widely used as a kind of antibacterial growth promoter in animal husbandry. The adrenal cortex was found to be one of the main toxic targets of QdNOs, accompanied by a decreased aldosterone level. However, the way in which QdNOs decrease production of the hormone aldosterone is far from clear. To illustrate the mechanism by which QdNOs damage the adrenal cortex and decrease aldosterone hormone levels, the QdNOs were screened to choose the drug with most toxic effects on aldosterone production, and then to reveal the mechanism between the gene and protein profiles in human adrenocortical cells (NCI-H295R cells). The results found that quinocetone (QCT) showed the highest adrenal toxic effect among QdNOs. After exposing H295R cells to 10 and 20μM QCT for 24h, compared with blank cells, the gene and protein expression profiles obtained were analyzed by microarray and MALDI TOF/TOF mass spectrometry, respectively. The results of microarray analysis suggested that ABCG1 and SREBF1, which were involved in the cholesterol biosynthetic and metabolic processes, and CYP17A1, NR4A2 and G6PD, which were related to aldosterone biosynthesis, were important molecular targets. It has been speculated that PKC and ERK pathways might be involved in the reduction of aldosterone production caused by QCT, through enhanced mRNA expression of CYP17A1. Additionally, JNK and p38MAPK signal transduction pathways might participate in apoptosis induced by QCT. Twenty-nine and 32 protein spots were successfully identified when cells were treated with 10 and 20μM QCT, respectively. These identified proteins mainly included material synthesis and energy metabolism-related proteins, transcription/translation processing-related proteins, signal transduction proteins, cytoskeletal proteins, molecular chaperones, proteins related to response to stress, and transport proteins. Further investigations suggested that oxidative stress caused by QCT was exacerbated through disruption of the Keap1/Nrf2/ARE anti-oxidative stress pathway. Taken together, the data demonstrated for the first time that the Keap1/Nrf2/ARE pathway plays a crucial role in adrenal toxicity, and that CYP17A1 was the key switch to reduce the aldosterone production induced by QCT. Furthermore, large numbers of genes and proteins and entry points for research in the inhibition of aldosterone synthesis induced by QCT were offered, which will provide new insight into the adrenal toxicity of QdNOs and help to provide a theoretical foundation for the formulation of safety controls for products obtained from animals and to design new QdNOs with less harmful effects.

PMID: 27046791 [PubMed - indexed for MEDLINE]

Alopecia and hirsutism in a postmenopausal woman as the presenting complaint of ovarian hilus (Leydig) cell tumor.

Thu, 05/18/2017 - 03:56
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Alopecia and hirsutism in a postmenopausal woman as the presenting complaint of ovarian hilus (Leydig) cell tumor.

Actas Dermosifiliogr. 2015 Oct;106(8):676-8

Authors: Berbegal L, Albares MP, De-Leon FJ, Negueruela G

PMID: 25910403 [PubMed - indexed for MEDLINE]

Retinoic acid post consolidation therapy for high-risk neuroblastoma patients treated with autologous hematopoietic stem cell transplantation.

Thu, 05/18/2017 - 03:56
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Retinoic acid post consolidation therapy for high-risk neuroblastoma patients treated with autologous hematopoietic stem cell transplantation.

Cochrane Database Syst Rev. 2015 Jan 29;1:CD010685

Authors: Peinemann F, van Dalen EC, Tushabe DA, Berthold F

Abstract
BACKGROUND: Neuroblastoma is a rare malignant disease and mainly affects infants and very young children. The tumors mainly develop in the adrenal medullary tissue and an abdominal mass is the most common presentation. About 50% of patients have metastatic disease at diagnosis. The high-risk group is characterized by metastasis and other characteristics that increase the risk for an adverse outcome. High-risk patients have a five-year event-free survival of less than 50%. Retinoic acid has been shown to inhibit growth of human neuroblastoma cells and has been considered as a potential candidate for improving the outcome of patients with high-risk neuroblastoma.
OBJECTIVES: To evaluate efficacy and adverse events of retinoic acid after consolidation with high-dose chemotherapy followed by bone marrow transplantation as compared to placebo or no therapy in patients with high-risk neuroblastoma (as defined by the International Neuroblastoma Risk Group (INRG) classification system). Our outcomes of interest were overall survival and treatment-related mortality as primary outcomes; and progression- and event-free survival, early and late toxicity, and health-related quality of life as secondary outcomes.
SEARCH METHODS: We searched the electronic databases CENTRAL (2014, Issue 8) on The Cochrane Library, MEDLINE (1946 to October 2014), and EMBASE (1947 to October 2014). Further searches included trial registries, conference proceedings, and reference lists of recent reviews and relevant articles. We did not apply limits on publication year or languages.
SELECTION CRITERIA: Randomized controlled trials (RCTs) evaluating retinoic acid post consolidation therapy for high-risk neuroblastoma patients treated with autologous hematopoietic stem cell transplantation (HSCT) compared to placebo or no further treatment.
DATA COLLECTION AND ANALYSIS: Two review authors performed the study selection, extracted the data on study and patient characteristics and assessed the risk of bias independently. We resolved differences by discussion or by appeal to a third review author. We performed analyses according to the guidelines of the Cochrane Handbook for Systematic Reviews of Interventions. The authors of the included study did not report the results specifically for the treatment groups relevant to this Cochrane Review. Therefore, we deduced the appropriate survival data from the published survival curves and calculated a hazard ratio (HR) based on the deduced data.
MAIN RESULTS: We identified one RCT (CCG-3891) that included patients with high-risk neuroblastoma who received high-dose chemotherapy followed by autologous HSCT (N = 98) after a first random allocation and who received retinoic acid (13-cis-retinoic acid; N = 50) or no further therapy (N = 48) after a subsequent second random allocation. These patients had no progressive disease after consolidation therapy. There was no clear evidence of difference between the treatment groups in both overall survival (HR 0.87, 95% CI 0.46 to 1.63; one trial; P = 0.66, low quality of evidence) and event-free survival (HR 0.86, 95% CI 0.50 to 1.49; one trial; P = 0.59, low quality of evidence). We calculated these HR values using the complete follow-up period of the trial. The study also reported five-year overall survival rates: 59% for the retinoic acid group and 41% for the no further therapy group (P value not reported). We did not identify results for treatment-related mortality, progression-free survival, early or late toxicity, or health-related quality of life. Also, we could not rule out the possible presence of selection bias, performance bias, attrition bias, and other bias.
AUTHORS' CONCLUSIONS: We identified one RCT that evaluated retinoic acid as a consolidation therapy versus no further therapy after high-dose chemotherapy followed by bone-marrow transplantation in patients with high-risk neuroblastoma. The difference in overall survival and event-free survival between both treatment alternatives was not statistically significantly different. This could be the result of low power. Information on other outcomes was not available. This trial was performed in the 1990s, since then many changes in for example treatment and risk classification have occurred. Therefore, based on the currently available evidence, we are uncertain about the effects of retinoic acid in patients with high-risk neuroblastoma. More research is needed for a definitive conclusion.

PMID: 25634649 [PubMed - indexed for MEDLINE]

Spontaneous regression of methotrexate-related diffuse large B-cell lymphoma following bladder lesion resection.

Thu, 05/11/2017 - 01:24
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Spontaneous regression of methotrexate-related diffuse large B-cell lymphoma following bladder lesion resection.

Rinsho Ketsueki. 2017;58(4):287-291

Authors: Yamada M, Kuroda H, Sato K, Miura S, Ameda S, Sakano H, Shibata T, Uemura N, Abe T, Fujii S, Maeda M, Fujita M, Kato J

Abstract
A 71-year-old woman who had been treated with methotrexate (MTX) and prednisolone for rheumatoid arthritis since 2010 presented with hematuria. Cystitis was diagnosed. Chest and abdominal CT images revealed a bladder tumor, with lung and bilateral adrenal metastases. Transurethral resection of the bladder tumor (TUR-BT) confirmed these findings in September 2014. Histological findings of the bladder included large atypical lymphoid cells indicating diffuse large B-cell lymphoma. After TUR-BT, CT imaging showed that the tumor had shrunk. Still, MTX was continued. She was diagnosed with MTX-related lymphoproliferative disorders in November 2014 and MTX was discontinued. Fluorodeoxyglucose-positron emission tomography on March 2015 showed a complete response.

PMID: 28484154 [PubMed - in process]

Genetics of Adrenocortical Development and Tumors.

Thu, 05/11/2017 - 01:24
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Genetics of Adrenocortical Development and Tumors.

Endocrinol Metab Clin North Am. 2017 Jun;46(2):419-433

Authors: Lodish M

Abstract
This article links the understanding of developmental physiology of the adrenal cortex to adrenocortical tumor formation. Many molecular mechanisms that lead to formation of adrenocortical tumors have been discovered via next-generation sequencing approaches. The most frequently mutated genes in adrenocortical tumors are also factors in normal adrenal development and homeostasis, including those that alter the p53 and Wnt/β-catenin pathways. In addition, dysregulated protein kinase A signaling and ARMC5 mutations have been identified as key mediators of adrenocortical tumorigenesis. The growing understanding of genetic changes that orchestrate adrenocortical development and disease pave the way for potential targeted treatment strategies.

PMID: 28476230 [PubMed - in process]

Management of a multiple endocrine neoplasia type 1 during pregnancy: A case report and review of the literature.

Thu, 05/11/2017 - 01:24
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Management of a multiple endocrine neoplasia type 1 during pregnancy: A case report and review of the literature.

J Exp Ther Oncol. 2016 Jul;11(3):217-220

Authors: Daglar HK, Kirbas A, Biberoglu E, Laleli B, Danisman N

Abstract
Multiple Endocrine Neoplasia Type 1 (MEN1) or Wermer's syndrome is a rare hereditary endocrine syndrome with high penetrance caused by mutations in MEN1 tumor suppressor gene. MEN1 is characterized by hyperplasia or tumoral enlargement in a number of endocrine organs (parathyroid glands, pancreas, pituitary gland, adrenal gland) and it could be hormonally active or inactive. MEN1 is a significant cause of morbidity due to hormone secretion and mass effect. Since it is a rare condition, there are no guidelines with respect to the follow-up of pregnant women with MEN1. Herein, we aimed to present the diagnosis and gestational follow-up of a 29-year-old pregnant with MEN1 syndrome.

PMID: 28471129 [PubMed - in process]

Recurrence of non-cardiogenic pulmonary edema and sustained hypotension shock in cystic pheochromocytoma.

Thu, 05/11/2017 - 01:24
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Recurrence of non-cardiogenic pulmonary edema and sustained hypotension shock in cystic pheochromocytoma.

J Zhejiang Univ Sci B. 2017 May;18(5):449-452

Authors: Dai J, Chen SJ, Yang BS, Lü SM, Zhu M, Xu YF, Chen J, Cai HW, Mao W

Abstract
Pheochromocytoma is a rare neuroendocrine tumor which derives from chromaffin cells of the adrenal gland or relevant to sympathetic nerves and ganglia. The clinical features of pheochromocytoma are various. Paroxysmal episodes of serious hypertension, headache, palpitation, and diaphoresis are the typical manifestations (Bravo, 2004). Hypotension shock, pulmonary edema, and acute coronary syndrome induced by pheochromocytoma are uncommon (Malindretos et al., 2008; Batisse-Lignier et al., 2015). In this study, we present a rare case of cystic pheochromocytoma causing recurrent hypotension shock, non-cardiogenic pulmonary edema, and acute coronary syndrome, and the possible mechanisms are discussed.

PMID: 28471119 [PubMed - in process]

Pheochromocytoma in a Child without Hypertension: A Contribution to the "Rule of 10s".

Thu, 05/11/2017 - 01:24
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Pheochromocytoma in a Child without Hypertension: A Contribution to the "Rule of 10s".

Indian J Med Paediatr Oncol. 2017 Jan-Mar;38(1):59-61

Authors: Tripathy PK, Pattnaik K, Nayak M, Mohanty HK

Abstract
Pheochromocytoma (PCC) is a neuroendocrine tumor originating from chromaffin tissue in adrenal medulla. Its diagnosis and treatment are well defined in adults, but experience in children is limited. Children constitute only 10% of reported cases, the average age at presentation being 11 years. The most common presentation is sustained hypertension, which is absent in only 10% of children. We managed a 14-month-old female child with PCC, but she was not hypertensive. We report two unusual features, in this case, an extremely young age at presentation and a childhood case of nonhypertensive PCC contributing for "rule of 10s."

PMID: 28469338 [PubMed - in process]

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