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Adrenal tumor

Hanane Eddaoualline, Khadija Mazouz, Bouchra Rafiq, Ghizlane El Mghari Tabib, Nawal El Ansari, Rhizlane Belbaraka, Abdelhamid El Omrani, Mouna Khouchani
BACKGROUND: Ewing sarcoma/primitive neuroectodermal tumor is a family of highly malignant proliferation of neuroectodermal origin, most often skeletal, adrenal localization is extremely rare. Only few cases have been reported in the literature. Classical management includes radical surgery with adjuvant chemotherapy or radiotherapy or both. This case report is the only one where recurrence was surgically removed, and it confirms the importance of adjuvant treatment, and the efficacy of neoadjuvant chemotherapy...
March 16, 2018: Journal of Medical Case Reports
Ying Pang, Garima Gupta, Chunzhang Yang, Herui Wang, Thanh-Truc Huynh, Ziedulla Abdullaev, Svetlana D Pack, Melanie J Percy, Terence R J Lappin, Zhengping Zhuang, Karel Pacak
BACKGROUND: The role of the hypoxia signaling pathway in the pathogenesis of pheochromocytoma/paraganglioma (PPGL)-polycythemia syndrome has been elucidated. Novel somatic mutations in hypoxia-inducible factor type 2A (HIF2A) and germline mutations in prolyl hydroxylase type 1 and type 2 (PHD1 and PHD2) have been identified to cause upregulation of the hypoxia signaling pathway and its target genes including erythropoietin (EPO) and its receptor (EPOR). However, in a minority of patients presenting with this syndrome, the genetics and molecular pathogenesis remain unexplained...
March 13, 2018: BMC Cancer
Mirjana Kocova, Vesna Janevska, Violeta Anastasovska
BACKGROUND: Testicular adrenal rest tumors (TARTs) are found in 30-94% of adult males with congenital adrenal hyperplasia (CAH). We sought to explore TART appearance through yearly ultrasound examination of testes in young boys with CAH, and its association with metabolic control and genetic mutations. METHODS: Twenty-five boys with 21-hydroxylase deficiency in the age group 4-18 years diagnosed during the period 2001-2016 were included in the study. ACTH, 17-hydroxyprogesterone, androstenedione and testosterone were measured at 4-month intervals...
March 12, 2018: Endocrine Connections
Yang Zhao, Hui Guo, Ying Zhao, Bingyin Shi
Ectopic adrenal adenoma causing chronic Cushing's syndrome (CS) is a rare phenomenon. Diagnosis is usually made years after disease onset because of the insidious nature of the ectopic adrenal gland and because it overlaps with common symptoms, such as overweight and hypertension, in the general population (Kreitschmann-Andermahr et al., 2015). Here, we report the case of a 46-year-old male with a 15-year history of severe hypertension, facial plethora, and centripetal obesity. During treatment for herpes zoster, the patient presented with severe hypokalemia and flaccid paralysis, characteristic changes associated with CS...
March 7, 2018: Annales D'endocrinologie
C Berthin, P Sibilia, J Martins-Hericher, A Donzeau, L Martin
BACKGROUND: Non-classical congenital adrenal hyperplasia (NC-CAH) is a recessive autosomal disease caused by a deficiency of adrenal steroidogenesis enzymes. It must be distinguished from classical CAH, either simple virilising or salt-wasting, diagnosed during the neonatal period and responsible for potentially lethal disorders of sexual differentiation. NC-CAH presents a simpler and less specific clinical picture. Herein, we present two cases comprising twin girls consulting for diffuse hypertrichosis...
March 7, 2018: Annales de Dermatologie et de Vénéréologie
J P Zhang, Q H Guo, Y M Mu, Z H Lyu, W J Gu, G Q Yang, J Du, J M Ba, J M Lu
Objective: To evaluate the clinical characteristics and etiologies of central diabetes insipidus (CDI). Methods: The clinical data of 230 patients with CDI in the Department of Endocrinology of Chinese PLA General Hospital from 2008 June to 2014 December were collected and analyzed retrospectively. Results: The three most common causes of CDI were idiopathic CDI, lymphocytic hypophysitis and intracranial germ cell tumors. Among all the CDI, the idiopathic CDI accounted for 37.48%. There were significant differences in age onset and gender distribution among the different causes of CDI...
March 1, 2018: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
Chrysanthi Aggeli, Alexander M Nixon, Christos Parianos, Georgios Vletsis, Labrini Papanastasiou, Theodora Kounadi, Georgrios Piaditis, Georgios N Zografos
OBJECTIVE: Resection of pheochromocytomas is a challenging procedure due to hemodynamic lability. Our aim was to evaluate surgical outcomes in 67 patients with pheochromocytoma and to validate the role of laparoscopic surgery in the treatment of these tumors. DESIGN: This study is a retrospective review. A total of 68 procedures for pheochromocytoma were performed between June 1997 and February 2017. All patients were investigated and operated on using an established departmental protocol...
October 2017: Hormones: International Journal of Endocrinology and Metabolism
Min Chen, Weibin Zhou, Zhe Zhang, Yuting Zou, Chengjiang Li
RATIONALE: The incidence of severe hyperandrogenism associated with masculinity in women is very low. While rare and difficult to diagnose, androgen secreting tumors should be suspected in women with hyperandrogenism and hirsutism, especially in the postmenopausal population. Herein we present one case of ovarian Leydig cell tumor (LCT) with markedly elevated serum testosterone levels and frank hirsutism. PATIENT CONCERNS: A 60-year-old woman, presented with increased hair growth and androgenic alopecia and the hormonal laboratory examination showed that she had elevated serum testosterone level and normal dehydroepiandrosterone sulfate (DHEAS), androstenedione, 17- hydroxyprogesterone, cortisol and thyroid stimulating hormone (TSH)...
March 2018: Medicine (Baltimore)
Fazli Yanik, Yekta Altemur Karamustafaoglu, Yener Yoruk
CONTEXT: Neuroblastoma is the most common extracranial malignant solid tumor that occurs during childhood. It arises from primitive cells and is seen in the adrenal medulla and sympathetic ganglia of the sympathetic nervous system. CASE REPORT: We present a rare case of a 40-year-old man who was diagnosed with the onset of neuroblastoma arising in the mediastinum. He was treated by means of surgical resection in the superior mediastinum after neoadjuvant chemotherapy...
March 5, 2018: São Paulo Medical Journal, Revista Paulista de Medicina
Masood Umer, Yasir Mohib, Muhammed Atif, Muhammad Nazim
Background: Renal cell carcinoma account for 3% of all cancers, with peak incidence between 60 and 70 years of age predominantly affecting male population. Renal carcinoma is the most common malignancy of kidney constitutes for 80-90% of renal neoplasm with an overall 45% five years survival rate. Majority are diagnosed incidentally during investigation for other disease process of abdomen. Classical triad of gross hematuria, pain and palpable mass in abdomen is rare accounting to only 6-10%...
March 2018: Annals of Medicine and Surgery
Yutaka Endo, Minoru Kitago, Akira Miyajima, Isao Kurihara, Kaori Kameyama, Masahiro Shinoda, Hiroshi Yagi, Yuta Abe, Taizo Hibi, Chisato Takagi, Yutaka Nakano, Wataru Koizumi, Osamu Itano, Yuko Kitagawa
INTRODUCTION: von Hippel-Lindau disease (vHL disease) is a hereditary disease in which tumors and cysts develop in many organs, in association with central nervous system hemangioblastomas, pheochromocytomas, and pancreatic tumors. We herein report a case of vHL disease (type 2A) associated with bilateral pheochromocytomas, pancreatic neuroendocrine tumors (PNET), and cerebellar hemangioblastomas treated via pancreatectomy after adrenalectomy. CASE PRESENTATION: A 51-year-old woman presented with a cerebellar tumor, bilateral hypernephroma, and pancreatic tumor detected during a medical checkup...
February 16, 2018: International Journal of Surgery Case Reports
Shiro Matsumoto, Yoshinori Hosoya, Alan Kawarai Lefor, Hidenori Haruta, Takashi Ui, Kentaro Kurashina, Shin Saito, Kentaro Ashizawa, Takahiro Sasaki, Joji Kitayama, Naohiro Sata
INTRODUCTION: Black adrenal adenoma (BAA) is a rare, benign adrenal lesion with a black or brown appearance. This is the first report of this lesion in a patient with a synchronous esophageal cancer and highlights the importance of considering a false positive finding on a Positron Emission Tomography (PET) scan, which might otherwise preclude resection. PRESENTATION OF CASE: A 73-year-old male was diagnosed with mid-esophagus carcinoma. Computed tomography scan revealed an enlarged left adrenal gland...
December 7, 2017: International Journal of Surgery Case Reports
Agathocles Tsatsoulis
Thyroid follicular cells, as well as adrenocortical cells, are endowed by an intrinsic heterogeneity regarding their growth potential, in response to various stimuli. This heterogeneity appears to constitute the underlying cause for the focal cell hyperplasia and eventually the formation of thyroid and adrenal nodules, under the influence of growth stimulatory factors. Among the main stimulatory factors are the pituitary tropic hormones, thyroid-stimulating hormone (TSH) or thyrotropin and adrenocorticotropic hormone (ACTH), which regulate the growth and function of their respective target cells, and the insulin/insulin-like growth factor system, that, through its mitogenic effects, can stimulate the proliferation of these cells...
February 26, 2018: Journal of Clinical Medicine
G Donatini, J L Kraimps, C Caillard, E Mirallie, F Pierre, Loïc De Calan, Hamy Antoine, O Larin, O Tovkay, S Cherenko
BACKGROUND: Pheochromocytoma (PHEO) in pregnancy is a life-threatening condition. Its management is challenging with regards to the timing and type of surgery. METHODS: A retrospective review of the management of ten patients diagnosed with pheochromocytoma during pregnancy was performed. Data were collected on the initial diagnostic workup, symptoms, treatment, and follow-up. RESULTS: PHEO was diagnosed in ten patients who were between the 10th and the 29th weeks of pregnancy...
February 27, 2018: Surgical Endoscopy
Rohit Bhattar, Vinay Tomar, Devendra Singh Dhakad, Neeraj Agarwal
Neurilemmomas are benign, slow growing, encapsulated nerve sheath tumor. These tumors arise from the schwann cells of neural crest. Neurilemmomas can manifest in various form according to site, extent and severity of involvement of organ. Diaphragmatic neurilemmomas are very unusual and even difficult to diagnose on preoperative imaging. We will report a case of 39 year old male, who presented with complaints of occasional left flank pain for one year and subsequently investigated, which showed left adrenal cyst with haemorrhagic fluid content...
January 2018: Turkish Journal of Urology
Levent Soydan, Ali Aslan Demir, Elif Sayman, Burcu Onomay Celik, Bala Basak Oven Ustaalioglu
Ewing sarcoma and peripheral primitive neuroectodermal tumor belong to the Ewing sarcoma (ES) family of tumors originating from a primitive neural tube. We report a 31-year-old man who was admitted to the urology clinic with complaints of fever, nausea, and dysuria. A right-sided adrenal mass was detected during ultrasonography. The lesion was then evaluated with magnetic resonance imaging, which showed areas of necrosis amid heterogeneous solid areas. Whole body scan with 2-deoxy-2-[fluorine-18]fluoro-D-glucose integrated with computed tomography and bone scan studies showed pulmonary and osseous metastatic foci...
December 2017: Radiology Case Reports
Jing Li, Pan Liang, Dandan Zhang, Jie Liu, Hongkai Zhang, Jinrong Qu, Jianbo Gao
BACKGROUND: Carcinosarcoma of the liver is a very rare tumor composed of a mixture of carcinomatous and sarcomatous elements. Less than 25 adequately documented cases have been reported, with inadequate description of imaging features. In order to improve the awareness of this rare tumor, this study aimed to analyze the clinicopathologic and imaging features of six cases of hepatic carcinosarcoma (HCS) confirmed by surgical pathologic evaluation. METHODS: We retrospectively studied the clinicopathologic and imaging features of six cases of HCS (matching the World Health Organization definition) and discussed the differential diagnosis on the basis of imaging findings...
February 27, 2018: Cancer Imaging: the Official Publication of the International Cancer Imaging Society
Anne Jouinot, Jerome Bertherat
Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis, the five-years overall survival being below 40%. However there is great variability of outcomes and we have now a better view of the heterogeneity of tumor aggressiveness. The extent of the disease at the time of diagnosis, best assayed by the European Network for the Study of Adrenal Tumors (ENSAT) staging score, is a major determinant of survival. The tumor grade, including the mitotic count and the Ki67 proliferation index, also appears as a strong prognostic factor...
February 23, 2018: European Journal of Endocrinology
Marie Marty, Delphine Gaye, Paul Perez, Claire Auder, Marie-Laure Nunes, Amandine Ferriere, Magalie Haissaguerre, Antoine Tabarin
CONTEXT: the recent recommendations of the European endocrine society states that the performance of computed tomography (CT) to characterize true adrenal incidentalomas (AIs) remains debatable. OBJECTIVE: to determine relevant thresholds for usual CT parameters for the diagnosis of benign tumors using robust reference standard amongst a large series of "true" AIs recruited in an endocrinological setting. DESIGN: retrospective study of 253 AIs in 233 consecutive patients explored in a single university hospital: 183 adenomas, 33 pheochromocytomas, 23 adrenocortical carcinomas, 5 other malignant tumors and 9 other benign tumors...
February 21, 2018: European Journal of Endocrinology
Edward Buitenwerf, Tijmen Korteweg, Anneke Visser, Charlotte Msc Haag, Richard A Feelders, Henri J Timmers, Letizia Canu, H R Haak, Peter H Bisschop, E Marelise W Eekhoff, Eleonora Pm van der Kleij-Corssmit, Nanda C Krak, Elise Rasenberg, Janneke van den Bergh, Jaap Stoker, Marcel Jw Greuter, Robin P F Dullaart, Thera P Links, Michiel N Kerstens
BACKGROUND: A substantial proportion of all pheochromocytomas is currently detected during the evaluation of an adrenal incidentaloma. Recently it has been suggested that biochemical testing to rule out pheochromocytoma is unnecessary in case of an adrenal incidentaloma with an unenhanced attenuation value ≤10 Hounsfield Units (HU) at computed tomography (CT). OBJECTIVES: We aimed to determine the sensitivity of the 10 HU threshold value to exclude a pheochromocytoma...
February 21, 2018: European Journal of Endocrinology
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