Raza M Ahmad, Kyle Ingram, Ralph Corsetti
Background: Feminizing adrenal tumors are rare and generally malignant tumors usually seen in male adults and children. We report the case of a benign feminizing adrenal tumor in a male patient. To our knowledge, only 2 other cases of benign, estrogen-only-secreting adrenal tumors have been reported. Case Report: A 44-year-old male with a history of hypertension presented to his primary care physician with chest tenderness, fatigue, and erectile dysfunction. Hormonal workup and imaging identified an estrogen-only-secreting adrenal adenoma...
2020: Ochsner Journal
Roberto Iglesias Lopes, Caio Vinicius Suartz, Rubens Pedrenho Neto, Ricardo Haidar Berjeaut, Berenice Mendonca, Madson Q Almeida, Maria Cândida Villares Fragoso, Francisco Tibor Dénes
AIM: The aim of this study is to present our experience in the management of hormonally active adrenal tumors in children. MATERIAL AND METHODS: We did a retrospective chart review of all children with hormonally active adrenal tumors evaluated at the endocrinology clinic and operated at our institution between 1983 and 2019. RESULTS: There were 75 patients included in the study, 58 with adrenocortical tumors (ACTs) and 17 with pheochromocytomas (PCCs)...
September 1, 2020: Journal of Pediatric Surgery
Kyungchul Song, Ahreum Kwon, Junghwan Suh, Han Saem Choi, Hyun Wook Chae, Ho-Seong Kim
Cushing syndrome (CS) occurs rarely in children. The clinical presentation of CS varies according to the extent and duration of glucocorticoid excess, and urolithiasis is one of the common complications of CS. We report the first case of a patient with CS associated with acute kidney injury (AKI) due to urolithiasis. A 6-year-old boy came to the emergency room with seizure. On physical examination, he had clinical features of CS and high blood pressure. Brain computerized tomography (CT) suggested posterior reversible encephalopathy syndrome due to hypertension...
August 7, 2020: Annals of Pediatric Endocrinology & Metabolism
Christina Tatsi, Megan Neely, Chelsi Flippo, Maria-Eleni Bompou, Meg Keil, Constantine A Stratakis
OBJECTIVES: The postoperative period of Cushing disease (CD) is complicated by a phase of adrenal insufficiency (AI). Factors that influence the duration of AI and its prognostic significance for CD recurrence in children have not been extensively studied. We investigated whether clinical or biochemical factors contribute to the duration of AI, and the correlation of the recovery process with the risk for recurrence. DESIGN: Patients with pediatric-onset CD who were followed up for at least 3 months after transsphenoidal surgery (TSS) (n=130) were included in the study...
July 29, 2020: Clinical Endocrinology
Chan Hon Chui, Kenneth Tou En Chang
PURPOSE: Loss of adrenal function is a major concern in the treatment of children with bilateral adrenal neuroblastoma (BAN). We aimed to evaluate the feasibility of adrenal-sparing surgery in this unique subgroup. METHOD: Retrospective review of our center's neuroblastoma database was conducted. Patients with synchronous BAN confirmed at surgery were included. Their demographic data and clinical charts were analyzed. RESULTS: Five patients were confirmed with BAN...
July 1, 2020: Journal of Pediatric Surgery
Mikołaj Danko, Anna Malinowska, Elżbieta Moszczyńska, Joanna Pawłowska, Maria Szarras-Czapnik, Małgorzata Walewska-Wolf, Urszula Wątrobińska, Mieczysław Szalecki
Objectives The main cause of hyperandrogenism in children is congenital adrenal hyperplasia, adrenal and gonadal tumors, polycystic ovary syndrome (PCOs) and Cushing's disease. In the last 20 years several descriptions of girls with hyperandrogenism and venous porto-systemic shunts appeared in literature. Case presentation First case is an eleven and a half-year-old girl, was admitted to Department of Endocrinology because of symptoms of hyperandrogenism. Laboratory tests revealed high serum testosterone, androstenedione, and dehydroepiandrosterone sulfate (DHEAS)...
July 13, 2020: Journal of Pediatric Endocrinology & Metabolism: JPEM
Emilia Modolo Pinto, Gerard P Zambetti, Carlos Rodriguez-Galindo
Childhood adrenocortical tumors (ACTs) are rare, representing ∼0.2% of all pediatric malignancies and having an incidence of 0.2-0.3 new cases per million per year in the United States, but incidences are remarkably higher in Southern Brazil. At diagnosis, most children show signs and symptoms of virilization, Cushing syndrome, or both. Less than 10% of patients with ACT exhibit no endocrine syndrome at presentation, although some show abnormal concentrations of adrenal cortex hormones. Pediatric ACT is commonly associated with constitutional genetic and/or epigenetic alterations, represented by germline TP53 mutations or chromosome 11p abnormalities...
May 2020: Best Practice & Research. Clinical Endocrinology & Metabolism
Alireza Mirsharifi, Mohammad Vasei, Ehsan Sadeghian, Ali Ghorbani-Abdehgah, Sara Naybandi Atashi
BACKGROUND: Adrenocortical carcinoma is a rare malignancy, with 43% being non-functional. These may arise from adrenal rest anywhere in the embryonic pathway of the adrenal glands. In the context of extra-adrenal and retroperitoneal tumors, the exact pathologic diagnosis is challenging. The case reported here, to the best of our knowledge, is the seventh reported case of extra-adrenal non-functional adrenocortical carcinoma. CASE PRESENTATION: We report a case of extra-adrenal non-functional adrenocortical carcinoma in a 15-year-old Persian boy who presented with an acute abdomen...
July 8, 2020: Journal of Medical Case Reports
Mohammed H Alomari, Harry P W Kozakewich, Cindy L Kerr, Wibke Uller, Scott L Davis, Gulraiz Chaudry, Marilyn G Liang, Darren B Orbach, John B Mulliken, Arin K Greene, Salim Afshar, Steven J Fishman, Amir H Taghinia, Alya Al-Ibraheemi, Ahmad I Alomari
OBJECTIVE: To describe the clinical, radiological and histopathological features of "congenital disseminated pyogenic granuloma" (CDPG) involving various organs with high morbidity related to cerebral hemorrhagic involvement. STUDY DESIGN: We searched the database of the Vascular Anomalies Center at Boston Children's Hospital from 1999 to 2019 for patients diagnosed as having multiple vascular lesions, visceral vascular tumors, congenital hemangiomatosis, multiple pyogenic granulomas or multiple vascular lesions without a definite diagnosis...
July 2, 2020: Journal of Pediatrics
Jamie M Shoag, Antonello Podda, Erin N Kobetz
Dinutuximab is a costly life-prolonging immunotherapy for high-risk neuroblastoma. We used a large pediatric inpatient database to analyze the use of antineoplastic immunotherapy in patients with malignant adrenal tumors 1 year after Food and Drug Administration approval of dinutuximab for high-risk neuroblastoma. On multivariate modeling, children of Black race (odds ratio [OR] 0.62, P = .04; referent non-Black) and the lowest ZIP code income quartile (OR 0.74, P = .03; referent wealthier 3 quartiles) were significantly less like to receive antineoplastic immunotherapy...
July 2, 2020: Pediatric Blood & Cancer
Zachary J Kastenberg, Eric R Scaife
Adrenocortical neoplasms are rare in childhood. Unlike their adult counterparts, they are often hormonally active and malignant. Despite being uncommon, adrenocortical neoplasms in children have significant associated morbidity and require complete surgical resection for effective management. Furthermore, the clinical overlap between adrenocortical neoplasms, adrenal medullary neoplasms, and functional disorders of the adrenal cortex requires that the practicing pediatric surgeon have a solid working knowledge of the presentation, diagnostic workup, and management of these anatomically related yet disparate pathologies...
June 2020: Seminars in Pediatric Surgery
Luojia Xu, Youyou Luo, Jindan Yu, Jingan Lou, Xiaofei Chen, Jie Chen
RATIONALE: Chronic radiation enteritis, a disease secondary to radiation exposure, has been widely reported in adults. However, few studies have described chronic radiation enteritis in children. Early diagnosis is essential, and nutrition management plays an important role in pediatric chronic radiation enteritis management. PATIENT CONCERNS: A Chinese 3-year-10-month-old boy was admitted with vomiting, weight loss (1-2 kg) after radiotherapy for a neuroblastoma...
June 19, 2020: Medicine (Baltimore)
Emilia Modolo Pinto, Katja Kiseljak-Vassiliades, Constanze Hantel
Adrenocortical carcinoma (ACC) is an uncommon and heterogeneous disease and may present differently in children and adults. Management of ACC is dependent on disease stage and complete surgical resection is the only potentially curative treatment. The first and most extensively used adrenocortical cancer cell line, as model system to examine mechanisms controlling normal and pathologic function of adrenal cortex, was initially isolated in 1980. Although NCI-H295 maintained steroid capabilities and adrenocortical characteristics, the lack of new cell lines and animal models of ACC has hampered the progress and development of new therapies...
October 2019: Current Opinion in Endocrine and Metabolic Research
Calogero Virgone, Marina Andreetta, Stefano Avanzini, Stefano Chiaravalli, Deborah De Pasquale, Alessandro Crocoli, Alessandro Inserra, Paolo D'Angelo, Rita Alaggio, Giuseppe Opocher, Giovanni Cecchetto, Andrea Ferrari, Gianni Bisogno, Patrizia Dall'Igna
BACKGROUND: Pheochromocytomas (PCs) are neuroendocrine tumors arising from the chromaffin cells of the adrenal gland, and paragangliomas (PGLs) are their extra-adrenal counterparts arising from ganglia along the sympathetic/parasympathetic chain. Surgery is the cornerstone of treatment. A sporatic or inherited germline mutation is commonly associated. MATERIALS AND METHODS: Among over 1000 patients registered into the Tumori Rari in Età Pediatrica-rare tumors in pediatric age project-from 2000 to 2019, 50 were affected by PC/PGL...
June 3, 2020: Pediatric Blood & Cancer
Marie de Tersant, Lucile Généré, Claire Freyçon, Sophie Villebasse, Rachid Abbas, Anne Barlier, Damien Bodet, Nadège Corradini, Anne-Sophie Defachelles, Natacha Entz-Werle, Cyrielle Fouquet, Louise Galmiche, Virginie Gandemer, Brigitte Lacour, Ludovic Mansuy, Daniel Orbach, Claire Pluchart, Yves Réguerre, Charlotte Rigaud, Sabine Sarnacki, Nicolas Sirvent, Jean-Louis Stephan, Estelle Thebaud, Anne-Paule Gimenez-Roqueplo, Laurence Brugières
Purpose: The purpose of this work is to assess the clinical outcome of pediatric patients diagnosed with pheochromocytoma and paraganglioma (PPGL) detected in France since 2000. Methods: A retrospective multicenter study was conducted that included all patients younger than 18 years with PPGL diagnosed in France between 2000 and 2016. Patients were identified from 4 different sources: the National Registry of Childhood Solid Tumors, the French Pediatric Rare Tumors Database, the French registry of succinate dehydrogenase (SDH)-related hereditary paraganglioma, and the nationwide TenGen network...
May 1, 2020: Journal of the Endocrine Society
Jane Pei-Chen Chang, Valeria Mondelli, Sentil Kumaran Satyanarayanan, Yi-Ju Chiang, Hui-Ting Chen, Kuan-Pin Su, Carmine M Pariante
BACKGROUND: Hypothalamus-Pituitary-Adrenal (HPA) axis dysregulation, inflammation and imbalance of neurotrophins have been suggested in attention deficit hyperactivity disorder (ADHD), but the results have not been conclusive. The aim of this study is to investigate the levels of salivary cortisol across 4-time points during the day, and of morning plasma inflammatory biomarkers and neurotrophins, in youth with ADHD and in typically developing youth (TD), with stratification by age, ADHD subtypes and oppositional defiant disorder (ODD) comorbidity in Taiwan...
May 8, 2020: Brain, Behavior, and Immunity
I V Poddubny, R S Oganesyan, K N Tolstov, M A Kareva
OBJECTIVE: To estimate the results of minimally invasive adrenalectomy in children and compare our data with worldwide results. MATERIAL AND METHODS: There were 65 patients aged from 3 months to 17 years with different organic diseases of adrenal glands. Children have undergone surgery for the period since 2003 to 2018. RESULTS: Incidentalomas accounted 33.8% of all resected tumors. Bilateral lesion was diagnosed in 12% of patients. The largest neoplasm (12 cm) was diagnosed in a 9-year-old child...
2020: Khirurgiia
Vania B Brondani, Maria Candida B V Fragoso
PURPOSE OF REVIEW: Adrenocortical tumor (ACT) is a rare disease with an annual worldwide incidence of 0.3-0.38/million children below 15 years old, and Brazilian population presents the highest incidence because of germline mutation in the TP53. Pediatric ACT is associated with virilizing features and hypercortisolism in most cases. Malignancy is defined when local invasion or metastasis is found, and it is associated with a poor prognosis. However, the correct and early diagnosis and treatment may impact on overall and disease-free survival...
June 2020: Current Opinion in Endocrinology, Diabetes, and Obesity
Hong Qin, Shen Yang, Siyu Cai, Qinghua Ren, Wei Han, Wei Yang, Haiyan Cheng, Xiaoli Ma, Huanmin Wang
BACKGROUND: Neuroblastoma (NB) tumor rupture is a rare oncology emergency with a poor prognosis. We aimed to evaluate patient clinical characteristics and risk factors for ruptured NB. METHODS: A retrospective study of 47 patients with confirmed NB rupture between January 2009 and January 2019 at Beijing Children's Hospital was conducted. To identify tumor rupture risk factors in high-risk NB patients, we included 93 consecutive non-ruptured high-risk NB patients from January 2017 to January 2019...
March 23, 2020: BMC Cancer
Kathryn Bradford, Alexander Nobori, Brittany Johnson, Wendy Allen-Rhoades, Bindi Naik-Mathuria, Eduard H Panosyan, Moran Gotesman, Joseph Lasky, Jerry Cheng, Alan Ikeda, Jeffrey Goldstein, Arun Singh, Noah Federman
The Ewing sarcoma family of tumors (ESFT) are high-grade small round blue cell malignancies traditionally presenting in children and adolescents. The most common site of primary disease is bone, though extraskeletal primary sites are well-recognized. We present 6 cases of primary ESFT of the kidney and 1 case of the adrenal gland. Patients were 11 to 18 years of age at diagnosis. Metastases at diagnosis were present in most cases (n=6). All patients underwent surgery, and most received radiation (n=5). Five patients relapsed after initial remission...
April 10, 2020: Journal of Pediatric Hematology/oncology
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