Search Our Scientific Publications & Authors

J Clin Endocrinol Metab 2019 Apr 12. Epub 2019 Apr 12.

Centre for Cancer Imaging, Peter MacCallum Cancer Centre, Victoria, Australia.

Purpose: Germline succinate dehydrogenase (SDHx) mutation carriers, especially SDHB, are at increased malignancy risk and require life-long surveillance. Current guidelines recommend periodic whole-body MRI imaging. We assessed the incremental value of GaTate PET/CT compared to conventional imaging in such patients. Read More

Asian J Surg 2019 Apr 5. Epub 2019 Apr 5.

Department of Pediatric Surgery, Cerrahpasa Medical Faculty, Istanbul University-Cerrahpasa, Turkey.

Background/objective: This study aims to evaluate the current surgical approach to adrenal masses in the pediatric age group.

Methods: We retrospectively analyzed cases that underwent surgery for adrenal masses between 2007 and 2017. Patients were assessed regarding age, sex, primary diagnosis, image defined risk factors (IDRF), surgical treatment method, complications, duration of hospital stay, and follow-up. Read More

Endocr Connect 2019 Mar 1. Epub 2019 Mar 1.

K Takase, Radiology, Tohoku University Hospital, Sendai, Japan.

Objective: Regional differences in cardiac magnetic resonance, which can reveal catecholamine-induced myocardial injury in patients with pheochromocytoma, have not yet been assessed using 3T magnetic resonance imaging. We evaluated these differences using myocardial T1-mapping and strain analysis.

Design And Methods: We retrospectively reviewed 16 patients newly diagnosed with catecholamine-producing tumors (CPT group) and 16 patients with essential hypertension (EH group), who underwent cardiac magnetic resonance imaging between May 2016 and March 2018. Read More

Intern Med J 2019 Apr;49(4):529-532

Department of Endocrinology and Diabetes, St Vincent's Hospital Melbourne, Melbourne, Victoria, Australia.

Carriers of succinate dehydrogenase (SDHx) mutations are at risk of developing phaeochromocytomas, catecholamine secreting extra-adrenal paragangliomas and non-secretory head and neck paragangliomas and require lifelong surveillance. There is no current consensus on the optimal surveillance strategy. This study describes the outcomes of a cohort of 50 SDHx mutation carriers followed at a tertiary Australian hospital using a surveillance protocol involving annual clinical review with plasma/urine metanephrines and biennial magnetic resonance imaging from skull base to pelvis. Read More

J Nucl Med 2019 Apr 6. Epub 2019 Apr 6.

University Hospital Heidelberg, Germany.

The recent development of quinoline based positron-emission-tomography (PET)-tracers that act as fibroblast-activation-protein inhibitors (FAPIs) demonstrated promising preclinical and clinical results. FAP is overexpressed by cancer associated fibroblasts (CAFs) of several tumor entities. Here we quantify the tumor-uptake in FAPI-PET/CT of various primary and metastatic tumors to identify the most promising indications for future application. Read More

JCO Precis Oncol 2018 Mar;2:1-12

Cancer Research UK Cambridge Institute, University of Cambridge, Li Ka Shing Centre, Robinson Way, Cambridge CB2 0RE, UK.

Purpose: Mutations in the mitochondrial enzyme succinate dehydrogenase (SDH) subunit genes are associated with a wide spectrum of tumours including phaeochromocytoma and paraganglioma (PPGL) 1, 2, gastrointestinal stromal tumours (GIST) 3, renal cell carcinoma (RCC) 4 and pituitary adenomas5. SDH-related tumorigenesis is believed to be secondary to accumulation of the oncometabolite succinate. Our aim was to investigate the potential clinical applications of MRI spectroscopy (H-MRS) in a range of suspected SDH-related tumours. Read More

Clin Endocrinol (Oxf) 2019 Apr 1. Epub 2019 Apr 1.

Department of Endocrinology, St Bartholomew's Hospital, Barts Health NHS Trust, London, UK.

Objective: There is consensus that asymptomatic carriers of SDHB mutations should undergo periodic surveillance imaging. MRI has the advantage of avoiding radiation exposure but its sensitivity and specificity for detecting phaeochromocytoma and paraganglioma (PPGL) are dependent on sequences performed and expertise of reporting radiologists. We aim to highlight the additional value of diffusion-weighted imaging (DWI) for MR based surveillance, demonstrating DWI's ability to identify small PPGLs at all body sites. Read More

Endocr Pract 2019 Mar;25(3):211-219

Objective: Most of the Indian studies on pheochromocytoma/paraganglioma (PCC/PGL) have focused on PCC, and there is a paucity of information regarding sympathetic paraganglioma (sPGL). Here, we describe the clinical, biochemical, and imaging features of sPGL compared with PCC.

Methods: This retrospective study included 75 patients with sPGL and 150 patients with PCC. Read More

Endocrinol Diabetes Metab Case Rep 2019 Mar 23;2019. Epub 2019 Mar 23.

St. Vincent's University Hospital and University College Dublin, Dublin, Ireland.

Context Adrenal incidentalomas (AI) represent an increasingly common problem in modern endocrine practice. The diagnostic approach to AIs can be challenging and occasionally reveals surprising features. Here we describe two rare cases of complex adrenal lesions consisting of phaeochromocytomas with synchronous metastases from extra-adrenal primaries. Read More

Nanomaterials (Basel) 2019 Mar 18;9(3). Epub 2019 Mar 18.

Department of Bioengineering, College of Engineering, Temple University, Philadelphia, PA 19122, USA.

Magnetic iron oxide (Magnetite, Fe₃O₄) nanoparticles are widely utilized in magnetic resonance imaging (MRI) and drug delivery applications due to their superparamagnetism. Surface coatings are often employed to change the properties of the magnetite nanoparticles or to modulate their biological responses. In this study, magnetite nanoparticles were fabricated through hydrothermal synthesis. Read More

J Pediatr Surg 2019 Feb 28. Epub 2019 Feb 28.

Department of Paediatric Surgery, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899. Electronic address:

Background/aims: Posterior retroperitoneoscoic adrenalectomy has been reported as an option for adrenal tumor resection but is not commonly performed in children owing to the extreme semikneeling position advocated to flatten the lumbar lordosis in order to achieve adequate retroperitoneal space. As children have smaller lordosis angles, flattening of the lordosis and creation of optimal retroperitoneal space may be achieved with less hip flexion. We used pediatric lumbar lordosis measurements to develop a modified prone jackknife position and report our experiences with this setup for posterior retroperitoneoscopic adrenalectomy for adrenal tumors. Read More

Front Oncol 2019 22;9:53. Epub 2019 Feb 22.

Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, United States.

Pheochromocytoma and paraganglioma (PHEO/PGL) are rare neuroendocrine tumors which may cause potentially life-threatening complications, with about a third of cases found to harbor specific gene mutations. Thus, early diagnosis, treatment, and meticulous monitoring are of utmost importance. Because of low incidence of succinate dehydrogenase complex subunit A ()-related metastatic PHEO/PGL, currently there exists insufficient clinical information, especially with regards to its diagnostic and treatment characteristics. Read More

Curr Opin Oncol 2019 May;31(3):243-246

Department of Medicine, Division of Endocrinology.

Purpose Of Review: Adrenal tumors are mostly encountered as incidentalomas in patients undergoing imaging not performed for suspected adrenal disease; although the majority are benign and nonfunctioning, malignant tumors and functioning tumors need to be excluded. The purpose of this review is to highlight recent advances in the evaluation of adrenal tumors.

Recent Findings: As a consequence of increased use of technologically improved imaging techniques, the detection of adrenal incidentalomas has continued to increase. Read More

J Clin Endocrinol Metab 2019 Mar 1. Epub 2019 Mar 1.

Department of Internal Medicine, sections of Endocrinology and Vascular Medicine, Radboud University Medical Centre, Nijmegen, The Netherlands.

Context: Cross sectional imaging with computed tomography (CT) or magnetic resonance imaging (MRI) is regarded as a first-choice modality for tumor localization in patients with pheochromocytoma and paraganglioma (PPGL). 123I-labeled metaiodobenzylguanidine (123I-MIBG) is widely used for functional imaging but the added diagnostic value is controversial.

Objective: To establish the virtual impact of adding 123I-MIBG scintigraphy to CT or MRI on diagnosis and treatment of PPGL. Read More

Korean J Intern Med 2019 Feb 28. Epub 2019 Feb 28.

Division of Cardiology, Department of Internal Medicine, Chungnam National University College of Medicine, Regional Cardiocerebrovascular Center, Chungnam National University Hospital, Daejeon, Korea.

Background/aims: Pheochromocytoma and paraganglioma (PPGL) are catecholamine-producing tumors that can cause blood pressure (BP) elevation and cardiovascular complications. Clinical presentation of these tumors may be changed through widespread use of imaging studies, which enables detection of PPGLs before onset of symptoms. We investigated clinical profiles of patients with surgically resected PPGLs. Read More

Int J Hematol Oncol Stem Cell Res 2018 Oct;12(4):249-252

Department of Pathology, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Adrenal involvement in the course of malignant lymphoma occurs in about 4% of patients, but primary adrenal lymphoma (PAL) is extremely rare. To the best of our knowledge, only one case study reported the combination of PAL with pheochromocytoma. In the current study, we present the second case who was a 63-year-old man admitted to our hospital with hematuria and abdominal discomfort. Read More

Chirurg 2019 Mar;90(Suppl 2):107

Sektion Endokrine Chirurgie, Klinik für Allgemein‑, Viszeral- und Transplantationschirurgie, Universitätsklinikum Essen, Hufelandstr. 55, 45147, Essen, Deutschland.

J Postgrad Med 2019 Jan-Mar;65(1):44-46

Division of Cardiology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.

We report a 49-year-old woman who presented with a hypertensive crisis and acute heart failure and reduced left ventricular systolic function. An abdominal ultrasonography revealed a huge lobulated heterogeneous mass at the lower pole of the right kidney and a mass over the left suprarenal area, which were further delineated by magnetic resonance imaging. The patient underwent laparoscopic right radical nephrectomy and left adrenalectomy. Read More

Int J Surg Case Rep 2019 9;55:7-10. Epub 2019 Jan 9.

Department of Surgery, Kendall Regional Medical Center, Miami, FL, United States.

Introduction: Pheochromocytoma are neuroendocrine tumors that arise from sympathetic chromaffin cells within the adrenal medulla. They principally secrete catecholamines, potentially causing life-threatening cardiovascular complications. A myriad of symptomatology and clinical findings are associated with pheochromocytoma, including a catecholamine-induced dilated cardiomyopathy. Read More

Rev Esp Med Nucl Imagen Mol 2019 Mar - Apr;38(2):94-99. Epub 2019 Jan 7.

Department of Nuclear Medicine, Medical University Innsbruck, Innsbruck, Austria.

Objective: F-Fluoro-L-dihydroxyphenylalanine (F-DOPA) PET offers high sensitivity and specificity in the imaging of non-malignant extra-adrenal paraganglioma (PGL) and pheochromocytoma (PHEO) but lower sensitivity in metastatic disease. These tumours are of neuroendocrine origin and can be detected by Ga-DOTA-Tyr3-octreotide (Ga-DOTA-TOC) PET. Therefore, we compared Ga-DOTA-TOC and F-DOPA as radiolabels for PET/CT imaging for the diagnosis of metastatic extra-adrenal PGL and PHEO. Read More

Health Sci Rep 2018 Mar 20;1(3):e22. Epub 2017 Dec 20.

Internal Medicine and Endocrinology, Department of Internal Medicine, Division of Endocrinology Cukurova University Medical Faculty Adana Turkey.

Background: Adrenal incidentalomas are adrenal masses that are discovered by imaging tests performed for other reasons.

Aims: In this retrospective study, we analysed 229 Turkish patients with adrenal masses and who presented with or without complaints.

Study Design: Descriptive retrospective study and review of the literature. Read More

J Med Case Rep 2019 Jan 5;13(1). Epub 2019 Jan 5.

Division of Diabetes, Metabolism, and Endocrinology, Department of Internal Medicine, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa, Tokyo, 142-8555, Japan.

Background: There are several reports of pheochromocytoma crisis triggered by systemic glucocorticoid administration. However, pheochromocytoma crisis after intra-articular glucocorticoid administration has been rarely reported.

Case Presentation: A 45-year-old Japanese man presented to our hospital with a sudden, severe headache. Read More

Pediatr Nephrol 2019 Jan 2. Epub 2019 Jan 2.

Division of Pediatric Nephrology and Hypertension, Children's Hospital of Michigan, Wayne State University School of Medicine, 5th Floor, Carl's Building, 3901 Beaubien Blvd, Detroit, MI, 48201, USA.

Pheochromocytomas and paragangliomas (PPGLs) generally grouped together are rare catecholamine-secreting endocrine tumors. Symptoms of catecholamine excess are non-specific and therefore a high index of suspicion in children with sustained hypertension, family history of endocrine tumors, or features of syndromes associated with PPGLs leads to a timely diagnosis and treatment. Free metanephrines in the plasma or 24-h urine are the preferred tests to establish catecholamine excess. Read More

J Biophotonics 2019 May 28;12(5):e201800289. Epub 2019 Jan 28.

Department of Physics, Korea Advanced Institute of Science and Technology, Daejeon, Republic of Korea.

Temporally low-coherent optical diffraction tomography (ODT) is proposed and demonstrated based on angle-scanning Mach-Zehnder interferometry. Using a digital micromirror device based on diffractive tilting, the full-field interference of incoherent light is successfully maintained during every angle-scanning sequences. Further, current ODT reconstruction principles for temporally incoherent illuminations are thoroughly reviewed and developed. Read More

Clin Endocrinol (Oxf) 2019 Apr 29;90(4):499-505. Epub 2019 Jan 29.

Department of Diabetes and Endocrinology, Cambridge University Hospital NHS Foundation Trust, Cambridge, UK.

The succinate dehydrogenase (SDH) enzyme complex functions as a key enzyme coupling the oxidation of succinate to fumarate in the citric acid cycle. Inactivation of this enzyme complex results in the cellular accumulation of the oncometabolite succinate, which is postulated to be a key driver in tumorigenesis. Succinate accumulation inhibits 2-oxoglutarate-dependent dioxygenases, including DNA and histone demethylase enzymes and hypoxic gene response regulators. Read More

Cancers (Basel) 2018 Dec 15;10(12). Epub 2018 Dec 15.

Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, 10 Center Drive, Building 10, Room 1E-3140, Bethesda, MD 20892, USA.

Adrenocortical carcinoma (ACC) and pheochromocytoma and paraganglioma (PPGL) are defined by clinicopathological criteria and can be further sub-divided based on different molecular features. Whether differences between these molecular subgroups are significant enough to re-challenge their current clinicopathological classification is currently unknown. It is also not fully understood to which other cancers ACC and PPGL show similarity to. Read More

Hinyokika Kiyo 2018 Nov;64(11):435-438

The Department of Urology, Osaka National Hospital.

A 69-year-old woman was admitted to the previous hospital because of a right adrenal tumor detected by a medical checkup. Although the tumor was diagnosed as non-functional adrenal adenoma, abdominal computed tomography (CT) revealed a left renal mass which was suspected to be renal cell carcinoma. Chest CT seeking for metastatic lesions revealed lung cancer of the left lung. Read More

Clinics (Sao Paulo) 2018 12 10;73(suppl 1):e756s. Epub 2018 Dec 10.

Unidade de Suprarrenal, Laboratorio de Hormonios e Genetica Molecular LIM/42, Servico de Endocrinologia e Metabologia, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR.

Malignancy must be considered in the management of adrenal lesions, including those incidentally identified on imaging studies. Adrenocortical carcinomas (ACCs) are rare tumors with an estimated annual incidence of 0.7-2 cases per year and a worldwide prevalence of 4-12 cases per million/year. Read More

EPMA J 2018 Dec 21;9(4):421-429. Epub 2018 Sep 21.

7Department of Radiology, Keck Medical Center of USC, Los Angeles, CA USA.

Objectives: This study aims to define a radiomic signature for pre-operative differentiation between subclinical pheochromocytoma (sPHEO) and lipid-poor adrenal adenoma (LPA) in adrenal incidentaloma. The goal was to apply a predictive, preventive, and personalized medical approach to the management of adrenal tumors.

Patients And Methods: This retrospective study consisted of 265 consecutive patients (training cohort, 212 (LPA, 145; sPHEO, 67); validation cohort, 53 (LPA, 36; sPHEO, 17)). Read More

Front Endocrinol (Lausanne) 2018 27;9:515. Epub 2018 Nov 27.

Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, United States.

Pheochromocytomas and paragangliomas (PCCs/PGLs) are rare commonly benign neuroendocrine tumors that share pathology features and clinical behavior in many cases. While PCCs are chromaffin-derived tumors that arise within the adrenal medulla, PGLs are neural-crest-derived tumors that originate at the extraadrenal paraganglia. Pheochromocytoma-paraganglioma (PPGL) syndromes are rapidly evolving entities in endocrinology and oncology. Read More

J Intern Med 2019 Feb 15;285(2):187-204. Epub 2019 Jan 15.

Clinical Genetics, Karolinska University Hospital, Stockholm, Sweden.

Pheochromocytoma and paraganglioma (PPGL) are rare tumours and at least 30% are part of hereditary syndromes. Approximately 20% of hereditary PPGL are caused by pathogenic germ line variants in genes of the succinate dehydrogenase complex (SDHx), TMEM127 or MAX. Herein we present guidelines regarding genetic testing of family members and their surveillance based on a thorough literature review. Read More

Hum Pathol 2019 Apr 8;86:66-75. Epub 2018 Dec 8.

Department of Pathology, University of Helsinki and HUSLAB, Helsinki University Hospital, Helsinki FIN-00014, Finland.

Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are neuroendocrine tumors that express somatostatin receptors (SSTRs), a phenomenon that constitutes a basis for tumor imaging and treatment with somatostatin analogues and peptide receptor radionuclide therapy. We studied the immunohistochemical expression of SSTR1-5 in 151 primary tumors, including 14 metastasized and 16 SDHB-deficient tumors. SSTR2 and SSTR3 were most abundantly present in these tumors, whereas the tumors were mostly negative for SSTR1, SSTR4, and SSTR5. Read More

Braz J Cardiovasc Surg 2018 Sep-Oct;33(5):522-524

Department of Cardiovascular Surgery, Muş State Hospital, Muş, Turkey.

This paper presents a case study of a patient that underwent surgery for a ruptured abdominal aneurysm. The postoperative course was complicated by resistant hypertension and tachycardia. A suprarenal mass was detected in the computed tomography scan with radiological suspicion of pheochromocytoma. Read More

Prog Urol 2018 Nov 27;28(12S):S175-S193. Epub 2018 Oct 27.

Service d'urologie, hôpital d'instruction des armées Sainte-Anne, BP 600, 83800 Toulon cedex 09, France; Service d'urologie, hôpital européen Georges-Pompidou, université Paris Descartes, AP-HP, 75015 Paris, France. Electronic address:

Objective: To update French oncology guidelines concerning adrenal cancer.

Methods: Comprehensive Medline search between 2016 and 2018 upon diagnosis, treatment and follow-up of adrenal cancer to update 2013 guidelines. Level of evidence was evaluated according to AGREE-II. Read More

J Clin Endocrinol Metab 2019 May;104(5):1386-1393

Division of Endocrinology, Diabetes, and Nutrition, Mayo Clinic, Rochester, Minnesota.

Context: Modern pheochromocytomas (PHEOs) are often discovered by incidental finding on cross-sectional imaging or mutation-based genetic case detection testing. Little is known about how these PHEOs behave.

Objective: To describe the characteristics and behavior of PHEOs discovered incidentally on imaging or through mutation-based genetic case detection testing. Read More

Radiol Med 2019 Mar 14;124(3):218-233. Epub 2018 Nov 14.

Department of Radiology, IEO, European Institute of Oncology IRCCS, Milan, Italy.

The past decade has witnessed a growing role and increasing use of whole-body magnetic resonance imaging (WB-MRI). Driving these successes are developments in both hardware and software that have reduced overall examination times and significantly improved MR imaging quality. In addition, radiologists and clinicians have continued to find promising new applications of this innovative imaging technique that brings together morphologic and functional characterization of tissues. Read More

Case Rep Gastrointest Med 2018 3;2018:8196051. Epub 2018 Oct 3.

Division of Gastroenterology and Hepatology, University of Florida and Malcom Randall VA Medical Center, Gainesville, FL, USA.

Primary adrenal leiomyosarcoma (PAL) is an extremely rare mesenchymal tumor with only a few isolated case reports in the medical literature. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) or endoscopic ultrasound-guided core biopsy (EUS-CB) is a safe, effective modality for sampling lesions in the gastrointestinal tract and adjacent organs, including the adrenal glands. We describe the case of a 50-year-old male presenting with abdominal pain and unintentional weight loss over the course of one year. Read More

Chin Med J (Engl) 2018 Nov;131(21):2614-2615

Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing 100730, China.

Chirurg 2019 Jan;90(1):3-8

Klinik für Endokrinologie, Diabetologie und Stoffwechsel, Universitätsklinikum Essen, Hufelandstr. 55, 45127, Essen, Deutschland.

An adrenal incidentaloma is an adrenal mass detected on imaging that was not performed for suspected adrenal disease. The prevalence is approximately 3% and increases up to 10% in older people. The risk of malignancy and a hormone excess have to be evaluated. Read More

Endocr Connect 2018 Sep 1. Epub 2018 Sep 1.

F Beuschlein, LMU München, Medizinische Klinik IV, Munich, Germany.

Context: Pheochromocytomas and paragangliomas (PPGLs) are rare but potentially harmful tumors that can vary in their clinical presentation. Tumors may be found due to signs and symptoms, as part of a hereditary syndrome or following an imaging procedure.

Objective: To investigate potential differences in clinical presentation between PPGLs discovered by imaging (iPPGLs), symptomatic cases (sPPGLs) and those diagnosed during follow-up because of earlier disease/known hereditary mutations (fPPGL). Read More

Surgery 2019 Jan 16;165(1):228-231. Epub 2018 Oct 16.

University of Tennessee Health Science Center, Department of Surgery, Division of Surgical Oncology, Memphis TN. Electronic address:

Background: Multifocal pheochromocytoma/paraganglioma presenting at an early age is commonly associated with a hereditary syndrome.

Case Report: A 29-year-old woman was referred for evaluation of multifocal pheochromocytoma/paraganglioma. Interestingly, her family history did not include pheochromocytoma/paraganglioma, and comprehensive genetic testing for the well-documented pheochromocytoma/paraganglioma susceptibility genes was negative. Read More

J Cancer 2018 8;9(19):3577-3582. Epub 2018 Sep 8.

Department of Radiology, Keck Medical Center of USC, Los Angeles, CA.

To evaluate the feasibility and accuracy of machine learning based texture analysis of unenhanced CT images in differentiating subclinical pheochromocytoma (sPHEO) from lipid-poor adenoma (LPA) in adrenal incidentaloma (AI). Seventy-nine patients with 80 LPA and 29 patients with 30 sPHEO were included in the study. Texture parameters were derived using imaging software (MaZda). Read More

Quant Imaging Med Surg 2018 Sep;8(8):853-875

Department of Radiology, Affiliated Hospital of Zunyi Medical University, Zunyi 563000, China.

Besides ultrasound and nuclear medicine techniques, computed tomography (CT) and magnetic resonance imaging (MRI) are commonly used to examine adrenal lesions in both symptomatic and asymptomatic patients. Some adrenal lesions have characteristic radiological features. If an adrenal nodule is discovered incidentally, determining whether the lesion is benign or malignant is of great importance. Read More

Clin Endocrinol (Oxf) 2019 Jan 28;90(1):31-46. Epub 2018 Nov 28.

Department of Endocrinology, St Bartholomew's Hospital, Barts Health NHS Trust, London, UK.

Objective: With the discovery that familial phaeochromocytoma and paraganglioma syndrome can be caused by mutations in each subunit of the succinate dehydrogenase enzyme (SDH), has come the recognition that mutations in the individual subunits have their own distinct natural histories. Increased genetic screening is leading to the identification of increasing numbers of, mostly asymptomatic, gene mutation carriers and the implementation of screening strategies for these individuals. Yet there is, to date, no international consensus regarding screening strategies for asymptomatic carriers. Read More

Indian J Pathol Microbiol 2018 Oct-Dec;61(4):587-589

Department of Surgery, Maulana Azad Medical College, New Delhi, India.

Sporadic adrenal medullary hyperplasia (AMH) is a rare entity and mimics pheochromocytoma clinically as well as pharmacologically. It is characterized by increase in adrenal medullary cells with the expansion of cells into areas normally not seen. A 59-year-old male presented with chronic hypertension and raised 24-h urinary normetanephrine levels. Read More

J Nucl Med 2018 Oct 5. Epub 2018 Oct 5.

Department of Endocrine Neoplasia and Hormonal Disorders, U. of Texas M.D. Anderson Cancer Center, United States.

Patients with metastatic or unresectable (advanced) pheochromocytoma or paraganglioma (PPGL) have poor prognoses and few treatment options. This multicenter, phase 2 trial evaluated the efficacy and safety of high-specific-activity I-131 meta-iodobenzylguanidine (HSA I-131 MIBG) in patients with advanced PPGL. In this open-label, single-arm study, 81 PPGL patients were screened for enrollment, and 74 received a treatment-planning dose of HSA I-131 MIBG. Read More

Endocr Relat Cancer 2018 10 5;25(12):993-1004. Epub 2018 Oct 5.

Department of Radiopharmaceutical and Chemical Biology, Helmholtz-Zentrum Dresden-Rossendorf, Institute of Radiopharmaceutical Cancer Research, Dresden, Germany.

Somatostatin receptor-targeting endoradiotherapy offers potential for treating metastatic pheochromocytomas and paragangliomas, an approach likely to benefit from combination radiosensitization therapy. To provide reliable preclinical in vivo models of metastatic disease, this study characterized the metastatic spread of luciferase-expressing mouse pheochromocytoma (MPC) cells in mouse strains with different immunologic conditions. Bioluminescence imaging showed that, in contrast to subcutaneous non-metastatic engraftment of luciferase-expressing MPC cells in NMRI-nude mice, intravenous cell injection provided only suboptimal metastatic spread in both NMRI-nude mice and hairless SCID (SHO) mice. Read More

Internist (Berl) 2018 Nov;59(11):1163-1179

Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, Campus Innenstadt, Ziemssenstr. 1, 80336, München, Deutschland.

Endocrine disorders are the most common causes of secondary hypertension. Early diagnosis and specific treatment are crucial for improvement of the prognosis. This article provides an overview on which clinical constellations point to an increased risk of secondary causes of hypertension. Read More

JAMA Surg 2019 Jan;154(1):40-45

Department of Surgery, Stanford University, Stanford, California.

Importance: Neuroendocrine tumors (NETs) express somatostatin receptors, which can be targeted with radiolabeled peptides. In a variety of solid tumors, radioguided surgery (RGS) has been used to guide surgical resection. Gallium 68 (68Ga) dota peptides have been shown to be more accurate than other radioisotopes for detecting NETs. Read More

Mayo Clin Proc Innov Qual Outcomes 2018 Mar 21;2(1):30-39. Epub 2017 Dec 21.

Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, MN.

Objective: To characterize large adrenal tumors (≥4 cm in diameter) and to identify features associated with malignancy.

Patients And Methods: We investigated the clinical, biochemical, and imaging characteristics in a large retrospective single-center cohort of patients with adrenal tumors of 4 cm or more in diameter during the period of January 1, 2000, through December 31, 2014.

Results: Of 4085 patients with adrenal tumors, 705 (17%) had adrenal masses measuring 4 cm or more in diameter; of these, 373 (53%) were women, with a median age of 59 years (range, 18-91 years) and median tumor size of 5. Read More