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Eur J Endocrinol 2021 May 1. Epub 2021 May 1.

F Beuschlein, Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, UniversitatsSpital Zurich, Zurich, 8091, Switzerland.

Objective: Within the past decade, important genetic drivers of pheochromocytoma and paraganglioma (PPGLs) development have been identified. The pathophysiological mechanism that translate these alterations into functional autonomy and potentially malignant behavior have not been elucidated in detail. Here we used MALDI-mass spectrometry imaging (MALDI-MSI) of formalin-fixed paraffin-embedded tissue specimens to comprehensively characterize the metabolic profiles of PPGLs. Read More

Kyobu Geka 2021 Feb;74(2):160-163

Department of General Thoracic Surgery, National Obihiro Hospital, Obihiro, Japan.

A 60-year-old woman with a lung tumor was admitted to our hospital for surgical treatment. Chest computed tomography (CT) showed a 23×21×20 mm nodule in the right lower lobe. Although the lung nodule was thought to be benign due to CT image findings, its size was apparently larger than that of CT image at four years ago. Read More

BMJ Case Rep 2021 May 10;14(5). Epub 2021 May 10.

Surgery, Kalafong Provincial Tertiary Hospital, University of Pretoria, Pretoria, South Africa

The association of pheochromocytoma in patients with neurofibromatosis type I has rarely been reported in low-income countries, especially on the African continent. A 43-year-old woman with neurofibromatosis type I was diagnosed with a right adrenal pheochromocytoma in Pretoria, South Africa. To our knowledge, this report is the first case to be published of a patient with neurofibromatosis type I diagnosed with a pheochromocytoma in Pretoria, and one of three cases on the African continent. Read More

Transl Androl Urol 2021 Apr;10(4):1813-1820

Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Pheochromocytoma (PHEO) is a rare neuroendocrine that tumor originated from the adrenal medulla that secrets catecholamines. Tumors from extra-adrenal chromaffin tissues are called extra-adrenal PHEO or paraganglioma (PGL). To our knowledge, adrenal PHEO and subclinical PGL with inferior vena cava (IVC) invasion had been sporadically reported, while functional PGL with IVC tumor thrombus has not been publicly reported yet. Read More

Curr Cardiol Rep 2021 May 7;23(6):60. Epub 2021 May 7.

Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic, 200 First St. SW, Rochester, MN, 55905, USA.

Purpose Of Review: Pheochromocytoma and paraganglioma (PPGL) in pregnancy is a rare entity and management of these patients is fraught with uncertainty. Our objective is to review current literature and discuss diagnosis and management of these patients.

Recent Findings: Outcomes of PPGL in pregnancy have improved in recent years. Read More

J Vet Emerg Crit Care (San Antonio) 2021 May 7. Epub 2021 May 7.

Emergency and Critical Care, VCA West Coast Specialty and Emergency Animal Hospital, Fountain Valley, California, USA.

Objective: To describe the clinical course of a dog with a pheochromocytoma multisystem crisis that exhibited autoresuscitation after cardiac arrest.

Case Summary: An approximately 10-year-old male neutered terrier mix dog presented for collapse. Abdominal imaging was suggestive of a pheochromocytoma, and clinical pathology data and thoracic imaging suggested the presence of a multisystem crisis. Read More

Clin Transl Oncol 2021 May 6. Epub 2021 May 6.

Endocrinology and Nutrition Department, Hospital Clinic Barcelona, University of Barcelona, IDIBAPS, Barcelona, Spain.

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the sympathetic/parasympathetic neural ganglia, respectively. The heterogeneity in its etiology makes PPGL diagnosis and treatment very complex. The aim of this article was to provide practical clinical guidelines for the diagnosis and treatment of PPGLs from a multidisciplinary perspective, with the involvement of the Spanish Societies of Endocrinology and Nutrition (SEEN), Medical Oncology (SEOM), Medical Radiology (SERAM), Nuclear Medicine and Molecular Imaging (SEMNIM), Otorhinolaryngology (SEORL), Pathology (SEAP), Radiation Oncology (SEOR), Surgery (AEC) and the Spanish National Cancer Research Center (CNIO). Read More

Case Rep Endocrinol 2021 14;2021:6699409. Epub 2021 Apr 14.

Department of Internal Medicine, HCA Healthcare/USF Morsani College of Medicine GME: Medical Center of Trinity, Trinity, FL, USA.

Pheochromocytoma (PCC) is a rare catecholamine-secreting tumor that arises from chromaffin cells of the adrenal medulla which are derived from the neural crest. This report illustrates a 51-year-old Caucasian male with a history of hypertension diagnosed two years ago who presented to the hospital due to acute onset of right testicular pain of 3-day duration. Laboratory results and imaging revealed a presumptive diagnosis of PCC. Read More

Arch Esp Urol 2021 May;74(4):445-449

Servicio de Oncología Médica. Hospital General Universitario de Elche. España. Unidad de Consejo Genético. Hospital General Universitario de Elche. España.

Objective: Description of two incidental cases of bladder paraganglioma in women and review of the published literature.

Methods: A bibliographic search was carried out in Medline over the last 10 years according to the terms "urinary bladder" and "paraganglioma".

Results: Bladder paraganglioma (BP) accounts for less than 0. Read More

Pancreas 2021 Apr;50(4):469-493

Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX.

Abstract: This manuscript is the result of the North American Neuroendocrine Tumor Society consensus conference on the medical management and surveillance of metastatic and unresectable pheochromocytoma and paraganglioma held on October 2 and 3, 2019. The panelists consisted of endocrinologists, medical oncologists, surgeons, radiologists/nuclear medicine physicians, nephrologists, pathologists, and radiation oncologists. The panelists performed a literature review on a series of questions regarding the medical management of metastatic and unresectable pheochromocytoma and paraganglioma as well as questions regarding surveillance after resection. Read More

Radiol Case Rep 2021 Jun 15;16(6):1493-1498. Epub 2021 Apr 15.

Department of Clinical Surgical Sciences, The University of the West Indies, St Augustine Campus, Trinidad, West Indies.

Retroperitoneal liposarcomas are rare mesenchymal tumors which carry a poor prognosis because of their late presentation. When symptoms do occur, they are usually nonspecific and related to mass effect or invasion of local structures. Rarely, retroperitoneal liposarcomas can clinically and biochemically mimic phaeochromocytomas. Read More

AJR Am J Roentgenol 2021 Apr 28:1-12. Epub 2021 Apr 28.

Department of Radiology and Imaging Sciences, University of Utah, Salt Lake City, UT.

The purpose of this article is to review the clinical manifestations, endocrine tumors types, and multimodality diagnostic tools available to physicians involved in the management of patients with multiple endocrine neoplasia (MEN) syndrome, in addition to discussing relevant imaging findings and appropriate imaging follow-up. Thorough knowledge of the spectrum of tumors associated with gene mutations aids in the screening, diagnostic workup, and posttreatment monitoring of patients with MEN-related gene mutations. Read More

BMC Surg 2021 Apr 26;21(1):210. Epub 2021 Apr 26.

Department of Urology, Affiliated Jinhua Hospital, Zhejiang University School of Medicine, Jinhua, 321000, China.

Background: Adrenal hemangioma is a rare benign adrenal tumor that is usually misdiagnosed preoperatively. We here present a case of adrenal cavernous hemangioma that was successfully treated with retroperitoneal laparoscopic adrenalectomy.

Case Presentation: A 67-year-old man with dull right back pain attended our clinic for examination of a mass on the right adrenal gland for 1 week. Read More

J Int Med Res 2021 Apr;49(4):3000605211007723

Department of Cardiology, Xinqiao Hospital, Army Medical University (Third Military Medical University), Chongqing, China.

Pheochromocytoma is a rare and usually benign tumor of the adrenal glands. We report a case of a 40-year-old woman with recurrent pheochromocytoma and catecholamine cardiomyopathy. She had no history of other types of tumors or connective tissue disease. Read More

N Engl J Med 2021 Apr;384(16):1542-1551

From the Division of General Surgery, Department of Surgery, Stanford University School of Medicine, Stanford, CA.

Cureus 2021 Mar 18;13(3):e13964. Epub 2021 Mar 18.

Department of Anaesthesiology, Ghulab Devi Hospital, Al-Aleem Medical College, Lahore, PAK.

Paragangliomas arise from neural cells and are found in different anatomical locations in the body. Paragangliomas in the adrenal glands are called pheochromocytoma, while the others are known as extra-adrenal paragangliomas. They are usually benign and are extremely rare in children. Read More

Eur J Case Rep Intern Med 2021 26;8(3):002421. Epub 2021 Mar 26.

Department of Radiology and Organ Imaging, United Christian Hospital, Hong Kong.

Colonic pseudo-obstruction is characterized by dilatation of the colon without a structural lesion causing the obstruction. It usually involves the caecum and right side of the colon and is commonly observed in patients with severe illness or after surgery; it is rarely caused by pheochromocytoma. The diagnosis of colonic pseudo-obstruction can be established by abdominal imaging including computed tomography (CT) of the abdomen or use of a water-soluble contrast enema. Read More

Front Endocrinol (Lausanne) 2021 29;12:625312. Epub 2021 Mar 29.

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

Neuroendocrine tumors overexpress somatostatin receptors, which serve as important and unique therapeutic targets for well-differentiated advanced disease. This overexpression is a well-established finding in gastroenteropancreatic neuroendocrine tumors which has guided new medical therapies in the administration of somatostatin analogs, both "cold", particularly octreotide and lanreotide, and "hot" analogs, chelated to radiolabeled isotopes. The binding of these analogs to somatostatin receptors effectively suppresses excess hormone secretion and tumor cell proliferation, leading to stabilization, and in some cases, tumor shrinkage. Read More

AACE Clin Case Rep 2021 Jan-Feb;7(1):72-74. Epub 2020 Dec 28.

Division of Endocrinology, Diabetes, & Metabolism, Department of Internal Medicine, University of Florida College of Medicine and the Malcom Randall VA Medical Center, Department of Internal Medicine, Division of Endocrinology, Diabetes & Metabolism, Gainesville, Florida.

Objectives: The evaluation of an adrenal mass is challenging. We present the case of a 33-year-old pregnant woman who was found to have an adrenal incidentaloma. Four months after the initial imaging, the mass vanished. Read More

Ergonomics 2021 May 3:1-11. Epub 2021 May 3.

Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.

This study proposes a risk analysis approach for complex healthcare processes that combines qualitative and quantitative methods to improve patient safety. We combine Healthcare Failure Mode and Effect Analysis with Computer Simulation (HFMEA-CS), to overcome widely recognised HFMEA drawbacks regarding the reproducibility and validity of the outcomes due to human interpretation, and show the application of this methodology in a complex healthcare setting. HFMEA-CS is applied to analyse drug adherence performance in the surgical admission to discharge process of pheochromocytoma patients. Read More

Abdom Radiol (NY) 2021 Apr 8. Epub 2021 Apr 8.

Department of Radiology, Harvard Medical School, Massachusetts General Hospital, 55 Fruit Street, White 270, Boston, MA, 02114-2696, USA.

Purpose: To investigate the diagnostic performance of dual-layer dual-energy CT (dlDECT) in the evaluation of adrenal nodules.

Methods: In this retrospective study, 66 patients with triphasic dlDECT (unenhanced, venous phase (VP), delayed phase (DP)) for suspected adrenal lesions were included. Virtual unenhanced images (VUE) were derived from VP acquisitions. Read More

J Headache Pain 2021 Apr 6;22(1):20. Epub 2021 Apr 6.

Department of medicine, Division of obstetrics & Gynecology, Milad hospital, Tehran, Iran.

Background: Reversible Cerebral Vasoconstriction Syndrome is a condition of transient cerebral vascular spasms, which usually presents with recurrent thunderclap headaches and recovers within 3 months. Several probable triggers and underlying factors, such as sex hormones, vasoactive drugs, head trauma or surgery, and tumors, have been implicated.

Case Presentation: In this paper, we present a 53-year-old woman with thunderclap headaches and normal lab tests who was radio-clinically diagnosed with reversible cerebral vasoconstriction syndrome and treated accordingly. Read More

N Engl J Med 2021 Mar;384(12):1145-1155

From the Departments of Pediatrics (K.R.O., R.W.C.), Medicine (K.R.O., N.S.), Radiology (K.N.), and Pathology (Z.O.), Massachusetts General Hospital, and the Departments of Pediatrics (K.R.O., R.W.C.), Medicine (K.R.O., N.S.), Radiology (K.N.), and Pathology (Z.O.), Harvard Medical School - both in Boston.

J Neuroendocrinol 2021 Mar 23:e12964. Epub 2021 Mar 23.

ENETs Centre of Excellence, Royal Free Hospital, London, UK.

Scanning for somatostatin receptors using Ga-dotatate positron emission tomography with co-registration with computed tomography or magnetic resonance imaging is now in widespread use for the identification of neuroendocrine tumours, phaeochromocytomas, and paragangliomas and their metastases. We present a case where a patient with a phaeochromocytoma showed uptake in her neck considered diagnostic of a head-and-neck paraganglioma, which was subsequently confirmed to be a metastatic papillary thyroid carcinoma. We alert clinicians to such falsely-identified tumours using this extensively used imaging technique. Read More

Curr Opin Endocrinol Diabetes Obes 2021 Jun;28(3):291-302

Adrenal Research Laboratory, The Discipline of Pharmacology and Therapeutics, Lambe Institute for Translational Research, School of Medicine, National University of Ireland Galway, Ireland.

Purpose Of Review: To summarise the emerging role of thermal ablation as a therapeutic modality in the management of functioning adrenal tumours and metastases to the adrenal gland.

Recent Findings: Observational evidence has demonstrated the benefit of thermal ablation in (i) resolving adrenal endocrinopathy arising from benign adenomas, (ii) treating solitary metastases to the adrenal and (iii) controlling metastatic adrenocortical carcinoma and phaeochromocytoma/paraganglioma.

Summary: Microwave thermal ablation offers a promising, minimally invasive therapeutic modality for the management of functioning adrenocortical adenomas and adrenal metastases. Read More

Medicine (Baltimore) 2021 Mar;100(10):e25088

Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Abstract: Diagnosis of unicentric Castleman disease (UCD) is not easy before the resection and obtainment of pathological result. We retrospectively summarized 10-year experience of clinical evaluation and management for retroperitoneal UCD in Peking Union Medical College Hospital (PUMCH) between December 1, 2009 and December 31, 2019. Seventy two UCD patients with pathological diagnosis after resection were screened out. Read More

Clin Endocrinol (Oxf) 2021 Mar 15. Epub 2021 Mar 15.

Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA.

Objective: To describe the presentation and outcomes of patients with adrenal ganglioneuromas (AGNs).

Design: Single-centre retrospective cohort study (1 January 1995 to 31 December 2019) and systematic review of literature (1 January 1980 to 19 November 2019).

Patients: Diagnosed with histologically confirmed AGN. Read More

Pol Arch Intern Med 2021 Mar 15. Epub 2021 Mar 15.

Intern Med 2021 Mar 15. Epub 2021 Mar 15.

Department of Endocrinology and Diabetes, Yamanashi Prefectural Central Hospital, Japan.

A 46-year-old woman with exacerbating hemoptysis and dyspnea was diagnosed with diffuse alveolar hemorrhage (DAH). High doses of glucocorticoids were initiated, but afterward, paroxysmal hypertension (210/140 mmHg) with headache and abdominal pain appeared. A 50-mm left adrenal tumor with an intense uptake by iodine-123 metaiodobenzylguanidine scintigraphy and catecholamine hypersecretion revealed complication with pheochromocytoma. Read More

EJNMMI Radiopharm Chem 2021 Mar 10;6(1):11. Epub 2021 Mar 10.

ICNAS/CIBIT - Institute for Nuclear Sciences Applied to Health, University of Coimbra, Pólo das Ciências da Saúde, Azinhaga de Santa Comba, 3000-548, Coimbra, Portugal.

The neurotracer 6-[18F] FDOPA has been, for many years, a powerful tool in PET imaging of neuropsychiatric diseases, movement disorders and brain malignancies. More recently, it also demonstrated good results in the diagnosis of other malignancies such as neuroendocrine tumours, pheochromocytoma or pancreatic adenocarcinoma.The multiple clinical applications of this tracer fostered a very strong interest in the development of new and improved methods for its radiosynthesis. Read More