Malignant pheochromocytoma

BACKGROUND: Malignant pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare tumors and available systemic therapies are limited. AIM: To explore the role of peptide receptor radionuclide therapy (PRRT) with Yttrium-90 (90 Y) and Lutetium-177 (177 Lu) peptides in pheochromocytomas (PCCs) and paragangliomas (PGLs). METHODS: We retrospectively analyzed more than 1500 patients with histologically proven neuroendocrine tumors treated with 177 Lu- or 90 Y-DOTA-TATE or -TOC between 1999 to 2017 at our Institute...

BACKGROUND: Pheochromocytomas (PHEOs) are rare catecholamine-secreting adrenal tumors. Approximately 60-90% of bilateral PHEOs are hereditary. We retrospectively analyzed the clinical characteristics of patients with bilateral PHEOs and the morbidity rate (malignancy, tumor recurrence and adrenal insufficiency (AI) rate) related to surgery technique and genetic status of the patients. RESULTS: Fourteen patients (12.5%, nine women, five men) had synchronous or metachronous bilateral PHEOs (out of 112 PHEO patients who underwent surgery between 1976 and 2021)...

INTRODUCTION AND IMPORTANCE: Phaeochromocytomas are neuroendocrine neoplasms arising from chromaffin cells of the adrenal medulla and are known as intra-adrenal paragangliomas. On the contrary, paragangliomas are non-epithelial neuroendocrine neoplasms that produce other peptide hormones such as norepinephrine, epinephrine, and dopamine. In a setting with limited resources, such a condition may result in inaccurate diagnosis and treatment, which may lose a patient life if left untreated...

BACKGROUND: Pediatric endocrine disorders requiring surgical intervention are rare and so are experienced surgeons dealing with these. The aim of the current study was to investigate disease profile and perioperative outcome of pediatric patients with surgical endocrine disorders in an endocrine surgery unit. METHODS: This retrospective study (Sep 1989-Aug 2019) consisted of pediatric endocrine surgery patients (<18 years) who were managed by a team of pediatric endocrinologists and endocrine surgeons at our center...

Data collected from approximately 1800 male and 1800 female Sprague-Dawley (SD) rats used in 104-week carcinogenicity studies were archived in a historical control database at Labcorp Early Development, Inc, and the neoplastic microscopic observation data from these rats were retrospectively evaluated. Historical control data can provide useful information on the range and incidence of spontaneously occurring background neoplasms in the species and strain of the test animal used in different types of toxicity studies, including studies of differing lengths, delivery of test article, and test animal...

PURPOSE: Silent pheochromocytoma refers to tumors without signs and symptoms of catecholamine excess. This study aimed to clarify the clinical, radiological characteristics, and perioperative features of silent pheochromocytomas diagnosed after adrenalectomy for adrenal incidentaloma. METHODS: Medical records of patients who underwent adrenalectomy for adrenal incidentaloma and were subsequently diagnosed with silent pheochromocytoma between January 2000 and December 2020 were retrospectively reviewed for demographic, diagnostic, surgical, and pathological findings...

INTRODUCTION: Incidentaloma is an adrenal tumor detected during diagnostic imaging performed for extra‑adrenal causes. Evaluation of metanephrine concentrations in a 24‑hour urine collection can be a significant challenge in patients with multiple medications and comorbidities. OBJECTIVES: The aim of this study was to evaluate the effect of commonly used groups of drugs on metanephrine levels in the 24‑hour urine collection. PATIENTS AND METHODS: A total of 1051 patients with adrenal mass below 10 Hounsfield units on unenhanced computed tomography were included in the study...

Background: Malignant pheochromocytomas are rare and aggressive tumors that arise from the adrenal medulla and secrete catecholamines. Patients exhibit episodic symptoms of hypertension, headaches, sweating, and palpitations. The diagnosis is supported by elevated levels of urinary metanephrines, and imaging is used to determine the stage. Treatment involves surgical resection when possible. Case Report: A 57-year-old male presented with hematemesis and melena, and endoscopy revealed upper gastrointestinal bleeding...

Multiple endocrine neoplasia 2B (MEN2B) is a rare syndrome with prevalence estimated at approximately 0.2 per 100,000; it is caused by mutation of the RET proto-oncogene. MEN2B is characterized by early-onset medullary thyroid carcinoma (MTC), ganglioneuromatosis of the aerodigestive tract, marfanoid habitus, ophthalmologic abnormalities, and pheochromocytoma in adulthood. Mutations in the RyR1 gene manifest clinically in congenital myopathies and/or malignant hyperthermia susceptibility. We present a case of a 4-year-old boy with an accidentally detected RET and RyR1 mutations in the course of diagnostic approach of short stature and delayed motor development...

Adrenal lesions (ALs) are often detected in patients with multiple endocrine neoplasia type 1 (MEN1). However, they are not well described in MEN1, making their clinical management unclear. This study examined the prevalence and outcomes of ALs found in MEN1. We performed a retrospective chart review of patients diagnosed with MEN1 from 1990 to 2021. ALs were diagnosed using abdominal or thoracic imaging and classified as being unilateral or bilateral, having single or multiple nodules, and as having diffuse enlargement or not...

UNLABELLED: Cancer immunotherapies have produced remarkable results in B-cell malignancies; however, optimal cell surface targets for many solid cancers remain elusive. Here, we present an integrative proteomic, transcriptomic, and epigenomic analysis of tumor specimens along with normal tissues to identify biologically relevant cell surface proteins that can serve as immunotherapeutic targets for neuroblastoma, an often-fatal childhood cancer of the developing nervous system. We apply this approach to human-derived cell lines (N=9) and cell/patient-derived xenograft (N=12) models of neuroblastoma...

OBJECTIVE: In the last years, robotic surgery was introduced in several different settings with good perioperative results. However, its role in the management of adrenal masses is still debated. In order to provide a contribution to this field, we described our step-by-step technique for robotic adrenalectomy (RA) and related modifications according to the type of adrenal mass treated. METHODS: We retrospectively analyzed 27 consecutive patients who underwent RA at Onze-Lieve-Vrouw hospital (Aalst, Belgium) between January 2009 and October 2022...

Paragangliomas are rare neuroendocrine tumors that arise from the extra-adrenal autonomic paraganglia, i.e., small organs consisting mainly of neuroendocrine cells that are derived from the embryonic neural crest and have the ability to secrete catecholamines. Paragangliomas can derive from either parasympathetic or sympathetic paraganglia. Most of the parasympathetic ganglia-derived paragangliomas are nonfunctional, and symptoms result from mass effect. Conversely, the sympathetic paragangliomas are functional and produce catecholamine...

This article aims to review current concepts in diagnosing and managing pheochromocytoma and paraganglioma (PPGL). Personalized genetic testing is vital, as 40-60% of tumors are linked to a known mutation. Tumor DNA should be sampled first. Next-generation sequencing is the best and most cost-effective choice and also helps with the expansion of current knowledge. Recent advancements have also led to the increased incorporation of regulatory RNA, metabolome markers, and the NETest in PPGL workup. PPGL presentation is highly volatile and nonspecific due to its multifactorial etiology...

Pheochromocytomas are rare neuroendocrine tumors that may secrete catecholamines, resulting in a wide array of clinical symptoms. While patients classically present with hypertension, headache, diaphoresis, and flushing, these symptoms are present in only 40% of cases. Here, we describe a 70-year-old woman whose predominant symptom was unexplained severe weight loss over a 12-month period associated with fatigue, anxiety, and palpitations at her endocrinologist and geriatrician visits. Diagnostic imaging was performed to assess for malignancy and demonstrated a 2...

COVID-19 has severely affected the delivery of surgical care worldwide. The aim of the present study was to evaluate its impact on adrenal surgery at our academic endocrine center. All primary adrenal surgeries performed at the University Hospital of Cologne, Germany between 01.01.2019 and 31.07.2022 were included. This time frame was divided into pre-Covid (before 02/20), acute Covid (until 05/21), and post acute period (after 05/2021). Demographics, clinic-pathologic characteristics and treatment of these patients were analyzed...

A 13-year-old was admitted to our clinic complaining about a vision loss of over 2 weeks. Bilateral optic disc edema, peripapillary flame-shaped hemorrhages, macular star pattern exudates, and cotton wool spots were found in fundoscopic examination. The OCT exam showed bilaterally serous retinal detachments in sub-foveal region with intraretinal exudates. A pediatric examination found a high systemic hypertension of 230/140 mm Hg, and laboratory tests revealed increased levels of plasmatic and urinary catecholamines...

BACKGROUND AND OBJECTIVES: Despite an increased adaptation of robotic adrenalectomy, its advantages over laparoscopic adrenalectomy (LA) have not been defined. The aim of the study was to compare perioperative outcomes of robotic versus laparoscopic lateral transabdominal adrenalectomy using a large single-center experience. METHODS: This was a retrospective single center study. Within 22 years, patients who underwent laparoscopic and robotic transabdominal lateral adrenalectomy were identified from a prospective institutional review board-approved database...

Von Hippel-Lindau (VHL) disease is an autosomal-dominant syndrome caused by mutations in the VHL gene, located on the short arm of chromosome 3. Patients with VHL are likely to manifest with a spectrum of multiple benign and malignant tumors involving various organ systems. We present a case of a 28-year-old female without a remarkable family history who presented with complaints of hematuria and abdominal discomfort. Initial laboratory investigations confirmed hematuria. Subsequent abdominal computed tomography scan revealed heterogeneous enhancing solid mass in bilateral kidneys, avidly enhancing mass in the right adrenal gland, bilateral simple renal cortical cysts, and a pancreatic cyst...

Pheochromocytoma (PCC) is a type of neuroendocrine tumor that originates from adrenal medulla or extra-adrenal chromaffin cells and results in the production of catecholamine. Paroxysmal hypertension and cardiovascular crises were among the clinical signs experienced by people with PCC. Five-year survival of advanced-stage PCC is just around 40% despite the identification of various molecular-level fundamentals implicated in these pathogenic pathways. MicroRNAs (miRNAs, miRs) are a type of short, non-coding RNA (ncRNA) that attach to the 3'-UTR of a target mRNA, causing translational inhibition or mRNA degradation...