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Diseases of the Esophagus : Official... Sep 2023Adrenal incidentalomas are regularly encountered during imaging for esophageal cancer patients, but their oncological significance remains unknown. This study aimed to...
Adrenal incidentalomas are regularly encountered during imaging for esophageal cancer patients, but their oncological significance remains unknown. This study aimed to describe the incidence and etiology of adrenal incidentalomas observed throughout the diagnostic workup. This retrospective cohort study included all esophageal cancer patients referred to or diagnosed in the Amsterdam UMC between January 2012 and December 2016. Radiology and multidisciplinary team meeting reports were reviewed for adrenal incidentalomas. In case of adrenal incidentaloma, the 18FDG-PET/CT was reassessed by a radiologist blinded for the original report. In case of a metachronous incidentaloma during follow-up, visibility on previous imaging was reassessed. Primary outcome was the incidence, etiology and oncological consequence of synchronous adrenal incidentalomas. This study included 1,164 esophageal cancer patients, with a median age of 66 years. Patients were predominantly male (76.1%) and the majority had an adenocarcinoma (69.0%). Adrenal incidentalomas were documented in 138 patients (11.9%) during the diagnostic workup. At primary esophageal cancer workup, 22 incidentalomas proved malignant. However, follow-up showed that four incidentalomas were inaccurately diagnosed as benign and three malignant incidentalomas were visible on staging imaging but initially missed. Stage migration occurred in 15 of 22 (68.2%), but this would have been higher if none were missed or inaccurately diagnosed. The oncological impact of adrenal incidentalomas in patients with esophageal cancer is significant as a considerable part of incidentalomas changed treatment intent from curative to palliative. As stage migration is likely, pathological examination of a synchronous adrenal incidentaloma should be weighted in mind.
Topics: Humans; Male; Aged; Female; Adrenal Gland Neoplasms; Positron Emission Tomography Computed Tomography; Retrospective Studies; Tomography, X-Ray Computed; Incidence; Esophageal Neoplasms
PubMed: 36722353
DOI: 10.1093/dote/doad003 -
Frontiers in Endocrinology 2020Pheochromocytoma (PCC) and paraganglioma (PGL) are rare neuroendocrine tumors associated with high cardiovascular morbidity and variable risk of malignancy. The current... (Review)
Review
Pheochromocytoma (PCC) and paraganglioma (PGL) are rare neuroendocrine tumors associated with high cardiovascular morbidity and variable risk of malignancy. The current therapy of choice is surgical resection. Nevertheless, PCCs/PGLs are associated with a lifelong risk of tumor persistence or recurrence. A high rate of germline or somatic mutations in numerous genes has been found in these tumors. For some, the tumorigenic processes are initiated during embryogenesis. Such tumors carry gene mutations leading to pseudohypoxic phenotypes and show more immature characteristics than other chromaffin cell tumors; they are also often multifocal or metastatic and occur at an early age, often during childhood. Cancer stem cells (CSCs) are cells with an inherent ability of self-renewal, de-differentiation, and capacity to initiate and maintain malignant tumor growth. Targeting CSCs to inhibit cancer progression has become an attractive anti-cancer therapeutic strategy. Despite progress for this strategy for solid tumors such as neuroblastoma, brain, breast, and colon cancers, no substantial advance has been made employing similar strategies in PCCs/PGLs. In the current review, we discuss findings related to the identification of normal chromaffin stem cells and CSCs, pathways involved in regulating the development of CSCs, and the importance of the stem cell niche in development and maintenance of CSCs in PCCs/PGLs. Additionally, we examine the development and feasibility of novel CSC-targeted therapeutic strategies aimed at eradicating especially recurrent and metastatic tumors.
Topics: Adrenal Gland Neoplasms; Animals; Humans; Neoplastic Stem Cells; Paraganglioma; Pheochromocytoma
PubMed: 32158431
DOI: 10.3389/fendo.2020.00079 -
Endocrine Sep 2022Almost 10 years have passed since the first attempts of liquid biopsy aimed at the characterisation of tumor cells present in the bloodstream from a regular sample of... (Review)
Review
Almost 10 years have passed since the first attempts of liquid biopsy aimed at the characterisation of tumor cells present in the bloodstream from a regular sample of peripheral blood were performed. Liquid biopsy has been used to characterise tumor heterogeneity in various types of solid tumors including adrenocortical carcinoma. The development of molecular biology, genetics, and methodological advances such as digital PCR and next-generation sequencing allowed us to use besides circulating tumor cells a variety of circulating cell-free nucleic acids, DNAs, RNAs and microRNAs secreted by tumors into blood and other body fluids as specific molecular markers. These markers are used for diagnosis, to check tumor development, selecting efficient therapies, therapy monitoring and even possess prognostic power. In adrenocortical carcinoma, there are some studies reporting analysis of circulating tumor cells, circulating cell free DNA and microRNAs for assessing tumor heterogeneity. Among microRNAs, hsa-miR-483-5p seems to be the most important player. Combined with other microRNAs like hsa-miR-195, their expression correlates with recurrence-free survival. Most studies support the applicability of liquid biopsy for assessing temporal tumor heterogeneity (i.e. tumor progression) in adrenocortical cancer. In this mini-review, the available findings of liquid biopsy for assessing tumor heterogeneity in adrenocortical cancer are presented.
Topics: Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Adrenocortical Carcinoma; Biomarkers, Tumor; Humans; Liquid Biopsy; MicroRNAs
PubMed: 35552979
DOI: 10.1007/s12020-022-03066-z -
Der Chirurg; Zeitschrift Fur Alle... Nov 2021Endocrine tumors and here in particular gastrointestinal neuroendocrine neoplasms (GEP-NET), pheochromocytomas (PC), paragangliomas (PGL) and thyroid tumors are prime... (Review)
Review
Endocrine tumors and here in particular gastrointestinal neuroendocrine neoplasms (GEP-NET), pheochromocytomas (PC), paragangliomas (PGL) and thyroid tumors are prime examples of the importance of molecular pathology and molecular biology for the diagnostics, classification and ultimately also the (surgical) treatment of these diseases. The GEP-NETs are graded using the Ki-67 index. This determines the type of molecular imaging (DOTA/DOPA/FDG-PET/CT), the possible treatment (surgical and/or radiopeptide therapy), antiproliferative and symptom-controlling treatment with somatostatin analogues and ultimately also the prognosis. The PC/PGLs can be hereditary (MEN2A, VHL, NF1, SDH mutations), which significantly influences the surgical treatment and preoperative medication. Molecular imaging is very important and can lead the way in cases of borderline biochemistry. Adrenal carcinomas can also be genetically determined. In the case of thyroid tumors, the pathology of the C‑cell (C-cell hyperplasia, medullary thyroid carcinoma) should be emphasized. In the case of hereditary diseases (FMTC, MEN2), early prophylactic surgery is often necessary and prevents the occurrence of advanced carcinomas; however, the determination of the extent of resection in follicular lesions or the distinction between noninvasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP) and follicular variants of papillary thyroid carcinoma can also be determined with the help of specific markers. Overall, molecular pathology has an increasingly more important role in these entities and is also the topic of ongoing research projects.
Topics: Adrenal Gland Neoplasms; Carcinoma, Neuroendocrine; Humans; Positron Emission Tomography Computed Tomography; Thyroid Cancer, Papillary; Thyroid Neoplasms
PubMed: 34618164
DOI: 10.1007/s00104-021-01512-8 -
Medicine Jan 2020Breast cancer is the most commonly diagnosed malignancies in females. The most common sites of metastasis are bone, lung, liver, and brain. Gastrointestinal and adrenal... (Review)
Review
RATIONALE
Breast cancer is the most commonly diagnosed malignancies in females. The most common sites of metastasis are bone, lung, liver, and brain. Gastrointestinal and adrenal gland metastasis from breast cancer are rare. Simultaneous metastases are extremely rare. Therefore, it is critically important to choose proper examination and treatment since the rapid diagnosis and primary treatment can significantly affect the survival of patients. To the best of our knowledge, this was the first case of initial dual metastasis.
PATIENT CONCERNS
The patient had a history of left breast cancer, and she underwent left breast-conserving surgery with sentinel lymph node biopsy 2 years ago. She was hospitalized in our center with the complaints of a stomach and lower back pain, which started suddenly and was progressively increased for half a month.
DIAGNOSIS
Computed tomography, gastroscopy, and immunohistochemical staining, especially GATA3 and mammaglobin, confirmed that there was simultaneous gastric and adrenal metastases.
INTERVENTIONS
She was eligible for the IMpassion131 clinical trials, a Phase 3 randomized, double-blind, placebo-controlled trial under treatment with atezolizumab/palcebo plus paclitaxel as adjuvant-therapy.
OUTCOMES
She was still undergoing the therapy and waiting for the further evaluation.
LESSONS
In order to better understand metastatic pathways of breast carcinoma, publications of individual patient cases diagnosed with rare metastatic sites should be encouraged, especially for the simultaneous rare metastatic sites. This might improve our understanding of metastatic behavior of breast cancer and promote further clinical research.
Topics: Adrenal Gland Neoplasms; Aged; Breast Neoplasms; Female; Humans; Randomized Controlled Trials as Topic; Stomach Neoplasms
PubMed: 32011488
DOI: 10.1097/MD.0000000000018812 -
Hormone and Metabolic Research =... Jul 2019Since Felix Fränkel's account of pheochromocytoma in 1886, great discoveries and vast advancements in the diagnosis, genetics, anatomical and functional imaging... (Review)
Review
Since Felix Fränkel's account of pheochromocytoma in 1886, great discoveries and vast advancements in the diagnosis, genetics, anatomical and functional imaging techniques, and surgical management of pheochromcytoma and paraganglioma (P-PGL) have been made. The improved insight in the pathophysiology of P-PGL and more accurate detection methods enable physicians to tailor the treatment plan to an individual based on the genetic profile and tumor behavior. This review will cover briefly the clinical features, diagnosis, genetic mutations, and imaging modalities that are used to guide current surgical management of these rare and interesting endocrinopathies.
Topics: Adrenal Gland Neoplasms; Humans; Mutation; Pheochromocytoma; Precision Medicine
PubMed: 31307109
DOI: 10.1055/a-0926-3618 -
Endocrine Sep 2022
Topics: Adrenal Gland Neoplasms; Humans; Italy
PubMed: 35867326
DOI: 10.1007/s12020-022-03147-z -
Cancer Feb 2022Von Hippel-Lindau disease (VHL) is a multineoplasm inherited disease manifesting with hemangioblastoma of the central nervous system and retina, adrenal... (Review)
Review
Von Hippel-Lindau disease (VHL) is a multineoplasm inherited disease manifesting with hemangioblastoma of the central nervous system and retina, adrenal pheochromocytoma, renal cell carcinoma, pancreatic neuroendocrine tumors and cysts, and neoplasms/cysts of the ear, broad ligament, and testicles. During 2018-2020, the VHL Alliance gathered several committees of experts in the various clinical manifestations of VHL to review the literature, gather the available evidence on VHL, and develop recommendations for patient management. The current report details the results of the discussion of a group of experts in the pancreatic manifestations of VHL along with their proposed recommendations for the clinical surveillance and management of patients with VHL. The recommendations subcommittee performed a comprehensive systematic review of the literature and conducted panel discussions to reach the current recommendations. The level of evidence was defined according to the Shekelle variation of the Grading of Recommendations, Assessment, Development, and Evaluation grading system. The National Comprehensive Cancer Network Categories of Evidence and Consensus defined the committee members' interpretation of the evidence and degree of consensus. The recommendations encompass the main aspects of VHL-related pancreatic manifestations and their clinical management. They are presented in a clinical orientation, including general planning of screening and surveillance for pancreatic neuroendocrine tumors, utility of biochemical biomarkers, the optimal choice for imaging modality, indirect risk stratification, indications for tissue sampling of VHL-related pancreatic neuroendocrine tumors, and interventions. These recommendations are designed to serve as the reference for all aspects of the screening, surveillance, and management of VHL-related pancreatic manifestations.
Topics: Adrenal Gland Neoplasms; Female; Hemangioblastoma; Humans; Kidney Neoplasms; Pancreatic Neoplasms; Pheochromocytoma; Von Hippel-Lindau Tumor Suppressor Protein; von Hippel-Lindau Disease
PubMed: 34735022
DOI: 10.1002/cncr.33978 -
Cancer Control : Journal of the Moffitt... Apr 2011Adrenal lesions are commonly identified in patients with extra-adrenal cancer. When lesions are present, it is important to identify if the lesion is a metastasis of the... (Review)
Review
BACKGROUND
Adrenal lesions are commonly identified in patients with extra-adrenal cancer. When lesions are present, it is important to identify if the lesion is a metastasis of the primary cancer or a primary adrenal neoplasm. If primary, the adrenal lesion must be evaluated for hypersecretion and its malignant potential determined for appropriate treatment planning.
METHODS
Recent literature was reviewed that focused on the normal investigation of adrenal lesions including radiographic imaging and hormonal evaluations as well as specific focused therapeutic options available for isolated metastatic adrenal lesions.
RESULTS
This review presents a pathway approach in investigating these lesions and also discusses various potential treatment options.
CONCLUSIONS
A proper investigative workup of an adrenal lesion in a cancer patient is critical for proper management. Isolated adrenal metastatic lesions in the cancer patient should be surgically removed when possible, but other options can be considered. In patients who do not have metastasis from extra-adrenal cancer, the decision for surgical resection is dependent on functionality of the tumor and it's potential for malignancy. Observation plays a key role in those tumors that are nonfunctioning and have a low risk of malignancy.
Topics: Adrenal Gland Neoplasms; Adrenal Glands; Catheter Ablation; Humans; Incidence; Laparoscopy; Neoplasms
PubMed: 21451454
DOI: 10.1177/107327481101800206 -
Hormones & Cancer Dec 2011Due to broader use of conventional imaging techniques, adrenal tumors are detected with increasing frequency comprising a wide variety of different tumor entities.... (Review)
Review
Due to broader use of conventional imaging techniques, adrenal tumors are detected with increasing frequency comprising a wide variety of different tumor entities. Despite improved conventional imaging techniques, a significant number of adrenal lesions remain that cannot be easily determined. A particular diagnostic challenge are lesions in patients with known extra-adrenal malignancy because these patients frequently harbor adrenal metastases. Furthermore, adrenal masses with low fat content and no detectable hormone excess are difficult to diagnose properly. Fine needle biopsy is invasive, often unsuccessful, and puts patients at risk, e. g., in cases of pheochromocytoma or adrenal cancer. Noninvasive characterization using radiotracers has therefore been established in recent years. (18)F-FDG PET helps to differentiate benign from malignant lesions. However, it does not distinguish between adrenocortical or nonadrenocortical lesions (e.g., metastases or adrenocortical carcinoma). More recently, enzyme inhibitors have been developed as tracers for adrenal imaging. Metomidate is most widely used. It binds with high specificity and affinity to CYP11B enzymes of the adrenal cortex. As these enzymes are exclusively expressed in adrenocortical cells, uptake of labeled metomidate tracers has been shown to be highly specific for adrenocortical neoplasia. (11)C-metomidate PET and (123)I-iodometomidate SPECT imaging has been introduced into clinical use. Both tracers not only distinguish between adrenocortical and nonadrenocortical lesions but are also able to visualize metastases of adrenocortical carcinoma. The very specific uptake has recently led to first application of (131)I-iodometomidate for radiotherapy in ACC. In conclusion, metomidate-based imaging is an important complementary tool to diagnose adrenal lesions that cannot be determined by other methods.
Topics: Adrenal Gland Neoplasms; Adrenal Glands; Adrenocortical Carcinoma; Animals; Carbon Radioisotopes; Diagnosis, Differential; Early Detection of Cancer; Etomidate; Humans; Neoplasm Metastasis; Pheochromocytoma; Positron-Emission Tomography; Radioactive Tracers; Steroid 11-beta-Hydroxylase; Tomography, Emission-Computed, Single-Photon
PubMed: 22124841
DOI: 10.1007/s12672-011-0093-3