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Int J Gynecol Pathol 2021 May 12. Epub 2021 May 12.

Department of Pathology, Yale-New Haven Hospital, Yale School of Medicine (L.I., K.E.F., V.P.) Department of Obstetrics and Reproductive Sciences, Yale University School of Medicine, New Haven (V.P.) Department of Obstetrics and Gynecology, Yale-New Haven Health, Bridgeport Hospital, Bridgeport, Connecticut (M.C.) Department of Pathology, University of California San Diego, San Diego, California (O.F.).

Patients with germline TP53 mutations are characterized by the occurrence of multiple early-onset malignancies. The characteristic syndrome is Li-Fraumeni syndrome (OMIM # 151623), an autosomal dominant disorder typified by premenopausal breast carcinoma, adrenal cortical tumors, bone and soft tissue sarcomas, leukemias, and tumors of the brain and spinal cord. Gynecologic malignancies are uncommonly reported in families harboring TP53 mutations, and the predominant tumor type reported is ovarian. Read More

Front Endocrinol (Lausanne) 2021 26;12:669426. Epub 2021 Apr 26.

Medical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia at ASST Spedali Civili di Brescia, Brescia, Italy.

Progesterone (Pg) and estrogen (E) receptors (PgRs and ERs) are expressed in normal and neoplastic adrenal cortex, but their role is not fully understood. In literature, Pg demonstrated cytotoxic activity on AdrenoCortical Carcinoma (ACC) cells, while tamoxifen is cytotoxic in NCI-H295R cells. Here, we demonstrated that in ACC cell models, ERs were expressed in NCI-H295R cells with a prevalence of ER- over the ER-. Read More

J Adolesc Young Adult Oncol 2021 May 12. Epub 2021 May 12.

Department of Internal Medicine, Guthrie Robert Packer Hospital, Sayre, Pennsylvania, USA.

Adrenal cortical carcinoma is a rare and aggressive cancer with poor prognosis. Cases usually present with signs and symptoms of excessive hormone production. Hyperglycemia and Cushing syndrome are common, but tumor-associated hypoglycemia due to paraneoplastic secretion of insulin-like growth factor-2 (termed Anderson's syndrome) is uncommon. Read More

Front Endocrinol (Lausanne) 2021 22;12:674039. Epub 2021 Apr 22.

Medical Oncology Unit, Azienda Socio Sanitaria Territoriale (ASST) Spedali Civili, Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, Brescia, Italy.

Background: In a recently published retrospective case series, Temozolomide was found active as second line approach in advanced ACC patients. The disease control rate obtained, however, was short-lived. We report here an ACC patient with extensive metastatic disease who obtained a remarkable long lasting response with this alkylating agent. Read More

BMJ Case Rep 2021 May 7;14(5). Epub 2021 May 7.

Medical Unit, National Hospital for Respiratory Disease, Welisara, Sri Lanka.

Small cell lung carcinoma, when associated with co-occurrence of complications such as paraneoplastic syndrome and superior vena cava syndrome, poses a greater management challenge to the clinical team. We report a 56-year-old man who was eventually diagnosed with stage III small cell lung carcinoma, presenting with respiratory distress, facial and upper body oedema, proximal muscle weakness, hypokalaemia, new-onset hypertension and hyperglycaemia. His medical management was complicated by associated superior vena cava syndrome and Cushing's syndrome leading to refractory hypokalemia, immunosuppression and depression. Read More

Horm Metab Res 2021 May 7;53(5):285-292. Epub 2021 May 7.

Department of Endocrinology, Diabetes and Metabolism, Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal.

Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy. For stage I and II tumors, surgery is a curative option, but even in these cases recurrence is frequent. Practical guidelines advocate a combination of mitotane with etoposide, doxorubicin, and cisplatin as first-line therapy for metastatic adrenocortical carcinoma. Read More

Am J Med Genet A 2021 May 7. Epub 2021 May 7.

Department of Pediatrics, Cedars-Sinai Medical Center, Los Angeles, California, USA.

Beckwith-Wiedemann syndrome (BWS) is a genetic overgrowth and cancer predisposition syndrome that can be associated with a spectrum of clinical features including isolated lateralized overgrowth, macrosomia, macroglossia, organomegaly, omphalocele/umbilical hernia, and distinct facial features. Because of a range of clinical presentations and molecular defects involving Chromosome 11p15, many cases will fall within what is now being defined as the Beckwith-Wiedemann spectrum (BWSp). Cushing syndrome (CS) in infants is a rare neuroendocrinological disease associated with hypercortisolism that has rarely been reported in patients with BWS. Read More

Cureus 2021 Apr 1;13(4):e14239. Epub 2021 Apr 1.

Faculty of Medicine, Memorial University of Newfoundland, St. John's, CAN.

Adrenocortical carcinoma (ACC) is a rare, highly malignant endocrine tumor, often associated with a poor prognosis. Most patients who develop ACC are either children of ages 1-6, or adults in their fourth to fifth decade of life. Individuals with a functional cortisol-secreting ACC frequently present with Cushing syndrome. Read More

BMJ Case Rep 2021 May 6;14(5). Epub 2021 May 6.

Department of Diabetes and Endocrinology, Mater Dei Hospital, Msida, Malta.

An elderly gentleman was admitted to hospital with severe hypokalaemia of 1.75mmol/L. A background of a recently diagnosed metastatic gastric carcinoma with a neuroendocrine component pointed towards the diagnosis of ectopic ACTH secretion causing this dangerous electrolyte imbalance. Read More

Case Rep Endocrinol 2021 14;2021:5584198. Epub 2021 Apr 14.

Division of Endocrinology and Metabolism, Japanese Red Cross Musashino Hospital, Tokyo, Japan.

Background: Adrenocortical carcinoma (ACC) is a rare and highly aggressive malignancy. ACCs often secrete adrenal steroid hormones including cortisol and androgens; however, aldosterone-producing ACC is very rare. Although adrenal production of aldosterone is assessed by adrenal venous sampling, the use of sampling from the relevant vein to assess aldosterone production from a tumor arising from ACC metastasis has not been previously reported. Read More

Aging (Albany NY) 2021 Apr 21;13(8):11919-11941. Epub 2021 Apr 21.

Department of Urology, The Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi 710000, China.

M6A-related genes have been proven to play an important role in many cancers. However, the role of that in adrenocortical carcinoma (ACC) has not been fully elucidated. In the present study, 77 ACC samples from TCGA database were divided into localized ( = 46) and metastatic ( = 31) groups. Read More

Cureus 2021 Apr 1;13(4):e14235. Epub 2021 Apr 1.

Endocrinology, Diabetes and Metabolism, Faculty of Medical Sciences, Lebanese University, Beirut, LBN.

Incidentalomas are commonly encountered adrenal lesions. However, adrenocortical carcinoma (ACC) represents a rare etiology of adrenal incidentalomas (AI). The diagnosis of AI is generally based on laboratory data and imaging results, Fine needle aspiration (FNA) is not usually indicated in the workup of incidentaloma. Read More

EClinicalMedicine 2021 May 26;35:100850. Epub 2021 Apr 26.

Departamento de Patologia, Faculdade de Medicina da Universidade de São Paulo. Av. Dr. Arnaldo, 455, 01246-903 São Paulo, SP, Brasil.

Background: COVID-19 in children is usually mild or asymptomatic, but severe and fatal paediatric cases have been described. The pathology of COVID-19 in children is not known; the proposed pathogenesis for severe cases includes immune-mediated mechanisms or the direct effect of SARS-CoV-2 on tissues. We describe the autopsy findings in five cases of paediatric COVID-19 and provide mechanistic insight into the mechanisms involved in the pathogenesis of the disease. Read More

Biomed Res Int 2021 15;2021:5546612. Epub 2021 Apr 15.

Department of Urology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.

Background: Neurovascular-related genes have been implicated in the development of cancer. Studies have shown that a high expression of neuropilins (NRPs) promotes tumourigenesis and tumour malignancy.

Method: A multidimensional bioinformatics analysis was performed to examine the relationship between NRP genes and prognostic and pathological features, tumour mutational burden (TMB), microsatellite instability (MSI), and immunological features based on public databases and find the potential prognostic value of NRPs in pancancer. Read More

Cureus 2021 Mar 28;13(3):e14159. Epub 2021 Mar 28.

Internal Medicine, Detroit Medical Center (DMC) Harper Hospital, Detroit, USA.

McCune Albright Syndrome (MAS) is caused by a mutation in the GNAS gene that results in multiple endocrinopathies such as Cushing syndrome, acromegaly, hyperthyroidism, and precocious puberty. Despite the presence of pleiotropy coupled with a GNAS gene mutation, malignancy is a rare occurrence in MAS. There is minimal literature showcasing squamous cell carcinoma (SCC) of the lung in patients with MAS. Read More

Cancers (Basel) 2021 Apr 9;13(8). Epub 2021 Apr 9.

First Department of Pathology, Medical School, National and Kapodistrian University of Athens, 115 27 Athens, Greece.

Adrenocortical carcinoma is a rare malignancy with aggressive behavior, with up to 40% of patients presenting with metastases at the time of diagnosis. Both conventional chemotherapeutic regimens and novel immunotherapeutic agents, many of which are currently being tested in ongoing clinical trials, have yielded modest results so far, bringing the need for a deeper understanding of adrenal cancer behavior to the forefront. In the recent years, the tumor microenvironment has emerged as a major determinant of cancer response to immunotherapy and an increasing number of studies on other solid tumors have focused on manipulating the microenvironment in the favor of the host and discovering new potential target molecules. Read More

Cancers (Basel) 2021 Apr 6;13(7). Epub 2021 Apr 6.

Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital Würzburg, 97080 Würzburg, Germany.

A clinically relevant proportion of adrenocortical carcinoma (ACC) cases shows a tendency to metastatic spread. The objective was to determine whether the epithelial to mesenchymal transition (EMT), a mechanism associated with metastasizing in several epithelial cancers, might play a crucial role in ACC. 138 ACC, 29 adrenocortical adenomas (ACA), three normal adrenal glands (NAG), and control tissue samples were assessed for the expression of epithelial (E-cadherin and EpCAM) and mesenchymal (N-cadherin, SLUG and SNAIL) markers by immunohistochemistry. Read More

J Pers Med 2021 Apr 4;11(4). Epub 2021 Apr 4.

Internal Medicine, Department of Clinical and Biological Sciences, San Luigi Gonzaga Hospital, University of Turin, 10043 Turin, Italy.

A relevant issue on the treatment of adrenocortical carcinoma (ACC) concerns the optimal duration of adjuvant mitotane treatment. We tried to address this question, assessing whether a correlation exists between the duration of adjuvant mitotane treatment and recurrence-free survival (RFS) of patients with ACC. We conducted a multicenter retrospective analysis on 154 ACC patients treated for ≥12 months with adjuvant mitotane after radical surgery and who were free of disease at the mitotane stop. Read More

In Vivo 2021 May-Jun;35(3):1805-1810

Department of Oncology-Radiotherapy, "Prof. Dr. Alexandru Trestioreanu" Institute of Oncology, "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.

Background/aim: Kidney cancers account for about 2% of human malignancies. In recent decades, the incidence of this cancer type has gradually increased, mainly due to advances in imaging. The metastatic potential of these cancers is significant: a quarter of patients will immediately present with metastases and more than one third of patients treated with nephrectomy for a localized disease will develop metastases during their course. Read More

Oncol Lett 2021 Jun 15;21(6):476. Epub 2021 Apr 15.

Department of Endocrinology, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi Zhuang Autonomous Region 530021, P.R. China.

Adrenocortical carcinoma (ACC) is an endocrine tumour with high malignancy, high invasiveness and poor prognosis. Curcumin, a major component in turmeric, has been reported to have good efficacy and biological safety in treating cancer. However, the role and mechanism of curcumin in ACC have not yet been fully investigated and were thus the focus of this study. Read More

Hum Pathol 2021 Apr 24. Epub 2021 Apr 24.

Department of Pathology, Yale School of Medicine, New Haven, CT. Electronic address:

Studies comparing the histomorphologic features and phenotypic heterogeneity between primary and its corresponding metastatic hepatocellular carcinoma (HCC) are lacking. The aim of this study was to assess and compare the histomorphologic features and heterogeneity between primary and metastatic HCC. A total of 39 cases with both primary and metastatic tissues were identified from pathology archives (2000-2019). Read More

In Vitro Cell Dev Biol Anim 2021 Apr 26. Epub 2021 Apr 26.

Department of Bioprocess Engineering and Biotechnology, Federal University of Paraná, P.O. box 19011, Curitiba, Paraná, 81531-990, Brazil.

This study presents an in vitro evaluation of the antitumor potential of a chitin-like exopolysaccharide (EPS, produced by Mortierella alpina) on Adrenocortical carcinoma cells (ACC) compared to mitotane, a commercial drug commonly used in ACC treatment, and known for its side effects. Techniques of cellular viability determination such as MTT and fluorescence were used to measure the cytotoxic effects of the EPS and mitotane in tumoral cells (H295R) and non-tumoral cells (VERO), observing high cytotoxicity of mitotane and a 10% superior pro-apoptotic effect of the EPS compared to mitotane (p < 0.05). Read More

Expert Opin Emerg Drugs 2021 May 3:1-14. Epub 2021 May 3.

Division of Medical Oncology, Department of Internal Medicine, The Ohio State University and Arthur G. James Cancer Center, Columbus, Ohio, USA.

: Adrenocortical cancer (ACC) is a rare and aggressive disease with a median survival of 14-17 months and 5-year survival of around 20% for advanced disease. Emerging evidence of sub-groups of ACC with specific molecular drivers indicate ACC may be amenable to inhibition of receptor tyrosine kinases involved in growth and angiogenic signaling. A significant subset of patients may also be responsive to immune strategies. Read More

Int J Surg Pathol 2021 Apr 23:10668969211012085. Epub 2021 Apr 23.

5803Memorial Sloan-Kettering Cancer Center, New York, USA.

Cancer-testis (CT) antigens were identified by their ability to elicit T- or B-cell immune responses in the autologous host. They are typically expressed in a wide variety of neoplasms and in normal adult tissues are restricted to testicular germ cells. PReferentially expressed Antigen of Melanoma (PRAME) is a member of the family of nonclassical CT antigens being expressed in a few other normal tissues besides testis. Read More

Oncoimmunology 2021 Apr 12;10(1):1908771. Epub 2021 Apr 12.

Department of Medical Oncology, Austin Health, Melbourne, Australia.

: Adrenocortical carcinoma is a rare malignancy, with poor prognosis and limited treatment options for patients with advanced disease. Chemotherapy is the current standard first-line treatment, providing only a modest survival benefit. There is only limited treatment experience with immunotherapy using single-agent anti-PD-1/PD-L1 therapy. Read More

Rare Tumors 2021 9;13:20363613211007792. Epub 2021 Apr 9.

Cardiology, Scripps Health, San Diego, CA, USA.

A case of pheochromocytoma producing vasoactive intestinal peptide (VIP) and left ventricular thrombus in the absence of cardiomyopathy or wall motion abnormalities on echocardiogram is presented along with a review of the relevant literature. A 30-year-old female of Afghani descent with past medical history of panic attacks presented with fever, cough, sore throat, vomiting, and was found to have an 11 cm adrenal mass consistent with primary adrenocortical adenoma versus carcinoma. Her tumor elicited catechols and vasoactive intestinal peptide. Read More

Oncotarget 2021 Apr 13;12(8):845-858. Epub 2021 Apr 13.

GMDx Group Ltd, Melbourne, Victoria, Australia.

Somatic mutation signatures are an informative facet of cancer aetiology, however they are rarely useful for predicting patient outcome. The aim of this study is to evaluate the utility of a panel of 142 mutation-signature-associated metrics (P142) for predicting cancer progression in patients from a 'TCGA PanCancer Atlas' cohort. The P142 metrics are comprised of AID/APOBEC and ADAR deaminase associated SNVs analyzed for codon context, strand bias, and transitions/transversions. Read More

Radiol Case Rep 2021 Jun 3;16(6):1329-1334. Epub 2021 Apr 3.

Department of Radiology, Hanoi Medical University, Hanoi, Vietnam.

Adrenocortical carcinoma (ACC) is a rare malignancy that arises from the adrenal cortex and can be classified as either non-functioning or functioning. A patient with non-functioning ACC may present no specific symptoms. Imaging analysis can provide some information to a clinician who suspects ACC, such as tumor size, density, washout, necrosis, hemorrhage, and calcification. Read More

Clin Med Insights Oncol 2021 8;15:11795549211007682. Epub 2021 Apr 8.

Division of Hematology-Oncology, Department of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon.

Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis. Treatment options for ACC are limited, with resection the main intervention. Most cases present in late metastatic cases, and data regarding effective therapies is limited. Read More

Expert Opin Drug Metab Toxicol 2021 May 4:1-7. Epub 2021 May 4.

Department of Internal Medicine, Máxima MC, Veldhoven, Eindhoven, The Netherlands.

Introduction: Mitotane is the only drug registered specifically for adrenocortical carcinoma. Finding the optimal dose for a patient is difficult due to large differences in bioavailability, toxicity and effect. We therefore look to improve personalized dosing of mitotane. Read More