China
Africa
Explore chapters and articles related to this topic
Paper 4
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
The main differential diagnosis is adrenocortical carcinoma. This has peak incidences in early childhood and in the fourth and fifth decade of life. This tumour is usually large at diagnosis and typically has central necrosis or haemorrhage. There is calcification in 30% and it can invade the inferior vena cava or the renal vein. However, adrenocortical carcinoma is relatively rare compared to metastatic adenocarcinoma.
Endocrine and Neuroendocrine Tumors
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Natasha Shrikrishnapalasuriyar, P.N. Plowman, Márta Korbonits, Ashley B. Grossman
The outlook for adrenocortical carcinoma remains grim, and it is not expected that there will be any major improvement in chemotherapy regimens. There is extensive research on the molecular genetics of such tumors, and it seems likely that only through a thorough understanding of the deranged pathways will effective targeted agents be developed. Unfortunately, there is little likelihood of such new agents appearing in the immediate future.
The Adrenal Glands
Published in E. George Elias, CRC Handbook of Surgical Oncology, 2020
This clinical syndrome is usually caused by benign conditions, and rarely by adrenocortical carcinoma. It has been estimated that 60% of primary aldosteronism is caused by unilateral adenoma, and less than 40% is caused by bilateral adrenocortical hyperplasia. Less than 5% is caused by adrenocortical carcinoma.3 Patients with primary aldosteronism present with hypertension, hypokalemia with metabolic alkalosis, high serum sodium, low serum calcium (secondary to the metabolic alkalosis), low hematocrit, polyuria, and polydypsia with high urinary potassium.
How good are the current models of adrenocortical carcinoma for novel drug discovery?
Published in Expert Opinion on Drug Discovery, 2022
Carmen Ruggiero, Mabrouka Doghman-Bouguerra, Enzo Lalli
During the last decade, significant advances have been made in the definition of the molecular mechanisms underlying the onset and progression of adrenocortical carcinoma (ACC). A better understanding of ACC tumorigenesis has come from the identification of several genetic and molecular drivers of this malignancy thanks to an extensive profiling analysis of ACC tumors. Unfortunately, the development of novel therapeutic options has been hampered by the relative lack of in vitro and in vivo preclinical models recapitulating the entire spectrum of ACC heterogeneity, molecular features, tumor microenvironment and sensitivity to the available treatments. The recent establishment and implementation of novel ACC cell lines, genetically engineered mouse models, patient-derived ACC xenografts (PDX) in mice and emerging preclinical in vivo models offer novel experimental possibilities for drug discovery.
Combination immunotherapy with ipilimumab and nivolumab in patients with advanced adrenocortical carcinoma: a subgroup analysis of CA209-538
Published in OncoImmunology, 2021
Oliver Klein, Clare Senko, Matteo S Carlino, Ben Markman, Louise Jackett, Bo Gao, Caroline Lum, Damien Kee, Andreas Behren, Jodie Palmer, Jonathan Cebon
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with a reported incidence of 0.5–2 cases per million population per year.1 The overall prognosis of patients with locally advanced or metastatic ACC not amendable to surgery is poor and treatment options are very limited. Expert consensus guidelines have been published to assist with the management of ACC patients.2 The current standard treatment is a polychemotherapy regimen combined with the adrenolytic agent mitotane that has demonstrated a progression free survival without an overall survival benefit and is associated with a high rate of treatment-related sided effects.3 There are no established second-line therapies and several clinical trials using different targeted agents have been unsuccessful.4
Delayed Metastasis of Clear Cell Sarcoma of Kidney to Bladder After 7 Disease-Free Years
Published in Fetal and Pediatric Pathology, 2018
The 5-year survival rate of childhood cancers in general is now 80% or greater (29,30). Currently, it is estimated that one in every 640–750 young adults between the ages of 20 and 39 is a survivor of pediatric cancer (30,31). With advances in the management of pediatric solid tumors, there has been an established significant risk for late mortality because of increased rates of treatment-related deaths in later decades (32–34). For example, the 25-year cumulative risk of late mortality of survivors of neuroblastoma is reported at 6% with the main causes of death being disease recurrence, secondary malignant neoplasms, and cardiac complications (35). Hertel et al. reported a case of an 8-year delayed recurrence of adrenocortical carcinoma (36). Late recurrence in pediatric solid tumors, though uncommon, still demonstrate the need for long-term clinical follow-up (1).