“Renal medullary carcinoma”

Renal medullary carcinoma (RMC) is a lethal disease that predominantly afflicts young individuals with sickle cell trait. Our recently reported molecular profiling of primary untreated RMC tissues elucidated distinct genomic and immune hallmarks of RMC, and identified MYC-induced replication stress as a targetable vulnerability for this disease.

BACKGROUND: To the authors' knowledge, AREN0321 is the first prospective clinical study of pediatric and adolescent renal cell carcinoma (RCC). Goals of the study included establishing epidemiological, treatment, and outcome data and confirming that patients with completely resected pediatric RCC, including lymph node-positive disease (N1), have a favorable prognosis without adjuvant therapy. METHODS: From 2006 to 2012, patients aged <30 years with centrally reviewed pathology of RCC were enrolled prospectively...

INTRODUCTION: Ipilimumab plus nivolumab has been approved for intermediate- and poor-risk metastatic renal cell carcinoma (RCC). However, the activity in non-clear cell RCC (nccRCC) is unknown. PATIENTS AND METHODS: Patients from Cleveland Clinic and the University of Texas Southwestern who had received ipilimumab plus nivolumab for metastatic nccRCC from October 2017 to May 2019 were retrospectively identified. Ipilimumab plus nivolumab was administered in accordance with the CHECKMATE 214 trial...

Clear cells in thyroid neoplasms can take two main forms: balloon-shaped and signet-ring cells. Balloon-shaped cell change in follicular adenoma is rare. A review of the literature has revealed only 20 previously published cases. We report herein a new case in the right thyroid lobe of a 45-year-old man. The clinicopathological data of the 21 cases including our paper have revealed that the ages of the patients ranged from 22-70 years, with a mean of 41.6 years. There was a clear predominance in women (M:F, 1:6)...

Angiogenesis has an essential role in tumor growth and metastasis, and blocking this pathway has been a successfully utilized strategy in the clinical treatment of cancer. Anlotinib (AL3818) is a novel oral receptor tyrosine kinase inhibitor targeting vascular endothelial growth factor receptor 2 and 3, fibroblast growth factor 1-4, platelet-derived growth factor receptor α and β, c-Kit and Ret. Anlotinib exerts inhibitory effects on tumor growth and angiogenesis and received its first approval as a third-line treatment for refractory advanced non-small-cell lung cancer in May 2018 and its second approval as a second-line treatment for advanced soft-tissue sarcoma in June 2019 in the People's Republic of China...

No abstract text is available yet for this article.

INTRODUCTION: Renal medullary carcinoma (RMC) is a highly lethal adenocarcinoma with a propensity for widespread metastatic disease in young patients. It is strongly associated with sickle cell trait and shows the loss of SMARCB1 (also known as INI1 or BAF47) protein expression. In the present study, we reviewed a series of 12 patients for whom the cytology specimens played a significant role in patient treatment. MATERIALS AND METHODS: We performed a retrospective case review of patients with a history of RMC from 3 large tertiary care pathology practices...

PURPOSE OF REVIEW: Sarcomas are a diverse group of rare solid tumors with limited treatment options for patients with advanced, inoperable disease. Cabozantinib is a tyrosine kinase inhibitor currently approved for advanced renal cell, hepatocellular, and medullary thyroid carcinoma. Cabozantinib has potent activity against a variety of kinases, including MET, vascular endothelial growth factor receptor, and AXL, that are associated with sarcoma growth and development. Here we review the preclinical findings and clinical development of cabozantinib in the treatment of soft tissue sarcoma, gastrointestinal stromal tumors (GIST), osteosarcoma, and Ewing sarcoma...

SUMMARY: We report a rare case of concurrent medullary thyroid cancer (MTC) and papillary thyroid cancer (PTC) with intermixed disease in several of the lymph node (LN) metastases in a patient who was subsequently diagnosed with clear cell renal cell carcinoma (RCC). A 56 year old female presented with dysphagia and was found to have a left thyroid nodule and left superior cervical LN with suspicious sonographic features. Fine needle aspiration biopsy (FNAB) demonstrated PTC in the left thyroid nodule and MTC in the left cervical LN...

PURPOSE OF REVIEW: Sarcomas are a diverse group of rare solid tumors with limited treatment options for patients with advanced, inoperable disease. Cabozantinib is a tyrosine kinase inhibitor currently approved for advanced renal cell, hepatocellular, and medullary thyroid carcinoma. Cabozantinib has potent activity against a variety of kinases, including MET, vascular endothelial growth factor receptor, and AXL, that are associated with sarcoma growth and development. Here we review the preclinical findings and clinical development of cabozantinib in the treatment of soft tissue sarcoma, gastrointestinal stromal tumors (GIST), osteosarcoma, and Ewing sarcoma...

Renal medullary carcinoma (RMC) is a highly lethal malignancy that mainly afflicts young individuals of African descent and is resistant to all targeted agents used to treat other renal cell carcinomas. Comprehensive genomic and transcriptomic profiling of untreated primary RMC tissues was performed to elucidate the molecular landscape of these tumors. We found that RMC was characterized by high replication stress and an abundance of focal copy-number alterations associated with activation of the stimulator of the cyclic GMP-AMP synthase interferon genes (cGAS-STING) innate immune pathway...

BACKGROUND: Cigarette smoking is causally linked to renal cell carcinoma (RCC). However, associations for individual RCC histologies are not well-described. Newly-available data on tobacco use from population-based cancer registries allow characterization of associations with individual RCC types. METHODS: We analyzed data for 30,282 RCC cases from 8 states that collected tobacco use information for a National Program of Cancer Registry project. We compared the prevalence and adjusted prevalence ratios (aPR) of cigarette smoking (current vs never, former vs never) among individuals diagnosed between 2011 and 2016 with clear cell RCC, papillary RCC, chromophobe RCC, renal collecting duct/medullary carcinoma, cyst-associated RCC, and unclassified RCC...

Renal medullary carcinoma is a rare and highly aggressive tumor seen almost exclusively in young individuals of African descent with sickle cell disease. Here, we describe a case of a 29-year-old man, who did not have sickle cell disease, with pathologically confirmed renal medullary carcinoma using dual-time FDG PET/CT.

Various pathologic neoplasms present in both childhood and adulthood. Multiple environmental and genetic factors with different targets at different ages have been implicated in prior literature. These tumors include Multi-Locular Cystic Renal Tumors (MCRT), Adrenocortical Carcinomas, Paragangliomas, Hepatic Hemangiomas, and Medullary Renal Cell Carcinomas. The pertinent imaging, pathological, and clinical features of these select entities are discussed to assist the General and Pediatric Radiologist in accurate interpretation and treatment planning...

Calcitonin (CT) is a marker for both initial diagnosis and monitoring of patients with residual or recurrent medullary thyroid carcinoma (MTC). In Japan, serum CT had been measured by radioimmunoassay (RIA) until recently. Electrochemiluminescence immunoassay (ECLIA) became commercially available in 2014, and this technique is now the only method used to examine CT concentration. The purposes of this study were to investigate the correlations between the CT concentration measured with ECLIA (ECLIA-CT) and RIA (RIA-CT) and to explore the clinical characteristics of patients with elevated ECLIA-CT...

No abstract text is available yet for this article.

Renal medullary carcinoma (RMC) is a rare, aggressive disease that predominantly afflicts individuals of African or Mediterranean descent with sickle cell trait. RMC comprises 1% of all renal cell carcinoma diagnoses with a median overall survival of 13 months. Patients are typically young (median age-22) and male (male:female ratio of 2:1) and tumors are characterized by complete loss of expression of the SMARCB1 tumor suppressor protein. Due to the low incidence of RMC and the disease's aggressiveness, treatment decisions are often based on case reports...

PURPOSE: We evaluated stage at presentation and cancer specific mortality according to variant histology relative to clear cell renal cell carcinoma. MATERIALS AND METHODS: Within the Surveillance, Epidemiology, and End Results registry (2001-2016) we identified variant histology and clear cell renal cell carcinoma cases. Cumulative incidence plots, multivariate Cox regression models matched for stage, grade and other patient characteristics addressed cancer specific mortality...

Renal medullary carcinoma (rmc) is a rare and aggressive renal malignancy that usually presents at an advanced stage, has a poor prognosis, and is associated with sickle cell trait. We present a case of rmc including radiologic and pathology findings, treatment, and outcome. A review of the literature is also presented, with an emphasis on the association of rmc with sickle cell trait, which was an unknown diagnosis in our patient preoperatively.

BACKGROUND Microscopic tumor foci have been detected incidentally on renal biopsy, including renal cell carcinoma and renomedullary interstitial cell tumor (medullary fibroma). A report is presented of a case of an incidental finding of microscopic renal angiomyolipoma that was diagnosed and completely excised on core needle biopsy. CASE REPORT A 44-year-old woman was referred to our hospital for evaluation of persistent mild proteinuria. Three years previously, she was diagnosed with Cushing's syndrome associated with a right adrenal cortical adenoma, which was successfully treated with unilateral adrenalectomy...