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Hereditary Papillary Renal Cell Carcinoma

HPRCC


Presentation

  • Version number changed to V.2. 15/01/2018 Protocol reviewed and presented at November 2017 RCM.[eviq.org.au]
  • Instead of that the patient primarily presented metastases of a papillary RCC in cervical lymph nodes.[bmcurol.biomedcentral.com]
  • The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.[orpha.net]
  • Considerations for testing Individuals—particularly adults—presenting with papillary renal carcinoma may be considered for analysis of the MET gene.[invitae.com]
Pathologist
  • Assay Assay and technical information Invitae is a College of American Pathologists (CAP)-accredited and Clinical Laboratory Improvement Amendments (CLIA)-certified clinical diagnostic laboratory performing full-gene sequencing and deletion/duplication[invitae.com]
  • Some pathologists postulate that there are two categories of Type 2 prcc, one generating indolent tumors, and the second presenting very aggressive tumors with poor survival rates.[ackc.org]
  • A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis.[dovemed.com]
  • To confirm a diagnosis of kidney cancer, doctors sometimes take a biopsy (a small tissue sample) of the kidney that is then examined by a pathologist.[nyp.org]
  • Recognition of histologic RCC subtypes is a frequent issue, and as pathologists become more familiar with current classification schemes, this will be less of an issue.[cancertherapyadvisor.com]
Fishing
  • Validation of a TFE3 break-apart FISH assay for Xp11.2 translocation renal cell carcinomas. Diagn Mol Pathol . 2011;20(3):129-137. 37. Malouf GG, Camparo P, Molinié V, et al.[hematologyandoncology.net]

Workup

  • Clinical Presentation Solid papillary renal tumors are the only clinical manifestation of this form of FRCC, and therefore these tumors are picked up incidentally, by screening of families known to be affected or on workup for gross or microscopic hematuria[abdominalkey.com]
  • A staging workup should be performed before treatment is initiated. Multiple enhancing lesions, or a family history of renal-cell carcinoma, particularly in persons younger than 50 years of age, suggests a hereditary predisposition to the disease.[aboutcancer.com]

Treatment

  • Management and treatment Management should include regular surveillance to allow early detection of carcinomas. Treatment options depend on the stage of the cancer at diagnosis.[orpha.net]
  • The care of affected individuals should be individualised based on their clinical situation, and the monitoring they need as part of their treatment and post-treatment follow up.[eviq.org.au]
  • Have you been given any follow up treatment or just monitoring, as it seems that you should be doing so,. However, knowing which is the best of the agents to use will again require that expert.[cancercompass.com]

Prognosis

  • One is the hereditary leiomyomatosis and renal cell carcinoma, which is characterized by cutaneous and uterine leiomyomas and renal cell carcinoma mostly consisting of the papillary renal cell carcinoma type II with a worse prognosis.[ncbi.nlm.nih.gov]
  • Prognosis Although 5-year survival rates for patients with RCC have improved in recent years, the outcome for patients with advanced stage disease remains poor. The documents contained in this web site are presented for information purposes only.[orpha.net]
  • The papillary RCC in HLRCC shows a penetrance of about 20-25% and mostly consists of a papillary RCC type II with a worse prognosis [ 12 ]; most patients die within five years [ 21 ].[bmcurol.biomedcentral.com]
  • Early stage RCC with tumor growth limited to the kidney has a very good prognosis.[amboss.com]

Etiology

  • Etiology The syndrome is associated with germline mutations in the MET proto-oncogene (7q31) encoding a hepatocyte growth factor-responsive tyrosine kinase receptor.[orpha.net]
  • You are here: Urology Textbook Kidneys Renal cell carcinoma Etiology Renal cell carcinoma (1/8): Definition and Epidemiology Renal cell carcinoma (2/8): Etiology Renal cell carcinoma (3/8): Pathology Renal cell carcinoma (4/8): Signs and symptoms Renal[urology-textbook.com]
  • Etiology Most renal cell carcinomas occur sporadically. However, approx. 4% of renal cell carcinomas are associated with hereditary factors.[amboss.com]
  • […] from region to region with the highest rates in the Czech Republic and North America In the United States, there are 64,000 new cases and almost 14,000 deaths from RCC each year ( CA Cancer J Clin 2017;67:7 ) Pathophysiology Various molecular pathways Etiology[pathologyoutlines.com]

Epidemiology

  • Summary Epidemiology The annual worldwide incidence of renal cell carcinomas (RCCs) is estimated at around 1 in 50,000. Papillary carcinomas represent 10-15% of all RCCs but the annual incidence of familial RCC syndromes is less than 1 in 1,500,000.[orpha.net]
  • So in epidemiological studies, it's been shown that there's an increased risk of renal cell carcinoma in the siblings of individuals who have renal cell carcinoma.[hstalks.com]
  • You are here: Urology Textbook Kidneys Renal cell carcinoma Etiology Renal cell carcinoma (1/8): Definition and Epidemiology Renal cell carcinoma (2/8): Etiology Renal cell carcinoma (3/8): Pathology Renal cell carcinoma (4/8): Signs and symptoms Renal[urology-textbook.com]
  • Epidemiology Most common malignancy of the renal parenchyma (85% of renal cancers in adults are RCC) Sex : ( 2:1) Age of onset : 60–80 years Ethnicity : slightly higher incidence in black populations in the US References: [1] [2] [3] [4] [5] Epidemiological[amboss.com]
Sex distribution
Age distribution

Pathophysiology

  • […] development RCC incidence varies widely from region to region with the highest rates in the Czech Republic and North America In the United States, there are 64,000 new cases and almost 14,000 deaths from RCC each year ( CA Cancer J Clin 2017;67:7 ) Pathophysiology[pathologyoutlines.com]
  • References: [6] [1] [7] [8] [2] [3] [4] [5] [9] Pathophysiology Renal cell carcinomas are adenocarcinomas that usually arise from the epithelial cells of the proximal convoluted tubule.[amboss.com]
  • Pathophysiology The molecular characteristics of RCC have been intensively studied during the past 20 years. Approximately 2 to 3% of RCC tumors are familial.[cancertherapyadvisor.com]
  • […] sickle cell disease. [23] The most significant disease affecting risk however is not genetically linked – patients with acquired cystic disease of the kidney requiring dialysis are 30 times more likely than the general population to develop RCC. [24] Pathophysiology[en.wikipedia.org]

Prevention

  • Cancer epidemiology, biomarkers & prevention: a publication of the American Association for Cancer Research, cosponsored by the American Society of Preventive Oncology. 2002, 11 (4): 393-400.[bmcurol.biomedcentral.com]
  • “The focus and goals of our current research projects are to develop better methods for diagnosis, treatment, and, ultimately, prevention of RCC,” he said.[gucasym.org]
  • Taking care of your kidneys and leading a healthy lifestyle could help prevent PRCC. These tips can help protect your kidneys: Maintain a healthy weight. Treat high blood pressure and keep your cholesterol in check. Exercise regularly.[healthline.com]

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