Symptoma

Papillary Renal Cell Carcinoma Type 1

Hereditary Papillary Renal Cell Carcinoma Type 1

Version number changed to V.2. 15/01/2018 Protocol reviewed and presented at November 2017 RCM. [eviq.org.au]

Clinical Presentation The earliest manifestations of VHL are usually not related to renal pathology. [abdominalkey.com]

MRI The tumors generally present with a pseudo-capsule. [radiopaedia.org]

Patients become symptomatic when the tumor has reached a large size (usually 10 cm ) and/or if metastases are present. Hematuria is the most common presenting symptom ( 40% of cases). [amboss.com]

Chromophobe RCC Chromophobe histology accounts for approximately 5% of RCCs. 1,2 In general, the growth pattern is solid, but tubular architecture can be present. Occasionally, focal calcifications and broad fibrotic septa are present. [hematologyandoncology.net]

  • Asymptomatic

    Papillary renal cell carcinomas were often detected incidentally in asymptomatic individuals or during screening of asymptomatic members of renal cell carcinoma families. [ncbi.nlm.nih.gov]

    As with other types of renal cell cancer, most are asymptomatic and incidentally discovered. [radiopaedia.org]

    […] imbalance (see below) Note no phenotypic sign Neoplastic risk multiple and/or bilateral papillary renal cell carcinomas, with median age 45 yrs at diagnosis (range 18-79 yrs, most cases being between 35 and 55 yrs old), sex ratio 29M/12F, the presence of asymptomatic [atlasgeneticsoncology.org]

    If the mass is less than 1 cm in size and asymptomatic, a watch and wait approach is recommended. All renal masses 1 cm in size are presumed to be renal cell carcinoma and treated as such! [amboss.com]

    VHL gene mutation analysis provides a method for early diagnosis of VHL in asymptomatic individuals or in clinical situations such as hereditary pheochromocytoma where the diagnosis is in doubt. [ommbid.mhmedical.com]

  • Falling

    Any variants that fall outside these regions are not analyzed. Any specific limitations in the analysis of these genes are also listed in the table below. [invitae.com]

    Most individuals will fall into this category. Treatment naïve, favorable risk metastatic clear cell carcinoma Patients in this category should have no poor risk factors (10 to 20% of patients). [cancertherapyadvisor.com]

  • Aggressive Behavior

    behavior Hybrid tumors have also been reported in non-syndromic cases (Petersson) Most others are clear cell carcinomas Frequently high grade Behavior same as non-syndromic cases Frequent microscopic foci of oncocytosis Clusters of cells with eosinophilic [surgpathcriteria.stanford.edu]

    Renal medullary carcinoma: rhabdoid features and the absence of INI1 expression as markers of aggressive behavior. Mod Pathol. 2008;21(6):647-652. 30. Elwood H, Chaux A, Schultz L, et al. [hematologyandoncology.net]

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]

  • Hepatocellular Carcinoma

    Pharmacokinetic studies of sorafenib have been conducted in patients with hepatocellular carcinoma. [cancertherapyadvisor.com]

    Clinical implication of long noncoding RNA NEAT1 expression in hepatocellular carcinoma patients. International journal of clinical and experimental pathology. 2015;8(5):5395. pmid:26191242 View Article PubMed/NCBI Google Scholar 16. [journals.plos.org]

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]

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]

Treatment Current treatment in renal cell cancer cures well over half the patients in stage I disease. Surgical resection is the accepted, often curative therapy for stage I. It may be simple or radical; partial or complete. [rare-cancer.org]

A tumor biopsy should be obtained prior to treatment to define histology. Post-treatment biopsy should also be considered. [cancertherapyadvisor.com]

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]

Early stage RCC with tumor growth limited to the kidney has a very good prognosis. [amboss.com]

[…] with treatment In cases where both kidneys are affected, the prognosis is not projected to be favorable. [dovemed.com]

Prognosis Defining the prognosis of renal-cell carcinoma is important for both therapeutic decision-making and counseling patients. [aboutcancer.com]

Have a good prognosis. Closely resembles normal kidney tissue. Grade 2 - Grade 4 are defined based on nuclear size, irregularity and nuclear prominence, with prognosis deterioration at each grade level. [rare-cancer.org]

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]

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]

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]

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]

[…] 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]

“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]

Nineteen of the patients had received at least one previous systemic therapy, such as sunitinib, that had not been successful in preventing their disease progressing. [esmo.org]