Renal Parenchymal Neoplasms Practice Flashcards

100 vocabulary flashcards covering renal neoplasms, including RCC, Wilms' tumour, neuroblastoma, and benign lesions as discussed in the lecture notes.

Renal Cell Carcinoma (RCC) Prevalence

The most common malignancy of the renal parenchyma, accounting for 85% of renal cancers in adults.

RCC Incidence (2020 US)

An estimated 73,750 new cases diagnosed.

RCC Sex Predilection

Occurs more frequently in males than females with a ratio of approximately $2:1$.

RCC Peak Incidence Age

Between 55 and 74 years.

RCC Median Age at Diagnosis

64 years.

RCC Mortality Fact

The most lethal of all urological tumours, with 50% of patients dying of the condition.

Hereditary RCC Percentage

Approximately 4% of renal cell carcinomas are associated with hereditary factors.

RCC Chromosomal Alteration

Structural alterations of the short arm of chromosome 3 ($3p$).

VHL Gene

A gene located on chromosome $3p$ whose subsequent alterations are involved in both sporadic and hereditary RCC.

Lifestyle Risk Factors for RCC

Smoking and obesity.

Associated Medical Conditions for RCC

Sickle cell disease, acquired cystic kidney disease, renal pelvic stones, hypertension, immunodeficiency, and chronic hepatitis C infection.

Occupational Carcinogens for RCC

Cadmium, Asbestos, and Petroleum by-products such as trichloroethylene.

Chemotherapeutic Risk Factor for RCC

Cisplatin exposure during childhood.

Analgesic Risk Factors for RCC

Chronic use of acetaminophen or aspirin.

Von Hippel-Lindau Syndrome

An autosomal dominant condition associated with phaeochromocytoma, renal and pancreatic cysts, and cerebellar haemangioblastoma.

Birt-Hogg-Dube Syndrome

A hereditary syndrome associated with the development of renal cell carcinoma.

HLRCC

Hereditary leiomyomatosis and renal cancer syndrome.

HPRCC

Hereditary papillary renal cell carcinoma.

RCC Pathological Origin

Adenocarcinomas that usually arise from the epithelial cells of the proximal convoluted tubule.

RCC Multifocality and Bilaterality

7% are multifocal and $1-2$% are bilateral.

RCC Calcification Rate

10–20% of tumors contain calcification.

Clear Cell RCC

The most common subtype of renal cell carcinoma, accounting for 70% of cases.

Papillary (Chromophilic) RCC

A subtype of RCC accounting for 10-15% of cases.

Chromophobe RCC

A specific histological subtype of renal cell carcinoma.

RCC Direct Extension: Adrenal Gland

Occurs in 7.5% of tumors larger than $5\,cm$.

RCC Direct Extension: Renal Capsule

Occurs in 25% of cases.

RCC Direct Extension: Renal Vein

Occurs in up to 44% of cases.

RCC Direct Extension: IVC and Heart

Extends into the IVC in 5% of cases and can reach the right atrium.

RCC Lymphatic Spread

Spreads to hilar and para-aortic lymph nodes.

RCC Lung Metastasis

The most common site of haematogenous spread, occurring in 75% of metastatic cases.

RCC Bone Metastasis

The second most common site of spread, occurring in 20% of metastatic cases.

RCC Liver and Brain Metastasis

Occurs in 18% and 8% of cases, respectively.

Early Stage RCC Symptoms

Usually asymptomatic; often detected incidentally on abdominal imaging.

RCC Symptom Threshold

Patients typically become symptomatic when the tumor is larger than $10\,cm$ or metastases are present.

RCC Primary Tumor Hematuria

Occurs in 50-60% of symptomatic patients.

RCC Primary Tumor Flank Pain

Occurs in 40% of symptomatic patients.

RCC Palpable Renal Mass

Found in 25% of symptomatic patients.

Left-Sided RCC Varicocele

A rare finding (2-5%) caused by malignant cells occluding the ostium of the left gonadal vein in the left renal vein.

Budd-Chiari Syndrome in RCC

Caused by involvement of the IVC, leading to lower limb edema, ascites, and hepatic dysfunction.

Pulmonary Metastasis Symptoms

Hemoptysis and dyspnea.

Bone Metastasis Symptoms

Bone pain and pathological fractures.

Secondary Hypercortisolism in RCC

A paraneoplastic syndrome due to ectopic ACTH release (Cushing's syndrome).

Secondary Polycythemia in RCC

Occurs in 5% of patients due to ectopic erythropoietin (EPO) secretion.

RCC Hypertension Etiology

Occurs in 25% of patients due to renin release, renal artery compression, or arteriovenous fistula.

RCC Hypercalcemia Etiology

Occurs in 10-20% of patients due to ectopic secretion of PTHrP (parathyroid hormone-related protein).

Stauffer's Syndrome

A paraneoplastic syndrome involving hepatic dysfunction, fever, and anorexia; resolves in 60-70% of patients post-nephrectomy.

Classical Triad of RCC

The combination of hematuria, flank pain, and a palpable flank mass.

Classical Triad Presentation Rate

Only 10–15% of patients present with all three components.

Clinical Stage T1a

Tumor limited to the kidney, size $\leq 4\,cm$.

Clinical Stage T1b

Tumor limited to the kidney, size $> 4\,cm$ but $\leq 7\,cm$.

Clinical Stage T2a

Tumor limited to the kidney, size $> 7\,cm$ but $\leq 10\,cm$.

Clinical Stage T2b

Tumor limited to the kidney, size $> 10\,cm$.

Clinical Stage T3a

Tumor extends into the renal vein or its segmental branches, or invades renal sinus fat, but not beyond Gerota's fascia.

Clinical Stage T3b

Tumor grossly extends into the vena cava below the diaphragm.

Clinical Stage T3c

Tumor grossly extends into the vena cava above the diaphragm or invades the wall of the vena cava.

Clinical Stage T4

Tumor invades beyond Gerota's fascia, including contiguous extension into the ipsilateral adrenal gland.

Preferred RCC Imaging

CT or MRI of the abdomen with IV contrast using a multiphase renal protocol.

First-line Investigation for Loin Pain

Abdominal Ultrasound (USS).

Bosniak Class I

Simple cyst with hairline-thin wall, no septa, no calcifications, and no contrast enhancement; very low malignancy risk.

Bosniak Class IIF

Cyst with minimally thickened wall, many thin septa, and coarse calcifications; requires follow-up imaging.

Bosniak Class III

Cyst with irregular and thick wall and septa, coarse calcifications, and enhancement; requires surgery.

Bosniak Class IV

Clearly defined soft lesions in wall and septa with enhancement; very high risk of malignancy.

Solid-Enhancing Renal Masses

Around 90% of these lesions will be RCCs.

RCC Bone Scan Indication

Indicated in patients with bone pain and/or elevated alkaline phosphatase (ALP).

RCC ESR Finding

Elevated ESR is found in 75% of cases.

Standard Treatment for Localized RCC

Partial or radical nephrectomy.

Partial Nephrectomy (PN) Gold Standard

The gold standard treatment for $cT1$ tumours.

Radical Nephrectomy Gold Standard

The gold standard treatment for $T2$-$4$ RCC.

Active Surveillance Candidates (RCC)

Patients with solid masses $< 2\,cm$, complex cystic masses, limited life expectancy, or high surgical risk.

RCC Radioresistance

The reason radiation therapy is not typically used for primary treatment, though it is used for metastatic palliation.

RCC Chemotherapy Resistance

RCC is highly resistant to most conventional chemotherapeutic agents.

VEGF Receptor Tyrosine Kinase Inhibitors

Targeted therapies such as Sunitinib or Sorafenib used to block tumor vascularization.

mTOR Inhibitors

Drugs like Everolimus used to block pathways involved in neovascularization.

Anti-PD-1 Antibodies

Immunotherapies such as Pembrolizumab or Nivolumab.

Leibovich Score

A scoring system used post-nephrectomy to predict RCC prognosis.

RCC 5-Year Survival: Stage I

70-94% depending on grade.

Wilms’ Tumour (Nephroblastoma)

The most common renal malignancy in children, often diagnosed between ages 2 and 4.

Wilms’ Tumour Survival Rate

Now reaches 90% overall survival.

WT1 Gene Location

Chromosome $11p13$. Deletion or mutation of both alleles results in tumorigenesis in 20% of cases.

WAGR Syndrome

Wilms’ tumour, Aniridia, GU anomalies, and Retardation; associated with bilateral tumors.

Denys-Drash Syndrome

Wilms’ tumour, male pseudohermaphrodism, and renal mesangial sclerosis; leads to ESRD.

Beckwith-Wiedemann Syndrome (BWS)

An overgrowth syndrome featuring macroglossia, hemihypertrophy, organomegaly, and omphalocele.

Most Common Anomaly with Wilms'

Undescended testis (UDT), occurring in 40% of cases.

Wilms’ Tumour Clinical Pearl

Suspect in a toddler with a firm, smooth, non-tender abdominal mass associated with hematuria and/or hypertension.

Wilms' Tumour Stage V

Bilateral tumor at diagnosis.

Standard Chemotherapy for Wilms'

Vincristine and doxorubicin.

Neuroblastoma

A malignant neuroendocrine neoplasm of the sympathetic nervous system originating from neural crest cells.

Commonest Extracranial Solid Tumour of Childhood

Neuroblastoma.

Neuroblastoma Primary Site

50% occur in the adrenal gland.

Opsoclonus-Myoclonus-Ataxia

A paraneoplastic syndrome in neuroblastoma characterized by 'dancing eyes dancing feet'.

Catecholamine Metabolites in Neuroblastoma

Increased Homovanillic acid (HVA) and Vanillylmandelic acid (VMA) in urine.

Raccoon Eyes (Periorbital Ecchymoses)

A typical finding in orbital neuroblastoma spread.

Blueberry Muffin Lesions

Bluish cutaneous/subcutaneous nodules associated with ganglioneuroblastoma.

Neuroblastoma Imaging (Specific)

MIBG (meta-iodo-benzyl-guanidine) scans are very sensitive for detection.

Neuroblastoma Midline Crossing

Unlike Wilms’ tumor, neuroblastoma masses often cross the midline.

Angiomyolipoma (AML)

The most common benign renal tumor, consisting of blood vessels, smooth muscle, and mature fat cells.

Angiomyolipoma Sex Ratio

Females are affected 4 times more than males ($4:1$).

Wunderlich’s Syndrome

Massive and life-threatening retroperitoneal bleeding occurring in up to 10% of angiomyolipoma cases.

Oncocytoma Definition

A benign epithelial tumor arising from the intercalated tubular cells in the collecting duct.

Oncocytoma CT Feature

Rarely exhibits a 'spoke-wheel' pattern caused by a stellate central scar.