rcc willims dehydration hypervolemia

an adenocarcinoma arising from the epithelial cells of the proximal tubular (90% of all kidney cancer)

renal cell carcinoma

renal cell carcinoma patho for acquired/somatic/sporatic type

most common, environmental exposures cause cells to mutate (more in men) (MOST COMMON)

renal cell carcinoma patho for hereditary type

genetic link, may pass on to kids

renal cell carcinoma subtypes/histology

clear cell = mots common adn agressive of the 4, clear cytoplasm

papillary = 2nd most common, finger like projections w central core

chromophobe = less common w better prognosis, large, polygonal w halo nucleus

oncocytoma = usually benign, uniform, round, and packed w bright pink granular cytoplasm

medullary/conducting duct = originates deeper inside kidneys and v aggressive

renal cell carcinoma risk factors

smoking , obesity, HTN, cystic disease, complex cyst, nephrolithiasis, analgesics, (NSAIDS), environmental/occupational exposures, dialysis, hx of renal cell cancer in 1 kidney inc risk in other kidney

hereditary link (von-hippel-lindau syndrome)

alc might be reno protective

renal cell carcinoma sx

25% asx, found on accident

CLASSIC TRIAD: hematuria, flank pain, abdominal mass

fever, night sweats, weight loss, anorexia, fatigue, worse HTN (activating RAAS)

blockage of IVC - lower extremity edema, ascites, hepatic dysfxn, pulm edema

paraneoplastic syndrome (stauffer syndrome = paraneoplastic elevation of LFTs w/o metastasees)

renal cell carcinoma ddx

renal pelvis urothelial cancer, hamarotomas (disorganized but benign), angiomyolipoma (blood+muscle+fat)(benign), renal oncocytomas (benign), renal abscesses, adrenal tumors (benign or malignant)

renal cell carcinoma complications

can trigger RVT and maybe block IVC → metastasize to adrenal glands/brain/bone/LN/lungs/liver

renal cell carcinoma hx and pe to dx

classic triad in 10% = hematuria, flank pain, abdominal mass (seen in advanced disease)

MOST COMMON PRESENTATION = HEMATURIA ALONE

pa=HTN, flank or abd pain, abd mass, varocele/engorged labia (usually if on left side), lower extremity edema

renal cell carcinoma labs

UA, culture, microscopy, cytology, CBC (see polycythemia first then anemia bc kidney gets bigger and then starts failing), CMP (check renal fxn adn LFT), hypercalcemia, alkalaine phosphatase (mets to liver and bone)

renal cell carcinoma imaging

abd US first study to see simple from complex cyst/mass

contrast CT or MRI to characterize mass, stage it, find mets, and eval conralateral kidney

CXR and bone scan to assess for mets

color duplex doppler to assess RVT or IVC compromise

before surgery, need CTA or MRA to assess vascular spread

starts to spread outside of kidney in stage 3 (goes to vasculature in stage 3, spreads far into abdomen and other places in stage 4) (1 and 2 still in kidney only)

renal cell carcinoma tx for localized cancer

main goal is to remove the cancer

active surveillance for small tumors, advanced disease, or pt not surgical candidate

partial nephrecotmy for perosn w 1 kidney, small tumor (3-4cm), in affected kidney and have a healthy kidney or they have bilateral renal cell carcinoma

radical nephrectomy for larger cancers or when partial isnt feasible

radiofrequency ablation or cryosurgery as alt to surg

renal cell carcinoma tx for metastatic cancer

goal is to control cancer adn imrpove survival

NO CHEMO!!!!

molecularly targeted therapies= block specific pathways cells need to grow and spread, good for clear cell RCC (vascular endothelial growth factor inhibitors =sunitinib, pazopanib, cabozantinib) (mammalian target of rapamycin aka mTOR inhibitors = everolimus)

immunotherapy to supercharge the immune system (nivolumab, pembrolizumab, ipilimumab)

combo = 2 immunotherapies OR one immuno and one molecularly targeted

cytoreductive nephrectomy (optional to remove kidney aka tx w meds then remove)

most common renal cancer, most common abdominal caner, and 4th most common peds cancer. can have unknown/sporatic etiology w slight genetic link. seen in kids 3-5

wilms tumor

wilms tumor patho

abnormal growth of metanephric blastema, also associated w chromosomal abnormalities (about 20% have WT1 or WT2 gene deletions)

genetic issue associated w wilms tumor with asymmetric overgrowth of half the body

hemihypertrophy/hemi-hyperplasia

genetic issue associated w wilms tumor and excessive growth of many body organs

beckwith-wiedermann syndrome

genetic issue associated w wilms tumor and wilms tumors, aniridia, GU abnormalities, intellectual disability

WAGR syndrome

genetic issue associated w wilms tumor and wilms tumor, ambiguous genitals, and diffuse glomerular disease

denys-drash syndrome

wilms tumor sx

most common = painless abdominal mass

might have pain due to distention, fever, HTN, hematuria, N/V, weight loss, lethargy, fatigue

wilms tumor dx

imaging and renal biopsy is key

PE = painless, palpable, firm, fixed abdominal mass friable, HTN, pallor, fever, varicocele/engorged labia, hepatosplenomegaly, distended abdominal vein

labs = hematuria, CBC (polycythemia and then anemia), CMP, LFTs

imaging = renal ultrasound (intrarenela mass is both cystic and solid) (ct scan of chest, abdomen and pelvis to see mets and staging

biopsy= provides definitive dx and prognosis, uniform and organized cells/undifferentiated cells/immature spindle cells/heterologous skeletal muscle/ osteoid/fat/glomeruli indicate favorable bx

wilms tumor tx

depends on staging, often do surgery first then chemo and RXT

bilateral tumors = chemo first, then radical nephrectomy of more involved kidney, partial nephrectomy for the lesser involved kidney to preserve some fxn

wilms tumor prognosis

lower stage, 5yr survival over 90%, more advanced unfavorable is 60% and favorable is 90%, 5% recurr in 2yrs but retreatment works

25% os survivors get complications from tx later on like cardiomyopathy, scoliosis, HTN, renal and bladder insufficiency, pulmoanry and hepatic dysfxn, infertility, and secondary cancers

state of net body water defecit, occurs when fluid out more than in, can involve water and/or electrolytes

dehydration

loss of extracellular fluid vol (both water and na)

hypovolemia (can coexist w dehydration but is distinct)

minimal water take for an adult

1600ml/d

water loss that is measurable (peeing, shitting, bleeding, wound drainage, gastric grainage, puking)

sensible

water loss that is not measureable (sweating, breathing, changes in humidity levels, fever)

insensible

causes of loss of total body water

gi loss = V/D, NG tube, external drainages

renal loss = diuretics, osmotic diuretics (DM), central and nephrogenic DI

skin = excessive sweating, bruns, SJS

third spacing = sepsis, trauma/crush injuries, pancreatitis/peritonitis, ascites

dehydration sx

mild = lost 3-4% (THIRSTY, dry mouth/lips, fatigue, lethragy, dec urine output)

moderate = lost 5-9% (inc thirst, HA, light headed, postural dizziness, fatigue, muscle cramps, dec urine output w dark urine)

severe = over 10% (medical emergency, profound weakness, AMS< disorientation, confusion, chest/abdominal pain, tachypnea, seizure, coma, anuria)

extreme = 15-20% lost (dead)

dehydration pe

lethragic, weak, sunken eyes, tachy, systolic under 90, ortho hypotension, tachypnea, dec skin turgor, dry lips/tongue/buccal mucosa, delayed cap refill, cool, clammy skin w shock, confused, disoriented, LOC, oliguria or anuria

dehydration dx

hx should tell you how they lost fluid, clinical dx w physical

labs= inc Hgb/HCT and albumin, dec GFR, inc BUN/SCr, hypo or hypernatremia, hypo/hyperkalemia, metabolic acisosis or alkalaosis, hyperglycemia if from DM, high serum osmolaity, dark urine w high specific gravity

dehydration tx

restore intravascular vol, fix electrolytes and rehydrate cells

oral water for mild/moderate

IV fluids is fastest (rapid bolus 1-2L os isotonic fluids to fix perfusion, followed by continuous infusion based on clinical response

can do normal saline (0.9%), or lactated Ringers (expand the extracelllular fluid col, try to fix deficit if known)

frequently reassess vitals, Pe, mental status and urine output (foley cath rec), daily weights monitor labs, fix underlying cause, special considerations for eldery, HF and renal failure pts to avoid fluid overload

crystalloids contain

water, electrolytes, maybe gluose

colloids contain

mainly albumin and blood products

isotonic crystalloids solutions are used to

raise extracellular fluid’s quantity

hypotonic crystalloids solutions are used to

when cells are dehydrated and fluid needs to be returned to them

hypertonic crystalloids solutions are used to

prevent cell edema

hypertonic colloid solutions (most common) are usually

albumin + fresh frozen plasma. used to prevent cell edema

reserved for severely hypovolemic pts bc inc intravascular vol by causing a high colloid osmotic pressure in blood vessels

advantages of crystalloids

cheap, readily available, safe, good for dehydration, non-allergenic, half life of 30-60min, vegetarian and vegan friendly

disadvantages of crystalloids

shifts in Na and osmolaltiy w hypotonic and hypertonic solutions, large volumes of normal saline and inc risk for hyperchloremic acidosis, lactated ringers inc risk of hyperglycemia and are contraindicated for hyperkalemia, avoid IVF w concurrent blood transfusion

advantages of colloids

replaces fluid vol, molecules too large to cross capillary walls so fluid remains intravascular longer, longer half life (hrs -days)

disadvantages of colloids

anaphalyctic risk, expensive, coagulation abnormalities (bleeding), excessive use and precipitate cardiac or renal failure, some preps are unsuitable fro vegitarians/vegans

a state of excess body water and na in blood vessels and surrounding tissues. can be from intaking more fluid than lost, excessive Na or fluid intake (IVF, blood transfusion, salty diet), dec output (renal failure), Na and water rentention (heart or liver failure, nephrotic syndrome, steroid use, low protien diet, hyperaldosteronism), or fluid shifts into intravascular space (burns, administrating albumin or hypertonic fluids)

hypervolemia

hypervolemia sx/pe/dx

puffy, swollen face/extremities, HTN, tachy, tachypnea, weight gain, JVP, S3 heart sound, crackles and rales on pulm auscultation, pitting edema, urinary output normal or inc

hypervolemia labs and imaging

dilute UA, CBC (dilutional anemia), inc BUN/SCr if renal failure, high BNP (if CHF), low serum osmolality due to dilutional hyponatremia

CXR = pulm edema w effusions, cardiomegaly, or bluntin costophrenic angles

echo - enlarged ventricles, muscle thickening, regurgitation

hypervolemia complications

feeling bloated, weight gain

CHF IS THE MOST COMMON CAUSE OF FLUID OVER LOAD (=WEG=IGHT GAIN, SWELLING FACE/LEGS/ARMS)

anasarca = severe generalized edema

pulmonary edema, plueral effusion (SOB, dyspnea on exertion, PND, orthopnea)

pericardial effusion (SOB, breathlessness, muffled heart sounds)

cerebral edema (lack of O2 to brain → drowsy, AMS, seizures, LOC)

cutaneous edema (inflammatory rxns like insect bites may cause pain to area, swellling, limited ROM)

periorbital edema (trauma or nephrotic syndromes)

lymphedema (block lymph drainage, elephantiasis in filariasis)

liver failure (ascites w fluid in peritoneal cavity)

hypervolemia tx

remove excess fluid, Na, tx cause

fluid and Na restriction, loop diuretics are most common, fix underlying (renal/cardiac/liver problem)

monitor: daily weights, strict I&Os, vitals, lung sounds, JVP, edema assessment, electrolytes (K), repeat imaging, elevate legs, intermittent pneumatic compression devices