RCC, Wilms, Dehydration, Hypervolemia

Renal Cell Carcinoma (RCC)

An adenocarcinoma that arises from the epithelial cells of the proximal tubulars that is more common in males in their 60s

Clear cell

Which subtype of RCC is most common and aggressive - characterized by clear cytoplasm

Papillary

Which subtype of RCC is the 2nd most common and characterized by finger like projections with a central core?

Chromophobe

Which subtype of RCC is characterized by a large, polygonal cells with halo nucleus - less common and has a better prognosis

Oncocytomas

Which subtype of RCC is usually benign and is characterized by uniform, round, and packed with bright pink granular cytoplasm

Medullary/collecting duct

Which subtype of RCC originates deeper inside the kidneys and is VERY aggressive

smoking, obesity, HTN, cystic disease, complex cyst, nephrolithiasis, analgesics (NSAIDs), environmental/occupational exposures, dialysis, von Hippel-Lindau syndrome

Risk factors for RCC (EtOH is renal protective)

Hematuria, flank pain, abdominal mass

Classic triad of RCC - seen with advanced disease

fever, night sweats, weight loss, anorexia, fatigue, HTN

Constitutional symptoms of RCC

Lower extremity edema, ascites, hepatic dysfunction, pulmonary edema

Symptoms of RCC that occur as a result of a blockage of the Inferior Vena Cava

Stauffer Syndrome

A paraneoplastic elevation of LFTs without mets

Renal pelvis urothelial cancer, hamartomas, angiomyolipomas, renal oncocytomas, renal abscess, adrenal tumors

DDX for RCC

RVT, IVC blockage, mets to adrenal glands, brain, bone, lymph nodes, lungs, liver

Complications of RCC

UA w/ culture and microscopy, CBC, CMP (renal function, LFTs, hypercalcemia), ALP (mets liver and bone)

Lab work up for RCC

ABD U/S 🥇 (determine simple vs. complex), CT/MRI (TMN staging, mets location), CXR/Bone scan (mets), Color doppler (RVT or IVC compromise), CTA/MRA (map vascular spread pre-op)

Imaging work up for RCC

stage 1

Mass is under 7cm and is limited to the kidney

stage 2

Mass is over 7cm and is limited to the kidney

Stage 3

Mass is starting to spread out of the kidney but has not hit any other organs yet

Stage 4

Mass has spread to other organs

Active surveillance

Which treatment is good for small, localized RCC tumors, advanced disease, or poor surgical patients

Partial nephrectomy

Which localized RCC treatment is good for homies with 1 kidney, small tumors (3-4 cm), and bilateral RCC

Radical nephrectomy

Which localized RCC treatment is good for larger cancers or when partial is not feasible

Radiofrequency or cyrosurgery ablation

Alternatives to surgery with RCC

Molecularly targeted therapy (VEGF/mTOR inhibitors), immunotherapy (Nivolumab, pembrolizumab, ipilimumab), Combo, Cytoreductive nephrectomy

Which treatments are prefered for metastatic RCC - NO CHEMO

90%+ (stage 1 or 2), 50-60% (stage 3), 0-15% (lymph node involvement), 15-30% (metastatic but just 1 spot)

Prognosis of RCC

Any renal suspicious mass (urology), RCC confirmed (urologic surge), metastatic (urology/oncology)

Referrals for RCC

Wilms tumor (nephroblastoma)

The most common renal cancer, the most common ped abdominal cancer, and the 4th most common ped cancer in general

Abnormal growth of metanephric blastema - associated with chromo abnormalities (deletions in WT1 or 2)

Patho for Wilms

Hemihypertrophy/hemi-hyperplasia

Asymmetric overgrowth of half the body associated with Wilms tumor

Beckwith-Wiedermann Syndrome

Microcephaly, macroglossia, and umbilical hernia associated with wilms tumor

Wilms Tumor, Aniridia, GU abnormalities, intellectual disability

WAGR syndrome is characterized by

Wilms tumor, ambiguous genitalia, diffuse glomerular disease

Denys-Drash syndrome is associated with

UA, CBC, CMP, Renal U/S, CT chest/abdomen/pelvis, renal biopsy

5 y/o male presents to the clinic for a well child check. Parents state that they’ve recently notice a lump in his stomach when they hug him. Vitals are stable with the exception of 100.4 temp and 144/88. On physical exam you note a non-tender firm, fixed mass in the abdomen, pallor, and a varicocele. What do you want to order?

uniform and organized cells, undifferentiated cells, immature spindle cells, heterologous skeletal muscle, osteoid, fat, glomeruli

5 y/o male presents to the clinic for a well child check. Parents state that they’ve recently notice a lump in his stomach when they hug him. Vitals are stable with the exception of 100.4 temp and 144/88. On physical exam you note a non-tender firm, fixed mass in the abdomen, pallor, and a varicocele. Labs reveal hematuria, polycythemia. U/S reveals an intrarenal mass that is BOTH cystic and solid. What would be a favorable biopsy result?

Cells are disorganized aka focal/diffuse anaplasia

What would a unfavorable biopsy of Wilms tumor look like?

surgery first then chemo

North America Approach for Wilms tumor

chemo → surgery → chemo and radiation

European approach for Wilms tumor

chemo → radical nephrectomy for the bad one, partial for the good one

Gameplan for bilateral Wilm tumor

lower stage 90%, unfavorable histology 60%

Prognosis for Wilms

cardiomyopathy, scoliosis, HTN, renal/bladder insufficiency, pulmonary and hepatic dysfunction, infertility, second malignancies

25% of survivors of Wilms tumor will get

hypovolemia

The loss of both water and salt

Dehydration

the state of net body water deficit

urination, defecation, bleeding, wound drainage, gastric draining, vomiting

What are some examples of sensible water loss?

sweat, respiration, changes in humidity, fever

What are some examples of insensible water loss?

GI loss (vomit, diarrhea, NGT, external drains), Renal (diuretics, osmotic diuresis, central/nephrogenic DI), Skin (excessive sweating, burns, severe skin disease), third-spacing (sepsis, trauma, pancreatitis, ascites)

Etiology for loss of Total body water

N/V, dysphagia, lack of access, AMS, fear of incontinence, pain, depression, eating disorders, post-op

Etiology for decreased fluid intake

thirst, dry mouth/lips, fatigue, lethargy, oliguria

Mild signs and symptoms of dehydration

increased thirst, HA, lightheaded, postural dizziness, fatigue, muscle cramps, oliguria with dark urine

Moderate signs and symptoms of dehydration

profound weakness, AMS, disorientation, confusion, chest/abd pain, tachypnea, seizures, coma, anuria

Severe signs and symptoms of dehydration

CBC, CMP, UA, VBG, serum osmolality

56 y/o male presents to the ED after passing out at work. His coworker states they had been working outside in 104 degree heat. Vitals are stable with the exception of 134 bpm and 22 RR. On physical exam you note decreased turgor, dry mucous membranes, delayed cap refill, and the patient is disoriented. What labs you want?

Rapid bolus of isotonic fluids (NS/LR), frequent reassessment, daily weights, monitor labs, treat the underlyin

56 y/o male presents to the ED after passing out at work. His coworker states they had been working outside in 104 degree heat. Vitals are stable with the exception of 134 bpm and 22 RR. On physical exam you note decreased turgor, dry mucous membranes, delayed cap refill, and the patient is disoriented. Labs are as follows, increased Hgb/Hct, hypernatremia, minor hyperkalemia, increased BUN/SCr, Serum osmolality is over 295, and the urine specific gravity is 1.030, What is your treatment plan?

oral hydration with oral rehydration solution (pedialyte)

Treatment plan for mild dehydration

elderly, HF, renal failure peeps

When treating dehydration which patients do we have to be careful to avoid fluid overload?

0.9% NS, 5% dex in saline, D5W

Give me some examples of isotonic crystalloids (raise ECF)

0.45% saline, 0.33% saline, 0.225% saline

Give me some examples of hypotonic crystalloids (cells in a hypertonic environment)

3-5% saline, 5% dextrose in 0.9% saline, 5% dextrose in 0.45% saline

Give me some examples of hypertonic crystalloids (cells in a hypotonic environment)

Albumin, FFP, hydroxyethyl starch, dextran, geletans (like blood)

Examples of Colloids - usually hypertonic and reserved for severely hypovolemic peeps

cheap, available, safe, good for rehydration, non-allergenic, t1/2 30-60

Pros for Crystalloids

Shifts in Na and osmolality, large volumes of NaCl increase the risk of hyperchloremic acidosis, increased risk of hyperglycemia and C/I with hyperkalemia (LR), Need a seperate line if blood is running

Cons for Crystalloids

replaces fluid volume, remain intravascular long, t1/2 is hours to days

Pros of Colloids

Anaphylaxis, expensive, coagulation abnormalities, excessive use can lead to cardiac or renal failure

Cons of Colloids

Hypervolemia

A state of excess body water and sodium blood vessels

intake exceeds loss, excessive sodium/fluid (or retention), decreased output, 3rd spacing

Etiology for hypervolemia

UA, CBC, CMP, BNP, Serum osmolality, CXR, echo

Wow you’re back in the Burn ICU at BAMC checking out your patient with full thickness burns on 55% of his body. Vitals are stable with the exception of 112 bpm. 144/92, and 24 RR. On physical exam you note pitting edema, bilateral crackles and rales, elevated JVP and an S3. What diagnostics you want?

CHF (most common cause of fluid overload), Pulmonary edema, pericardial effusion, cerebral edema, cutaneous edema, periorbital edema, lymphedema, liver failure

Complications of Hypervolemia

Fluid and Na restriction, loop diuretics (most common), treat the underlying; monitor daily weights, strict I&Os, vitals, lung sounds, JVP, edema assessment, electrolytes, intermittent pneumatic compression devices

Treatment plan for hypervolemia