Before We Begin

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• This presentation segment shows a great example
  of
• Simplifing the case H P into a few words
• Using opportunities in a case to reinforce
  previously learned medical school topics (e.g.
  the Anatomy Quiz)
• Embeding audio/visual aids, that is acceptable
  for these presentations! (the SGS rooms should be
  equipped to broadcast audio)
• Again, the use of humor really keeps students
  engaged!
• Best viewed as a slidshow! F5!

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Before We Begin
A Joke
Two old men were arguing the merits of their
doctors. The first one said, "I don't trust your
fancy doctor. He treated old Jake Waxman for a
kidney ailment for nearly a year, and then Jake
died of a liver ailment. "So what makes you
think your doctor is any better?" asked his
friend.
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Before We Begin
"Because when my doctor treats you for a kidney
ailment, you can be sure you'll die of a kidney
ailment."
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Case 2

• Renal Cell Carcinoma
• von Hippel-Lindau

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Case 2

• 32 year old while man
• On routine physical exam
• Palpable masses in the left and right flanks.
• No pain
• Normal urinary and bowel function

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Case 2
Cant miss the kidneys
Anatomy Quiz Time
CT scan of abdomen. Identify major abdominal
organs. Identify kidneys and describe
abnormalities present. Can a CT scan
differentiate between cystic fluid filled
structures and solid masses?
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Gas in Colon
Hepatic Portal Vein
SM Artery and Vein
Abd Aorta
Transverse / Descending Colon
Infer. VC
Psoas Major
Right Kidney
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Case 2
See multiple, bilateral cysts variable density
(mostly cystic), liver cysts
CT scan of abdomen. Identify major abdominal
organs. Identify kidneys and describe
abnormalities present. Can a CT scan
differentiate between cystic fluid filled
structures and solid masses?
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Case 2
Renal angiogram. Identify radiographic
appearance of neovascularization occurring in
renal masses. Correlate areas of
neovascularization with abnormalities identified
on CT scan.
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Case 2
Neovascularization close to capsule.
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Case 2
- The patient didnt want a bilateral
nephroectomy (hated dialysis). - A kidney
transplantation was rejected by the patient (pun
intended.) So what to do? Patient underwent an
extensive bilateral kidney operation to preserve
minimal function without dialysis.
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Case 2
Gross pathologic specimen of the left kidney.
Identify normal renal parenchyma and multiple
renal masses. Differentiate between solid and
cystic lesions within the kidney. Identify
renal capsule and relationship of renal masses to
the renal capsule and renal fat.
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Case 2
Representative microscopic sections of some of
the masses shown in Slide 20. What type of
neoplastic cells do you see beneath the cyst
lining?
Still see clear cells (but not as many), cells
layered in sheets. Characteristic of renal cell
carcinoma.
Proteinacous cyst fluid.
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Case 2
Clear cytoplasms, rich (neo)vascularity
Representative microscopic sections of some of
the masses shown in Slide 20. What two features
characteristic of renal cell carcinoma are seen?
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Case 2
Representative microscopic sections of some of
the masses shown in Slide 20. What two features
characteristic of renal cell carcinoma are seen?
Possible mitosis
Obviously something to know about RCC.
Clear cytoplasms, rich (neo)vascularity
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Case 2
Renal Cell Carcinoma 4 Fuhrman grades
Our patient have RCC, Clear cell carcinoma,
nuclear grade I
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Case 2
von Hippel-Lindau loves to spurt other lesions
outside the kidney. Where are the most likely
sites?
Cerebral hemangioblastoma
Angiomatosis of the retina
Pancreatic cysts and carcinomas
Cystadenoma of the epididymis
He has this.
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Case 2
Cerebellar hemangioblastoma is a well
circumscribed tumor consisting of thin-walled,
closely packed capillaries separated by large
pale stromal cells.
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Case 2
Cerebellar hemangioblastoma. An
immuno-histochemical stain for factor
VIII-related antigen delineates the capillary
network.
Apparently F8 is a marker for vascular growth.
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Case 2 Summary

• What are the anatomic lesions commonly observed
  in such patients?
• Hemangioblastomas in cerebrellum, spinal cord,
  and retina.
• Pancreatic cysts and carcinomas
• Cystadenoma of the epididymis
• Very rarely pheochromocytoma
• What is the vHL genetic characterization and mode
  of inheritance?
• Autosomal dominant, virtually complete
  penetrance!
• Loss of vHL tumor suppressor gene on 3p

• Most common RCC etiology (including vHL)
  Tobacco.
• RCC (including vHL) microscopic features
• clear cytoplasm,
• neovascularization
• hyperchromatic nuclei
• pleomorphic nuclei