Transcript Case Study - University of Pittsburgh

Case Study 39
Henry Armah, M.D., M.Phil.
Question 1
Clinical history: 59-year-old male with past medical
history of von Hippel Lindau syndrome, bilateral renal cell
carcinomas, bilateral nephrectomies, and right kidney
transplant 10 years prior. He presented with a 3-week
history of lower extremity numbness, instability and
difficulty walking, left leg worse than right. Describe the
abnormal spinal MRI findings?
Sag T1
Sag T2
Sag T1+C
Answer
Intradural extramedullary oval homogenously contrast
enhancing mass at the T7 vertebral body level
compressing and displaying the spinal cord within the
thecal space.
Question 2
What are your differential diagnoses based on the
patients’ age, past medical history, and the radiological
findings?
Answer
1. Hemangioblastoma
2. Metastatic renal cell carcinoma
3. Meningioma
4. Schwannoma
5. Hemangiopericytoma
6. Solitary fibrous tumor
7. Paraganglioma
Question 3
The neurosurgeon performs a laminectomy with resection
of the mass, and requested an intraoperative consultation.
Describe the microscopic findings on this frozen section
slide?
Click here to view slide.
Answer
Neoplasm composed of numerous thin-walled vessels
and intervening vacuolated stromal cells.
Question 4
What is your intraoperative diagnosis? (A: Category such
as Defer, Reactive/Non-neoplastic, or Neoplastic; B: More
specific diagnosis or statement)
Answer
A. Neoplasm
B. Hemangioblastoma
Question 5
The permanent section has returned from histology.
Describe the microscopic findings on this H&E slide?
Click here to view slide.
Answer
Fragments of a neoplasm composed of numerous thinwalled vessels lined with abundant vascular cells, and
interspersed vacuolated stromal cells. Occasional stromal
cells with large atypical and hyperchromatic nuclei are
identified. There is no evidence of anaplasia, mitotic
figures, or necrosis. There are focal areas of hemorrhage
and hemosiderin deposition.
Question 6
What additional immunohistochemical studies would you
need to rule out other important differential diagnoses and
confirm the final diagnosis in this case?
Answer
1. Factor XIIIa
2. Inhibin alpha
3. CD31
4. Vimentin
5. CD10
6. RCC
Question 7
What do you see on this Factor XIIIa immunostain slide?
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Answer
Factor XIIIa is patchy positive in the neoplastic stromal
cells.
Question 8
What do you see on this Inhibin alpha immunostain slide?
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Answer
Inhibin alpha is strongly and diffusely positive in the
neoplastic stromal cells.
Question 9
What do you see on this CD31 immunostain slide?
Click here to view slide.
Answer
CD31 is negative in the neoplastic stromal cells, but
highlights the rich vasculature of the tumor.
Question 10
What do you see on this Vimentin immunostain slide?
Click here to view slide.
Answer
Vimentin is strongly and diffusely positive in tumor cells.
Question 11
What do you see on this CD10 immunostain slide?
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Answer
CD10 is negative in tumor cells.
Question 12
What do you see on this RCC immunostain slide?
Click here to view slide.
Answer
RCC is negative in tumor cells.
Question 13
What is your final diagnosis in this case?
Answer
Hemangioblastoma
Question 14
What is the corresponding WHO grade of this lesion?
Answer
WHO Grade 1