Here are some scenario-based multiple choice questions related to Von Hippel Lindau Syndrome (VHL)
Emily is a 32-year-old female who presents to her physician with complaints of a headache and blurred vision. Further examination reveals bilateral retinal angiomas and a cerebellar hemangioblastoma. What is the most likely diagnosis?
a) Multiple sclerosis
b) Brain tumor
c) Von Hippel Lindau Syndrome
d) Migraine headache
Answer: c) Von Hippel Lindau Syndrome
John is a 40-year-old male who has a family history of VHL syndrome. He is asymptomatic but undergoes genetic testing and is found to have a mutation in the VHL gene. What is the most appropriate next step?
a) Start annual screening for VHL-associated tumors
b) Do nothing, as he is asymptomatic
c) Start treatment with an angiogenesis inhibitor
d) Start treatment with chemotherapy
Answer: a) Start annual screening for VHL-associated tumors
Lisa is a 25-year-old female who presents to her physician with complaints of abdominal pain and distention. Further examination reveals a large cystic mass in her left kidney. What is the most likely diagnosis?
a) Ovarian cyst
b) Renal cell carcinoma
c) Polycystic kidney disease
d) Renal cysts associated with VHL syndrome
Answer: d) Renal cysts associated with VHL syndrome
Mark is a 50-year-old male who was diagnosed with VHL syndrome at age 30. He has a history of multiple surgeries for VHL-associated tumors, including a pheochromocytoma and a hemangioblastoma. He now presents with a new adrenal mass. What is the most likely diagnosis?
a) Adrenal adenoma
b) Adrenal carcinoma
c) Metastatic pheochromocytoma
d) Recurrent pheochromocytoma associated with VHL syndrome
Answer: d) Recurrent pheochromocytoma associated with VHL syndrome
Sarah is a 28-year-old female who has been diagnosed with VHL syndrome. She is interested in starting a family but is concerned about passing on the VHL mutation to her children. What is the most appropriate counseling for her?
a) She should avoid having children to prevent passing on the VHL mutation
b) She can undergo preimplantation genetic testing to select embryos without the VHL mutation
c) She can undergo in vitro fertilization with donor sperm to prevent passing on the VHL mutation
d) She should have children and have them tested for the VHL mutation after birth
Answer: b) She can undergo preimplantation genetic testing to select embryos without the VHL mutation.
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