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Hereditary Disorders: Von-Hippel-Lindau Syndrome, Sturge-Weber-Syndrome, Neuroblastoma, MEN2A & MEN2B – Andrenal Medulla

by Carlo Raj, MD
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    Hereditary Pheos, here is your Von Hippel-Lindau, angiomatosis, cerebellar hemangioblastomas, pheochromocytoma; renal cell carcinoma is huge. The molecular mechanism for that is already discussed in great detail. Once again, we talked about the hypoxia-inducible factor 1-alpha and its influence on VEGF and you can have the cyst in the kidney, in the liver, in the pancreas, all over the place. In Sturge-Weber, cavernous hemangiomas of fifth cranial nerve, pheochromocytoma. Sturge-Weber is a child, half the face might have your port wine stain. What does that mean? Half the face looks reddened, erythematosus. More hereditaries… these are neuroblastomas. Malignant catecholamine producing tumour from the adrenal medulla association. Remainder arise along the sympathetic chain. Neuroblastoma… most common solid tumour in infants less than one year of age, causes hypertension. The most important… the two most important points out of neuroblastoma - less than one year of age. We’re thinking about solid tumour and number two, pheochromocytoma. Look for episodic hypertension. Often differentiated into more mature form called ganglioneuromas; amplification of N-myc… N neuroblastoma… this is bad. You know about myc, myc especially your C-myc, here’s N-myc… neuroblastoma, we’re still continuing the topic of neuroblastoma. Labs will show increased urinary excretion of your catecholamines in the form of metabolites. Once again, we have our vanillylmandelic acid, the neuroblastomas that we’re seeing in the images to your right. Continue our discussion of hereditary pheochromocytomas, here we have MEN part of both 2A and 2B. We have more than one endocrine organ which we will walk through in great detail. There is only 2A and 2B that we’ll take a look at with pheochromocytoma at this juncture. Autosomal dominant 2A and 2B, familial medullar thyroid cancer could be found in 2A… medullary. Genetic individuals here with 2A you want to keep in mind that RET...

    About the Lecture

    The lecture Hereditary Disorders: Von-Hippel-Lindau Syndrome, Sturge-Weber-Syndrome, Neuroblastoma, MEN2A & MEN2B – Andrenal Medulla by Carlo Raj, MD is from the course Adrenal Gland Disorders.


    Included Quiz Questions

    1. Congenital adrenal insufficiency
    2. Pheochromocytoma
    3. Renal cell carcinoma
    4. Pancreatic cysts
    5. Cerebellar hemangioblastomas
    1. Neuroblastoma
    2. Hemangioma
    3. Astrocytoma
    4. Glioma
    5. Pituitary adenoma
    1. MEN
    2. von Hippel-Lindau syndrome
    3. Neurofibromatosis
    4. Sturge Webber syndrome
    5. Neuroblastoma
    1. MEN2A
    2. MEN1
    3. MEN2B
    4. Sturge Webber syndrome
    5. von Hippel-Lindau

    Author of lecture Hereditary Disorders: Von-Hippel-Lindau Syndrome, Sturge-Weber-Syndrome, Neuroblastoma, MEN2A & MEN2B – Andrenal Medulla

     Carlo Raj, MD

    Carlo Raj, MD


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