subependymal giant cell astrocytoma - USMLE Forums
USMLE Forums Logo
USMLE Forums         Your Reliable USMLE Online Community     Members     Posts
Home
USMLE Articles
USMLE News
USMLE Polls
USMLE Books
USMLE Apps
Go Back   USMLE Forums > USMLE Step 1 Forum

USMLE Step 1 Forum USMLE Step 1 Discussion Forum: Let's talk about anything related to USMLE Step 1 exam


Reply
 
Thread Tools Search this Thread Display Modes
  #1  
Old 10-04-2011
USMLE Forums Addict
 
Steps History: ---
Posts: 125
Threads: 59
Thanked 183 Times in 74 Posts
Reputation: 194
Question subependymal giant cell astrocytoma

A 10-year-old boy has a small tumor in the wall of the right lateral ventricle. A biopsy of this tumor is consistent with subependymal giant cell astrocytoma. Which of the following lesions may also be present in this patient?
A. Café-au-lait spots
B. Cortical tubers
C. Hemangioblastoma
D. Lisch nodules
E. Schwannoma of the 8th cranial nerve
Reply With Quote Quick reply to this message
The above post was thanked by:
docusmle9 (10-04-2011), m82_ghasemi (10-04-2011)



  #2  
Old 10-04-2011
USMLE Forums Scout
 
Steps History: CK Only
Posts: 20
Threads: 4
Thanked 14 Times in 7 Posts
Reputation: 24
Default

B(Tuberos sclerosis)
Reply With Quote Quick reply to this message
  #3  
Old 10-04-2011
USMLE Forums Addict
 
Steps History: CK Only
Posts: 178
Threads: 61
Thanked 250 Times in 105 Posts
Reputation: 260
Correct Answer Answer

Subependymal giant cell astrocytoma (SEGA) is almost always associated with tuberous sclerosis.

A, D, and E are associated with Neurofibromatosis.

Germline mutations in a tumor suppressor gene, the Von Hippel-Lindau (VHL) gene, put affected individuals at risk for developing a variety of tumors, including hemangioblastomas of the retina (often referred to as retinal angiomas) and of the central nervous system, clear cell renal cancer, pheochromocytoma, pancreatic neuroendocrine tumors, endolymphatic sac tumors, and papillary cystadenomas of the epididymis (males) or broad ligament (females).
Reply With Quote Quick reply to this message
The above post was thanked by:
usluipek (10-05-2011)
  #4  
Old 10-04-2011
USMLE-Syndrome's Avatar
USMLE Forums Master
 
Steps History: ---
Posts: 1,203
Threads: 180
Thanked 1,251 Times in 441 Posts
Reputation: 1265
Default

B. Cortical tubers
Reply With Quote Quick reply to this message
  #5  
Old 10-04-2011
USMLE Forums Addict
 
Steps History: ---
Posts: 125
Threads: 59
Thanked 183 Times in 74 Posts
Reputation: 194
Default

The correct answer is B. This item tests your knowledge of neurocutaneous syndromes, a group of hereditary conditions characterized by concomitant neoplastic or hamartomatous lesions predominantly affecting the skin and nervous system. Subependymal giant cell astrocytoma is a peculiar astrocytic tumor that grows from the walls of the lateral ventricles. It is pathognomonic of tuberous sclerosis, which is caused by mutations of TS1 or TS2 genes. Tuberous sclerosis manifests with multiple hamartomatous lesions in the skin, CNS, and visceral organs. Cortical tubers are malformed (hamartomatous) nodules of the cortex, probably resulting from faulty cortical development. Other lesions include shagreen patches and ash-leaf spots on the skin, cardiac myomas, and renal angiomyolipomas.
Café-au-lait spots (choice A) are found in both types of neurofibromatosis. Lisch nodules (choice D) are small pigmented nodular lesions of hamartomatous nature that are present in the iris of patients with neurofibromatosis type 1. Schwannomas of the 8th cranial nerve (choice E), especially when bilateral, are typically associated with neurofibromatosis type 2.
Hemangioblastoma (choice C) is a vascular tumor of unknown histologic origin that frequently develops in the cerebellum of patients with von Hippel-Lindau syndrome. Renal cell carcinomas are also common in this disease. Von Hippel-Lindau syndrome is caused by autosomal dominant mutations of the VHL gene, a tumor suppressor gene. You may recall that mutations of VHL gene are also found in the majority of sporadic renal cell carcinomas
Reply With Quote Quick reply to this message



Reply

Quick Reply
Message:
Options

Register Now

In order to be able to post messages on the USMLE Forums forums, you must first register.
Please enter your desired user name, your email address and other required details in the form below.
User Name:
Password
Please enter a password for your user account. Note that passwords are case-sensitive.
Password:
Confirm Password:
Email Address
Please enter a valid email address for yourself.
Email Address:
Medical School
Choose "---" if you don't want to tell. AMG for US & Canadian medical schools. IMG for all other medical schools.
USMLE Steps History
What steps finished! Example: 1+CK+CS+3 = Passed Step 1, Step 2 CK, Step 2 CS, and Step 3.

Choose "---" if you don't want to tell.

Favorite USMLE Books
What USMLE books you really think are useful. Leave blank if you don't want to tell.
Location
Where you live. Leave blank if you don't want to tell.

Log-in

Human Verification

In order to verify that you are a human and not a spam bot, please enter the answer into the following box below based on the instructions contained in the graphic.



Thread Tools Search this Thread
Search this Thread:

Advanced Search
Display Modes


Similar Threads
Thread Thread Starter Forum Replies Last Post
HPV and koilocytic cell!! salz USMLE Step 1 Forum 3 09-07-2011 05:41 AM
Cell transport.. surez18 USMLE Step 1 Forum 3 07-31-2011 02:37 PM
Astrocytoma Oncogen or Cause? Rawalian USMLE Step 1 Forum 2 05-03-2011 05:47 PM
T cell defect anoop_1198 USMLE Step 2 CK Forum 6 05-25-2010 11:49 PM
Giant Cell Tumor Mnemonic aungawa USMLE Step 1 Mnemonics 0 04-12-2010 07:45 AM

RSS Feed
Find Us on Facebook
vBulletin Security provided by vBSecurity v2.2.2 (Pro) - vBulletin Mods & Addons Copyright © 2017 DragonByte Technologies Ltd.

USMLE® & other trade marks belong to their respective owners, read full disclaimer
USMLE Forums created under Creative Commons 3.0 License. (2009-2014)