Difference between revisions of "IPLab:Lab 5:Neurofibromatosis"

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== Clinical Summary ==
 
== Clinical Summary ==
This 45-year-old divorced white male came to the emergency room with severe hepatic cirrhosis and aspiration pneumonia. Shortly after admission he developed cardiac arrhythmias and died. Significant past history included alcohol abuse, cirrhosis, and neurofibromatosis. He had no family history of neurofibromatosis, but his condition was diagnosed at age 17 when he developed neurofibromas along the lateral chest wall. There was no history of continued follow-up after this initial diagnosis.
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A 45-year-old divorced white male came to the emergency room with severe hepatic cirrhosis and aspiration pneumonia. Shortly after admission he developed cardiac arrhythmias and died. Significant past history included alcohol abuse, cirrhosis, and neurofibromatosis. He had no family history of neurofibromatosis, but his condition was diagnosed at age 17 when he developed neurofibromas along the lateral chest wall. There was no history of continued follow-up after this initial diagnosis.
  
== Autopsy Findings ==
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The patient was covered with variably sized subcutaneous nodules ranging from 0.5 to 2.5 cm in diameter. Other significant findings included micronodular hepatic cirrhosis, ascites (500 ml), and splenomegaly.
The patient was covered with variably sized subcutaneous nodules ranging from 0.5 to 2.5 cm in diameter. Other significant findings included micronodular hepatic cirrhosis (liver weight: 1760 grams), ascites (500 ml), and splenomegaly (weight: 260 grams).  
 
  
 
== Images ==
 
== Images ==
 
<gallery heights="250px" widths="250px">
 
<gallery heights="250px" widths="250px">
File:IPLab5Neurofibromatosis1.jpg|This photograph, taken at autopsy, demonstrates the distribution of neurofibromas on the skin of this patient.  
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File:IPLab5Neurofibromatosis1b.JPG|This photograph, taken at autopsy, demonstrates the distribution of neurofibromas on the skin of this patient.  
File:IPLab5Neurofibromatosis2.jpg|This is another view taken at autopsy demonstrating the neurofibromas. Some lesions can be seen as subcutaneous swellings (arrow) and others form pedunculated masses. Most are hyperpigmented.  
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File:IPLab5Neurofibromatosis2b.JPG|This is another view taken at autopsy demonstrating the neurofibromas. Some lesions can be seen as subcutaneous swellings (arrow) and others form pedunculated masses. Most are hyperpigmented.  
File:IPLab5Neurofibromatosis3.jpg|This is a closer view of neurofibromas on the skin.  
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File:IPLab5Neurofibromatosis3b.JPG|This is a closer view of neurofibromas on the skin.  
 
File:IPLab5Neurofibromatosis4.jpg|This is a low-power photomicrograph of a subcutaneous neurofibroma (1). Note the increased pigmentation in the skin (2).  
 
File:IPLab5Neurofibromatosis4.jpg|This is a low-power photomicrograph of a subcutaneous neurofibroma (1). Note the increased pigmentation in the skin (2).  
 
File:IPLab5Neurofibromatosis5.jpg|This is a higher-power photomicrograph of the neurofibroma (1) with the overlying skin (2).  
 
File:IPLab5Neurofibromatosis5.jpg|This is a higher-power photomicrograph of the neurofibroma (1) with the overlying skin (2).  
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File:IPLab5Neurofibromatosis8.jpg|This is a high-power photomicrograph of the cells in the neurofibroma.  
 
File:IPLab5Neurofibromatosis8.jpg|This is a high-power photomicrograph of the cells in the neurofibroma.  
 
</gallery>
 
</gallery>
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== Virtual Microscopy ==
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<peir-vm>IPLab5Neurofibromatosis</peir-vm>
  
 
== Study Questions ==
 
== Study Questions ==
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=== Images ===
 
=== Images ===
* [http://peir.path.uab.edu/library PEIR Digital Library]
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* [{{SERVER}}/library/index.php?/tags/2145-neurofibromatosis PEIR Digital Library: Neurofibromatosis Images]
** Try the following searches:
 
*** neurofibromatosis
 
*** von recklinghausen's disease
 
 
* [http://library.med.utah.edu/WebPath/CNSHTML/CNSIDX.html#11 WebPath: CNS Neoplasms]
 
* [http://library.med.utah.edu/WebPath/CNSHTML/CNSIDX.html#11 WebPath: CNS Neoplasms]
  

Latest revision as of 19:29, 8 July 2020

Clinical Summary[edit]

A 45-year-old divorced white male came to the emergency room with severe hepatic cirrhosis and aspiration pneumonia. Shortly after admission he developed cardiac arrhythmias and died. Significant past history included alcohol abuse, cirrhosis, and neurofibromatosis. He had no family history of neurofibromatosis, but his condition was diagnosed at age 17 when he developed neurofibromas along the lateral chest wall. There was no history of continued follow-up after this initial diagnosis.

The patient was covered with variably sized subcutaneous nodules ranging from 0.5 to 2.5 cm in diameter. Other significant findings included micronodular hepatic cirrhosis, ascites (500 ml), and splenomegaly.

Images[edit]

Virtual Microscopy[edit]

Study Questions[edit]


Additional Resources[edit]

Reference[edit]

Journal Articles[edit]

Images[edit]

Cirrhosis is a liver disease characterized by necrosis, fibrosis, loss of normal liver architecture, and hyperplastic nodules.

In alcoholics, aspiration pneumonia is common--bacteria enter the lung via aspiration of gastric contents.

Arrhythmias are abnormal heart rhythms.

Ascities is the accumulation of fluid with in the abdominal cavity. There should be less than 30 ml of fluid in the peritoneal cavity.