An onion that melts like snow!!!

Does an onion melt like snow?
It would in EWING’S SARCOMA.

Nah, I am not drunk drafting this article. “Ewing’s sarcoma is an onion that melts like a snow.”
It is a figure of speech to describe Ewing’s sarcoma!!!

It is so described since it appears as an “onion peel” in X-ray and
is highly radiosensitive (Patient responds to treatment as fast as a snow transforming itself to water).

 = 
How to read this X-ray?

  1. Look at the brightest (white) and darkest (black) regions in the X-ray. They are the reference colors. 
  2. The cortex of the bone appears white in color. This figure shows Ewing’s sarcoma in the HUMERUS.
  3. Follow the outline of the bony structure. You will find a disruption somewhere half way in the structure (indicated with arrows.)
  4. Ewing’s sarcoma has no borders as opposed to other osteoblastic lesions. Hence this will be a bit difficult to spot.
  5. Being an osteoblastic lesion, the lesion itself will be dark colored and in this case merge with the background.
  6. The diagnostic feature is the Layered Periosteal Reaction (aggressive) and the lesion seems to resemble onion.
Although the disease is curable, however headaches as a result of trying to memorize the treatment modalities may seem incurable. Here is perhaps one easy way to remember the drugs for chemotherapy. Look at the picture below:




Disclaimer: This figure has been created to help medical professionals remember the drug names. It is in no way intended to hurt anyone’s sentiments.



There are five drugs used in chemotherapy of the disease:

  1. Chris gardner = vin CRIS tine
  2. Cycle = CYCLO phosphamide
     
  3. Ruby = Doxo RUBI cin 
  4. Dark Tin = DAC TIN omycin 
  5. Top side sign = E TOP o SIDE 
Relapsed patients (responds to treatment but disease recurs): Topotecan/ irinotecan in combination with an alkylating agent. "Top Irene" may be used to remember the drugs.

Refractory cases (Do not respond to treatment): Anti IGF1r antibody.

Local treatment includes surgical resection with limb salvage/radiation therapy.

A classical presentation of the patient is localised pain and tenderness with a palpable mass. Fever may sometimes accompany the disease. 

A microscopic examination of the tissue sample reveals monotonous, small, round sheets of blue cells in the H/E stain.

Other facts about the disease are as follows:
  • Occurs most often in the second decade of life.
  • Mic-2 gene is the cell surface marker for the disease.
  • PAS stained cell detects “Glycogen filled cytoplasm” which is characteristic for Ewing’s sarcoma.
  • Since the disease is very aggressive, it is considered a “Systemic disease.”
  • Common sites of occurrence of the disease are pelvis, femur, humerus, ribs and clavicle.
  • Common sites of metastases are Lung, bones and bone marrow.
After having learnt almost everything about Ewings a thought lingers in my mind... "What are the odds of meeting a patient of Ewings?" It is 2.6:1,000,000. Interesting!

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