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DSRCT and Diagnostics

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Warning: This information is provided for information purposes. You should always discuss your individual case with your own physician. Keep in mind that there are different stages of DSRCT tumors, from one local tumor to metastatic disease. Please find out which stage your physician considers your tumor to be.

Introduction

  • The following are the tools used by the medical field to diagnose or keep an eye on DSRCT tumors (well, all types of cancer). Please be aware that in stage IV disease some of these diagnostic tools do not pick up the extent of cancer that can be present within the abdomen. This information is based on numerous reports from patients with DSRCT who have had an abdominal CT or PET scan and than during surgery it is discovered there is widespred disease. This is possibly due to the fact that DSRCT seems to come in a variety of sizes from volley ball sized abdominal tumors to golf ball size to nodules to microscopic seeding.
  • Patients should be aware that CT and PET scan reports of 'no visible evidence' of disease can mean that the diagnostic tools can't spot the cancer because it is too small. The only way to determine if a patient has no disease is from biopsy or exploratory surgery or from the passage of time (nothing shows up). They needed to have a few consecutive CT scans with 3 month in between to see if more lung meets are getting seen. One of the reasons for this type of delay is that sarcomas (like DSRCT) are often already disseminated when the primary is respected and microscopic disease can stay dormant for an uncertain period of time, thus there is no way to know the real number. Treatment will depend on the location and the number of the mets and how agressive are they, so they need to wait for the next scan to find it out.
  • Patients should also be aware that it is possible for some MRI machines to pick up the path of peritoneal spread caused by tumor shedding (the path flow of malignant ascitis). Whether this information is valuable for treatment should be determined by

the oncologist.

  • It is hoped that this information will help patients establish a dialogue with their physicians as they try to determine the best course of treatment.

Biopsy

  • Sarcoma tumors are rare. Desmoplastic small round cell tumors are even rarer. Because of this a biopsy from a suspected sarcoma should be reviewed by a sarcoma pathology specialist.
  • It is critical before treatment begins to know exactly which type of sarcoma you have.
  • There are two types of biopsies that can be done, either a needle biopsy in the doctors office or incisional biopsy in the hospital. In the majority of DSRCT cases the biopsy is done in the operating room. A small incision will be made and a sample of tissue removed.
  • With DSRCT diagnosis has been known to take up to a week or more. It is important to get a correct diagnosis as quickly as possible. Sometimes DSRCT can be mistaken for Rhabdomyosarcoma, Germ Cell tumor, or Uterine sarcoma. Because DSRCT is rare the biopsy may need a second or third opinion.

X-Ray

  • Helpful for the initial test to spot tumors. Usually a patient will have an x-ray first before being recommended for a PET, CT, or MRI.
  • Chest x-rays are also used for spotting metastatic cancer in the lungs.

Computed Tomography (CT)

  • Computed tomography (CT)is often used to locate tumors which are in the abdomen, pelvis, or chest. In the case of DSRCT it should be noted that the scans can pick up large tumors, but often misses smaller nodules and will not pick up the extent of microscopic disease.
  • Your oncologist will probably order a 'baseline' CT to be used as your treatment begins. The baseline CT will be compared to future CT to see if there is any change in your tumors as you undergo chemotherapy or radiation.
  • The CT is a useful tool for the oncologist to keep track of disease progress. Oncologists will use a CT to see if the chemotherapy or radiation treatments are reducing the size and amount of disease. When the tumors reach a certain size than the oncologist will often recommend surgery to remove the bulk of the tumors. If the CT reveals that the tumors are progressing, than the oncologist will determine that the chemotherapy is not working and will try to change the protocol.
  • CT can also be used in conjunction with a PET scan to give the oncologist even more information about the location and size of tumors.

Positron Emission Tomography (PET)

  • Positron emission tomograpy (PET) produces a three dimensional image or map of functional processes in the body. This machine is used in Nuclear Medicine and works for cancer by looking at the metabolic activity of cells in the body. Malignant tumor cells have an active metabolism and therefore can be seen on a PET scan.
  • In the case of DSRCT, if there are any active cancer cells than the image will 'light' up as spots.
  • As in other diagnostics, PET scans may not be able to detect very small lesions and microscopic disease. DSRCT cancer cells can stay dormant for an undetermined amount of time and than reappear (reoccurence).
  • An oncologist will order a PET scan to use as a baseline and compare the image with future PET scans. Sometimes a PET scan will be used in conjunction with a CT to give the oncologists more information about the disease.
    • PET scans require careful preparation, so please follow instructions carefully.

24 hours prior to your appointment:

    • Refrain from consuming any caffeine, including any decaffeinated products for 24 hours prior to your exam. Note: Decaffeinated products still contain small amounts of caffeine.

12 hours prior to your appointment:

  • Stay on a low-carbohydrate diet. (See below for a guide on what to eat.)

Do NOT engage in any strenuous exercise. Do NOT chew gum. Avoid mints.

6 hours prior to your appointment:

  • Do NOT eat anything.
  • Drink several glasses of water (2-6).
  • Take any prescribed medications as usual.
  • If you need to eat, please stick to a small protein-only meal.
  • If you are diabetic, please consult your doctor for questions regarding diet and medication.
  • Low-Carbohydrate Diet Guide:

Foods allowed:

  1. All meats
  2. Tofu
  3. Hard cheeses
  4. Eggs
  5. Unsweetened peanut butter
  6. Oil, margarine, butter
  7. Diet soda (be careful to avoid caffeinated and #decaffeinated sodas.)
  8. Non-starchy vegetables (such as: broccoli, spinach, green beans.)

Foods not allowed:

  1. Cereals
  2. Breads
  3. Pasta
  4. Rice
  5. Dry beans
  6. Gravies
  7. Fruit and fruit juices
  8. Jams and jellies
  9. Sugar and candy
  10. Honey
  11. Starchy vegetables (such as: peas, corn, potatoes)
  12. Milk (including non-dairy milk)
  13. Alcohol
  14. Coffee or Tea

Magnetic Resonance Imaging(MRI)

  • In general this tool is useful to image tumors in the extemeties and soft tissue. It gives the oncologist a detailed view of the tumor and the surrounding organs and structures within the body.

This can be a useful tool for locating peritoneal implants within the abdomen.

Malignant involvement of the peritoneum may occur as a result of intraperitoneal seeding of tumor cells from primary malignancies of the ovary, abdominal cavity, and gastrointestinal tract. These tumor cells will then spread according to the pathways of ascitic flow. Eventually, dissemination of intraperitoneal tumor may involve all of the peritoneal surfaces of the abdomen and pelvis, including the free peritoneal surfaces, the bowel, perihepatic and perisplenic ligaments, mesentery, and omentum.
Alternatively, these same peritoneal structures may serve as pathways of direct tumor spread from tumors within the abdomen and pelvis. Primary tumors of the pancreas, liver, gallbladder, stomach, or spleen, for example, often spread to organs via the peritoneal reflections of the upper abdomen.
Primary tumors of the stomach, pancreas, colon, and appendix often spread by intraperitoneal tumor shedding and subsequent peritoneal carcinomatosis. Accurate depiction of sometimes subtle peritoneal tumor can completely alter patient management. In patients with pancreatic cancer, for instance, surgical resection is not indicated if metastatic peritoneal tumor is confirmed on preoperative MR imaging. Similarly, in the patient with colon cancer metastatic to the liver, possible hepatic resection of isolated liver metastases may prolong survival. With concurrent peritoneal metastases, however, hepatic tumor resection is obviously contraindicated. In the patient with gastric cancer, although we are all familiar with the drop metastases to the pelvis producing large complex Krukenberg tumors, it is more common to find subtle peritoneal metastases elsewhere in abdomen on MR images.

Spread of Tumors

The peritoneal reflections also form the ligaments that connect the abdominal organs and viscera to one another and to the retroperitoneum and abdominal wall. In the upper abdomen, a complex network of peritoneal reflections surround and connect the liver, stomach, spleen, kidneys, and duodenum. These peritoneal reflections thus serve as important potential pathways for the spread of abdominal malignancies.
The gastrohepatic ligament is also known as the lesser omentum. It extends from the lesser curvature of the stomach to the left lobe of the liver. On the liver surface, the gastrohepatic ligament extends into the fissure for the ligamentum venosum, which separates the caudate lobe from the left hepatic lobe. The hepatoduodenal ligament is located along the free margin of the lesser omentum or gastrohepatic ligament. It extends from the porta hepatis to the duodenal sweep and contains the components of the portal triad: the portal vein, hepatic artery, and bile ducts. The hepatoduodenal ligament is an important pathway of spread of inflammation or tumor from the retroperitoneum to the liver or from the liver to the retroperitoneum.
Once tumor gains access to the liver, it can spread along the periportal space, which then communicates with the left intersegmental fissure and the falciform ligament. The falciform ligament connects the liver with the anterior abdominal wall. Using these peritoneal reflections, a continuous pathway is thus established from the retroperitoneum through the liver to the abdominal wall. A pancreatic cancer, for example, can spread along the hepatoduodenal ligament from the retroperitoneum to the liver, and then along the periportal tissues to the falciform ligament and finally to the anterior abdominal wall.
The gastrohepatic, gastrosplenic, and splenorenal ligaments, greater omentum, and transverse mesocolon are additional peritoneal reflections that also serve as potential pathways for tumors to spread to the liver, stomach, spleen, kidneys, and colon. - Body MRI: Diagnostic Imaging

Exploratory Surgery

  • This type of surgery is sometimes undertaken to observe the tumor or tumor location. A biopsy may be taken at this time.
  • This type of surgery is not without risk and should be done after major surgery to remove all visible signs of tumor.
  • A second look exploratory surgery and biopsy may be the only reliable way to tell if there is any more visible disease present. Patients should remember that no visible evidence of disease does not account for microscopic or disease.

Possible Markers

  • Desmoplastic small round cell tumor is a unique, chimeric disease that shares common genetic features with Ewings Sarcoma and Wilms tumor. This distinguishing feature helps in the final diagnosis of the disease.
  • At the moment there is no known 'marker' for DSRCT that would alert a patient that the disease is live and present.

Chemosensitivity Testing

  • There are some labs that offer the service of testing a patients tumor sample to see which chemicals the tumor will respond to. Patients are encouraged to discuss this issue with their oncologists to see if this may be of benefit in their treatment plan.
  • Patients should use this type of testing as a tool and not as a final decision on which chemo drugs to use. There are many factors to consider, including the fact that some DSRCT tumors may respond differently within the body. Also, patients should be aware that the test may not always be reliable. Much depends upon how much tumor sample is used, the weight or amount of chemicals tested on the tumor, the drug combinations tested. Also once the patient has undergone chemotherapy and wishes to have the tumor retested, new samples will need to be retreived. The second testing may than conflict with the first test.

Facilities that offer Chemosensitivity Testing for soft tissue sarcoma:

    • Oncotech
      • 15501 Red Hill Avenue
      • Tustin, CA 92780
      • Phone: (800) 576-6326
      • Fax: (714) 566-0421
        • Client Services
        • Phone: (800) 662-6832
        • Fax: (714) 566-0423
    • Caris Molecular Profiling Institute
      • Dr. Arlet Alarcon, Manager
      • 445 N. 5th Street, 3rd Floor
      • Phoenix, AZ 85004
        • Phone: 602-358-8982
        • Fax: 602-358-8920
        • Email: aalarcon@carismpi. com

DSRCT References

Contact: dsrct.wiki@gmail.com

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