Rare Tumors Patient & Family Handbook

n TUMOR NODE METASTASIS The most common staging system is the tumor node metastasis (TNM) staging system. The TNM system is based on the extent of the tumor (T), the extent of spread to the lymph nodes (N), and the presence of distant metastasis (M). A number is added to each letter to indicate the size or extent of the primary tumor and how much the cancer has spread. For many cancers, TNM combinations correspond to one of five stages. Criteria for stages differ for different types of cancer.

Stage 0

Carcinoma in situ (contained in original site)

Higher numbers indicate more extensive disease: larger tumor size or spread of the cancer beyond the organ in which it first developed (to nearby lymph nodes or organs adjacent to the location of the primary tumor).

Stage I, Stage II, and Stage III

Stage IV

The cancer has spread to other organ(s).

n WHAT TESTS AND PROCEDURES WILL MY CHILD NEED? To diagnose a particular rare cancer and determine the extent of your child’s disease, a number of tests and procedures are necessary. To outline the best treatment possible, it is very important to identify the exact type of cancer and its presence throughout the body. Your physician will determine which tests and procedures will be required. The chart below lists the tests most commonly done for specific rare tumors.

Germ Cell Tumor

Thyroid Cancer

Nasopharyngeal Cancers

Malignant Melanoma

Adrenocortical Carcinoma

Pleuropulmonary Blastoma

TEST

Liver Cancer

Biopsy

4

4 4

4

4

4

4

X ray

4

4 4

4

4

4

CT scan

4

4

4

4

4

4

Endoscopy

4

PET scan

4

4

4

4

4

4

MRI

4

4

4

4

Bone scan

4

4

Ultrasound

4

4 4 (neck)

4

Blood tests

4

4 4

4

4

4

4

Biopsy A needle biopsy is a diagnostic procedure in which a needle is used to obtain tissue samples from an organ, bone, lymph node, or mass. Removing tissue samples is necessary to make a diagnosis and determine what treatment should be chosen. Sometimes this is done in the operating room and sometimes in the radiology department.

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