Rare Tumors Patient & Family Handbook

The common elements considered in most staging systems are site of the primary tumor tumor size and number of tumors

lymph node involvement (spread of cancer into lymph nodes) cell type and tumor grade (how closely the cancer cells resemble normal tissue cells) the presence or absence of metastasis.

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)

Stage I, Stage II, and Stage III

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 IV

The cancer has spread to other organ(s).

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.

Liver Cancer

Germ Cell Tumor

Thyroid Cancer

Nasopharyngeal Cancers

Malignant Melanoma

Adrenocortical Carcinoma

Pleuropulmonary Blastoma

TEST

✓ ✓ ✓

✓ ✓ ✓

✓ ✓

✓ ✓ ✓ ✓ ✓ ✓ ✓

✓ ✓ ✓

✓ ✓ ✓

Biopsy

X ray

CT scan

Endoscopy

✓ ✓

✓ ✓

✓ ✓

PET scan

MRI

Bone Scan

✓ ✓

✓ ✓

✓ (neck)

✓ ✓

Ultrasound

Blood Test

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