used in medical language to describe fairly powerful disease

not malignant

small pieces of tissue removed for examination under the microscope; biopsy is usually a small surgical procedure

biopsy: needle biopsy
also called fine needle or fine needle aspiration biopsy; using a small needle to obtain a very small amount of tissue for exam under a microscope

out-of-control cells, proliferating and creating damage to normal cells and organs

congenital cystic adenomatoid malformation (CCAM)
a type of congenital lung cyst which has occasionally been associated with later appearance of PPB

use of chemicals (drugs, medications) to treat cancer; almost always several drugs are combined into a carefully scheduled program called a "regimen"

chemotherapy, intracavitary
chemotherapy given into a body cavity such as the chest cavity (between the chest wall and the lungs); used to directly bathe cancer cells in the drug used.

chemotherapy, neo-adjuvant
chemotherapy used before major surgery to shrink a large amount of disease to make surgery easier or safer

chemotherapy, systemic
chemotherapy given by mouth or intravenously in order to get into the bloodstream; the drugs get to the cancer through the blood.

chest wall
the skin, muscles, and bone of the outer chest; the "ribcage"

a kind of malignant tissue found under the microscope in PPB; looks like malignant cartilage tissue

present at birth; may be genetic ("in the genes") but may be an "accident" during formation of the baby

when NO tumor remains in a person and enough time has passed that if any tumor did exist it would have shown up. Remission also means there is no known tumor, but not enough time has passed to be sure it is all gone.

an abnormal cavity filled by air or fluid and occupying space in some organ such as the lung. The cyst cavity wall is lined by a layer of cells.

cystic nephroma
cystic nephroma is a condition found in about 9-10% of PPB patients or in their families. Cystic nephroma is a benign abnormality in which fluid-filled cysts appear in one or both kidneys. The cysts may be small or very large. If the cysts are small, nothing might be done. For very large cysts which prevent a kidney from functioning normally, the kidney or parts of it might be removed with surgery. Cystic nephroma tends to appear in children under age 3 years.

the divider between the chest space and the abdominal space; made of muscle; works in breathing, coughing, etc.

DICER1 gene
this gene, in the nucleus of a cell on chromosome 14, instructs cells how to make a protein called DICER1. This protein is an enzyme. DICER1 protein processes molecules to make microRNA (miRNA) which are very important controller molecules inside cells. Mutations in DICER1 gene have been discovered in some individuals and families with PPB and some of the diseases associated with PPB such as rhabdomyosarcoma and cystic nephroma. A mutation in DICER1 may be a part of the cause for these conditions in certain PPB patients and their families.

a tendency for diseases to be present in more than one person in a family and the near relatives; the opposite of familial is "sporadic" familial usually means genetic

a kind of malignant tissue found under the microscope in PPB; looks like malignant fibrous/connective tissue

fine needle (aspiration) biopsy
using a small needle to obtain a very small amount of tissue for exam under a microscope

indicates that a gene abnormality is responsible for the disease or condition

a tendency for diseases to be inherited from one generation to the next; this implies that an abnormality in the genes allows for or causes the disease to occur in at least some family members. Inheritable, genetic.

used in medical language to describe slower-moving diseases, which may or may not still be dangerous in the long run

Institutional Review Board - a committee found in most hospitals and research institutions, responsible for reviewing all medical research to make sure it is ethical and that patients' rights and privacy are protected

a kind of malignant tissue found under the microscope in PPB; looks like malignant fatty tissue

a clump of tissue, a mass; may be benign or malignant

lymph nodes
clumps of lymph tissue throughout the body along lymph channels; nodes act as filters or sieves along those channels to prevent infections or cancers from spreading


the "edge" of a surgical removal of a mass; if the margin is normal tissue, there is no tumor at the edge of the surgical site

a lump of tissue, a tumor; usually abnormal but may be benign or malignant

the MIDDLE of the chest space containing the heart, esophagous (swallowing tube), the "bronchuses" (breathing tubes into the lungs), the major blood vessels (aorta, etc), lymph tissue (thymus and lymph nodes) and some nerves

the spread of cancerous tissue beyond the organ in which it started. Cancer may metastasize by direct extension into nearby organs, by getting into lymph channels and spreading to nearby lymph nodes, and by getting into the blood stream and spreading to distant organs.

a mutation is an error in the DNA code of a cell. Chromosomes are made up of DNA. DNA is the library of instructions for all cells' activities. Some mutations interfere strongly with how cells function. Other mutations are unimportant. Mutations can be inherited from mothers or fathers, but some mutations occur for the first time in a child and affect only that child -- or the mutation could be passed on to the offspring of the child from that point on.

no evidence of disease

a small mass, perhaps 1 - 2 centimeters in diameter

a thin membrane surrounding the heart

the coverings of the lung AND of the inside surface of the chest wall.

pleura, parietal
the pleura covering the inside of the chest wall

pleura, visceral
the pleura covering the lungs

pleuropulmonary blastoma (PPB)
the medical name for a rare childhood cancer of lung tissue and/or pleural tissue; it is different from the cancer called "pulmonary blastoma" which is a rare lung cancer of adults.

pneumothorax (pleural: pneumothoraces)
an abnormal condition in which air has leaked into the "pleural" space (the space between the chest wall and the lung) collapsing the lung

PPB Family Tumor Syndrome
PPB is a disease which is associated with several other conditions either in the PPB patient or in family members. Because most of these conditions are rare, it suggests that there is a genetic abnormality which predisposes the patient and family members to having the conditions. Many, many family members are completely normal, so the genetic predisposition is just that - only a predisposition. Conditions associated with PPB are other cases of PPB, lung cysts, cystic nephroma, some other childhood cancers, some non-cancerous conditions like small intestine polyps, and some ovarian and testicular tumors. A mutation in DICER1 gene has been discovered in some PPB families and may be, in part, responsible for this familial tumor predisposition syndrome. Additional conditions in patient or family occur in about 1/3 of PPB cases.

pulmonary blastoma
a rare cancer of lung tissue occurring in adults; different from PPB

radiation therapy
a way to kill malignant cells or tissue by focusing penetrating and damaging x-rays directly on such tissue; the same kind of x-rays are used for common diagnostic purposes but common x-rays involve MUCH LESS energy than radiation therapy

relapse; the return of disease

the return of disease; recurrence

usually used for leukemia but can be used for any cancer. Means that there is no known disease, but microscopic or hidden disease may be present and may show up later. A remission becomes a cure when enough time has passed that any disease would have shown up. In PPB, it usually takes 3 or 4 years to be sure it won't come back, but rarely PPB has come back even after 3 - 4 years

surgical removal of a mass

resection, complete
usually means that a surgeon believes he/she has removed all visible abnormal tissue; there may be microscopic amounts remaining

resection, sub-total
less than "complete" or "total" resection: the surgeon knows that there is some abnormal tissue remaining that it wasn't possible or safe to remove

resection, total
usually means that a surgeon believes he/she has removed all visible abnormal tissue; there may be microscopic amounts remaining

a kind of malignant tissue found under the microscope in PPB; looks like malignant muscle tissue

a situation in which tumor is very fragile and fragments of it are unavoidably "spilled" into the space around where a tumor is being operated upon; means that microscopic (or more) amounts of tumor remain

physical removal of cancerous or other tissue

a large lymph organ in the middle of the chest

a clump of tissue, a mass; may be benign or malignant