About pulmonary rhabdomyosarcoma

What is pulmonary rhabdomyosarcoma?

Pleuropulmonary blastoma (PPB) is a rare childhood cancer occurring in the chest, specifically in the lungs or in the coverings of the lungs called "pleura". PPB occurs almost exclusively in children under the age of approximately 7-8 years and rarely in older children, in teenagers and more rarely in adults. Three subtypes of PPB exist and are called type I, type II, and type III PPB. Type I PPB takes the form of cysts in the lungs (air-filled pockets) and occurs in the youngest children with PPB (from birth to about 2 years of age). Type II PPB has cysts and solid tumor. Type III PPB is entirely solid tumor. Types II and III PPB tend to occur after age 2 years. Children with type I PPB can have the disease come back. If type I PPB comes back, it usually happens within about 4 years of the original diagnosis, and it is usually type II or III when it recurs. Children with type I PPB have a better outlook ("prognosis") than children with types II and III PPB; most type I PPB patients are cured (more than 80%). Treatment for type I consists of surgery and possibly chemotherapy; for Types II and III PPB surgery, chemotherapy and possibly radiation therapy is recommended. At present, about 50-60% of types II and III children are cured.

PPB is a childhood cancer in the family of cancers called soft tissue cancers, which are scientifically called sarcomas. PPB is, therefore, a soft tissue sarcoma. PPB is related to, or in the broad family of, cancers such as cancers of muscle, cartilage, and connective tissues. Physicians classify diseases this way in order to compare features and to compare treatments. Often treatments that are effective for one member of a family of cancers are effective for other members of that family.

PPB occurs in the lungs so PPB can be called a lung cancer, but PPB has no connection whatsoever with lung cancers in adults that are often related to tobacco use.

Like many cancers, PPB can spread through the blood to other areas of the body. When a cancer spreads to another part of the body it is called a "metastasis" of the cancer. Types II and III PPB can metastasize. The most common location for a PPB metastasis is the brain. Less common metastasis occurs in the bones, the liver, remaining parts of the lung, and other organs. It is not known why PPB has this pattern of spread to brain, liver, or bones. PPB can also spread by growing directly into tissues next to the lung like the diaphragm.

What are the symptoms for pulmonary rhabdomyosarcoma?

For example, if the cancer is in the head or neck area, signs and symptoms may include, among others:

  • Headache
  • Bulging or swelling of the eyes
  • Bleeding in the nose, throat or ears

If the cancer is in the urinary or reproductive system, signs and symptoms may include, among others:

  • Trouble urinating and blood in the urine
  • Difficulty with bowel movements
  • A mass or Bleeding in the vagina or rectum

If the cancer is in the arms or legs, signs and symptoms may include, among others:

  • Swelling or a lump in the arm or leg
  • Pain in the affected area, though sometimes there is no pain

What are the causes for pulmonary rhabdomyosarcoma?

It's not clear what causes rhabdomyosarcoma.

Doctors know that rhabdomyosarcoma begins when a cell develops changes in its DNA. A cell's DNA contains the instructions that tell a cell what to do. The changes tell the cell to multiply quickly and to go on living when healthy cells would normally die. The result is a mass (tumor) of abnormal cells that can invade and destroy healthy body tissue. The abnormal cells can break away and spread (metastasize) throughout the body.

What are the treatments for pulmonary rhabdomyosarcoma?

Rhabdomyosarcoma treatment usually involves a combination of treatments, including chemotherapy, surgery and radiation therapy.

Which treatments your doctor recommends will depend on the location of the cancer, the size of the tumor, whether the cells are aggressive, and whether the cancer is confined to one area (localized rhabdomyosarcoma) or it has spread to other areas of the body (metastatic rhabdomyosarcoma).


The goal of surgery is to remove all of the cancer cells. But it's not always possible to do that if the rhabdomyosarcoma has grown around or near organs or other important structures. When the cancer can't be removed completely with surgery, doctors may remove as much as possible and then use other treatments, such as chemotherapy and radiation, to kill any cancer cells that might remain.


Chemotherapy uses powerful drugs to kill cancer cells. The treatment usually involves a combination of drugs, which are usually administered through a vein. Which drugs are given and how often varies depending on your particular situation.

Chemotherapy is often used after surgery or radiation therapy to kill any cancer cells that might remain. It can also be used before other treatments to shrink a tumor to make surgery or radiation therapy more effective.

Radiation therapy

Radiation therapy uses high-energy beams, such as X-rays and protons, to kill cancer cells. It's usually done with a machine that moves around you as it aims the radiation at precise points on the body.

Radiation therapy might be recommended after surgery to kill any cancer cells that remain. It can also be used instead of surgery when the rhabdomyosarcoma is located in an area where surgery isn't possible because of nearby organs or other important structures.

Clinical trials

Clinical trials are studies to investigate new ways of treating cancer. Ask your doctor or your child's doctor about whether you may be eligible to join a trial.

What are the risk factors for pulmonary rhabdomyosarcoma?

Factors that may increase the risk of rhabdomyosarcoma include:

  • Family history of cancer. The risk of rhabdomyosarcoma is higher in children with a blood relative, such as a parent or sibling, who has had cancer, particularly if that cancer occurred at a young age. But most children with rhabdomyosarcoma have no family history of cancer.
  • Genetic syndromes that increase the risk of cancer. In rare instances, rhabdomyosarcoma has been linked to genetic syndromes that are passed from parents to children, including neurofibromatosis 1, Noonan syndrome, Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome and Costello syndrome.

Is there a cure/medications for pulmonary rhabdomyosarcoma?

A soft-tissue malignancy, in simple terms, is a type of cancer, while common in children, is comparatively rare in middle-aged adults (45+). It is said to be the result of translocation of primitive mesenchymal cells. This transformation occurs when parts of the DNA are swapped from one chromosome to another, and tends to occur at the time of a single cell splitting into two independent ones.

Pulmonary Rhabdomyosarcoma is a rare kind of cancer that develops in soft tissues, specific to skeletal muscle tissue or, at times, hollow organs like the bladder and the uterus. It commonly affects children.

Before the prognosis is determined, it is important to identify what type it is, and where it begins (head and neck area, urinary system, reproductive systems such as the vagina or uterus or testes, arms and legs) growth of the tumor and its range of host cells.

Cure/medications for Pulmonary Rhabdomyosarcoma
Yes, sometimes it can be cured. However, remission, as cancer goes, is almost inevitable. Survival rates of children are higher than those of adults.

Medications/drugs approved by the FDA are listed below:
1. Cosmegen (Dactinomycin)
2. Dactinomycin
3. Vincristine Sulfate
among others that are not mentioned here

Further divided into mild and severe risks:
Low Risk:
1. VA (vincristine, dactinomycin, which is also termed actinomycin-D)
2. VAC (vincristine, dactinomycin, cyclophosphamide)
Intermediate Risk:
1. VA (vincristine, dactinomycin, cyclophosphamide)
2. VAC/VI (vincristine, dactinomycin, cyclophosphamide, alternating vincristine with irinotecan)

Treatment of Pulmonary Rhabdomyosarcoma includes:

1. Surgery (Biopsy)
2. Chemotherapy
3. Radiation Therapy
4. High dosage chemotherapy
5. Stem cell transplant

A lump or swelling in the area where the tumor is and can be painful,Tumors in the head might cause vision problems or headaches,Tumors in lower parts of the body might cause bleeding, belly pain, or vomiting
Soft-tissue malignancies in children
Chemotherapy,Radiation therapy,Surgery

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