What are the other Names for this Condition? (Also known as/Synonyms)

• Intimal Sarcoma of Pulmonary Artery

What is Pulmonary Artery Intimal Sarcoma? (Definition/Background Information)

• An intimal sarcoma of artery is a connective tissue tumor that originates in large blood vessels (the major arteries) and in the heart-lung blood circulation system, in the body
• There are 2 main types of intimal sarcoma, based on the location it affects: Intimal Sarcoma of Pulmonary Artery, and intimal sarcoma of aorta. The former (pulmonary type) is twice as common as the latter (aortic type)
• Pulmonary Artery Intimal Sarcoma is an extremely rare tumor that may be an undifferentiated tumor (undifferentiated pleomorphic sarcoma) or show components of other sarcomas including osteosarcoma and chondrosarcoma
• The tumor is mostly seen in adults (young, middle-aged, and older). The cause of Pulmonary Artery Intimal Sarcoma is unknown and no relevant risk factors have been yet identified
• The tumor can present a host of signs and symptoms including breathlessness, chest pain, and recurrent cough. Metastatic disease can result in weight loss, fatigue, and severe anemia
• Pulmonary Artery Intimal Sarcoma is a highly-malignant tumor. It has the tendency to spread to the bones, liver, kidney, and other major organs. A wide age range of individuals may be affected by this tumor
• The treatment of the tumor may involve surgery, chemotherapy, and/or radiation therapy. However, the prognosis of Pulmonary Artery Intimal Sarcoma depends upon several factors including the response to treatment and the stage of the tumor. In a majority of cases, the prognosis is poor

Who gets Pulmonary Artery Intimal Sarcoma? (Age and Sex Distribution)

• Pulmonary Artery Intimal Sarcoma may be present in individuals of all age group, though the tumor is normally observed in adults. It is rarely found in infants and very young children
• The average age of incidence is 56 years and age range is between 26-78 years
• Both males and females are reported to be equally affected
• There is no racial or ethnic preference observed

What are the Risk Factors for Pulmonary Artery Intimal Sarcoma? (Predisposing Factors)

• Risk factors are currently unknown or unidentified for Pulmonary Artery Intimal Sarcoma
• Certain morphological or genetic abnormalities may be potential factors that lead to development of the condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one's chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Pulmonary Artery Intimal Sarcoma? (Etiology)

The exact cause of Pulmonary Artery Intimal Sarcoma is unknown.

• It is thought to occur spontaneously, due to some genetic or morphological abnormality
• Amplification of MDM2 gene is reported in many tumor specimens, along-with PGDFRA gene amplification

What are the Signs and Symptoms of Pulmonary Artery Intimal Sarcoma?

Wide-ranging signs and symptoms of Pulmonary Artery Intimal Sarcoma may be observed. These include the following:

• Shortness of breath , which is the most common presenting symptom
• Chest and/or back pain
• Frequent cough with blood in cough/sputum
• Fatigue, tiredness
• Fainting spells
• Progressive weight loss
• Fever
• Anemia

Other signs and symptoms may include:

• Right-side heart failure; heart murmur
• Presence of aneurysm in the pulmonary artery
• Cyanosis or bluish discoloration of skin
• Swelling of feet
• Swollen liver
• Symptoms that are similar to Takayasu disease may be present

Features of the tumor include:

• The tumor is normally located either on the right or left, or on both branches of the main pulmonary trunk (in 80% of the cases), or is found on the pulmonary valve
• Presence of a solitary tumor mass with a polypoid growth pattern inside the artery
• The lumen of the involved blood vessels are often dilated and obstructed
• The tumor may extend into the lung tissues or mediastinum from the pulmonary artery
• High-grade sarcomas may show hemorrhage and tumor necrosis

Pulmonary arterial embolism (both acute and chronic) is frequently and incorrectly diagnosed as Pulmonary Intimal Sarcoma, due to similar presentations. The signs and symptoms of the tumor can also cause it to be misdiagnosed as pulmonary hypertension, in some cases.

How is Pulmonary Artery Intimal Sarcoma Diagnosed?

A diagnosis of Pulmonary Artery Intimal Sarcoma would involve the following procedures and tests:

• Physical examination and complete medical history screening: During the physical exam, the healthcare provider may listen to the lung  with a stethoscope, to detect the presence of any abnormal lung sounds
• Imaging studies that may include a chest X-ray, MRI or CT scan of the lungs
• Arterial blood gases
• Lung function test
• Sputum cytology: This procedure involves the collection of mucus (sputum), coughed-up by the patient, which is then examined in a laboratory by a pathologist

A tissue biopsy refers to a medical procedure that involves the removal of cells or tissues, which are then examined by a pathologist. This can help establish a definitive diagnosis. The different biopsy procedures may include: 

• Bronchoscopy: During bronchoscopy, a special medical instrument called a bronchoscope is inserted through the nose and into the lungs to collect small tissue samples. These samples are then examined by a pathologist, after the tissues are processed, in an anatomic pathology laboratory
• Thoracoscopy: During thoracoscopy, a surgical scalpel is used to make very tiny incisions into the chest wall. A medical instrument called a thoracoscope is then inserted into the chest, in order to examine and remove tissue from the chest wall, which are then examined further
• Thoracotomy: Thoracotomy is a surgical invasive procedure with special medical instruments to open-up the chest. This allows a physician to remove tissue from the chest wall or the surrounding lymph nodes of the lungs. A pathologist will then examine these samples under a microscope after processing the tissue in a laboratory
• Fine needle aspiration biopsy (FNAB): During fine needle aspiration biopsy, a device called a cannula is used to extract tissue or fluid from the lungs, or surrounding lymph nodes. These are then examined in an anatomic pathology laboratory, in order to determine any signs of abnormality. Nevertheless, FNAB is not a preferred method for the biopsy of lung tumors
• Autofluorescence bronchoscopy: It is a bronchoscopic procedure in which a bronchoscope is inserted through the nose and into the lungs and measure light from abnormal precancerous tissue. Samples are collected for further examination by a pathologist

Note: A diagnosis of Intimal Sarcoma is very difficult and most often the actual cause is evaluated and understood post-mortem.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Pulmonary Artery Intimal Sarcoma?

The complications of Pulmonary Artery Intimal Sarcoma could include:

• The metastasizing or malignant nature of intimal sarcoma and its potency of affecting many vital organs and blood vessels
  • Frequently, lung metastasis, or spread of the cancer to outside of the chest region (to the kidney, pancreas, lymph nodes, brain, skin) is observed
  • Metastasis to the spine resulting in cauda equina syndrome
• Blood loss during invasive treatment methods may be heavy
• Damage of vital nerves and surrounding structures during surgery
• Side effects due to chemotherapy and radiation therapy
• Tumor recurrence following surgery

How is Pulmonary Artery Intimal Sarcoma Treated?

Pulmonary Artery Intimal Sarcomas are aggressive lesions that require early and accurate diagnosis, followed by prompt and appropriate treatment. The treatment measures may include:

• Wide surgical excision of the tumor (removal of the entire tumor) followed by chemotherapy and/or radiotherapy
• When it is at an inaccessible location, or is unsafe for surgical intervention, radiation therapy and/or chemotherapy is employed
• Post-operative care is important: A minimum activity level is to be ensured, until the surgical wound heals
• Follow-up care with regular screening and check-ups are important

How can Pulmonary Artery Intimal Sarcoma be Prevented?

• Current medical research have not established a method of preventing Pulmonary Artery Intimal Sarcoma
• Regular medical screening at periodic intervals with blood tests, scans, and physical examinations are mandatory for those who have already endured the tumor, due to its high metastasizing potential and possibility of recurrence

What is the Prognosis of Pulmonary Artery Intimal Sarcoma? (Outcomes/Resolutions)

• Pulmonary Artery Intimal Sarcoma is an extremely rare, but high-malignancy tumor that is not yet completely understood. The long-term prognosis depends on a combination of factors such as:
  • Age of the individual at the time of diagnosis
  • Tumor location, size, and stage at detection
  • And most importantly its early and accurate identification
• The best outcome is when the tumor is detected early, it can be surgically removed, and when it has not metastasized to other body regions. Favorable factors may include the presence of low-grade tumor components and positive response to multimodal therapy
• The 3 year survival rate is around 70% following surgery (complete removal of tumor). The survival rate, when the tumor is removed incompletely, is around or less than 12 months
• However, despite intense treatment and management of the condition, the prognosis remains poor given its highly-infiltrative metastasizing ability. Pulmonary Artery Intimal Sarcoma is also known to infrequently result in sudden death

Additional and Relevant Useful Information for Pulmonary Artery Intimal Sarcoma:

The management of Pulmonary Artery Intimal Sarcoma is very difficult for the following reasons:

• The extreme rarity of the tumor
• The non-specific and misleading symptoms it presents
• Its high-malignancy behavior
• The difficulty in performing complete surgical resection
• The several regions and organs of the body it can affect
• And a lack proper medical literature on the subject