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

• Congenital Bronchopulmonary Leiomyosarcoma
• Congenital Peribronchial Myofibroblastic Tumour
• Neonatal Pulmonary Hamartoma

What is Congenital Peribronchial Myofibroblastic Tumor? (Definition/Background Information)

• Congenital Peribronchial Myofibroblastic Tumor (CPMT) is a very uncommon tumor affecting the lung airways. It has microscopic and macroscopic similarities with congenital infantile fibrosarcoma, but is unrelated to the tumor
• Congenital Peribronchial Myofibroblastic Tumor is typically present at birth. The cause of formation of the tumor is unknown and presently the risk factors are not well-established
• The tumor is often present as a large mass enveloping the bronchus with a potential to severely damage the airway. It may present obstructive signs and symptoms including cough and breathlessness
• The treatment of Congenital Peribronchial Myofibroblastic Tumor involves surgery, which can be curative. However, regular follow-up is advised following a surgical removal of the tumor
• In a majority of cases, the prognosis of Congenital Peribronchial Myofibroblastic Tumor is good and recurrence is not observed. However, the outcomes may be adversely affected by pregnancy-related complications (such as hydrops fetalis)

Who gets Congenital Peribronchial Myofibroblastic Tumor? (Age and Sex Distribution)

• Congenital Peribronchial Myofibroblastic Tumor is an extremely rare tumor with only around 20 cases being reported in the medical literature
• The condition is observed in newborn children (congenital manifestation); both males and females are affected
• No specific racial or ethnic group predilection is noted

What are the Risk Factors for Congenital Peribronchial Myofibroblastic Tumor? (Predisposing Factors)

• Currently, no risk factors have been identified for Congenital Peribronchial Myofibroblastic Tumor
• Presently, no cases have been associated with any genetic disorder or syndrome. Also, so far, no family history of this lung tumor has been reported

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones 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 Congenital Peribronchial Myofibroblastic Tumor? (Etiology)

• The cause of development of Congenital Peribronchial Myofibroblastic Tumor is generally unknown. It is reported to form in the fetus, while it is still in the mother’s womb
• The tumor is observed to develop spontaneously and no genetic anomalies have been reported
• Unlike other congenital tumors (such as congenital infantile fibrosarcoma or congenital mesoblastic nephroma) which this peribronchial tumor resembles, no chromosomal translocations seen in the former have been reported

What are the Signs and Symptoms of Congenital Peribronchial Myofibroblastic Tumor?

The signs and symptoms of Congenital Peribronchial Myofibroblastic Tumor may include the following:

• Presence of a well-circumscribed mass in the chest or thorax, visible through imaging studies (chest X-ray)
• Some tumors are between 5-10 cm in size; weight of the tumor may exceed 0.1 kg
• Large tumors may involve both the lungs
• The growth of the tumor mass typically obstructs the airway (bronchus) and may even completely destroy/distort it
• Lung tissues near the tumor may be involved/invaded into
• Tumors affecting the bronchus can cause obstructive symptoms including chest pain, cough, breathing difficulties, and blood in sputum

Some tumors may remain asymptomatic and present no respiratory or gastrointestinal system symptoms.

How is Congenital Peribronchial Myofibroblastic Tumor Diagnosed?

There are a variety of tests the healthcare provider may employ to diagnose Congenital Peribronchial Myofibroblastic Tumor, which may include:

• 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
• Prenatal ultrasound scan can reveal the presence of the tumor

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

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 Congenital Peribronchial Myofibroblastic Tumor?

The complications of Congenital Peribronchial Myofibroblastic Tumor may include the following:

• Severe obstruction of the airways in case of a large-sized tumor
• Pneumonia
• Severe chest pain
• Large tumors may also compress the heart and impede its functioning
• Pregnancy-related complications that may include excess amniotic fluid (polyhydramnios) and fetal hydrops

How is Congenital Peribronchial Myofibroblastic Tumor Treated?

The treatment of Congenital Peribronchial Myofibroblastic Tumor may involve the following:

• The treatment of choice is complete surgical excision, which can result in a cure
• Follow-up care with regular screening and check-ups are important and encouraged

How can Congenital Peribronchial Myofibroblastic Tumor be Prevented?

Currently, there are no known methods to prevent the development of Congenital Peribronchial Myofibroblastic Tumor.

What is the Prognosis of Congenital Peribronchial Myofibroblastic Tumor? (Outcome/Resolutions)

• The prognosis of Congenital Peribronchial Myofibroblastic Tumor is generally good with appropriate treatment. No recurrence of the tumor following surgery has been recorded
• However, the outcome may be complicated by the presence of hydrops fetalis (severe accumulation of fluid in the fetus) during pregnancy, which can be a fatal condition

Additional and Relevant Useful Information for Congenital Peribronchial Myofibroblastic Tumor:

The following article link will help you understand other lung conditions:

http://www.dovemed.com/healthy-living/healthy-lungs/