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Pulmonary Sclerosing Hemangioma

Last updated Dec. 23, 2018

Approved by: Maulik P. Purohit MD, MPH

Pulmonary Sclerosing Hemangioma (PSH) is a generally solitary tumor that is often observed in women, who are in their 50s.

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

  • Pneumocytoma of Lung
  • PSH (Pulmonary Sclerosing Hemangioma)
  • Sclerosing Hemangioma of Lung

What is Pulmonary Sclerosing Hemangioma? (Definition/Background Information)

  • Pulmonary Sclerosing Hemangioma (PSH) is a generally solitary tumor that is often observed in women, who are in their 50s. Hemangiomas are typically benign malformation of blood vessels that can occur in a number of tissues such as the brain, kidney, liver, skin, and lung
  • Also known as Pneumocytoma of Lung, PSH is a benign tumor of the lung, which can occur either in the lung tissue or occasionally in the pleura. The exact cause of formation of this hemangioma type is unknown; no specific risk factors have also been identified
  • Most Pulmonary Sclerosing Hemangiomas are asymptomatic, meaning that no signs and symptoms are generally present. Due to this reason, majority of them are diagnosed incidentally, while a diagnostic work-up is being performed for other medical conditions
  • The healthcare provider may undertake treatment (surgical removal of the tumor) in case it causes worrisome signs and symptoms. Even in the absence of any symptoms, a Pulmonary Sclerosing Hemangioma is removed, since some are known to metastasize
  • The prognosis of Pulmonary Sclerosing Hemangioma is typically excellent with treatment, since these are normally benign tumors

Who gets Pulmonary Sclerosing Hemangioma? (Age and Sex Distribution)

  • Pulmonary Sclerosing Hemangioma is usually observed in adults around the age of 50 years; though individuals of any age may be affected
  • The condition affects both males and females, but there is a strong preference for females (female-male ratio is 5:1)
  • This condition is observed worldwide and there is no racial or ethnic group predilection

What are the Risk Factors for Pulmonary Sclerosing Hemangioma? (Predisposing Factors)

  • Currently, no risk factors have been identified for Pulmonary Sclerosing Hemangioma

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 Pulmonary Sclerosing Hemangioma? (Etiology)

  • The cause of development of Pulmonary Sclerosing Hemangioma is generally unknown
  • Research is currently being undertaken to identify the relevant causal factors

What are the Signs and Symptoms of Pulmonary Sclerosing Hemangioma?

Pulmonary Sclerosing Hemangiomas can arise in the following lung parts:

  • The condition commonly involves the lung tissue or lung parenchyma
  • Rarely, it can involve the lung lining or pleura

A majority of Pulmonary Sclerosing Hemangiomas present no clinical indications and remain asymptomatic. The following features may be observed in PSH:

  • Pulmonary Sclerosing Hemangioma consists of blood vessels with surrounding fibrosis
  • It is a slow-growing tumor that is usually single
  • The tumor size is usually less than 3 cm, though some may grow to larger sizes
  • In some cases, the hemangioma can cause chest pain, coughing, and blood in sputum

How is Pulmonary Sclerosing Hemangioma Diagnosed?

There are a variety of tests the healthcare provider may employ to diagnose Pulmonary Sclerosing Hemangioma. These 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 scan 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 a patient, which is then examined in a laboratory by a pathologist. This procedure may be performed, although no tumor cells may be noted

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
  • 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 tissue biopsy is preferred to a fine needle aspiration biopsy, because of the uncommon nature of the tumor.

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 Sclerosing Hemangioma?

There are no significant complications typically associated with Pulmonary Sclerosing Hemangioma. However, in some cases the following may be observed:

  • A vast majority of tumors are benign; however, about 2% of the tumors metastasize to the lymph nodes
  • Large-sized tumor may cause obstruction of the airways
  • Large tumors may also compress the heart and impede its functioning
  • Severe chest pain
  • Emotional stress in the individual since the lung tumor may resemble lung cancer

How is Pulmonary Sclerosing Hemangioma Treated?

The treatment of Pulmonary Sclerosing Hemangioma may involve the following:

  • The treatment of choice is complete surgical excision, which can result in a cure
  • In a majority of cases, removal of the tumor is generally not necessary, unless it causes bothersome signs and symptoms. However, since there is a possibility of metastasis, these tumors are commonly removed
  • Follow-up care with regular screening and check-ups are important and encouraged

How can Pulmonary Sclerosing Hemangioma be Prevented?

Currently, there are no known methods to prevent the development of Pulmonary Sclerosing Hemangioma.

What is the Prognosis of Pulmonary Sclerosing Hemangioma? (Outcomes/Resolutions)

  • The prognosis for Pulmonary Sclerosing Hemangioma is generally excellent with appropriate treatment, since it is a benign tumor in most cases
  • Even with lymph node metastasis, the outcome is typically good

Additional and Relevant Useful Information for Pulmonary Sclerosing Hemangioma:

Based on the pathology examination of the tissue, there are 4 patterns namely:

  • Papillary Pulmonary Sclerosing Hemangioma
  • Sclerotic Pulmonary Sclerosing Hemangioma
  • Solid Pulmonary Sclerosing Hemangioma
  • Hemorrhagic Pulmonary Sclerosing Hemangioma

However, some tumors can have a mixed pattern of presentation.

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: March 13, 2016
Last updated: Dec. 23, 2018