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

• Primary Malignant Lung Teratoma
• Primary Malignant Pulmonary Teratoma
• Pulmonary Germ Cell Tumor - Primary Malignant Teratoma

What is Primary Malignant Teratoma of Lung? (Definition/Background Information)

• A teratoma of lung is the most common type of germ cell tumor of the lung. However, the lung is a very uncommon site for such a tumor, and hence, the origin of the teratoma from other common sites (such as gonads) should be eliminated prior to establishing a diagnosis of primary teratoma of lung
• There are 2 main forms of pulmonary teratomas:
  • Mature teratoma of lung
  • Immature teratoma of lung
• Teratoma of lung may be cancerous or benign. A majority of benign teratomas are mature teratomas, while a majority of malignant teratomas are immature teratomas
• Teratoma of lung is typically seen in childhood and young adulthood. The cause of formation of Primary Malignant Teratoma of Lung is unknown. Also, presently the risk factors are not well-established
• The tumors may grow to large sizes and obstruct the lung airways. The presenting signs and symptoms of Primary Malignant Teratoma of Lung may include shortness of breath, chest pain, and blood in sputum
• The treatment of Primary Malignant Teratoma of Lung involves surgery. They may additionally be treated using chemotherapy and/or radiation therapy
• The prognosis of Primary Malignant Lung Teratoma depends upon various factors and is usually poor, in a majority of cases

Who gets Primary Malignant Teratoma of Lung? (Age and Sex Distribution)

The specific demographics on Primary Malignant Teratoma of Lung are unavailable. However, the following may be noted:

• Teratoma of lung is a rare type of tumor that is mostly observed in older children and young adults; the peak age range is between 10-40 years
• However, infants, very young children, and older adults may also be affected. The overall age range is between 10 months to 68 years
• Both males and females are affected, although a slight preference for females is observed
• No specific racial or ethnic group predilection is noted

What are the Risk Factors for Primary Malignant Teratoma of Lung? (Predisposing Factors)

• Currently, no definite risk factors have been identified for Primary Malignant Teratoma of Lung

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 Primary Malignant Teratoma of Lung? (Etiology)

The cause of development of Primary Malignant Teratoma of Lung is generally unknown.

• It is believed that abnormal differentiation of germ cells gives rise to cancerous cells that lead to the formation of this condition
• The tumor is thought to arise from abnormally-placed pharyngeal tissues (from the pharyngeal pouch or Zenker’s diverticulum); pharyngeal pouches are known to form during embryological development

What are the Signs and Symptoms of Primary Malignant Teratoma of Lung?

The signs and symptoms of Primary Malignant Teratoma of Lung may include the following:

• Presence of a well-circumscribed lesion in the upper lobes of the lung; the cystic tumors may involve the airways/bronchus
• Immature teratomas are mostly solid and are known to be locally invasive
• The tumors may range in size from 3 to 30 cm
• The growth of the tumor mass may obstruct the airway
• Tumors affecting the bronchus can cause obstructive symptoms including chest pain, cough, breathing difficulties, and blood in sputum
• Trichoptysis: It is the most unusual and ‘specific’ sign of a teratoma, where the individual coughs-up hair or hair-like particles. This may occur from pulmonary teratomas or mediastinal teratomas (that has invaded the lung)
• When metastasis is noted, it may result in weight loss, fatigue, and other systemic symptoms

How is Primary Malignant Teratoma of Lung Diagnosed?

There are a variety of tests the healthcare provider may employ to diagnose Primary Malignant Teratoma of Lung, 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
• 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

Tissue biopsy from the affected lung:

• A biopsy of the tumor is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
• Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
• Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, flow cytometric analysis and very rarely, electron microscopic studies, to assist in the diagnosis

A differential diagnosis with respect to other lung cancer types may be necessary prior to establishing a definite diagnosis, by excluding the following cancers:

• Teratoma that has spread from other body sites
• Other pulmonary germ cell tumors
• Pulmonary hamartoma
• Glial heteropia affecting the lung (observed in children with neural tube defects)

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 Primary Malignant Teratoma of Lung?

The complications of Primary Malignant Teratoma of Lung may include the following:

• Some immature teratomas are known to rupture and cause associated complications including bronchial or bronchopleural fistulas, lung inflammation, and fibrosis
• Severe obstruction of the airways in case of a large-sized tumor
• Metastasis of the malignant teratoma to distant sites
• Recurrence of the tumor following its incomplete surgical removal. It is generally difficult to surgically remove the tumors completely. And thus, they are more likely to reappear after treatment

There may be complications related to the methods used in treating the condition and may include:

• Side effects of radiation therapy that may include sunburn-like rashes, where radiation was targeted, red or dry skin, heaviness of the breasts, and general fatigue
• Side effects of chemotherapy, which may include nausea, vomiting, hair loss, decreased appetite, mouth sores, fatigue, low blood cell counts, and a higher chance of developing infections
• The treatment can also cause infertility in men and women. Hence, measures to protect the individual’s fertility must be considered, before starting chemotherapy

How is Primary Malignant Teratoma of Lung Treated?

The treatment of Primary Malignant Teratoma of Lung may involve the following:

• Generally, the treatment of choice is complete surgical excision for all teratomas (mature or immature)
• However, while it is usually possible to surgically remove a mature teratoma, the complete removal of an immature teratoma may be difficult
• Additionally, chemotherapy and/or radiation therapy may be necessary
• Follow-up care with regular screening and check-ups are important and encouraged

How can Primary Malignant Teratoma of Lung be Prevented?

Currently, there are no known methods to prevent the development of Primary Malignant Teratoma of Lung.

What is the Prognosis of Primary Malignant Teratoma of Lung? (Outcomes/Resolutions)

• The prognosis of Primary Malignant Teratoma of Lung depends upon the stage of the tumor, severity of signs and symptoms, and whether it is a mature or immature type of teratoma
• If the malignant tumors can be completely removed through surgery, then the prognosis is generally improved
• Immature teratomas are aggressive, difficult to treat (complete tumor resection may not be possible), and can spread to other sites. The prognosis of these tumors is usually poor; case studies indicate that the average survival following diagnosis in such cases is about 6 months
• The prognosis of lung cancer, in general, depends upon a set of several factors that include:
  • The grade of the lung tumor such as grade1, grade2, and grade 3. Grade1 indicates a well-differentiated tumor, grade 2 a moderately-differentiated tumor, whereas grade 3 indicates a poorly-differentiated tumor. Grade 3 tumors are higher prone to metastasis to the lymph nodes and recurrence
  • Stage of lung cancer: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
  • Histological subtype of the tumor: Some subtypes have better prognosis than others
  • The size of the lung tumor: Individuals with small-sized tumors fare better than those with large-sized tumors
  • Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
  • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
  • Individuals with bulky disease of the lung cancer have a poorer prognosis
  • Involvement of the lymph node, which can adversely affect the prognosis
  • Involvement of vital organs may complicate the condition
  • The surgical respectability of the tumor (meaning, if the tumor can be removed completely)
  • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
  • Response to treatment of lung cancer: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
  • Progression of the condition makes the outcome worse
• An early diagnosis and prompt treatment of the tumor generally yields better outcomes than a late diagnosis and delayed treatment
• The combination chemotherapy drugs used, may have some severe side effects (like cardio-toxicity). This chiefly impacts the elderly adults, or those who are already affected by other medical conditions. Tolerance to the chemotherapy sessions is a positive influencing factor

It is important to have follow-up appointments with a physician, to evaluate the effects of the current treatment method, and to monitor for any returning tumors.

Additional and Relevant Useful Information for Primary Malignant Teratoma of Lung:

The following article link will help you understand other cancers and benign tumors:

http://www.dovemed.com/diseases-conditions/cancer/