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

• Esophageal Lymphangioma
• Lymphangioma of Oesophagus
• Oesophageal Lymphangioma

What is Lymphangioma of Esophagus? (Definition/Background Information)

• Lymphangioma of Esophagus is an extremely rare benign tumor of lymphatic blood vessels. The esophagus is a part of the upper gastrointestinal tract and is also known as the ‘food-pipe’
• A lymphangioma is generally seen among a wide age range of adults and can occur at various locations in the body. But, the esophagus is a very uncommon location for the tumor (only about 1% of the lymphangiomas are seen within the GI tract)
• Esophageal Lymphangiomas may be either of the two types - cavernous (in many cases) or cystic. These are mostly observed in elderly men and women
• The cause of formation and risk factors for Lymphangioma of Esophagus are unknown. It is believed to abnormally arise during fetal growth and development
• Most small tumors are asymptomatic and painless, though the larger ones may compress the surrounding structures and cause related signs and symptoms
• Lymphangioma of Esophagus may be diagnosed by evaluating the clinical history and a thorough physical exam, followed by imaging studies, such as CT and MRI scans of the chest
• The treatment techniques for tumors causing significant symptoms may include removal through endoscopic surgery. In some cases, the healthcare provider may recommend a ‘wait and watch’ approach to monitor the tumor. The prognosis of Lymphangioma of Salivary Gland is good with appropriate treatment

Who gets Lymphangioma of Esophagus? (Age and Sex Distribution)

• Lymphangioma of Esophagus is an extremely uncommon tumor that is mostly observed in older adults. Some cases have been rarely reported in infants and children
• Both males and females are affected
• No racial or ethnic predilection is observed

What are the Risk Factors for Lymphangioma of Esophagus? (Predisposing Factors)

• Currently, no definitive risk factors are known for Lymphangioma of Esophagus
• It may occur when there is a positive family history 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 Lymphangioma of Esophagus? (Etiology)

The exact cause and mechanism of Lymphangioma of Kidney formation is unknown.

• The tumors arise from lymphatic blood vessels due to congenital malformation
• The tumor may be associated with a family history of the condition

What are the Signs and Symptoms of Lymphangioma of Esophagus?

Some small-sized Lymphangiomas of Esophagus may not cause any significant symptoms and are detected incidentally. In others, the following signs and symptoms may be noted:

• Swallowing difficulty may occur from large-sized tumors
• Tumors may be small or large masses (polyps) and occur beneath the mucosal surface
• Some appear as large pedunculated masses (a bag-like structure)
• Large masses can affect the mediastinum and surrounding structures
• They are mostly observed in the middle-third to lower-third portion of the esophagus
• Tumors have been seen in small children/infants in the upper/cervical region of esophagus

How is Lymphangioma of the Esophagus Diagnosed?

A diagnosis of Lymphangioma of Esophagus would involve:

• Physical exam and evaluation of medical history
• X-ray of the chest
• CT or MRI scan of the chest
• Upper GI endoscopy: An endoscopic procedure is performed using an instrument called an endoscope, which consists of a thin tube and a camera. Using this technique, the radiologist can have a thorough examination of the insides of the upper gastrointestinal tract
• Endoscopic ultrasonography: During this procedure, fine needle aspiration biopsy (FNAB) can be performed on the affected area. This is good technique for tumor detection
• A tissue biopsy of the tumor (polyp) is performed and sent to a laboratory for a pathological examination
  • A pathologist examines the biopsy under a microscope. If it is indeed a polyp, a distinct appearance is noted by the pathologist. 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, and very rarely, electron microscopic studies to assist in the diagnosis

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 Lymphangioma of Esophagus?

The complications of Lymphangioma from large-sized tumors may include:

• Large-sized tumors may compress adjoining tissues and structures resulting in additional complications
• Ulceration and bleeding
• Severe obstruction of the food-pipe and pain, leading to difficulties in eating
• Stricture formation of esophagus
• Damage to the muscles, vital nerves, and blood vessels, during surgery
• Post-surgical infection at the wound site is a potential complication

How is Lymphangioma of Esophagus Treated?

The treatment options vary from one individual to another. Majority of asymptomatic tumors are not surgically removed after the tumor is determined to be benign on a biopsy. The treatment measures for Lymphangioma of Esophagus may include the following:

• The healthcare provider may recommend a ‘wait and watch’ approach for small-sized tumors presenting mild signs and symptoms, after a diagnosis of Lymphangioma of Esophagus is made through a fine needle aspiration or a core biopsy
• A complete surgical resection of the tumor (endoscopic polypectomy) is usually curative. It is normally undertaken when significant symptoms are observed
• Arterial embolization of the tumor may be used to provide temporary relief from the symptoms, and reduce blood loss during ‘tumor removal’ surgical procedure
• Radiation therapy may be planned on a case-by-case basis
• Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals
• Follow-up care with regular screening and check-ups are important

How can Lymphangioma of Esophagus be Prevented?

• Current medical research has not established a method of preventing the formation of Lymphangioma of Esophagus
• Regular medical screening at periodic intervals with tests and physical examinations are strongly recommended

What is the Prognosis of Lymphangioma of Esophagus? (Outcomes/Resolutions)

• The prognosis of Lymphangioma of Esophagus is typically good with adequate treatment; a full recovery is often ensured and no long-term complications are noted
• In most cases, the prognosis of small-sized solitary tumors is excellent with surgical intervention or appropriate treatment, since these are benign

Additional and Relevant Useful Information for Lymphangioma of Esophagus:

• A lymphangioma can occur at various locations in the body, such as the soft tissues of the head/neck region, liver, and lung

The following DoveMed website links are useful resources for additional information:

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