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

• Esophageal Granular Cell Tumor
• GCT of Esophagus
• Oesophageal Granular Cell Tumor

What is Granular Cell Tumor of Esophagus? (Definition/Background Information)

• Granular cell tumor (GCT) is a common and mostly benign tumor of the soft tissue, which can occur anywhere in the body. Some researchers note that granular cell tumor may be a tumor of neural origin (relating to a nerve). Occasionally, cases of malignant GCTs are noted
• Granular Cell Tumor of Esophagus is an extremely rare tumor affecting the esophagus. The esophagus is a part of the upper gastrointestinal tract and is also known as the ‘food-pipe’
• The cause of formation and risk factors for Granular Cell Tumor of Esophagus are unknown. It is researched that the tumor may be due to certain genetic defects
• Most tumors are small, asymptomatic and painless, though the larger ones may compress the surrounding structures and cause related signs and symptoms, such as swallowing difficulties
• Esophageal Granular Cell Tumor 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. The prognosis of Granular Cell Tumor of Esophagus is good with appropriate treatment. However, in some rare cases, a malignant transformation of the tumor from a benign GCT has been documented

Who gets Granular Cell Tumor of Esophagus? (Age and Sex Distribution)

• Granular Cell Tumor of Esophagus is mostly observed in middle-aged and older adults
• Both males and females are affected
• No racial or ethnic predilection is observed; although, a higher number of cases are recorded in African Americans

It is reported that between 6-10% of the granular cell tumors are seen in the gastrointestinal tract.

What are the Risk Factors for Granular Cell Tumor of Esophagus? (Predisposing Factors)

• Currently, no definitive risk factors are known for Granular Cell Tumor of Esophagus
• In rare cases, granular cell tumors are known to run in families

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 Granular Cell Tumor of Esophagus? (Etiology)

Currently, there are no identified causes for the development of Granular Cell Tumor of Esophagus.

• Researchers have documented certain genetic changes within the tumor. However, cases where these specific genetic mutations have been observed are rare. Thus, studies regarding genetic changes are limited
• GCT is not associated with any known congenital syndrome

What are the Signs and Symptoms of Granular Cell Tumor of Esophagus?

The following signs and symptoms of Granular Cell Tumors of Esophagus may be noted:

• Small-sized tumors are typically less than 10 mm at detection; in such cases, the detection is usually incidental
• Most small-sized tumors are asymptomatic
• Larger tumor sizes are rare; swallowing difficulty may occur from large-sized tumors
• In about 10% of the cases, more than one tumor may be present
• Some tumors are noted to grow along the surface of the esophagus

How is Granular Cell Tumor of Esophagus Diagnosed?

A diagnosis of Granular Cell Tumor 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 Granular Cell Tumor of Esophagus?

The complications of Granular Cell Tumor of Esophagus are normally rare, but in some cases, large tumors may cause the following complications:

• Large-sized tumors may compress adjoining tissues and structures resulting in additional complications
• Obstruction of the food-pipe and pain, leading to difficulties in eating
• Stricture formation of esophagus
• Transformation of the benign GCT to malignant GCT is a rare possibility. Malignant tumors may metastasize to local and distant regions
• Damage to the muscles, vital nerves, and blood vessels, during surgery
• Post-surgical infection at the wound site is a potential complication

How is Granular Cell Tumor of Esophagus Treated?

The treatment of Granular Cell Tumor of Esophagus may involve the following measures:

• A complete surgical resection of the tumor (endoscopic polypectomy) is usually curative
• Sometimes, it is difficult to distinguish between a benign GCT and a malignant GCT. Hence, a complete removal of the tumor is usually recommended
• 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 Granular Cell Tumor of Esophagus be Prevented?

• Current medical research has not established a method of preventing the formation of Granular Cell Tumor of Esophagus
• Regular medical screening at periodic intervals with blood tests, radiological scans, and physical examinations are recommended

What is the Prognosis of Granular Cell Tumor of Esophagus? (Outcomes/Resolutions)

• The prognosis of Granular Cell Tumor of Esophagus is typically good with adequate treatment; a full recovery is often ensured and no long-term complications are noted
• In the rare case of a malignant transformation, the prognosis depends upon several factors such as stage of the tumor and overall health of the patient

Additional and Relevant Useful Information for Granular Cell Tumor of Esophagus:

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

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