Benign Rare Tumors Of Esophagus

Benign Tumors of the Esophagus

These are less common as compared to esophageal cancer. Tumors originating from the submucosa and muscularis propria are more common than those arising from the mucosa. Patients are asymptomatic, and it is diagnosed incidentally during endoscopy. On the barium esophagogram, they appear as a smooth defect in the lumen.

Granular Cell Tumor

These are found in the skin, respiratory tract, GIT, breast, and tongue. These tumors are derived from Schwann cells, and most commonly, they originate from mucosa and submucosa. The most common location of granular cell tumors in GIT is the distal third of the esophagus. They appear as sessile or bulging whitish-gray lesions with normal overlying mucosa. Largely benign and Multiple tumors are seen in 11% of patients 

Diagnosis

• In endoscopic ultrasound
  • Regular borders
  • They arise from the first and second sonographic layers.
• Tunneled biopsy
  • Might reveal eosinophilic granules.
  • These tumors stain positive for S-100

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Indications for Surgical Excision

Atypical features in endoscopic ultrasound.

• Large tumor size (>2cm).
• Symptomatic patient.

Treatment

Endoscopic resection is done when the diagnosis is not confirmed or malignancy has to be ruled out.

Fibrovascular Polyp

These are soft tissue tumors, most often found in the cervical esophagus (at or near the cricopharyngeus). They appear cylindrical or elongated with a stalk. Rarely symptomatic. Large tumors can cause dysphagia. If there is prolapse of the tumor in the hypopharynx, it can lead to airway obstruction. Large tumors can be resected endoscopically after securing the airways.

Squamous Papillomas

Most often, it occurs in the distal esophagus. These are associated with inflammation and human papillomavirus infection. These are colorless, exophytic lesions with wart-like projections along with a crossing vessel on the surface of the lesion.

Treatment

Complete resection of the tumor is performed to rule out malignancy. This resection can be performed endoscopically.

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Benign Submucosal Tumors

It includes

• Lipomas
• Hemangiomas
• Neural tumors

Lipoma

On endoscopic ultrasound: Homogenous, hyperechoic, smooth appearance. These are asymptomatic until and unless the tumors are large; hence, resection is seldom warranted.

Hemangioma

They appear as purple or reddish nodules. On endoscopic ultrasound: Smooth, hypoechoic submucosal mass. Most tumors are asymptomatic. If the tumor is causing dysphagia or bleeding, it should be treated endoscopically.

Neural Tumors

It includes neurofibromas and schwannomas (rarely in the esophagus). The majority of these tumors are benign. If symptomatic: Enucleation is performed. Large tumors may require esophagectomy.

Leiomyoma

It is the most common benign tumor of the esophagus. More common in males during the 4th to 5th decade. It originates in smooth muscle, particularly from muscularis propria. Most commonly, it affects the distal 2/3rd of the esophagus. It remains intramural, and the bulk of mass protrudes towards the outer wall, leading the majority of the patients to be asymptomatic.

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Clinical Features

Most commonly asymptomatic. The most common symptoms are dysphagia and pain.

Diagnosis

The investigation of choice for diagnosis is a barium swallow, which gives a smooth contour punch-out effect. On endoscopy: Round protrusion into the lumen with smooth, normal mucosa. On endoscopic ultrasound: hypoechoic with regular border arising from 4th endosonographic layer. Since there is adhesion between the tumor and the mucosa, a biopsy should not be done as it may lead to mucosal perforation at the time of enucleation.

Treatment

In patients with small asymptomatic lesions with typical appearance, only observation should be done. Treatment of choice: enucleation in symptomatic lesions. In case of larger lesions, video-assisted thoracoscopic surgery (VATS) can be done. Endoscopic resection with submucosal tunnel formation can be done in tumors up to 5.5cm.

Rare Malignant Tumors of the Esophagus

Small Cell Carcinoma of Esophagus

Accounts for only 0.6% of esophageal tumors. It is highly aggressive. Histologic appearance: Poorly differentiated neuroendocrine tumors. Patients typically present with lymph node metastasis in the advanced stage. In patients having early-stage tumors, surgical resection can be done. Systemic chemotherapy is given to improve survival. The most important prognostic factor is the stage of the tumor; thus, these patients have poor prognosis.

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Primary Melanoma of Esophagus

Accounts for only 0.1-0.2% of esophageal malignancies. Generally, patients present at a late stage, hence having poor prognosis.

Leiomyosarcoma

These are rare tumors that often erode through mucosa. On endoscopy: Ulcerated or exophytic masses. On endoscopic ultrasound: Irregular borders with heterogeneous appearance. Treatment of choice: Esophagectomy with radical lymphadenectomy

GIST: Gastrointestinal Stromal Tumors

The most common site is stomach > small intestine > colon > rectum > esophagus. The appearance of GIST in the esophagus is like leiomyoma. It is distinguished from leiomyomas.

• Histologically, it is positive for CD-117 (C-KIT) and CT-34
• Tends to be larger.
• Uptake of IV contrast on CT
• Significant PET avidity

Treatment

• Enucleation if negative margins can be achieved.
• Formal esophagectomy if there is a concerning margin status or risk of recurrence.
• Imatinib, if the size of the tumor is >3 cm, there are high-risk features and locally advanced tumors (neoadjuvant settings).

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