Beyond the two main carcinomas, the esophagus gives rise to benign epithelial tumors such as papilloma and adenoma, and to non-epithelial tumors that range from leiomyoma to lymphoma, sarcoma and metastatic disease.
Benign epithelial tumors
Esophageal papilloma is an asymptomatic, benign epithelial tumor. Endoscopically it appears as a solitary exophytic lesion in the distal third of the esophagus, white to pink with a soft to slightly rough surface, and histologically it shows finger-like projections with an increased number of squamous cells. Its exact pathophysiology is not determined; it is hypothesized to arise from either inflammation of the esophagus or HPV infection.
Adenoma of the esophagus is a very rare tumor and is almost exclusively associated with Barrett’s esophagus. Barrett’s mucosa mostly transforms into adenocarcinoma, and only in very rare cases into adenoma.
Inflammatory fibroid polyp is characterized histologically by submucosal-based polypoid lesions, perivascular concentric fibroblastic proliferation, and infiltration of eosinophils.
Malignant non-epithelial tumors
Lymphoma of the esophagus accounts for less than 1% of all esophageal malignancies, and it can be either Hodgkin or non-Hodgkin lymphoma. Diffuse large B-cell lymphoma (DLBCL) is the most common non-Hodgkin subtype. Lymphoma appears in the esophagus in two forms: primary lymphoma, the far less common form, arises from the esophagus without involvement of the mediastinum, spleen or liver, with a normal chest radiograph and a normal white blood cell (WBC) count; secondary lymphoma is more common.
Sarcoma of the esophagus arises from the mesenchymal cells, and its most common form is leiomyosarcoma. Most esophageal sarcomas are submucosal, so biopsy of the mucosa is not diagnostic and the main diagnostic tool is endoscopic ultrasound (EUS) with fine-needle aspiration (FNA). Sarcomas generally have a poor prognosis, with a 5-year survival of 35%.
Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the GI tract, though not of the esophagus, and 1–3% of all GIST cases occur in the esophagus. GIST typically arises from the muscularis propria. EUS is the most accurate modality for diagnosis and can help predict malignant potential, which is lowest when the tumor is less than 2 cm and its mitotic count is less than 5 in 50 high-power fields (HPF).
Metastasis to the esophagus is very rare; the two most common cancers that metastasise there are melanoma and breast cancer.
Benign non-epithelial tumors
Leiomyoma is the most common benign non-epithelial tumor of the esophagus. It arises from the muscularis propria and is therefore located in the submucosa. Endoscopic biopsy is not diagnostic and EUS is needed. Only masses that are symptomatic and larger than 5 cm should be resected surgically.
Granular cell tumors are mostly located in the head and neck, especially the oropharynx, and are less common in the GI tract; within the GI tract their most common location is the esophagus.
Fibrovascular polyps are lesions of the cervical portion of the esophagus, mostly pedunculated and containing vessels and adipose tissue. Because of the vessels, it is recommended to check them with EUS before resection. They can reach a large size and cause dysphagia and a globus sensation.
Hamartomas look endoscopically very similar to fibrovascular polyps and are only distinguishable by histological study; resection is recommended.
Hemangioma may also occur.
Lipomas are rare benign tumors of the GI tract, mostly located in the colon, small intestine or stomach rather than the esophagus. They are usually asymptomatic and found incidentally during endoscopy as yellowish nodules, but a large lipoma can become symptomatic. They are positive for the Pillow sign, the indentation of the lipoma produced by touch.