Sections

Medication

Medication Summary

Most of the chemotherapy agents currently used for the treatment of esophageal cancer have not been approved by the US Food and Drug Administration (FDA) for this specific indication. Drug classes used in these regimens include alkylating, antimetabolite, anthracycline, and antimicrotubular agents. However, several biologic agents, such as programmed death ligand 1 (PD-L1) inhibitors have received FDA approval for use in esophageal cancer, and trastuzumab has been approved for use in esophagogastric junction adenocarcinoma that overexpresses HER2.

Class Summary

These agents inhibit cell growth and proliferation. They interfere with DNA synthesis by blocking the methylation of deoxyuridylic acid.

Fluorouracil (Adrucil)

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Fluorouracil is a pyrimidine antimetabolite. Several mechanisms of action have been proposed, including inhibition of thymidylate synthase and inhibition of RNA synthesis. This agent is also a potent radiosensitizer.

Capecitabine (Xeloda)

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Capecitabine is a pyrimidine antimetabolite and a prodrug of fluorouracil. It forms the active moiety, fluorouracil, by undergoing hydrolysis in the liver and tissues. Capecitabine is used for the treatment of esophageal cancer, which is an off-label indication.

Class Summary

These agents inhibit cell growth and proliferation, interfering with DNA synthesis by the formation of DNA cross-links. Alkylating agents can have serious adverse effects such as bone marrow suppression, anaphylactic-like reactions, ototoxicity, renal toxicity, and vomiting.

Cisplatin

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Intrastrand cross-linking of DNA and inhibition of DNA precursors are among the proposed mechanisms of action for cisplatin. This agent is used in combination with radiation therapy. Cisplatin has black box warnings for adverse reactions, including anaphylactic-like reactions, ototoxicity, and renal toxicity.

Carboplatin

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Carboplatin is a platinum alkylating agent that interferes with the function of DNA by producing interstrand DNA cross-links. It can be used in combination with paclitaxel for the treatment of esophageal cancer, which is an off-label indication. Carboplatin has black box warnings including bone marrow suppression, anaphylactic reactions, and vomiting.

Oxaliplatin (Eloxatin)

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Oxaliplatin is a platinum alkylating agent that inhibits DNA replication and transcription, resulting in cell death. It can be used in combination chemotherapy for the treatment of esophageal cancer, which is an off-label indication. It has a black box warning for anaphylactic reactions, which can be managed with epinephrine, corticosteroids, and antihistamines.

Class Summary

PD-1 and related target PD-ligand 1 (PD-L1) are expressed on the surface of activated T cells under normal conditions. PD-L1/PD-1 interaction inhibits immune activation and reduces T-cell cytotoxic activity when bound.

Nivolumab (Opdivo)

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Pembrolizumab (Keytruda)

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Indicated in combination with fluoropyrimidine- and platinum-containing chemotherapy as first-line treatment for advanced or metastatic gastric cancer, gastroesophageal junction cancer, or esophageal adenocarcinoma that is not amenable to surgical resection or definitive chemoradiation. Also indicated as a single agent for recurrent, locally advanced or metastatic, squamous cell carcinoma of the esophagus (ESCC) whose tumors express programmed death ligand 1 (PD-L1) with a Combined Positive Score (CPS) 10 or greater, as determined by an FDA-approved test, with disease progression after 1 or more prior lines of systemic therapy.

Class Summary

These agents prevent cell growth and proliferation. They work by enhancing tubulin dimers, stabilizing existing microtubules, and inhibiting microtubule disassembly.

Docetaxel (Taxotere, Docefrez)

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Docetaxel inhibits the depolymerization of tubulin, which inhibits DNA, RNA, and protein synthesis. It can be used in combination with cisplatin and fluorouracil for the treatment of esophageal cancer, which is an off-label indication. It has several black box warnings such as bone marrow suppression, fluid retention, and hypersensitivity reactions.

Use of docetaxel is not recommended in certain patients with hepatic impairment. Patients receiving treatment with docetaxel should be premedicated with corticosteroids the day before administration to help reduce fluid retention and hypersensitivity reactions.

Paclitaxel

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Paclitaxel promotes microtubule assembly, interferes with the G2 mitotic phase, and inhibits cell replication. Although not FDA approved, it has been used in combination chemotherapy for the treatment of esophageal cancer. Paclitaxel has an off-label indication for the treatment of adenocarcinoma. Black box warnings for this drug include bone marrow suppression and hypersensitivity reactions.

Class Summary

Anthracycline antineoplastics inhibit DNA and RNA synthesis by steric obstruction. They intercalate between DNA base pairs and trigger DNA cleavage by topoisomerase II.

Epirubicin (Ellence)

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Epirubicin inhibits DNA and RNA synthesis. It can be used off label as part of a combination chemotherapy regimen for the treatment of esophageal cancer. It has several black box warnings, including bone marrow suppression, extravasation, myocardial toxicity, and secondary malignancy. Dosage reduction is recommended in patients with mild to moderate hepatic impairment.

Class Summary

These agents prevent cell growth and proliferation. They work by binding to topoisomerase and causing single-strand DNA breaks.

Irinotecan (Camptosar)

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Irinotecan binds reversibly to the topoisomerase I–DNA complex and prevents the ligation of the cleaved DNA strand. It can be used as part of combination chemotherapy for the treatment of esophageal cancer, which is an off-label indication. Black box warnings for irinotecan include bone marrow suppression and diarrhea.

Class Summary

Following uptake into cancer cells, trifluridine is incorporated into DNA, interferes with DNA synthesis and inhibits cell proliferation.

Tipiracil/trifluridine (Lonsurf)

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Tipiracil is a thymidine phosphorylase inhibitor that increases trifluridine exposure by inhibiting its metabolism. Trifluridine is a thymidine-based nucleoside analog that incorporates into DNA, interferes with DNA synthesis, and inhibits cell proliferation. It is indicated for metastatic gastric or gastroesophageal junction (GEJ) adenocarcinoma previously treated with at least 2 prior lines of chemotherapy that included a fluoropyrimidine, a platinum, either a taxane or irinotecan, and if appropriate, HER2/neu-targeted therapy.

Class Summary

This category includes miscellaneous antineoplastic agents that cause cytotoxic activity by various mechanisms of action.

Porfimer (Photofrin)

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Porfimer is a photodynamic therapy that causes cytotoxic activity by producing oxygen free-radicals in the presence of laser light. It also can release thromboxane A2, leading to necrosis and vascular occlusion. It is indicated for palliation in patients with partially or completely obstructing esophageal cancer.

Trastuzumab (Herceptin, Herzuma, Kanjinti)

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Monoclonal antibody, inhibits growth of tumor cells that overexpress HER2

Questions

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Media Gallery

Esophageal cancer. Endoscopy demonstrating intraluminal esophageal cancer.
Esophageal cancer. Cascade of events that lead from gastroesophageal reflux disease to adenocarcinoma.
Esophageal cancer. Barium swallow demonstrating stricture due to cancer.
Esophageal cancer. Barium swallow demonstrating an endoluminal mass in the mid esophagus.
Esophageal cancer. Chest CT scan showing invasion of the trachea by esophageal cancer.
Esophageal cancer. Transhiatal esophagectomy in which (a) is the abdominal incision, (b) is the cervical incision, and (c) is the stomach stretching from abdomen to the neck.
Esophageal cancer. Five-year survival for esophageal cancer based on TNM stage.
Esophageal cancer. Hematoxylin and eosin stain, high power, showing junction of benign glands in the lower right, Barrett columnar cell metaplasia with a large goblet cell containing blue mucin in the lower center, and adenocarcinoma on the left.
Esophageal cancer. Macroscopic image of a resection of the gastroesophageal junction. On the right is non-neoplastic esophagus, consisting of tan, smooth mucosa. On the left is the non-neoplastic rugal folds of the stomach. In the center of the picture is an ulcer with a yellow-green fibrinous exudate surrounded by irregular, heaped-up margins with almost a cobblestone appearance. The latter represents mucosal adenocarcinoma with probably some Barrett metaplasia in the background.
Esophageal cancer. Hematoxylin and eosin stain, high power, demonstrating invasive esophageal squamous cell carcinoma. This carcinoma does not form glands and instead shows features of squamous differentiation, including keratinization and intercellular bridges.
Esophageal cancer. Macroscopic image of an esophageal resection. A polypoid squamous cell carcinoma is visible protruding from the esophageal mucosal surface (left center of specimen).
Esophageal cancer. On this positron emission computed tomography (PET) scan, esophageal cancer is evident as a golden lesion in the chest.
Palmoplantar keratoderma (tylosis) of palms (A) and soles (B). Courtesy of The American Journal of Gastroenterology, Nature Publishing Group.
Esophageal cancer. Diagram showing T1,T2 and T3 stages of esophageal cancer. Courtesy of Cancer Research UK and Wikimedia Commons.
Esophageal cancer. Micrograph of squamous cell carcinoma of the esophagus (H&E stain). Courtesy of Wikimedia Commons.
Esophageal cancer. Low-magnification micrograph of an intramucosal esophageal adenocarcinoma (H&E stain). Endoscopic mucosal resection specimen. Courtesy of Wikimedia Commons.

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Table 2. Five-year esophageal cancer survival rates by stage at diagnosis in the US, 2012-2018 ^[60]

Stage	Survival Rate (%)
Localized	47.3
Regional	26.3
Distant	5.7
All Stages	20.6

Table 3. Clinical Staging Classification (Squamous Cell Carcinoma)

Stage 0	Tis	N0	M0
Stage I	T1	N0-1	M0
Stage II	T2	N0	M0
	T3	N0	M0
Stage III	T3	N1	M0
	T1-3	N2	M0
Stage IVA	T4	N0-2	M0
	Any T	N3	M0
Stage IVB	Any T	Any N	M1

Table 4. Clinical Staging Classification (Adenocarcinoma)

Stage 0	Tis	N0	M0
Stage I	T1	N0	M0
Stage IIA	T1	N1	M0
Stage IIB	T2	N0	M0
Stage III	T2	N1	M0
	T3	N0-1	M0
	T4a	N0-1	M0
Stage IVA	T1-4a	N2	M0
	T4b	N0-2	M0
	Any T	N3	M0
Stage IVB	Any T	Any N	M1

Table 5. Hereditary Cancer Predisposition Syndromes Associated with an Increased Risk for Esophageal Cancers.

Syndrome	Gene(s)	Inheritance Pattern	Clinical Manifestation	Surveillance Recommendations
Tylosis (non-Epidermolytic palmoplantar keratosis (PPK) or Howel Evans syndrome)	RHBDF2	Autosomal dominant	Skin thickening of palms and soles. Non-epidermolytic PPK is associated with high risk of developing SCC of esophagus (40-90% by age of 70 years)^{[45, 42]}	Surveillance by upper GI endoscopy is recommended for family members with tylosis after 20 years of age^[45]
Familial Barrett esophagus (FBE)	Genes not identified	Autosomal dominant	Familial aggregation of Barrett esophagus, adenocarcinoma of the esophagus and EGJ^{[133, 134]}	Potential family history of Barrett esophagus, and adenocarcinoma of the esophagus,should be determined for patients presenting with GERD, especially white men older than 40 years of age.
Bloom syndrome	BLM/RECQL3	Autosomal recessive	Increased predisposition to a wide variety of malignancies^[135]. Acute myeloid leukemia, acute lymphoblastic leukemia, lymphoid neoplasms, and Wilms tumor are the predominant cancers diagnosed before 25 years of age, whereas carcinomas of different anatomic sites including SCC of the esophagus are diagnosed after 20 years of age. Chromosomal quadraradials with breakage may be used for the diagnosis of BS^[45]	Screening for GERD with or without endoscopy after 20 years of age may be considered to detect cancer early.
Fanconi anemia (FA)	FANC^[136]	Autosomal recessive	Characterized by congenital malformations, progressive pancytopenia, easy bruising, chromosomal breakage and an increased predisposition to the development of hematologic malignancies as well as solid tumors^[45]. AML is the most common cancer type in patients with FA. However, patients with FA are also at an increased risk of developing SCC of head, neck and esophagus, cervical cancer, and brain tumors.^{[45, 137, 138]}Enhanced mitomycin C–induced chromosomal breakage analysis can help in diagnosis.^[139]	Endoscopy of the esophagus may be considered as a surveillance strategy in individuals identified with FA.

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Esophageal Cancer Medication

Medication Summary

Antineoplastics, Antimetabolite

Class Summary

Fluorouracil (Adrucil)

Capecitabine (Xeloda)

Antineoplastics, Alkylating

Class Summary

Cisplatin

Carboplatin

Oxaliplatin (Eloxatin)

PD-1/PD-L1 Inhibitors

Class Summary

Nivolumab (Opdivo)

Pembrolizumab (Keytruda)

Antineoplastics, Antimicrotubular

Class Summary

Docetaxel (Taxotere, Docefrez)

Paclitaxel

Antineoplastics, Anthracycline

Class Summary

Epirubicin (Ellence)

Antineoplastics, Topoisomerase Inhibitors

Class Summary

Irinotecan (Camptosar)

Thymidine Analog

Class Summary

Tipiracil/trifluridine (Lonsurf)

Antineoplastics, Other

Class Summary

Porfimer (Photofrin)

Antineoplastics, Anti-HER2

Trastuzumab (Herceptin, Herzuma, Kanjinti)

References

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