eMedicine Specialties > Gastroenterology > Esophagus
Esophageal Lymphoma: Treatment & Medication
Updated: Jun 13, 2007
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
Data vary regarding therapy and prognosis in primary esophageal lymphoma. Choice of therapy depends on the histologic grade of the tumor and the extent of esophageal involvement. The initial therapy for primary esophageal lymphoma has included chemotherapy, surgical resection, and radiotherapy. Some authors prefer combined therapy, with local resection plus chemotherapy and/or radiotherapy as the initial therapy. Others prefer chemotherapy alone as the therapeutic modality of choice. Recent studies have indicated that nonsurgical approaches have had equivalent outcomes to that of surgical strategies in patients with GI lymphoma.
- Chemotherapy
- The chemotherapy regimen most commonly used is a combination of cyclophosphamide, prednisone, doxorubicin, and vincristine (also referred to as CHOP). Other chemotherapy regimens have been used, but none have demonstrated greater efficacy than CHOP. Recent data indicate that the addition of rituximab, a chimeric anti-CD20 IgG1 monoclonal antibody approved by the US Food and Drug Administration (FDA) for use in various B-cell lymphoma subtypes, results in higher response rates and improved survival rates, although the experience is limited in primary GI lymphoma.
- The regimen can be modified, depending on the patient's performance status and other comorbid conditions.
- Radiotherapy
- Radiotherapy can be used as a single modality, but it is most often used in conjunction with chemotherapy.
- Risks of radiotherapy include development of esophagotracheal or esophago-aortic fistulae.
Surgical Care
- Surgical resection is reportedly curative in some cases of primary GI lymphoma, though surgery is primarily used for diagnosis and treatment of complications.
- The role of surgery greatly depends on the stage of disease at the time of diagnosis and on the underlying medical condition of the patient.
Medication
The primary medical therapy for esophageal lymphoma is chemotherapy, with the most common regimen consisting of cyclophosphamide, prednisone, doxorubicin, and vincristine. Cyclophosphamide, doxorubicin, and vincristine are administered on day 1 of a 5-day cycle. Prednisone is administered on days 1-5 of the 5-day cycle. Cycles are repeated every 21-28 days for a total of 6 or more cycles. Rituximab may be added as an adjunct therapy.
Antineoplastics
Inhibit cell growth and proliferation.
Cyclophosphamide (Cytoxan)
Antineoplastic activity is mediated by its 2 active metabolites. These metabolites are alkylating agents that prevent cell division by cross-linking DNA strands. Absorbed almost completely from the GI tract, making it bioavailable in either PO or IV forms. Excretion is primarily via urine. Because of the rarity of esophageal lymphoma, determine dose on a case-by-case basis.
Adult
2-3 mg/kg/d PO/IV (average dose used in susceptible neoplasms); alternatively, 30 mg/kg as a single dose has been effective in lymphomas
Pediatric
Not established
Allopurinol may increase risk of bleeding or infection and enhance myelosuppressive effects; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones; chloramphenicol may increase half-life while decreasing metabolite concentrations; may increase effect of anticoagulants; coadministration with high doses of phenobarbital may increase rate of metabolism and leukopenic activity; thiazide diuretics may prolong cyclophosphamide-induced leukopenia and neuromuscular blockade by inhibiting cholinesterase activity
Documented hypersensitivity; severely depressed bone marrow function
Pregnancy
D - Unsafe in pregnancy
Precautions
Monitor for hematopoietic suppression (regularly examine hematologic profile, particularly neutrophils and platelets); regularly examine urine for RBCs, which may precede hemorrhagic cystitis; adverse reactions include cardiac toxicity, nausea and vomiting, hemorrhagic cystitis, leukopenia, thrombocytopenia, and alopecia
Doxorubicin (Adriamycin)
Anthracycline antibiotic that can intercalate with DNA, affecting many of the functions of DNA, including synthesis. Administered intravenously. Distributes widely into bodily tissues, including heart, kidneys, lungs, liver, and spleen. Does not cross the blood-brain barrier and is excreted primarily in bile.
Adult
Variable; maximum cumulative dose is 550 mg/m2; in patients who have received chest irradiation, maximum cumulative dose is 450 mg/m2
Pediatric
Not established
Increased toxicity with cyclophosphamide, cyclosporine, mercaptopurine, verapamil, streptozocin, paclitaxel, progesterone; phenobarbital decreases effect; decreased toxicity with digoxin; phenytoin levels are decreased
Documented hypersensitivity; severe CHF; cardiomyopathy; preexisting myelosuppression; impaired cardiac function; complete cumulative doses of daunorubicin, doxorubicin, idarubicin
Pregnancy
D - Unsafe in pregnancy
Precautions
May produce severe local toxicity in irradiated tissues even when the 2 therapies are not administered concomitantly; caution in patients who have received radiotherapy; cardiomyopathy is a well-known characteristic of doxorubicin, monitor for drug-induced cardiomyopathy, mortality rate is >50% once cardiomyopathy has developed
Vincristine (Oncovin)
Vinca alkaloid that is cell cycle–specific (M phase). The mitotic apparatus is arrested in metaphase via disruption of the microtubules. Absorption via the GI tract is variable; therefore, administer intravenously. Metabolized extensively in the liver and excreted primarily via bile. Neurotoxicity is the limiting factor during therapy. Peripheral neuropathy is most common adverse effect at usual doses.
Adult
1.4 mg/m2 IV
Pediatric
Not established
Acute pulmonary reaction may occur when taken concurrently with mitomycin-C; increases the therapeutic effect of methotrexate; decreases digoxin levels; may decrease plasma phenytoin levels
Documented hypersensitivity; demyelinating form of Charcot-Marie-Tooth disease
Pregnancy
C - Safety for use during pregnancy has not been established.
Precautions
Caution in patients diagnosed with severe cardiopulmonary or hepatic impairment and patients with preexisting neuromuscular disease; necrosis may result from extravasation, monitor closely; monitor for neurotoxicity; dose reduction may decrease neurotoxic effects
Corticosteroids
Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
Prednisone (Deltasone)
Glucocorticoid that acts as an immunosuppressant by stimulating the synthesis of enzymes needed to decrease inflammatory response. Also acts as an anti-inflammatory agent by inhibiting the recruitment of leukocytes and monocyte-macrophages into affected areas via inhibition of chemotactic factors and factors that increase capillary permeability. Readily absorbed via the GI tract and is metabolized in the liver. Inactive metabolites of prednisone are excreted via the kidneys. Most of the adverse effects of corticosteroids are dose or duration dependent.
Adult
100 mg PO qd
Pediatric
Not established
Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective-tissue infections; fungal or tubercular skin infections; GI disease
Pregnancy
C - Safety for use during pregnancy has not been established.
Precautions
Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use
Monoclonal antibodies
Antibodies targeted to specific antigenic determinants. Can be specific to growth factors, cytokines, and cell surface molecules found on tumor cells.
Rituximab (Rituxan)
The rituximab antibody is genetically engineered chimeric mouse/human monoclonal antibody directed against the CD20 antigen found on the surface of normal and neoplastic B lymphocytes. The most common adverse reactions to rituximab were infusion reactions, some of which were fatal. Bowel perforation has been reported with rituximab. Patients reporting abdominal pain during therapy should be evaluated for perforation of the intestinal tract. Reactivation of hepatitis B has been demonstrated. Patients at high risk for hepatitis B should be screened prior to initiation of therapy. No studies have been conducted to determine if dose adjustment is necessary in patients with hepatic or renal dysfunction.
Adult
375 mg/m2 IV qwk for 4-8 wk
Pediatric
Not established
Coadministration with cisplatin is known to cause severe renal toxicity, including acute renal failure; may interfere with immune response to live virus vaccine (MMR) and reduce efficacy (do not administer within 3 mo of vaccine)
Documented hypersensitivity; IgE mediated reaction to murine proteins
Pregnancy
C - Safety for use during pregnancy has not been established.
Precautions
Caution in patients with dormant infections, such as hepatitis B, hepatitis C, or CMV, because of risk of reactivation; hypotension, bronchospasm, and angioedema may occur; premedication with acetaminophen and diphenhydramine may decrease incidence; discontinue treatment if life-threatening cardiac arrhythmias occur; must administer by slow IV infusion; do not administer IV push or bolus
More on Esophageal Lymphoma |
| Overview: Esophageal Lymphoma |
| Differential Diagnoses & Workup: Esophageal Lymphoma |
 Treatment & Medication: Esophageal Lymphoma |
| Follow-up: Esophageal Lymphoma |
| References |
| « Previous Page | Next Page » |
References
Dawson IMP, Cornes JS, Morson BC. Primary malignant lymphoma of the intestinal tract. British Journal of Surgery. 1961;49:80-9.
Gupta NM, Goenka MK, Jindal A, Behera A, Vaiphei K. Primary lymphoma of the esophagus. J Clin Gastroenterol. Oct 1996;23(3):203-6. [Medline].
Agha FP, Schnitzer B. Esophageal involvement in lymphoma. Am J Gastroenterol. Jun 1985;80(6):412-6. [Medline].
Bernal A, del Junco GW. Endoscopic and pathologic features of esophageal lymphoma: a report of four cases in patients with acquired immune deficiency syndrome. Gastrointest Endosc. Apr 1986;32(2):96-9. [Medline].
Bolondi L, De Giorgio R, Santi V, Paparo GF, Pileri S, Di Febo G, et al. Primary non-Hodgkin's T-cell lymphoma of the esophagus. A case with peculiar endoscopic ultrasonographic pattern. Dig Dis Sci. Nov 1990;35(11):1426-30. [Medline].
Brady LW, Asbell SO. Malignant lymphoma of the gastrointestinal tract. Erskine Memorial Lecture, 1979. Radiology. Nov 1980;137(2):291-8. [Medline].
Cappell MS, Botros N. Predominantly gastrointestinal symptoms and signs in 11 consecutive AIDS patients with gastrointestinal lymphoma: a multicenter, multiyear study including 763 HIV-seropositive patients. Am J Gastroenterol. Apr 1994;89(4):545-9. [Medline].
Chadha KS, Hernandez-Ilizaliturri FJ, Javle M. Primary esophageal lymphoma: case series and review of the literature. Dig Dis Sci. Jan 2006;51(1):77-83. [Medline].
Chow DC, B1eikh SH, Eickhoff L, Soloway GN, Saul Z. Primary esophageal lymphoma in AIDS presenting as a nonhealing esophageal ulcer. Am J Gastroenterol. Mar 1996;91(3):602-3. [Medline].
Field SP, Sachar DB, Childs CC, Rubin KP. Steroid-responsive dysphagia: a clue to the diagnosis of esophageal lymphoma. Mt Sinai J Med. Jul-Aug 1984;51(4):451-4. [Medline].
Finn DG. Lymphoma of the head and neck and acquired immunodeficiency syndrome: clinical investigation and immunohistological study. Laryngoscope. Apr 1995;105(4 Pt 2 Suppl 68):1-18. [Medline].
Gaskin CM, Low VH, Ho LM. Isolated primary non-hodgkin's lymphoma of the esophagus. AJR Am J Roentgenol. Feb 2001;176(2):551-2. [Medline].
Gilman AG, Rall TW, Nies AS. The Pharmacological Basis of Therapeutics. 8th ed. New York: McGraw-Hill Inc; 1993.
Herrmann R, Panahon AM, Barcos MP, Walsh D, Stutzman L. Gastrointestinal involvement in non-Hodgkin's lymphoma. Cancer. Jul 1 1980;46(1):215-22. [Medline].
Hosaka S, Nakamura N, Akamatsu T, Fujisawa T, Ogiwara Y, Kiyosawa K, et al. A case of primary low grade mucosa associated lymphoid tissue (MALT) lymphoma of the oesophagus. Gut. Aug 2002;51(2):281-4. [Medline].
Hricak H, Thoeni RF, Margulis AR, Eyler WR, Francis IR. Extension of gastric lymphoma into the esophagus and duodenum. Radiology. May 1980;135(2):309-12. [Medline].
Kaplan KJ. Primary esophageal lymphoma: a diagnostic challenge. South Med J. Apr 2004;97(4):331-2. [Medline].
Kirsch HL, Cronin DW, Stein GN, Latour F, Herrera AF. Esophageal perforation. An unusual presentation of esophageal lymphoma. Dig Dis Sci. Apr 1983;28(4):371-4. [Medline].
Kurtin PJ, Pinkus GS. Leukocyte common antigen--a diagnostic discriminant between hematopoietic and nonhematopoietic neoplasms in paraffin sections using monoclonal antibodies: correlation with immunologic studies and ultrastructural localization. Hum Pathol. Apr 1985;16(4):353-65. [Medline].
Levine AM. Acquired immunodeficiency syndrome related lymphoma. Blood. 1992;80:8-20.
Maipang T, Panjapiyakul C, Sriplung H. Primary lymphoma of the esophagus: a case report. J Med Assoc Thai. May 1992;75(5):299-303. [Medline].
Marnejon T, Scoccia V. The coexistence of primary esophageal lymphoma and Candida glabrata esophagitis presenting as dysphagia and odynophagia in a patient with acquired immunodeficiency syndrome. Am J Gastroenterol. Feb 1997;92(2):354-6. [Medline].
Matsuura H, Saito R, Nakajima S, Yoshihara W, Enomoto T. Non-Hodgkin's lymphoma of the esophagus. Am J Gastroenterol. Dec 1985;80(12):941-6. [Medline].
Mengoli M, Marchi M, Rota E, Bertolotti M, Gollini C, Signorelli S. Primary non-Hodgkin's lymphoma of the esophagus. Am J Gastroenterol. Jun 1990;85(6):737-41. [Medline].
Moses AE, Rahav G, Bloom AI, Okon E, Polliack A, Maayan S, et al. Primary lymphoma of the esophagus in a patient with AIDS. J Clin Gastroenterol. Dec 1995;21(4):327-8. [Medline].
Moss S, Valentine CB, Carey PB, Hind CR. Dysphagia in an HIV-positive man. Postgrad Med J. Apr 1995;71(834):247-8. [Medline].
Nagrani M, Lavigne BC, Siskind BN, Knisley RE, Traube M. Primary non-Hodgkin's lymphoma of the esophagus. Arch Intern Med. Jan 1989;149(1):193-5. [Medline].
Orvidas LJ, McCaffrey TV, Lewis JE, Kurtin PJ, Habermann TM. Lymphoma involving the esophagus. Ann Otol Rhinol Laryngol. Nov 1994;103(11):843-8. [Medline].
Safai B, Diaz B, Schwartz J. Malignant neoplasms associated with human immunodeficiency virus infection. CA Cancer J Clin. Mar-Apr 1992;42(2):74-95. [Medline].
Salerno CT, Kreykes NS, Rego A, Maddaus MA. Primary esophageal lymphoma: a diagnostic challenge. Ann Thorac Surg. Oct 1998;66(4):1418-20. [Medline].
Soon MS, Yen HH, Soon A, Lin OS. Primary esophageal B-cell lymphoma: evaluation by EUS. Gastrointest Endosc. Jun 2005;61(7):901-3. [Medline].
Weeratunge CN, Bolivar HH, Anstead GM, Lu DH. Primary esophageal lymphoma: a diagnostic challenge in acquired immunodeficiency syndrome--two case reports and review. South Med J. Apr 2004;97(4):383-7. [Medline].
Wotherspoon AC. Extragastric MALT lymphoma. Gut. Aug 2002;51(2):148-9. [Medline].
Further Reading
Keywords
human immunodeficiency virus, acquired immunodeficiency syndrome, HIV, AIDS, Epstein-Barr virus, EBV, tracheoesophageal fistula, esophageal ulcerations, esophagitis
