Esophageal Lymphoma Treatment & Management
- Author: Vivek V Gumaste, MD; Chief Editor: BS Anand, MD more...
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. Studies have indicated that nonsurgical approaches have had equivalent outcomes to those of surgical strategies in patients with GI lymphoma.
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.
Surgical resection is reportedly curative in some cases of primary GI lymphoma, though surgery is primarily used for the 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.
A 2014 case report of a Japanese patient with esophageal mucosa-associated lymphoid tissue (MALT) lymphoma appeared to be successfully treated with endoscopic submucosal dissection (ESD). The authors suggested that this procedure may be superior to endoscopic mucosal resection for early esophageal MALT lymphoma with respect to the lateral and vertical margins of the resected specimen.
Carefully monitor adverse effects of chemotherapy or radiotherapy. In addition, carefully follow the patient's nutritional status. Consider placement of a gastric or jejunal feeding tube if the patient cannot maintain his or her nutritional status because of dysphagia or odynophagia.
Transfer to a specialized cancer center may be required for further treatment (eg, chemotherapy, radiotherapy, surgical intervention).
Chemotherapy and Radiotherapy
The chemotherapy regimen most commonly used is a combination of cyclophosphamide, doxorubicin, vincristine, and prednisone (a regimen referred to as CHOP). Other chemotherapy regimens have been used, but none have demonstrated greater efficacy than CHOP. 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 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.
Taal BG, Van Heerde P, Somers R. Isolated primary oesophageal involvement by lymphoma: a rare cause of dysphagia: two case histories and a review of other published data. Gut. 1993 Jul. 34(7):994-8. [Medline]. [Full Text].
Madabhavi I, Patel A, Revannasiddaiah S, et al. Primary esophageal Burkitt's lymphoma: a rare case report and review of literature. Gastroenterol Hepatol Bed Bench. 2014 Fall. 7(4):230-7. [Medline]. [Full Text].
Kalogeropoulos IV, Chalazonitis AN, Tsolaki S, et al. A case of primary isolated non-Hodgkin's lymphoma of the esophagus in an immunocompetent patient. World J Gastroenterol. 2009 Apr 21. 15(15):1901-3. [Medline]. [Full Text].
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. 2004 Apr. 97(4):383-7. [Medline].
Dawson IMP, Cornes JS, Morson BC. Primary malignant lymphoma of the intestinal tract. British Journal of Surgery. 1961. 49:80-9.
Orvidas LJ, McCaffrey TV, Lewis JE, Kurtin PJ, Habermann TM. Lymphoma involving the esophagus. Ann Otol Rhinol Laryngol. 1994 Nov. 103(11):843-8. [Medline].
Gaskin CM, Low VH, Ho LM. Isolated primary non-hodgkin's lymphoma of the esophagus. AJR Am J Roentgenol. 2001 Feb. 176(2):551-2. [Medline].
Zhu Q, Xu B, Xu K, Li J, Jin XL. Primary non-Hodgkin's lymphoma in the esophagus. J Dig Dis. 2008 Nov. 9(4):241-4. [Medline].
Neshatian L, Katzka DA. A hidden cause of dysphagia. primary esophageal lymphoma. Gastroenterology. 2015 Sep. 149 (3):549-50. [Medline].
Ahmadzadehfar H, Sabet A, Nake K, et al. Dual-time F-18 FDG-PET/CT imaging for diagnosis of occult non-Hodgkin lymphoma in a patient with esophageal cancer. Clin Nucl Med. 2009 Mar. 34(3):168-70. [Medline].
Tirumani H, Rosenthal MH, Tirumani SH, Shinagare AB, Krajewski KM, Ramaiya NH. Imaging of uncommon esophageal malignancies. Dis Esophagus. 2015 Aug-Sep. 28 (6):552-9. [Medline].
Kinoshita M, Sanuki T, Yamada Y, et al. A case of esophageal mucosa-associated lymphoid tissue lymphoma diagnosed using endoscopic ultrasound-guided fine-needle aspiration. Nihon Shokakibyo Gakkai Zasshi. 2016. 113 (1):63-70. [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. 2002 Aug. 51(2):281-4. [Medline].
Wotherspoon AC. Extragastric MALT lymphoma. Gut. 2002 Aug. 51(2):148-9. [Medline].
Wu N, Pang L, Chen Z, et al. Primary esophageal CD30-positive ALK-positive anaplastic large cell lymphoma: a case report and literature review. J Gastrointest Cancer. 2011 Mar. 42(1):57-60. [Medline].
Kudo K, Ota M, Narumiya K, Shirai Y, Ohki T, Yamamoto M. Primary esophageal mucosa-associated lymphoid tissue lymphoma treated by endoscopic submucosal dissection. Dig Endosc. 2014 May. 26(3):478-81. [Medline].
Agha FP, Schnitzer B. Esophageal involvement in lymphoma. Am J Gastroenterol. 1985 Jun. 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. 1986 Apr. 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. 1990 Nov. 35(11):1426-30. [Medline].
Brady LW, Asbell SO. Malignant lymphoma of the gastrointestinal tract. Erskine Memorial Lecture, 1979. Radiology. 1980 Nov. 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. 1994 Apr. 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. 2006 Jan. 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. 1996 Mar. 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. 1984 Jul-Aug. 51(4):451-4. [Medline].
Finn DG. Lymphoma of the head and neck and acquired immunodeficiency syndrome: clinical investigation and immunohistological study. Laryngoscope. 1995 Apr. 105(4 Pt 2 Suppl 68):1-18. [Medline].
Gilman AG, Rall TW, Nies AS. The Pharmacological Basis of Therapeutics. 8th ed. New York: McGraw-Hill Inc; 1993.
Gupta NM, Goenka MK, Jindal A, Behera A, Vaiphei K. Primary lymphoma of the esophagus. J Clin Gastroenterol. 1996 Oct. 23(3):203-6. [Medline].
Herrmann R, Panahon AM, Barcos MP, Walsh D, Stutzman L. Gastrointestinal involvement in non-Hodgkin's lymphoma. Cancer. 1980 Jul 1. 46(1):215-22. [Medline].
Hricak H, Thoeni RF, Margulis AR, Eyler WR, Francis IR. Extension of gastric lymphoma into the esophagus and duodenum. Radiology. 1980 May. 135(2):309-12. [Medline].
Kirsch HL, Cronin DW, Stein GN, Latour F, Herrera AF. Esophageal perforation. An unusual presentation of esophageal lymphoma. Dig Dis Sci. 1983 Apr. 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. 1985 Apr. 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. 1992 May. 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. 1997 Feb. 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. 1985 Dec. 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. 1990 Jun. 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. 1995 Dec. 21(4):327-8. [Medline].
Moss S, Valentine CB, Carey PB, Hind CR. Dysphagia in an HIV-positive man. Postgrad Med J. 1995 Apr. 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. 1989 Jan. 149(1):193-5. [Medline].
Park S, Jeen YT, Kwon YD, et al. Successfully cured primary esophageal lymphoma in a patient with acquired immune deficiency syndrome (AIDS). Endoscopy. 2009. 41 suppl 2:E148-9. [Medline]. [Full Text].
Safai B, Diaz B, Schwartz J. Malignant neoplasms associated with human immunodeficiency virus infection. CA Cancer J Clin. 1992 Mar-Apr. 42(2):74-95. [Medline].
Salerno CT, Kreykes NS, Rego A, Maddaus MA. Primary esophageal lymphoma: a diagnostic challenge. Ann Thorac Surg. 1998 Oct. 66(4):1418-20. [Medline].
Saddoughi SA, Taswell J, Harmsen WS, et al. Surgical resection of rare esophageal cancers. Ann Thorac Surg. 2016 Jan. 101 (1):311-5. [Medline].
Ye ZY, Cao QH, Liu F, et al. Primary esophageal extranasal NK/T cell lymphoma with biphasic morphology: a case report and literature review. Medicine (Baltimore). 2015 Jul. 94 (28):e1151. [Medline].