Esophageal Lymphoma Workup
- Author: Vivek V Gumaste, MD; Chief Editor: BS Anand, MD more...
No specific laboratory blood tests are required for the diagnosis of esophageal lymphoma. In primary esophageal lymphoma, the WBC count is, by definition, within the reference range.
Anemia and thrombocytopenia are common in AIDS patients who present with lymphoma. The CD4 count is usually low. In a series of 22 AIDS patients with non-Hodgkin lymphoma, 2 of whom had esophageal involvement, the mean CD4 cell count was 80.8 ± 119.6/µL.
Initial staging studies include a complete blood count (CBC), a bone marrow biopsy, and computed tomography (CT) scans of the chest, abdomen, and pelvis. Classification of esophageal lymphoma as a primary lesion can only be made once the criteria of Dawson et al are fulfilled. (See Overview.)
Radiography and CT Scanning
The radiographic appearance of esophageal lymphoma varies and is somewhat nonspecific; therefore, esophageal lymphoma is a difficult diagnosis to confirm with radiographic studies.
Esophageal lymphoma has no pathognomonic appearance. Barium swallow studies of the esophagus may reveal thickened folds with nodular, polypoid, ulcerated, or stenotic features. A radiographic picture consistent with pseudoachalasia may also be present. However, these features cannot help to differentiate esophageal lymphoma from other benign or malignant esophageal diseases.
CT scanning is not diagnostic in esophageal lymphoma; instead, it is used in staging of the disease and in evaluating response to therapy.
Esophagogastroduodenoscopy (EGD) is the only way to directly visualize and biopsy esophageal lymphoma. The morphologic appearance of the tumor may be of little help, especially in patients with AIDS, in whom candidal superinfection is common. Endoscopically, the tumor may appear as an ulcerated, polypoid, or submucosal mass.
Routine endoscopic biopsies may not be useful in making a diagnosis because of the submucosal nature of the lesion. In one study, all patients required repeat endoscopy and biopsy to confirm the diagnosis. Endoscopic biopsies may have a false-negative rate of greater than 30%. Endoscopic mucosal resection of the esophageal lesion has a greater diagnostic yield.
In one series, 27 patients with lymphomatous involvement of the esophagus were identified. Of these, 3 had primary esophageal lymphoma. EGD confirmed the diagnosis in 81% of patients, although 19% required surgical exploration to establish the diagnosis.
If suspicion for esophageal lymphoma is high, consider other diagnostic modalities (eg, CT scans, surgical biopsy).
In one case report of primary esophageal lymphoma, the endosonographic features were reported as diffuse, homogenous, hypoechogenic esophageal wall thickening. Esophageal lymphomas may demonstrate anechoic areas.
Endoscopic ultrasonographically guided fine-needle aspiration (EUS-FNA) of suspicious submucosal lesions may help to obtain tissue and to establish a diagnosis, such as mucosa-associated lymphoid tissue lymphoma (MALT). In patients who receive chemotherapy or radiation for primary esophageal lymphoma, EUS may be helpful in evaluating the response to treatment.
Most reported cases of primary esophageal lymphoma are diffuse large cell lymphomas of the B-cell immunotype. In general, surface markers of the tumor cells reveal positive immunofluorescent staining results for immunoglobulin G (IgG) and kappa light chain.
Mucosa-associated lymphoid tissue (MALT) lymphoma has been found in the esophagus and is being increasingly reported, although still very rare. Unlike MALT lymphoma of the stomach, MALT lymphoma of the esophagus does not appear to be associated with Helicobacter pylori.[13, 14]
Another histologic variant is anaplastic large cell lymphoma, with 3 cases being reported in the literature.
Gupta et al summarized the histologic findings of 17 patients with primary esophageal lymphoma. All but 1 had non-Hodgkin lymphoma, with large cell lymphoma being the most common histologic subtype.
If the tumor type is difficult to determine on hematoxylin and eosin staining, using monoclonal antibodies to the leukocyte common antigen may help to differentiate hematopoietic from nonhematopoietic malignancies. This may be useful for patients in whom it is difficult to distinguish a poorly differentiated carcinoma or sarcoma from a lymphoma.
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].