|
|
|||
|
| |||
|
| |||
|
| |||
|
|
|||
|
Ioannis V Kalogeropoulos, Sofia Tsolaki, Dimitra Rontogianni, Department of Radiology "Evagelismos" General Hospital, Hypsilantou 45-47, 10676, Athens, Greece Athanasios N Chalazonitis, Fotios Laspas, Nikolaos Ptohis, Ioannis Neofytou, Department of Radiology "Hippocratio" General Hospital, Thisseos 29, 15234, Halandri, Athens, Greece Author contributions: Kalogeropoulos IV and Tsolaki S wrote the manuscript; Chalazonitis AN organized and revised the manuscript; Laspas F assisted in writing the manuscript and is the corresponding author; Ptohis N and Neofytou I carried out the literature analysis and assisted in writing the manuscript; Rontogianni D provided data and figures relating to the patient. Correspondence to: Fotios Laspas, MD, MSc, Department of Radiology, "Hippocratio" General Hospital, Thisseos 29, 15234, Halandri, Athens, Greece. fotisdimi@yahoo.gr Telephone: +30-21-06011821 Fax: +30-21-06857071 Received: December 24, 2008 Revised: March 18, 2009 Accepted: March 25, 2009 Published online: April 21, 2009
Abstract Primary non-Hodgkin’s lymphoma of the esophagus is a rare disease. A case of primary isolated non-Hodgkin’s lymphoma of the esophagus in a 77-year-old man without acquired immunodeficiency syndrome is presented. We describe the clinical features and the imaging findings (barium swallow, endoscopic ultrasonography and CT) of a biopsy proven B-cell lymphoma with diffuse transmural involvement of the esophagus wall, which was discovered incidentally. We also briefly review the literature.
© 2009 The WJG Press and Baishideng. All rights reserved.
Key words: Endoscopic ultrasonography; Esophagus; Lymphoma; Non-Hodgkin’s lymphoma
Peer reviewers: George Triadafilopoulos, Professor, Clinical Professor of Medicine, Division of Gastroenterology and Hepatology, Alway Building, Room M 211, 300 Pasteur Drive, MC: 5187, Stanford, CA 94305-5187, United States; Jia-Yu Xu, Professor, Rui Jin Hospital, Shanghai Jiao Tong University School of Medicine, 197 Rui Jin Er Road, Shanghai 200025, China
Kalogeropoulos IV, Chalazonitis AN, Tsolaki S, Laspas F, Ptohis N, Neofytou I, Rontogianni D. A case of primary isolated non-Hodgkin’s lymphoma of the esophagus in an immunocompetent patient. World J Gastroenterol 2009; 15(15): 1901-1903 Available from: URL: http://www.wjgnet.com/1007-9327/15/1901.asp DOI: http://dx.doi.org/10.3748/wjg.15.1901
INTRODUCTION Although lymphomas are malignant neoplasms which are usually confined to the lymph nodes, one fifth of lymphomas present with extranodal localization[1]. The esophagus is an uncommon localization accounting for less than 1% of patients with lymphoma and is usually seen secondary to mediastinal nodes or gastric lymphoma[2,3]. Isolated primary lymphoma of the esophagus is exceptionally rare, and when seen, is usually the non-Hodgkin’s type[4]. The imaging findings of esophageal lymphoma have shown a diverse spectrum of abnormalities, similar to those of lymphoma elsewhere in the gastrointestinal tract. We present a distinctly rare case of primary diffuse non-Hodgkin’s lymphoma of the esophagus with a unique, to our knowledge, endoscopic ultrasonographic appearance, of a mainly hyperechoic mass.
CASE REPORT A 77-year old man was admitted because of paroxysmal atrial fibrillation and epigastric pain. His physical examination was normal without clinical evidence of lymph node enlargement or hepatomegaly. Complete blood cell count and routine serum chemistry levels were also normal. A double-contrast barium swallow showed enlargement of the mucosal folds and mild dilatation of the esophageal lumen below the level of the aortic arch (Figure 1); the stomach and duodenum were normal. Chest CT showed extensive thickening of the esophagus wall extending from the middle to the lower portion, without enlargement of mediastinal lymph nodes (Figure 2). CT of the abdomen and pelvis were negative. Endoscopic ultrasonography (EUS) was performed in order to evaluate the esophageal wall thickening. EUS showed a transmural wall thickening along the middle and lower portion of the esophagus with a heterogeneous, mainly hyperechoic mass confined to the sub-mucosal layer, without erosions or ulcers. There was no enlargement of adjacent lymph nodes (Figure 3). Endoscopy showed excessive rigid folds extending 20-30 cm from the incisors, along the length of the middle and lower esophagus. The stomach and duodenum were normal. Biopsy of the lesion was performed. Histologic examination revealed the presence of a B-cell non-Hodgkin’s lymphoma (Figure 4A-C). Bone marrow biopsy specimens showed no evidence of lymphoma; hence, the patient was diagnosed as isolated B-cell lymphoma with diffuse primary involvement of the esophagus.
DISCUSSION The gastrointestinal tract is the most common extranodal site of non-Hodgkin’s lymphoma accounting for 5%-20% of all cases[1,5,6]. Non-Hodgkin’s lymphoma of the esophagus is an extremely uncommon localization of non-Hodgkin’s disease, accounting for less than 1% of patients with lymphoma and occurs more often in the distal esophagus[1,4,7]. In a review of the literature, Okerbloom et al[8] found only 4 primary lymphomas of the esophagus in a large series of 1235 cases of non-Hodgkin’s lymphoma, representing an incidence of 0.3%. Freeman et al[1], in another large series found only 3 esophageal lymphomas. Taal et al[9] found 37 cases of esophageal non-Hodgkin’s lymphoma and only two were primary lymphomas. Isolated primary lymphoma of the esophagus without an extra-esophageal location is very rare and only about 20 cases have been described in the literature[10,11]. Although lymphoma of the esophagus is often asymptomatic, most patients present with dysphagia. Other less common clinical manifestations of esophageal lymphoma are odynophagia, fever and weight loss[12-15]. Although this disease is extremely rare, it should be considered in the differential diagnosis of patients with acquired immunodeficiency syndrome presenting with dysphagia and weight loss. In our case, the patient complained of epigastric pain. This symptom is nonspecific and should be differentiated from other esophageal or gastric diseases. Our patient had no fever, dysphagia or odynophagia and he was not immunocompromised. Furthermore, our patient fulfilled all Dawson’s criteria to identify primary gastrointestinal lymphoma, such as, no palpable superficial lymphadenopathy, no enlargement of mediastinal or hilar lymph nodes and no splenic involvement[16]. Previously described cases have reported a variety of radiographic appearances for primary esophageal lymphoma. The most common include polypoid masses with or without erosions, stricture with ulceration mimicking esophageal cancer, thickening of the mucosal folds producing a varicoid appearance, narrowed distal segments (achalasia-like appearance), or submucosal nodules[5,12,17,18]. Given such a diverse spectrum of radiographic appearance, EUS and CT must be performed and biopsy of the esophageal wall are needed in order to confirm the diagnosis. In our case double-contrast esophagography showed diffuse mucosal fold thickening, without luminal narrowing, suggesting an intramural, benign lesion. CT revealed the precise extent of the lesion, involving the middle and lower third of the esophagus and the absence of mediastinal lymph nodes. With the clinical application of EUS, great progress has been made in the diagnostic accuracy of structural abnormalities and depth of invasion in various gastrointestinal diseases, including lymphoma[19]. EUS provides more accurate information on the involvement of the wall layers by such an intrinsic process. In our case, all layers were thickened, but not disrupted. Furthermore, a submucosal-located heterogeneous, mainly hyperechoic mass was imaged, which had gradually elevated margins. In a previous study esophageal lymphoma was described to involve the whole length of the esophagus[20]. However, in this study, EUS showed lymphomatous involvement of the gastrointestinal wall to produce a typical hypo-echogenic transmural thickening. To our knowledge, our case of primary diffuse esophageal lymphoma is the first described in the literature to have a mainly hyperechoic appearance on EUS. Given the non-specific clinical and radiological appearances, endoscopic biopsy of the lesion was necessary. Endoscopy showed extensive rigid folds without erosions and biopsy was performed. Pathologic evaluation of the endoscopic biopsy showed a B-cell non-Hodgkin’s lymphoma. The treatment of esophageal lymphoma depends on the histological tumor type and its initial location. Because of the extent of the lesion, our patient was treated with chemotherapy and showed clinical improvement. The chemotherapy regimen was a combination of chemotherapeutic agents. The cycle was repeated every 28 d. The patient received six cycles and achieved complete remission (CT scan of thorax and barium swallow did not reveal any residual lesion). In summary, esophageal lymphomatous involvement should be considered in the differential diagnosis of immunocompetent patients presenting with epigastric pain. A transmural, mainly hyperechoic, diffuse thickening of the esophageal wall in the EUS is another possible appearance of gastrointestinal lymphoma.
REFERENCES 1 Freeman C, Berg JW, Cutler SJ. Occurrence and prognosis of extranodal lymphomas. Cancer 1972; 29: 252-260 PubMed DOI 2 Oğuzkurt L, Karabulut N, Cakmakci E, Besim A. Primary non-Hodgkin's lymphoma of the esophagus. Abdom Imaging 1997; 22: 8-10 PubMed DOI 3 Jones AS, Roland NJ, Hamilton J, Rowley H, Nandapalan V. Malignant tumours of the cervical oesophagus. Clin Otolaryngol Allied Sci 1996; 21: 49-53 PubMed DOI 4 Coppens E, El Nakadi I, Nagy N, Zalcman M. Primary Hodgkin's lymphoma of the esophagus. AJR Am J Roentgenol 2003; 180: 1335-1337 PubMed 5 Herrmann R, Panahon AM, Barcos MP, Walsh D, Stutzman L. Gastrointestinal involvement in non-Hodgkin's lymphoma. Cancer 1980; 46: 215-222 PubMed DOI 6 Papaxoinis G, Papageorgiou S, Rontogianni D, Kaloutsi V, Fountzilas G, Pavlidis N, Dimopoulos M, Tsatalas C, Xiros N, Economopoulos T. Primary gastrointestinal non-Hodgkin's lymphoma: a clinicopathologic study of 128 cases in Greece. A Hellenic Cooperative Oncology Group study (HeCOG). Leuk Lymphoma 2006; 47: 2140-2146 PubMed DOI 7 Aozasa K, Tsujimoto M, Inoue A, Nakagawa K, Hanai J, Kurata A, Nosaka J. Primary gastrointestinal lymphoma. A clinicopathologic study of 102 patients. Oncology 1985; 42: 97-103 PubMed 8 Okerbloom JA, Armitage JO, Zetterman R, Linder J. Esophageal involvement by non-Hodgkin's lymphoma. Am J Med 1984; 77: 359-361 PubMed DOI 9 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; 34: 994-998 PubMed DOI 10 Golioto M, McGrath K. Primary lymphoma of the esophagus in a chronically immunosuppressed patient with hepatitis C infection: case report and review of the literature. Am J Med Sci 2001; 321: 203-205 PubMed 11 Gupta S, Pant GC, Gupta S. A clinicopathological study of primary gastrointestinal lymphoma. J Surg Oncol 1981; 16: 49-58 PubMed DOI 12 Carnovale RL, Goldstein HM, Zornoza J, Dodd GD. Radiologic manifestations of esophageal lymphoma. AJR Am J Roentgenol 1977; 128: 751-754 PubMed 13 George MK, Ramachandran V, Ramanan SG, Sagar TG. Primary esophageal T-cell non-Hodgkin's lymphoma. Indian J Gastroenterol 2005; 24: 119-120 PubMed 14 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; 97: 383-387 PubMed DOI 15 Gaskin CM, Low VH, Ho LM. Isolated primary non-hodgkin's lymphoma of the esophagus. AJR Am J Roentgenol 2001; 176: 551-552 PubMed 16 Dawson IM, Cornes JS, Morson BC. Primary malignant lymphoid tumours of the intestinal tract. Report of 37 cases with a study of factors influencing prognosis. Br J Surg 1961; 49: 80-89 PubMed DOI 17 Salerno CT, Kreykes NS, Rego A, Maddaus MA. Primary esophageal lymphoma: a diagnostic challenge. Ann Thorac Surg 1998; 66: 1418-1420 PubMed DOI 18 Levine MS, Sunshine AG, Reynolds JC, Saul SH. Diffuse nodularity in esophageal lymphoma. AJR Am J Roentgenol 1985; 145: 1218-1220 PubMed 19 Shim CS, Lee JS, Kim JO, Cho JY, Lee MS, Jin SY, Youm W. A case of primary esophageal B-cell lymphoma of MALT type, presenting as a submucosal tumor. J Korean Med Sci 2003; 18: 120-124 PubMed 20 Bolondi L, De Giorgio R, Santi V, Paparo GF, Pileri S, Di Febo G, Caletti GC, Poggi S, Corinaldesi R, Barbara L. Primary non-Hodgkin's T-cell lymphoma of the esophagus. A case with peculiar endoscopic ultrasonographic pattern. Dig Dis Sci 1990; 35: 1426-1430 PubMed DOI
S- Editor Tian L L- Editor Webster JR E- Editor Lin YP
| |||
|
|
