2 Department of Pathology, New Royal Infirmary of Edinburgh, Little France Crescent, Edinburgh, EH16 4TJ.

 

Introduction:

Primary Hodgkin’s lymphoma of the gastro-intestinal tract is an uncommon pathological entity and the sole involvement of the oesophagus is extremely rare.  We present the case of a 71-year-old gentleman presenting with dysphagia, who was subsequently diagnosed with isolated Hodgkin’s lymphoma of the oesophagus and provide a brief discussion of oesophageal Hodgkin’s Lymphoma.

 

Case Report:

A 71-year-old gentleman presented with a 6-month history of progressive dysphagia, weight loss and nausea.  He had a background of cognitive impairment, cerebrovascular disease and residual right arm weakness secondary to a previous head injury.  Examination revealed a cachexic, frail gentleman who had clear dysphagia to solids and liquids.  He had a soft abdomen with no palpable organomegaly and no lymphadenopathy.  Examination of the cardiovascular and respiratory systems was unremarkable and his neurological system examination revealed only his residual right arm weakness. His abbreviated mental test score was 4/10.

 

His initial blood investigations revealed a Hb of 125 g/L, WCC 3.6 x10⁹/L (neutrophils 2.14, lymphocytes 1.03, eosinophils 0.07), Plt 132 x10⁹/L.  Urea and electrolytes, liver function tests, albumin, calcium and phosphate were all normal.  Erythrocyte sedimentation rate was 26 mm and c-reactive protein was 43 mg/dL.

 

Given the suspicion of gastro-intestinal pathology he underwent an upper gastro-intestinal endoscopy, which revealed a sinister stricture at 25 cm (figure 1a).   The subsequent biopsy showed glandular epithelium with a polypoidal architecture (figure 2).  In the deep aspect of the biopsy there was a mixed infiltrate of inflammatory cells admixed with large cells.  These large cells were predominantly mononuclear Hodgkin cells with prominent nucleoli but also contained classical binucleate Reed-Sternberg cells with “owls-eye” nuclei.  The background inflammatory infiltrate contained small T cells and granulocytes.  The large cells were positive for CD30 and CD20 and weakly positive for CD15 and CD79a.  The majority of large cells expressed Ki67.  The tumour cells were positive for EBV-LMP1 by immunohistochemistry and EBER by in-situ hybridisation.  A diagnosis was made of classical Hodgkin Lymphoma of mixed-cellularity subtype.

 

Figure 1: Initial endoscopy (a), and repeat procedure with stent insertion (b).

 

Figure 2: a) Haematoxylin and Eosin stained section showing glandular epithelium on right with dense infiltrate on left (x100), b) Mixed inflammatory cell infiltrate containing large mononuclear Hodgkin cells [arrowheads] (x400), c) CD30 marking large cells with occasional bi-nucleate forms [arrow] (x400), d) EBV EBER-ISH (x100).

 

A staging CT of chest and abdomen was performed revealing a generally thickened oesophagus, with no significant lymphadenopathy in the chest or abdomen.  The solid intra-abdominal organs were unremarkable.  A diagnosis was made of isolated oesophageal Hodgkin’s lymphoma (Ann Arbor Stage 1A).

 

Given this gentleman’s frailty and cognitive impairment a decision was made to undertake treatment for symptom control and the lesion was stented endoscopically (figure 1b).  He underwent a period of rehabilitation in hospital and subsequently was discharged to a nursing home.

 

Discussion:

Primary lymphoma of the oesophagus is an extranodal lymphoma arising within the oesophageal wall with the main bulk of the disease within the oesophagus.[i]^,[ii] This clinical picture is rare and involvement of the oesophagus in lymphoma is usually secondary; as a result of mediastinal nodes causing extrinsic compression or direct spread into the oesophagus.² The incidence of oesophageal involvement in gastro-intestinal lymphoma is about 1%[iii]^{, [iv]} and most cases are low grade B-cell mucosa associated lymphoid tissue (MALT lymphoma) or diffuse large B-cell types.   Hodgkin’s lymphoma with the primary site of disease as the oesophagus is an exceedingly rare entity.^4,[v],[vi]

 

No definite specific aetiological factors and oesophageal infections have been identified as predisposing factors, although there remains an increased risk of lymphoma in patients with immunodeficiency,¹ with one case report of oesophageal lymphoma in a patient with AIDS.⁵ 

 

The primary symptom of oesophageal Hodgkin’s lymphoma is dysphagia,² although it may present as an incidental finding on endoscopy in an asymptomatic patient. Symptoms and complications of oesophageal lymphoma are related to the locations and spread of the mass.  Upper oesophageal involvement may cause laryngeal symptoms like hoarseness (33%), cough (15%) or dyspnoea (15%).  Mid oesophageal lymphoma may cause broncho-oesophageal fistula (22%) while those of the lower oesophagus can cause stricture in 30% of cases.¹

 

Endoscopy is the usual procedure of detection; superficial lesions show either as oedematous and swollen areas or as multiple erythematous areas in the oesophageal mucosa.  Diagnosis is established on morphological and immunotypical features on biopsy although multiple biopsies may be required as single biopsies may miss the characteristic Reed-Sternberg cells.[vii] If clinical suspicion remains high despite negative biopsies then deep biopsies via rigid endoscopy should be considered, before considering considerably more invasive procedures such as thoracotomy.⁷

 

Surgery has a limited place in the treatment of primary oesophageal lymphoma. The standard treatment is tailored to the stage and usually includes a combination of chemo and radiotherapy.¹ However, in our frail elderly gentleman with advanced cognitive impairment, a palliative approach was taken and hence the lesion was stented for symptomatic treatment of dysphagia.

 

Conclusion:

Although rare, the differential of a primary lymphoma should be considered when a patient presents with dysphagia and a stricture on endoscopy.

 

References

1. Scheppach W, Bresalier RS and Tytgat GN (Editors). Gastrointestinal and Liver Tumours. Springer-Verlag, Berlin (2004) pp30-31 (ISBN 3-540-43462-3). 

2. Taal BG, P 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. 

3. Wodzinski MZ, Gavalas M, Brown MJ, Thorpe JA. Hodgkin's disease of the oesophagus. Acta Haematol. 1988;79(4):221-3. 

4. E Coppens E, Nakadi IE, Nagy N, Zalcman M. Primary Hodgkin's Lymphoma of the Esophagus. Am J Roentgenol. 2003 May;180(5):1335 - 1337. 

5. Loeb DS, Ribeiro A, Menke DM. Hodgkin's disease of the esophagus: report of a case. Am J Gastroenterol. 1999 Feb;94(2):520-2. 

6. Gelb AB, Medeiros LJ, Chen YY, Weiss LM, Weidner N. Hodgkin's disease of the esophagus. Am J Clin Pathol. 1997 Nov;108(5):593-8 

7. Horne G, Medlicott SA, Mansoor A et al. A definitive diagnosis of primary Hodgkin lymphoma on endoscopic biopsy material utilizing in-depth immunohistochemical analysis. Can J Gastroenterol. 2007 Mar;21(3):185-8.

 

 

Acknowledgements: We would like to thank the Royal Infirmary of Edinburgh Endoscopy department for providing images of the procedure. 

Consent was obtained from the patient’s next of kin for publication of this case report.