Introduction

Lymphomatous involvement of the gastrointestinal (GI) tract is usually seen in the setting of disseminated disease. Primary GI involvement is usually seen in non-Hodgkin's lymphoma. Primary Hodgkin's lymphoma of the stomach is a rare entity with data from the NCI reporting just six cases of Hodgkin's lymphoma of the GI tract between 1953 and 1990.[1] Herein, we report a case of Hodgkin's lymphoma of the stomach.

Case Report

A 48-year-old woman presented to our department with a history of epigastric pain, early satiety, loss of appetite, and weight loss of 10 kg in the preceding 6 months. She had taken a course of proton pump inhibitors as prescribed by her family physician with no improvement in symptoms. No history of any nausea, vomiting, hematemesis, or melena was noted. On examination, she had good performance status, vitals were normal, and there was no peripheral lymphadenopathy. Examination of the abdomen did not reveal any mass, hepatosplenomegaly, or ascites. Complete blood counts and renal and liver functions were normal. Lactate dehydrogenase was elevated (350 U/L).

Question 1

What are the most common malignant causes of such presentation?

Answer: (1) Carcinoma of the stomach; (2) lymphoma of the stomach; and (3) GI stromal tumor.

She underwent upper GI (UGI) endoscopy which revealed multiple ulcers in the fundus along the lesser curvature of the stomach [Figure 1]a]. Biopsy from the lesion showed gastric mucosa with ulceration and infiltration by large atypical lymphoid cells admixed with inflammatory cells.

| Figure 1  Endoscopy and histology. (a) Ulcer in the fundus of the stomach. (b) Low‑power view showing ulcerated gastric mucosa and aggregation of atypical lymphoid cells in an inflammatory background. (c) High‑power view of large atypical neoplastic cells. (d) The neoplastic cells are cytokeratin negative whereas the gastric epithelial cells are positive. (e) The neoplastic cells and the B cells are PAX5 positive. (f) The neoplastic cells are CD30 positivE

Question 2

What are the most common types of lymphoma of the stomach?

Answer: (1) Diffuse large B-cell lymphoma; (2) marginal zone lymphoma.

Immunohistochemistry (IHC) was done and these cells expressed PAX5, CD30, and MUM1. They were negative for CK, LCA, CD15, ALK, CD20, CD3, and LMP1 [Figure 1]b], [Figure 1]c], [Figure 1]d], [Figure 1]e], [Figure 1]f]. In situ hybridization for EBER was negative in the atypical cells. In view of the above findings, a diagnosis of Hodgkin's lymphoma was made.

A whole-body FDG positron emission tomography–computed tomography (PET-CT) was done, which showed increased uptake and thickening of the stomach wall with perigastric and aortocaval lymph nodes and another 4 cm × 3 cm lesion in the left adrenal with increased uptake and diffuse uptake in the bone marrow. Bone marrow biopsy did not show any lymphomatous involvement.

The patient was diagnosed and staged as Hodgkin's lymphoma Stage IV (Ann Arbor Staging); IPSS score 2/7.

Question 3

How common is Hodgkin's lymphoma of the stomach?

Answer: Hodgkin's lymphoma of the stomach is a rare entity. Colucci et al. reviewed 721 cases of primary gastric lymphoma diagnosed between 1973 and 1990 and identified only 17 as Hodgkin's lymphoma. If IHC would have been done, probably, many cases would be recategorized as non-Hodgkin's lymphoma.

She was started on ABVD chemotherapy regimen. After three cycles, the patient's symptoms showed improvement and PET-CT showed residual disease. She received three more cycles of ABVD. PET- CT done after six cycles showed residual disease in the stomach and left adrenal gland. UGI and stomach biopsy were repeated. Biopsy revealed similar morphology and IHC staining of neoplastic cells. The patient did not consent for biopsy of adrenal lesion or perigastric lymph nodes. Currently, the patient is being planned for second-line salvage therapy with GDP followed by autologous stem cell transplantation if favorable response is noted.

Discussion

GI involvement by lymphoma may be seen in advanced disseminated cases. In 1961, Dawson et al. proposed a set of criteria to distinguish primary GI lymphoma from secondary involvement in the context of disseminated disease. These are (1) absence of peripheral lymphadenopathy at the time of presentation, (2) lack of enlarged mediastinal lymph nodes, (3) normal results for a complete blood count and differential, (4) predominance of the bowel lesion, despite the presence of disease in adjacent lymph nodes, and (5) absence of any lymphomatous involvement of the liver or spleen.[2] This case fits into the above criteria except for the fact that adrenal lesion was present.

Moreover, Hodgkin's lymphoma of the stomach is a rare entity. It constitutes <1 class="i" xss=removed>et al. reviewed 721 cases of primary gastric lymphoma diagnosed between 1973 and 1990 and identified only 17 as Hodgkin's lymphoma. If IHC would have been done, probably, many cases would be recategorized as non-Hodgkin's lymphoma.[3] In addition, literature review identified only eight other cases of Hodgkin's lymphoma of the stomach excluding the current case [Table 1].

1.  Devaney K, Jaffe ES. The surgical pathology of gastrointestinal Hodgkin's disease. Am J Clin Pathol 1991; 95: 794-801
2.  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: 80-9
3.  Colucci G, Giotta F, Maiello E, Fucci L, Caruso ML. Primary Hodgkin's disease of the stomach. A case report. Tumori 1992; 78: 280-2
4.  Jung JH, Kim J, Park G, Chin HM. Primary gastric Hodgkin's lymphoma. J Korean Surg Soc 2012; 83: 111-4
5.  Ogawa Y, Chung YS, Nakata B, Muguruma K, Fujimoto Y, Yoshikawa K. et al. A case of primary Hodgkin's disease of the stomach. J Gastroenterol 1995; 30: 103-7
6.  Mori N, Yatabe Y, Narita M, Hayakawa S, Ishido T, Kikuchi M. et al. Primary gastric Hodgkin's disease. Morphologic, immunohistochemical, and immunogenetic analyses. Arch Pathol Lab Med 1995; 119: 163-6
7.  Zippel K, Hoksch B, Zieren HU. A rare stomach tumor--Hodgkin's lymphoma of the stomach. Chir Z Alle Geb Oper Medizen 1997; 68: 540-2
8.  Venizelos I, Tamiolakis D, Bolioti S, Nikolaidou S, Lambropoulou M, Alexiadis G. et al. Primary gastric Hodgkin's lymphoma: A case report and review of the literature. Leuk Lymphoma 2005; 46: 147-50
9.  Penázová M, Dedic K, Ryska A. Primary Hodgkin lymphoma of the stomach-a case report. Cesk Patol 2007; 43: 23-6
10.  Saito M, Tanaka S, Mori A, Toyoshima N, Irie T, Morioka M. Primary gastric Hodgkin's lymphoma expressing a B-cell profile including Oct-2 and Bob-1 proteins. Int J Hematol 2007; 85: 421-5
11.  Hossain FS, Koak Y, Khan FH. Primary gastric Hodgkin's lymphoma. World J Surg Oncol 2007; 5: 119

1.  Devaney K, Jaffe ES. The surgical pathology of gastrointestinal Hodgkin's disease. Am J Clin Pathol 1991; 95: 794-801
2.  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: 80-9
3.  Colucci G, Giotta F, Maiello E, Fucci L, Caruso ML. Primary Hodgkin's disease of the stomach. A case report. Tumori 1992; 78: 280-2
4.  Jung JH, Kim J, Park G, Chin HM. Primary gastric Hodgkin's lymphoma. J Korean Surg Soc 2012; 83: 111-4
5.  Ogawa Y, Chung YS, Nakata B, Muguruma K, Fujimoto Y, Yoshikawa K. et al. A case of primary Hodgkin's disease of the stomach. J Gastroenterol 1995; 30: 103-7
6.  Mori N, Yatabe Y, Narita M, Hayakawa S, Ishido T, Kikuchi M. et al. Primary gastric Hodgkin's disease. Morphologic, immunohistochemical, and immunogenetic analyses. Arch Pathol Lab Med 1995; 119: 163-6
7.  Zippel K, Hoksch B, Zieren HU. A rare stomach tumor--Hodgkin's lymphoma of the stomach. Chir Z Alle Geb Oper Medizen 1997; 68: 540-2
8.  Venizelos I, Tamiolakis D, Bolioti S, Nikolaidou S, Lambropoulou M, Alexiadis G. et al. Primary gastric Hodgkin's lymphoma: A case report and review of the literature. Leuk Lymphoma 2005; 46: 147-50
9.  Penázová M, Dedic K, Ryska A. Primary Hodgkin lymphoma of the stomach-a case report. Cesk Patol 2007; 43: 23-6
10.  Saito M, Tanaka S, Mori A, Toyoshima N, Irie T, Morioka M. Primary gastric Hodgkin's lymphoma expressing a B-cell profile including Oct-2 and Bob-1 proteins. Int J Hematol 2007; 85: 421-5
11.  Hossain FS, Koak Y, Khan FH. Primary gastric Hodgkin's lymphoma. World J Surg Oncol 2007; 5: 119