Clinical Profile:

A 50 year old lady presented with epigastric pain after eating food and early satiety since three months. The pain was dull aching in nature. She also complained of significant loss of weight since one month.

Radiological Investigations:

Spot radiographs from a upper gastrointestinal study (fig 1, 2, 3 and 4) show circumferential nodular thickening of gastric folds in the fundus and the proximal body of the stomach. There is no outlet obstruction.

CT enterography (Fig 5,6,7 and 8) shows circumferential , transmural thickening of the gastric wall involving the fundus and and the proximal body of the stomach with a maximum diameter of 29mm. It shows homogenous hypo-enhancement with absence of normally enhancing overlying mucosa. There are multiple hypo-enhancing rounded enlarged lymph nodes noted in the peri gastric region.

Radiological Diagnosis:

Primary gastric lymphoma with local nodal involvement ( stage IIa)

Pathological diagnosis

Endoscopic fundic growth biopsy.

Intermediate and high power photomicrograph of H-E stain of the gastric biopsy specimen (fig 9 and 10) shows an infiltrate of blue lymphoid cells through out with few gastric glands. The cells show high nuclear to cytoplasmic ratio.

Intermediate and high power photomicrograph of Immunostain CD 20 ( fig 11 and 12) show pleomorphic cells stain positively with CD 20, a marker for B cells.

Intermediate power photomicrograph of Immunostain CD3 (fig 13) show no uptake of CD3 stain by pleomorphic cells, normal T cell are stained positively by CD 3.

Intermediate power photomicrograph of Immunostain pan cytokeratin (fig 14) shows no uptake of stain by the cells suggestive non epithelial origin of tumor.

Pathological diagnosis – Diffuse large B cell (DLBC) lymphoma (Non Hodgkin lymphoma).

Final diagnosis :

Primary DLBC gastric lymphoma with local nodal involvement (stage IIa)

Treatment – Patient is on CHOP- R chemotherapy regimen

Discussion:

Primary non-Hodgkin's lymphoma of the gastrointestinal tract is the most commonly involved extra-nodal site and represents 10%–15% of all NHL cases and 30%–40% of all extra-nodal sites. The most commonly involved site is the stomach (60%–75% of cases), followed by the small bowel, ileum, cecum, colon and rectum¹.

Dawson et al cited five criteria that must be met for the diagnosis of a primary gastrointestinal lymphoma to be made ^2. -

1.No palpable superficial lymph nodes are seen.

2.Chest radiographic findings are normal.

3.The white blood cells count are normal.

4.There is no involvement of liver and spleen.

5.At laparotomy, the alimentary lesion is predominantly involved, with lymph nodes involvement (if any) confined to the drainage area of the involved segment of the gut.

Clinical presentation:

Symptoms of primary gastric lymphoma are usually vague and nonspecific. Abdominal pain and cramping is the most common finding. Additional symptoms like early satiety, unintended weight loss, abdominal tenderness and sometimes gastric bleeding can occur.

Pathological features:

All histological categories of nodal lymphomas may arise in the gastrointestinal (GI), but the main two histological subtypes (>90% of cases) are mucosa-associated lymphoid tissue (MALT) non Hodgkin lymphoma (NHL) and diffuse large B-cell (DLBC) NHL³.

Staging:

The classification of primary gastrointestinal lymphomas is best made with the classification system adopted at the Consensus Conference in Lu-guano in 1993⁴:

•stage I, tumor confined to gastrointestinal tract, single primary site, and multiple noncontiguous lesions;

•stage II, tumor extends into the abdominal cavity from the primary gastrointestinal site (IIa, local nodal involvement; IIb, distant nodal involvement);

•stage III, penetration through serosa to involve adjacent organs or tissues;

•stage IV, disseminated extra-nodal involvement or a gastrointestinal tract lesion with supradiaphragmatic nodal involvement. Most patients present with stage II disease.

Follow up

• A breath test is performed at approximately 2 months post-treatment to evaluate successful H pylori eradication. Also, 3 to 6 months after H pylori eradication, an endoscopic examination with multiple biopsies should be done to assess lymphoma regression.

•Endoscopic examination should be repeated every 6 months for 2 years and then yearly to monitor histological remission⁹.

•There are no indications for surveillance CT scans or PET CTs unless patients have new symptoms ¹⁰.

Prognostic factors:

•Female gender, age less than 60 years, H pylori associated MALT lymphoma , disease limited to mucosa and submucosa are associated with good overall survival.

•More than 5% of diffuse large blast in MALT lymphoma, DLBC lymphoma , high LDH, advance stage and poor performance status associated with poor prognosis⁵.

Treatment:

•Early stage, H pylori positive, gastric MALT type lymphoma – triple therapy based on combination of proton pump inhibitors, clarithromycin and metronidazole for 14 days⁶.

•H pyloric negative and antibiotic resistant gastric MALT type lymphoma- radiation therapy of total dose of 30 Gy over 4 weeks⁷.

•DLBC gastric lymphoma - R- CHOP chemotherapy regimen for every 14 to 21 days for 6 to 8 cycles⁸.

• Today, surgery is now limited to cases of perforation, haemorrhage, or obstruction due to the tumor.

References

1.D'Amore F, Brincker H, Grønbaek K, Thorling K, Pedersen M, Jensen MK, Andersen E, Pedersen NT, Mortensen LS. Non-Hodgkin's lymphoma of the gastrointestinal tract: a population-based analysis of incidence, geographic distribution, clinicopathologic presentation features, and prognosis. Danish Lymphoma Study Group. Journal of clinical oncology. 1994 Aug;12(8):1673-84

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. British Journal of Surgery. 1961 Jul;49(213):80-9.

3.Koch P, del Valle F, Berdel WE, Willich NA, Reers B, Hiddemann W, Grothaus-Pinke B, Reinartz G, Brockmann J, Temmesfeld A, Schmitz R. Primary gastrointestinal non-Hodgkin’s lymphoma: I. Anatomic and histologic distribution, clinical features, and survival data of 371 patients registered in the German Multicenter Study GIT NHL 01/92. Journal of Clinical Oncology. 2001 Sep 15;19(18):3861-73.

4.Rohatiner A. Report on a workshop convened to discuss the pathological and staging classifications of gastrointestinal tract lymphoma. Annals of Oncology. 1994 May 1;5(5):397-400.

5. Wang YG, Zhao LY, Liu CQ, Pan SC, Chen XL, Liu K, Zhang WH, Yang K, Chen XZ, Zhang B, Chen ZX. Clinical characteristics and prognostic factors of primary gastric lymphoma: a retrospective study with 165 cases. Medicine. 2016 Aug;95(31).

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10. Diagnosis and evaluation of gastric cancer by positron emission tomography.Wu CX, Zhu ZH

World J Gastroenterol. 2014 Apr 28; 20(16):4574-85. [PubMed] [Ref list]

Acknowledgement:

We are grateful to the Department of Surgery at our institution for sharing intraoperative images and to the Department of Pathology for the histopathological images and providing the description for them.