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Year : 2021  |  Volume : 14  |  Issue : 1  |  Page : 73-79  

An unusual case of gastric lymphoma presenting as linitus plastica

Department of Pathology, Dr. DY Patil Medical College Hospital and Research Centre, DY Patil Vidyapeeth, Pune, Maharashtra, India

Date of Submission27-Jul-2020
Date of Decision30-Dec-2020
Date of Acceptance04-Jan-2021
Date of Web Publication22-Jan-2021

Correspondence Address:
Vidya Vishwanathan
Department of Pathology, DY Patil Medical College, Pune-411018
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjdrdypu.mjdrdypu_274_20

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Disorders of the stomach are frequently seen as clinical diseases. Of all the lesions of the stomach, inflammatory conditions and neoplastic lesions are most common. Primary malignant lymphoma of the stomach accounts for only a small percentage (about 10%) of all malignant tumors of the stomach, and majority of them arise from B-cells. Most common among these are indolent extranodal marginal zone lymphomas, and in the stomach, they are called MALTomas. Occasionally, gastric lymphomas of one type or another are seen adjacent to adenocarcinoma. Sometimes, lymphoma precedes carcinoma by several years. The aim was to study the microscopic patterns of gastric biopsies and mastectomy specimens received for the case in our department. Multiple gastric biopsy specimens as well as gastrectomy specimens were received in our department. Multiple sections were taken and stained with hematoxylin and eosin. Immunohistochemical (IHC) stains were used as applicable. Studies posed a diagnostic difficulty by showing picture of gastritis from biopsy of the cardia; signet ring cell adenocarcinoma was seen in small gastrointestinal biopsies from lesser curvature and gastric lymphoma with gross picture of linitis plastica on gastrectomy specimen. For confirmation and to know the type of gastric lymphoma, multiple B- and T-cell IHC markers were used. CD20, CD10, BCL2, and c-Myc were positive in tumor cells, CD3 positivity was seen in reactive T-cells, and MIB1 positivity was seen in 70%–75% of tumor cells. Hence, it was confirmed to be high-grade large cell lymphoma (double-expresser type). Hence, it can be concluded that IHC markers are necessary to solve the diagnostic difficulties.

Keywords: Immunohistochemistry, linitis plastica, lymphoma

How to cite this article:
Vishwanathan V, Malhotra A, Dharwadkar A, Vimal S, Gore C, Kumar H. An unusual case of gastric lymphoma presenting as linitus plastica. Med J DY Patil Vidyapeeth 2021;14:73-9

How to cite this URL:
Vishwanathan V, Malhotra A, Dharwadkar A, Vimal S, Gore C, Kumar H. An unusual case of gastric lymphoma presenting as linitus plastica. Med J DY Patil Vidyapeeth [serial online] 2021 [cited 2021 Mar 9];14:73-9. Available from: https://www.mjdrdypv.org/text.asp?2021/14/1/73/307672

  Introduction Top

Incidence of lymphomas is on the rise in the past 2–3 decades. This increase is observed in extranodal forms.[1],[2] Extranodal forms refer to principally non-Hodgkin's lymphoma (NHL) of the central nervous system followed by stomach, intestinal, and cutaneous lymphomas.[3] Primary gastric lymphoma most commonly occurs in patients who are older than 50 years of age, but it can as well be seen in patients in their second decade of life.[4],[5] Males are 2–3 times more likely to develop them than females.[6],[7] These account for only a small percentage (about 10%) of all malignant tumors of the stomach, and majority of them arise from B-cells.[8] Most common among these are indolent extranodal marginal zone lymphomas, and in stomach, these are called MALTomas (mucosa-associated lymphoid tissue). Most common translocation seen is t (11; 18)(q21; q21). There can be Helicobacter pylori-induced inflammation which triggers the pathway to MALTomas. Occasionally, gastric lymphomas of one type or another are seen adjacent to adenocarcinoma. Sometimes, lymphoma precedes carcinoma by several years.[8] Further, MALTomas can transform into more aggressive tumors identical to diffuse large B-cell lymphoma (DLBCL). Common clinical presentation is similar to any other gastrointestinal (GI) malignancy such as hematemesis, melena, and constitutional symptoms of malaise and weight loss. There can be dyspepsia, epigastric pain, and H. pylori-induced gastritis.[9]

  Case Report Top

A 65-year-old male patient came to the outpatient department with complaints of vomiting within 10 min following food and liquid intake for the past 6 months, which was nonprojectile in nature along with one episode of hematemesis. The patient also had heartburn, regurgitation, and halitosis. There was significant amount of weight loss of 27 kg in a span of 6 months. Further, the patient gave a history of passage of black stools on and off along with itching over the abdomen. The patient consumed tobacco for the past 20 years and is also a known case of tinea corporis over abdomen. On general examination, the patient was afebrile. On abdominal inspection, shape was pendulous, flanks showed no fullness, umbilicus was inverted and central, and all quadrants moved equally.

On palpation, abdomen was soft and nontender, no organomegaly was noted, and hernial orifices were free. On percussion, no free fluid was noted. On auscultation, bowel sounds were present.

Esophagogastroscopy was done which showed a mass lesion in the cardia and lower esophagus. On high-resolution CT of the chest, esophagus showed no obvious mass lesion. Oral contrast was given which did not show any obvious obstruction to the flow. No abnormal dilation of esophagus was noted. On CT thorax, abdomen, and pelvis, the study showed circumferential asymmetric enhancing mural thickening of distal body, antrum, and pylorus of the stomach. Thickening was noted along lesser and greater curvature. Gastroesophageal junction was normal. There was no obstruction to the passage of orally administered contrast across the thickened segment. Fat planes of the thickened segment with adjacent structures were well maintained. Ryle's tube was noted in the stomach. Rest other organs did not show any abnormality. It was concluded that mural thickening of distal body, antrum, and pylorus of the stomach could be of neoplastic etiology. Histological correlation was therefore advised.

Repeat esophagogastroscopy showed normal upper and middle esophagus with mild esophagitis in the lower third, while cardia, fundus, and body of the stomach showed irregular mucosal thickening starting from esophagogastric junction extending below throughout lesser curvature of stomach and body of the stomach with some extensions in greater curvature as well. Proliferative lesion was extending till pylorus. Multiple biopsies were taken. Stomach was shrunken and thickened and nondistensible. Report was suggestive of carcinoma of the stomach below esophagogastric junction till pyloric with gastric outlet obstruction.

Histopathological examination of the biopsy from lesser curvature of the stomach showed features of chronic gastritis. One tissue bit on deep sectioning showed solid nests and glands of malignant cells along with scattered isolated signet ring cells. Background showed inflammation and fibrosis, suggesting a diagnosis of adenocarcinoma of the stomach (signet ring type).

Following this histopathological report, the patient was operated. Diagnostic laparoscopy with total gastrectomy with D1 dissection and esophagojejunostomy was done.

First biopsy was done during esophagogastroscopy on which a diagnosis of benign adenoma of the stomach was made. Further, multiple biopsies from esophagus and cardia of the stomach were studied which showed two tissue bits with normal gastric mucosa and unremarkable overlying mucosal lining with underlying lamina propria showing infiltration by inflammatory cells along with preservation of rest of the histological architecture. A diagnosis of gastritis was made on the basis of the above histopathological findings.

Biopsy from lesser curvature of the stomach showed multiple tissue bits with most of them showing features of chronic gastritis. One tissue bit on deep sectioning showed solid nests and glands of malignant cells along with scattered isolated signet ring cells. Background showed inflammation and fibrosis, suggesting a diagnosis of adenocarcinoma of the stomach signet ring type.

Repeat biopsy from cardia of the stomach showed multiple tissue bits of normal gastric mucosa with chronic inflammatory infiltrate and a tumor in the submucosa. The tumor was composed of cells arranged in sheets and gland-like pattern with cells being round to polygonal having vacuolated cytoplasm and peripherally pushed nuclei. Tumor was invading the underlying muscle layer. Vascular emboli were noted as well. Hence, a diagnosis of adenocarcinoma of the stomach signet ring type was made.

Subsequently, we received the gastrectomy specimen along with omentum in a single tissue piece which measured 29 cm × 8 cm × 2 cm [Figure 1]. Stomach measured 18 cm × 2 cm × 1.5 cm. External surface of the stomach was grayish-white; multiple congested dilated blood vessels were noted. Internal surface of stomach was grayish-white with few brownish areas. Two ulcers were noted each of size 2 cm in diameter. Multiple rugae were noted which were flattened. Entire wall of the stomach was diffusely thickened and measured 1 cm in thickness and had leather-bottle appearance, leading to a presumptive diagnosis of linitus plastica.
Figure 1: Gross picture of gastrectomy specimen with omentum

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Sixteen sections for histological examination were taken from the gastrectomy specimen. Multiple sections showed diffusely ulcerated mucosa that was composed of predominately lymphoid cells. The cells were admixed with pleomorphic large round-to-oval cells having vesicular nucleus, prominent eosinophilic nucleoli, high nucleus–cytoplasm ratio, and minimal cytoplasm. Mitosis was occasional. Cells were largely restricted to mucosa. Few areas of normal gastric mucosa were noted as well which showed infiltration of muscularis by inflammatory infiltrate [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]. Sections from proximal and distal surgical margins were free of tumor. A total of 11 lymph nodes were identified and dissected which were all free from tumor.
Figure 2: Scanner view (×4) of high-grade lymphoma

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Figure 3: Low-power (×10) view of high grade lymphoma

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Figure 4: ×40 view of high-grade lymphoma

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Figure 5: ×40 view showing positive staining for LCA

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Figure 6: ×40 view showing negative staining for epithelial marker

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Histopathological examination of the biopsy from jejunal and esophageal end of gastrectomy specimen showed no tumor deposits.

To confirm the diagnosis of lymphoma, multiple immunohistochemical (IHC) markers such as leukocyte common antigen, epithelial markers, and mucicarmine were studied along with B-cells and T-cell markers. IHC results showed positivity for leukocyte common antigen [Figure 7]. Epithelial markers [Figure 8] and mucicarmine stain showed negative staining. CD 20, CD10, BCL2, and c-Myc showed positivity in tumor cells [Figure 9], [Figure 10], [Figure 11], [Figure 12]; CD3 was positive in reactive T-cells [Figure 13]. MIB1 showed positivity in 70%–75% of cells [Figure 14]. Final diagnosis of high-grade large cell lymphoma (double-expresser type) was made.
Figure 7: Low-power view of high-grade large cell lymphoma

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Figure 8: ×40 view of high-grade large cell lymphoma

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Figure 9: CD10-positive tumor cells

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Figure 10: CD20-positive tumor cells

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Figure 11: BCL-positive tumor cells

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Figure 12: CD3-positive reactive T-cells

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Figure 13: MIB1-positive staining in 70%–75% tumor cells

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Figure 14: c-Myc-positive tumor cells

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  Discussion Top

The term lymphoma refers to cancer that arises in the lymphatic system. Lymphatic system is a part of immune system which helps protect body against infections and diseases. It consists of network of tubes known as lymph vessels that drain thin watery fluid lymph from different areas of body into the bloodstream. Lymph collects extracellularly and contains proteins, fats, and white blood cells lymphocytes. Lymph while moving through lymphatic system gets filtered by a network of small structures known as lymph nodes that help remove microorganisms and other foreign bodies from bloodstream.

Most cases of lymphomas arise in the lymph nodes. When they arise outside the lymph nodes, they are known as extranodal lymphomas. Primary gastric lymphoma is the most common form of extranodal lymphoma. Primary gastric lymphomas can be Hodgkin's type or non-Hodgkin's type. Among non-Hodgkin's type, B-cell subtypes are more common than T-cell subtypes.[8],[9]

Gastric lymphomas are as compared to adenocarcinoma of the stomach. Their incidence is increased in recent times. Patients with lymphoma presented with symptoms which are similar to those seen with adenocarcinoma. Lymph node involvement was usually seen along with lymphoma; however, in our study, the lymph nodes were not involved. It was noted that lymph node involvement was not seen when the tumor invades beyond the submucosa as in our case where was tumor seen beyond the muscularis.[5],[6]

Linitis plastica of the stomach is a long known condition, with initial reports from the 16th and 17th centuries.[10] It was defined as a distinct entity in 1859 by Dr. William Brinton, who described it as benign disease with peculiar characteristics, stomach as macroscopically thickened with inconsistent evidence of mucosal ulceration. Histologically, it showed prominent submucosal hypertrophy due to increase in connective tissue and prominent muscular hypertrophy. “Linitis” term was used due to the presence of irregular bands of filamentous tissue in hypertrophic submucosa resembling fibers of linen.[10],[11] Linitis plastica does not always present as complete involvement of the stomach. It may appear in plaques which gives the appearance of segmental lack of distensibility.[12],[13] Cell–stroma interactions have been associated to activate transforming growth factor-beta, hepatocyte growth factor (c-met ligand), fibroblast growth factor 7, and other soluble factors.[14],[15]

It was seen that linitis plastica is a carcinoma with scirrhous stroma that involves submucosal and muscular layers of the stomach even in the absence of mucosal alteration. In most of the cases, primary cancer cells are signet ring cells or scattered cells in the context of poorly differentiated carcinoma.[16]

Lymphoepithelial lesions were the hallmark of all cases of low-grade and secondary high-grade malignant MALT-derived lymphomas, while reactive lymphoid follicles were found in 80% and 48% of these cases, respectively. Macroscopically, 50% of these tumors showed ulcerous growth pattern, 25% showed polypoid–exophytic growth, and other 25% showed diffuse, linitis-like growth pattern.[7]

Reactive lymphoid cells should be differentiated from neoplastic cells. Dense lymphocytic infiltrate occupying most of the lamina propria along with lymphoepithelial lesions and Dutcher bodies point toward lymphoma. In high-grade lymphoma, large blasts are seen in clusters with minimum 20 large cells or as sheet-like proliferations. A proliferation marker-like Ki67 can be helpful in delineating the large cell component as it did in our study. Further, different immunostains are helpful in diagnosing and differentiated B from T-cell lymphoma.[17]

The term “double-hit” lymphoma is used when c-Myc and BCL2 and/or BCL6 rearrangement is identified by either cytogenetic testing or by fluorescence in situ hybridization. Further, terms such as “double-protein” or “double-expresser” refer to coexpression of two oncogenes (c-Myc and BCL2) on a high percentage of tumor cells based on IHC staining.[18],[19] Double-hit lymphomas were a rare subtype among primary gastric lymphomas.[20] Above two oncogenes along with CD10 and CD20 were positive in our study.

In our study, it was a very rare presentation of linitis plastica in DLBCL type of primary gastric lymphoma, “double-expresser type.” Usually, linitis plastica is a presentation of carcinoma of the stomach or metastasis from other sites.

  Conclusion Top

GI tract NHL is a heterogeneous disease, characterized by a variety of clinicopathological and demographic features. Primary gastric NHL is by far the most common type, with distinct histopathological features. Stomach is the most common site to be involved in the GI tract as extranodal lymphomas. Gastric lymphoma accounts for 3%–5% of all malignant tumors of the stomach. In our case, diagnostic difficulties were seen because of multiple biopsies from various sites and gastrectomy specimen showing different histopathological lesions, ranging from simple gastritis to high-grade lymphoma. The diagnostic dilemma was overcome by studying multiple B-cell and T-cell IHC markers which confirmed the lesion to be high-grade large-cell lymphoma (double-expresser type) presenting as linitis plastica. Thus, it can be concluded that IHC markers study is necessary for definitive diagnosis.

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Conflicts of interest

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  References Top

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Lyle HH. VIII. Linitis plastica (cirrhosis of stomach): With a report of a case cured by gastro-jejunostomy. Ann Surg 1911;54:625-68.  Back to cited text no. 10
Stout AP. Tumors of the Stomach. Washington: Armed Forces Institute of Pathology; 1953.  Back to cited text no. 11
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Yashiro M, Hirakawa K. Cancer-stromal interactions in scirrhous gastric carcinoma. Cancer Microenviron 2010;3:127-35.  Back to cited text no. 14
Inoue T, Chung YS, Yashiro M, Nishimura S, Hasuma T, Otani S, et al. Transforming growth factor-beta and hepatocyte growth factor produced by gastric fibroblasts stimulate the invasiveness of scirrhous gastric cancer cells. Jpn J Cancer Res 1997;88:152-9.  Back to cited text no. 15
Agnes A, Estrella JS, Badgwell B. The significance of a nineteenth century definition in the era of genomics: Linitis plastica. World J Surg Oncol 2017;15:123.  Back to cited text no. 16
Doglioni C, Ponzoni M, Ferreri AJ, Savio A, Gruppo Italiano Patologi Apparato Digerente (GIPAD), Società Italiana di Anatomia Patologica e Citopatologia Diagnostica/International Academy of Pathology, Italian division (SIAPEC/IAP), et al. Gastric lymphoma: The histology report. Dig Liver Dis 2011;43 Suppl 4:S310-8.  Back to cited text no. 17
Horn H, Ziepert M, Becher C, Barth TF, Bernd HW, Feller AC, et al. MYC status in concert with BCL2 and BCL6 expression predicts outcome in diffuse large B-cell lymphoma. Blood 2013;121:2253-63.  Back to cited text no. 18
Swerdlow SH. Diagnosis of 'double-hit' diffuse large B-cell lymphoma and B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma: When and how, FISH versus IHC. Hematol Am Soc Hematol Educ Program 2014;2014:90-9.  Back to cited text no. 19
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13], [Figure 14]


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