LGCmain
Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 206
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size


 
  Table of Contents    
CASE REPORT  
Year : 2021  |  Volume : 64  |  Issue : 5  |  Page : 89-91
Malignant gastrointestinal stromal tumor in association with Russell body gastritis—A case report


1 Department of General and Clinical Pathology, Medical University of Plovdiv; UMHAT “Kaspela”, Plovdiv, Bulgaria
2 Department of General and Clinical Pathology, Medical University of Plovdiv; St. George University Hospital, Plovdiv, Bulgaria
3 Department of General and Clinical Pathology, Medical University of Plovdiv, Plovdiv, Bulgaria; Service d'Anatomie et Cytologie Pathologiques, Grand Hôpital de l'Est Francilien, Jossigny, France

Click here for correspondence address and email

Date of Submission27-Oct-2019
Date of Decision12-Dec-2019
Date of Acceptance06-Jan-2020
Date of Web Publication7-Jun-2021
 

   Abstract 


Russell body gastritis (RBG) is an unusual form of chronic inflammation characterized by accumulation of plasma cells containing Russell bodies (RB) in the gastric mucosa. Although its pathogenesis has not been fully evaluated, there is evidence to support a strong association with Helicobacter pylori infection. Only four cases of RBG in association with malignant epithelial gastric tumors were reported. We report the first case of RBG in peritumoral mucosa of a malignant gastrointestinal stromal tumor in association with coccoid form of Helicobacter pylori and a follow-up.

Keywords: Gastrointestinal stromal tumors, Helicobacter pylori, plasma cells, Russell body gastritis, stomach

How to cite this article:
Bozhkova DM, Koleva-Ivanova MS, Belovejdov VT, Dikov DI. Malignant gastrointestinal stromal tumor in association with Russell body gastritis—A case report. Indian J Pathol Microbiol 2021;64, Suppl S1:89-91

How to cite this URL:
Bozhkova DM, Koleva-Ivanova MS, Belovejdov VT, Dikov DI. Malignant gastrointestinal stromal tumor in association with Russell body gastritis—A case report. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 Jun 23];64, Suppl S1:89-91. Available from: https://www.ijpmonline.org/text.asp?2021/64/5/89/317929





   Introduction Top


Russell bodies (RB), the condensed immunoglobulin (Ig) variants in plasma cells, are frequently observed in chronic infections, plasma cell dyscrasias, and autoimmune diseases. Small number of RB are often observed in the mucosa in H. pylori gastritis, but long-standing overstimulation by H. pylori can result in diffuse infiltration of plasma cell with RB. The term “Russell body gastritis” (RBG) has first been used by Tazawa K et al. to describe accumulation of RB containing plasma cells in gastric mucosa with H. pylori infection.[1] To date, only four cases of RBG and malignant gastric tumors of epithelial origin were reported. There are no data concerning the association between RBG and mesenchymal tumors in the literature. We report the first case of H. pylori-associated RBG in peritumoral mucosa of a malignant gastrointestinal stromal tumor (GIST) with a follow-up. In addition, in the reported case RBG was associated with coccoid form of H. pylori.


   Case History Top


A 60-year-old woman presented with abdominal discomfort and a partial resection of the stomach was performed due to abnormal gastric lesion. Macroscopically the gastric fragment was with a length of 5 cm on the small stomach curvature, 17 cm on the large curvature, and 7 cm thick. A nodular tumor formation at the level of the small curvature with dimensions 6 × 6× 1 cm was observed. A fragment of omentum with dimensions 25 × 25 × 1 cm was also submitted for investigation.

Histologically a tumor mass was found, composed of spindle cells with hyperchromatic nuclei, showing 25 mitosis/50 HPF [Figure 1]a. Immunohistochemically, the tumor cells showed positive expression of c-kit [Figure 1]b and DOG1 (data not shown). The final diagnosis was malignant gastrointestinal stromal tumor–spinde cell histological type, with high risk of progression according to the classification of Miettinen et al. Lasota, pT3 pN0 staging in TNM (AJCC, 2017). There was no evidence of carcinomatous lymphangitis and vascular tumor embolism. Infiltration of visceral peritoneum was observed. The excision was complete with longitudinal margins of 3 cm. Peritumoral lymph nodes were negative: 15 N-/15.
Figure 1: (a) Malignant GIST with chronic gastritis in the adjacent mucosa, HE, ×50. (b) Malignant GIST: Tumor cell with positive immunohistochemical reaction for c-kit, c-kit ×200

Click here to view


In the peritumoral mucosa, especially in the fundus, chronic gastritis was found, rich in RB, so-called RBG. The inflammatory infiltrate was represented by quite a number of plasma cells and Mott cells with areas consisting entirely of RB [Figure 2]a and [Figure 2]b. Several hyperplastic lymphoid follicles were also observed. No neutrophilic leucocytes were found.
Figure 2: (a) RB gastritis: Gastric mucosa densely infiltrated by Russell body-containing plasma cells, HE, ×200. (b) Higher magnification of RB gastritis with Mott cells: HE, ×400. (c) Plasma cells with positive expression of CD79a, CD79a, ×200. (d) Positive immunohistochemical reaction in plasma cells with kappa, kappa, ×630. (e) Positive immunohistochemical reaction in plasma cells with lambda, lambda, x630, IgM. (f) Presence of coccoid form of H pylori. IHC Hp, ×200

Click here to view


In fundic mucosa plasma cells expressed CD79a [Figure 2]c, CD138 (data not shown), kappa [Figure 2]d, and IgM lambda [Figure 2]e.

After additional immunohistochemical examination in the area of the peritumoral mucosa the presence of H. pylori in coccoid form was found [Figure 2]f.

For the needs of differential diagnosis of RBG and signet ring cell carcinoma we performed simultaneous PAS staining and cytokeratin immunostaining. RB-containing plasma cells were PAS positive [Figure 3]a and cytokeratin AE1/AE3 negative [Figure 3]b.
Figure 3: Russell body-containing plasma cells are PAS positive (a) and cytokeratin AE1/AE3 negative (b), ×400

Click here to view


The first follow-up gastroscopy with a biopsy was performed 8 months after the surgical intervention, treatment with immunotherapy, and H. pylori eradication. Normal gastrojejunal anastomosis with focal foveolar hyperplasia was found. The upper 1/3 of the stomach was presented by a normal fundus. In cardia the presence of mild inflammatory infiltrate without activity with single RB and focal intestinal metaplasia without dysplasia (data not shown) was observed. There was no evidence of a tumor process or H. pylori. The second and the third follow-up gastroscopy were performed 4 and 5 years after the surgical intervention, with no evidence of disease.


   Discussion Top


Since the introduction of the term RBG in the literature were published 42 cases. 27 of them were associated with H. pylori infection.[2],[3] In the presented case we proved the presence of H. pylori infection by immunohistochemical staining. We found that H. pylori was of coccoid form. When exposed to environmental stress conditions, H. pylori is able to change its morphology from a spiral to coccoid form. This form contributes to treatment failures and recurrence. The form of H. pylori in RBG was not mentioned in previous studies and might be an interesting fact for correlation.

Four cases of total 42 cases of RB gastritis described in the literature were associated with gastric carcinomas in the adjacent tissues—tree cases of adenocarcinoma[3],[4],[5] and one of signet ring cell carcinoma.[6]

Although it is now well established that H. pylori infection predisposes individuals toward gastric adenocarcinoma later in life, the relationship between RBG and gastric carcinoma remains unclear. The significantly higher density of plasma cells containing RB in gastric biopsies of patients with gastric carcinoma in comparison with patients without gastric carcinoma led to the statement that these cells could be important for adenocarcinoma development.[7] There are reports that suggest association between the production of chemokines in tumor cells and the development of RB.[8]

The association of Helicobacter pylori (HP) with gastritis, gastric dysplasia, gastric carcinoma, and gastric lymphoma (mucosa-associated lymphoid tissue) has been previously demonstrated. However, the association of HP with GISTs has not been well explored. According to some studies there is a prevalence of patients with GIST in a combination with H. pylori gastritis: 62.9% of cases with GIST were positive for H. pylori.[9] Other studies suggest that gastric GIST arises in cases of nonatrophic or mildly atrophic gastric mucosa with no prevalence of H. pylori infection: 50% of the patients with GIST had evidence of H. pylori.[10] The literature analysis showed diverse results—some investigations report prevalence of H. pylori infection in cases with GIST, while others suggest no connection between these two entities. More studies are needed to establish the association of HP with GISTs. In the presented case we proved the presence of H. pylori infection, which might be in connection with RB gastritis rather than with the malignant GIST.

As in our case, larger tumors present with vague abdominal discomfort. The complications include chronic gastrointestinal bleeding, intestinal obstruction or altered bowel habits, rarely perforation.[11]

In one study, a total of 15 cases (15/30) of RBG with polyclonal plasma cells, containing RB (Mott cells) have been described.[2] Our case also showed polyconal proliferation of plasma cells with RB with uneventful clinical followup. The decreased number of Mott cells in the stomach after H. pylori eradication shows that the factor causing RB formation is H. pylori. Future studies are needed to investigate the distinction between RBG with monoclonal Mott cells and plasmacytoma or mucosa-associated lymphoid tissue (MALT)-lymphoma. RBG can be differentiated from MALT lymphoma by the plasmacytic differentiation with the absence of cytologic atypism, lymphoepithelial lesions, centrocyte-like cells, and monocytoid cells.[12]

Diffuse infiltration of plasma cells with RB in the gastric mucosa requires differential diagnosis with several diseases. Cytokeratin negativity and CD79a and CD138 positivity exclude signet ring cell carcinoma of the stomach. The simultaneous performing of PAS or PAS-AB staining and cytokeratin immunostaining in such cases has significant role for setting the correct diagnosis (unpublished own data). Kappa and lambda polyclonal immunoreactive pattern excludes lymphoplasmacytic lymphoma, plasmacytoma, and monoclonal gammopathy of undetermined significance.


   Conclusion Top


Little is known about the connection between RBG and malignant epithelial or mesenchymal gastric tumors. We report the first case of RBG in peritumoral mucosa of a malignant GIST.

The association between RBG and H. pylori infection remains unclear, although the majority of the reported cases showed H. pylori positivity. In our case we presented a rare combination of RBG with coccoid form of H. pylori. Further case reports or large series investigations are required in order to prove or reject the correlation between RBG and H. pylori infection and the connection between RBG and stomach malignancies.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Tazawa K, Tsutsumi Y. Localized accumulation of RB containing plasma cells in gastric mucosa with Helicobacter pylori infection: 'Russell body gastritis'. Pathol Int 1998;48:242-4.  Back to cited text no. 1
    
2.
Yorita K, Iwasaki T, Uchita K, Kuroda N, Kojima K, Iwamura S, et al. Russell body gastritis with Dutcher bodies evaluated using magnification endoscopy. World J Gastrointest Endosc 2017;9:417-24.  Back to cited text no. 2
    
3.
Altindag SD, Cakir E, Ekinci N, Avci A, Dilek FH. Analysis of clinical and histopathological findings in Russell body gastritis and duodenitis. Ann Diagn Pathol 2019;40:66-71.  Back to cited text no. 3
    
4.
Altun E, Turhan G, Taskin M. Russell body cervicitis: A case report and literature review. Med Sci Int Med J 2018;7:225-8.  Back to cited text no. 4
    
5.
Choi J, Lee H, Byeon S, Nam K, Kim M, Kim W. Russell body gastritis presented as a colliding lesion with a gastric adenocarcinoma: A case report. Basic Appl Pathol 2012;5:54-7.  Back to cited text no. 5
    
6.
Wolf EM, Mrak K, Tschmelitsch J, Langner C. Signet ring cell cancer in a patient with Russell body gastritis – A possible diagnostic pitfall. Histopathology 2011;58:1178-80.  Back to cited text no. 6
    
7.
Johansen A, Sikjär B. The diagnostic significance of Russell bodies in endoscopic gastric biopsies. Acta Pathol Microbiol Scand A 1977;85:245-50.  Back to cited text no. 7
    
8.
Takahashi Y, Shimizu S, Uraushihara K, Fukusato T. Russell body duodenitis in a patient with retroperitoneal metastasis of ureteral cancer. World J Gastroenterol 2013;19:125-8.  Back to cited text no. 8
    
9.
Nonaka K, Ban S, Hiejima Y, Narita R, Shimizu M, Aikawa M. Status of the gastric mucosa with endoscopically diagnosed gastrointestinal stromal tumor. Diagn Ther Endosc 2014:429761.  Back to cited text no. 9
    
10.
Raza M, Shahab A, Paul Mazzara P. Gastrointestinal stromal tumors: Association with Helicobacter pylori gastritis. Am J Clin Pathol 2012;138(Suppl 1):A351.  Back to cited text no. 10
    
11.
Jagtap SV, Nikumbh DB, Kshirsagar AY, Bohra A, Khatib W. Malignant gastrointestinal stromal tumor of the sigmoid colon with perforation and peritonitis - An unusual presentation. Int J Health Sci Res 2012;2:104-9.  Back to cited text no. 11
    
12.
Midi A, Ataizi ÇC, Kaya H. “Russell body” gastritis: A case report. Turk J Pathol 2010;26:159-61.  Back to cited text no. 12
    

Top
Correspondence Address:
Desislava M Bozhkova
Department of General and Clinical Pathology, Medical University – Plovdiv, Vassil Aprilov Blvd., Plovdiv
Bulgaria
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_842_19

Rights and Permissions


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case History
   Discussion
   Conclusion
    References
    Article Figures

 Article Access Statistics
    Viewed124    
    Printed2    
    Emailed0    
    PDF Downloaded7    
    Comments [Add]    

Recommend this journal