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  Table of Contents    
CASE REPORT  
Year : 2014  |  Volume : 57  |  Issue : 2  |  Page : 326-328
Enteropathy associated T cell lymphoma-monomorphic variant, presenting as bilateral ovarian masses


1 Department of Pathology, Regional Cancer Centre, Trivandrum, Kerala, India
2 Centre for Excellence in Histopathology, SRL Diagnostics, Mumbai, Maharashtra, India
3 Department of Surgical Oncology, Regional Cancer Centre, Trivandrum, Kerala, India

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Date of Web Publication19-Jun-2014
 

   Abstract 

Enteropathy associated T cell lymphoma (EATL) is a rare type of T-cell lymphoma, often associated with a history of celiac disease. It usually arises in the jejunum, but can involve other gastrointestinal tract sites such as stomach and colon. Monomorphic variant of EATL often occurs without a history of celiac disease, has variable histologic evidence of enteropathy, and is usually CD56 + . We report a case of EATL in a 49-year-old female presenting as bilateral ovarian masses. The morphology and immunophenotypic features were compatible with monomorphic variant of EATL.

Keywords: Enteropathy associated T cell lymphoma, ovary, monomorphic variant

How to cite this article:
Jacob PM, Nair RA, Mehta J, Borges AM, Suchetha S. Enteropathy associated T cell lymphoma-monomorphic variant, presenting as bilateral ovarian masses. Indian J Pathol Microbiol 2014;57:326-8

How to cite this URL:
Jacob PM, Nair RA, Mehta J, Borges AM, Suchetha S. Enteropathy associated T cell lymphoma-monomorphic variant, presenting as bilateral ovarian masses. Indian J Pathol Microbiol [serial online] 2014 [cited 2021 Feb 24];57:326-8. Available from: https://www.ijpmonline.org/text.asp?2014/57/2/326/134733



   Introduction Top


Lymphomas presenting with primary female genital tract (FGT) symptomatology or involving the FGT at initial presentation are unusual. Most reported cases are of the B cell type. Only four cases of ovarian T cell lymphoma have been reported in the literature. [1] To the best of our knowledge, this is the first case of enteropathy associated T cell lymphoma (EATL) presenting as bilateral ovarian masses. The monomorphic variant is by itself is very rare and comprises 10-20% of all EATLs. [2]


   Case report Top


The case is that of a 49-year-old female presenting with symptoms of loss of appetite and mild gastrointestinal symptoms of 1 month duration. The patient denied any family history of gastrointestinal or autoimmune diseases. Physical examination was unremarkable. The gastrointestinal work-up, including upper endoscopy and colonoscopy was unremarkable. Laboratory examination revealed hemoglobin of 12.3 g/dL, white blood cell count of 10.9 × 10 9 /L and a platelet count of 594 × 10 9 /L. An ultrasound abdomen revealed bilateral ovarian masses. Postcontrast axial computed tomography sections of the abdomen and pelvis with coronal reformats were performed, which revealed bilateral predominantly solid ovarian mass lesions [Figure 1]a]. Small bowel thickening was noted in the left iliac fossa with adjacent spiculations in the mesentery - suggestive of desmoplastic reaction [Figure 1]b]. Among the tumor markers, cancer antigen-125 = 298 u/mL (normal range: 0-35 U/mL) and carcinoembryonic antigen = 1.43 mcg/L (normal range: 0-2.5 mcg/L). An exploratory laparotomy was done with bilateral ovarian mass excision, mesenteric lymph node biopsy and omental biopsy. Peroperatively, bilateral ovaries were enlarged by solid cystic tumors, 5 cm × 4 cm on the left side and 6 cm × 4 cm on the right side. The uterus appeared normal. Small bowel loops were dilated in its entire length. Multiple intraluminal tumors were palpable in the small bowel with serosal involvement and desmoplastic reaction. The most distal tumor was palpated around 20 cm from the ileocecal junction. Multiple mesenteric lymphnodes were present. There was no ascites. Frozen section of the ovary was reported as poorly differentiated malignant neoplasm possibly lymphoma, with differential diagnosis of metastatic lobular breast carcinoma. The resected specimen contained a right sided ovarian mass measuring 5 cm × 5 cm × 3 cm, left sided ovarian mass measuring 4.5 cm × 3 cm × 3cm both with an intact capsule. A portion of the  Fallopian tube More Details, mesentery and a lymph node were also submitted. The cut section of both ovarian masses was grey white, slimy, and edematous [Figure 2]. Histologically, the tumor cell infiltrate was a diffuse and monotonous population of small to medium sized lymphoid cells with round nuclei, condensed chromatin, and inconspicuous nucleoli [Figure 3]a-c]. Immunohistochemical staining of the tumor cells revealed positivity for CD45, CD3, CD7, CD8, CD43, granzyme and CD56 (focally). CD4, CD5, CD25, CD20, PAX5, CD34, TdT, MPO, CD33, CD68, CD117 and CD11c were negative. Epstein-Barr virus was negative by EBER transcript [Figure 3]d-h]. The morphology and immunophenotypic features are compatible with monomorphic variant of EATL. Correlating with radiological evidence of small bowel thickening and desmoplastic reaction, and intraoperative evidence of multiple intraluminal tumors palpated in the small bowel with serosal involvement and desmoplastic reaction, a diagnosis of monomorphic variant of EATL involving bilateral ovaries and fallopian tube was made.
Figure 1: Postcontrast axial computed tomography sections of the abdomen and pelvis with coronal reformats. (a) Bilateral predominantly solid ovarian mass lesions. (b) Small bowel thickening in the left iliac
fossa with adjacent spiculations in the mesentery - suggestive of desmoplastic reaction


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Figure 2: Gross appearance of bilateral ovarian masses. Cut section is grey white and edematous

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Figure 3: (a) H and E, ×100 section from ovary showing diffuse infiltrate of tumor cells. (b) H and E, ×200 monomorphic tumor cells. (c) H and E, ×400 tumor cells are intermediate sized with scanty
cytoplasm, round nuclei and inconspicuous nucleoli. (d-h) The tumor cells are CD3 positive, CD5 negative, CD7 positive, CD8 positive, CD56 focally positive


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   Discussion Top


Malignant lymphoma, especially peripheral T/natural killer (NK)-cell lymphoma, often present in unusual locations, which may lead to misdiagnosis of such tumors, causing delayed correct diagnosis, and inappropriate management of patients. The involvement of the FGT can present either as a primary lesion or as a focus of involvement in cases of disseminated lymphomas. Indeed, secondary involvement of the FGT was reported to occur in as many as 40% of cases of disseminated lymphomas in an article published in 1961. [3] However, a literature review shows that lymphomas presenting with primary FGT symptomatology or involving the FGT at initial presentation are unusual. Most of the cases previously reported are of B-cell type, although immunophenotypic studies were done on a limited number of cases in prior studies. To the best of our knowledge, only four cases of ovarian T-cell lymphoma have been reported in the English literature. In the first two cases, their clinicopathologic and immunophenotypic evaluations differed somewhat from what is practiced today. [4],[5] A study by Shigeo Nakamura et al. (2001), documents five cases of peripheral T/NK-cell lymphomas affecting the FGT of which two were ovarian masses and one was bilateral. [1]

EATL is an uncommon intestinal tumor arising from T lymphocytes that reside in intraepithelial space, [6] comprising <1% of non-Hodgkin's lymphoma cases [7] and accounts for fewer than 5% of all gastrointestinal tract lymphomas. [2] The intestinal intraepithelial T lymphocytes supposed to be a normal counterpart of EATL have dendritic morphology and express surface CD3 and CD8, but not CD4 antigens. The subtype of T cell receptor expressed on intestinal intraepithelial T lymphocytes is mostly gamma delta. The main function of these T cells is to repair damaged intestinal epithelial cells. The median age of patients with EATL at the time of diagnosis is 60 years. Men and women are affected with similar frequency. [8] EATL most commonly arises in the proximal jejunum and less frequently elsewhere in the small intestine, stomach, or colon. In relation to the growth site of the tumor, EATL patients frequently complain of appetite loss or abdominal pain. Septicemia due to acute peritonitis accounts for the dismal clinical prognosis in these cases. In patients without intestinal perforation at diagnosis, common clinical symptoms are abdominal pain, weight loss, malabsorption, and protein losing enteropathy. [9] At disease manifestation, approximately two-thirds of EATL cases are limited to the gastrointestinal tract and mesenteric lymph nodes. In the course of the disease, dissemination can occur, with spread to the liver, spleen, skin, and other organs. Chott et al. classified EATL into two types or histological variants. [10],[11] Pleomorphic anaplastic EATL is usually associated with a history of celiac disease, histologic evidence of enteropathy and the tumor cells are morphologically heterogeneous and positive for CD3 and CD7, but negative for CD4, CD8, or CD56. Monomorphic EATL which comprises 10-20% of EATL cases, often occurs without a history of celiac disease, has variable histologic evidence of enteropathy, and is usually CD56 + and CD8 + . [2]


   Conclusion Top


In the last decade, much interest has been focused on the clinicopathological and biological features of extranodal NK/T cell lymphomas and several site specific entities such as hepatosplenic gamma delta T cell lymphoma, nasal type NK/T cell lymphoma, and EATL. These entities appear to be closely linked with their unusual anatomic sites. When these tumors arise in other areas, they pose a diagnostic challenge and need a multidisciplinary approach to arrive at a diagnosis. A single missing piece in the puzzle can change the picture altogether.


   Acknowledgments Top


The author would like to thank Dr. Anila KR for providing frozen section details and also Dr. Sumod Mathew Koshy for contributing radiological images.

 
   References Top

1.Nakamura S, Kato M, Ichimura K, Yatabe Y, Kagami Y, Suzuki R, et al. Peripheral T/natural killer-cell lymphoma involving the female genital tract: A clinicopathologic study of 5 cases. Int J Hematol 2001;73:108-14.  Back to cited text no. 1
    
2.Zettl A, deLeeuw R, Haralambieva E, Mueller-Hermelink HK. Enteropathy-type T-cell lymphoma. Am J Clin Pathol 2007;127:701-6.  Back to cited text no. 2
    
3.Rosenberg SA, Diamond HD, Jaslowitz B, Craver LF. Lymphosarcoma: A review of 1269 cases. Medicine (Baltimore) 1961;40:31-84.  Back to cited text no. 3
[PUBMED]    
4.Lamoureux D, Daya D, Simon GT. Cell junctions in lymphomas: Study of a primary ovarian T-cell lymphoma and review of fifty-six other cases of lymphoma. Ultrastruct Pathol 1990;14:247-52.  Back to cited text no. 4
    
5.Monterroso V, Jaffe ES, Merino MJ, Medeiros LJ. Malignant lymphomas involving the ovary. A clinicopathologic analysis of 39 cases. Am J Surg Pathol 1993;17:154-70.  Back to cited text no. 5
    
6.Sieniawski M, Angamuthu N, Boyd K, Chasty R, Davies J, Forsyth P, et al. Evaluation of enteropathy-associated T-cell lymphoma comparing standard therapies with a novel regimen including autologous stem cell transplantation. Blood 2010;115:3664-70.  Back to cited text no. 6
    
7.Gale J, Simmonds PD, Mead GM, Sweetenham JW, Wright DH. Enteropathy-type intestinal T-cell lymphoma: Clinical features and treatment of 31 patients in a single center. J Clin Oncol 2000;18:795-803.  Back to cited text no. 7
    
8.Paul WE. Fundamental Immunology. 6 th ed. Philadelphia: Lippincott Williams & Wilkins; 2008. p. 989-91.  Back to cited text no. 8
    
9.Kim JB, Kim SH, Cho YK, Ahn SB, Jo YJ, Park YS, et al. A case of colon perforation due to enteropathy-associated T-cell lymphoma. World J Gastroenterol 2013;19:1841-4.  Back to cited text no. 9
    
10.Chott A, Vesely M, Simonitsch I, Mosberger I, Hanak H. Classification of intestinal T-cell neoplasms and their differential diagnosis. Am J Clin Pathol 1999;111:S68-74.  Back to cited text no. 10
    
11.Chott A, Haedicke W, Mosberger I, Födinger M, Winkler K, Mannhalter C, et al. Most CD56+ intestinal lymphomas are CD8+CD5-T-cell lymphomas of monomorphic small to medium size histology. Am J Pathol 1998;153:1483-90.  Back to cited text no. 11
    

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Correspondence Address:
Rekha A. Nair
Department of Pathology, Regional Cancer Centre, Trivandrum - 695 011, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.134733

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    Abstract
   Introduction
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