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Year : 2021  |  Volume : 64  |  Issue : 1  |  Page : 168-170
Primary anaplastic large cell lymphoma of caecum and ascending colon

1 Department of Pathology and Cytology, Aware Gleneagles Global Hospital, Hyderabad, Telangana, India
2 Department of Gastroenterology and Hepatology, Aware Gleneagles Global Hospital, Hyderabad, Telangana, India

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Date of Submission21-Mar-2019
Date of Decision02-Jun-2019
Date of Acceptance06-Jun-2019
Date of Web Publication8-Jan-2021


Lymphomas are hematological malignancies with a wide variety of histological subtypes, varied clinical manifestations and behaviour and have a wide range of organ involvement. About 40 per cent of lymphomas are extra nodal. The most common extra nodal site is gastrointestinal tract (GIT). In the GIT, stomach is the most common organ involved accounting for 50–60 per cent of the lesions. Colorectal lymphomas are rare and account for 15–20 per cent of GIT lymphomas. They constitute 1 per cent of colorectal malignancies. Most common histological type of lymphoma involving GIT is diffuse large B-cell lymphoma, followed by MALT lymphoma; T-cell lymphomas are very rare and have an incidence of 3 per cent of Non Hodgkins Lymphoma (NHL). We report a case of anaplastic large cell lymphoma in the caecum and ascending colon with review of literature.

Keywords: ALK-1 positive, anaplastic large cell lymphoma, ascending colon, caecum

How to cite this article:
Radha S, Afroz T, Reddy R. Primary anaplastic large cell lymphoma of caecum and ascending colon. Indian J Pathol Microbiol 2021;64:168-70

How to cite this URL:
Radha S, Afroz T, Reddy R. Primary anaplastic large cell lymphoma of caecum and ascending colon. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 May 10];64:168-70. Available from: https://www.ijpmonline.org/text.asp?2021/64/1/168/306500

   Introduction Top

Primary colonic lymphomas are more common in men. The commonest site of involvement in colon is caecum because of rich lymphoid tissue. Intestinal lymphomas are commonly B-cell type. T-cell lymphomas are rare and the ratio of B- to T-cell lymphomas is 6:1.[1] There are two subsets of systemic anaplastic large cell lymphoma (ALCL) in WHO classification. One subset expresses the oncogenic protein anaplastic lymphoma kinase (ALK) and the other is ALK negative. ALK positive ALCL patients have a better prognosis of 79.8 per cent five-year survival rate compared to 32.9 per cent for ALK negative patients. The peak incidence is fourth to sixth decades. The case under discussion is a young female. The most common extra nodal site (s) of involvement in ALCL is/are skin, bone and/or soft tissues. We present a rare case of ALK positive ALCL in young female involving caecum and ascending colon.

   Case History Top

A 22-year-old unmarried lady presented with complaints of fever of one-month duration, history of abdominal distension and anorexia of four–five days duration. Fever was continuous low grade not associated with chills or rigors. There were no complaints of altered bowel habits. On examination, she was a moderately built lady who was febrile. She was pale, no icterus or clubbing noted. There was no peripheral lymphadenopathy. Her heart and lungs were clear. There was rigidity and vague mass in the right side of abdomen.

Her routine hematological tests revealed normocytic normochromic anemia, there was neutrophil leukocytosis. No abnormal cells were seen. Platelets were adequate. Viral markers including HIV were negative. Liver and renal function tests were normal. Lactic dehydrogenase was 295 u/l and uric acid was 7.4 mg/dl. Chest X-ray was normal. Computed tomography scan (CT scan) of abdomen revealed thickened caecum and ascending colon with minimal fat stranding. No lymph nodes noted. Liver and spleen were normal.

In view of CT scan findings for further evaluation, a colonoscopy was done. Colonoscopy revealed oedematous mucosa with nodularity in caecum and ascending colon [Figure 1]a. Biopsy was taken from the nodular lesions in caecum and ascending colon.
Figure 1: (a) Colonoscopy reveals edematous mucosa with nodularity. (b) H and E stain shows diffuse infiltrate in the lamina propria separating the crypts. (×200). (c) H and E stains shows diffuse infiltrate comprising of round to polygonal cells with anisonucleosis with coarse nuclear chromatin. (×400). (d) Cytokeratin stain highlighting the tubular crypts. (×200)

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Histopathology revealed fragments of colonic mucosa with sheets of cells in lamina propria. These cells are large, round to oval with scanty cytoplasm [Figure 1]b and [Figure 1]c. There is moderate anisonucleosis with coarse chromatin. These cells are CD45 positive [Figure 2]a, CD20 and CD 3 negative. CD30 positive [Figure 2]b, ALK-1 positive [Figure 2]c, PAX-5 and CD43 negative [Figure 2]d Ki 67 was 90 per cent. Cytokeratin was negative in the cells in the lamina propria but clearly highlighted the tubular crypts [Figure 1]d. Based on the morphology and immunohistochemistry, a diagnosis of ALK-1 positive A large cell lymphoma was given, favouring null cell type as T-cell markers were negative.
Figure 2: (a) Diffuse CD45 positivity (×200). (b) Infiltrate in lamina propria shows diffuse C30 positivity (×200). (c) Infiltrate in lamina propria shows diffuse ALK-1 positivity (×200). (d) Negative CD43 in the lamina propria infiltrate (×200)

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Morphological differential diagnosis includes carcinoma, melanoma, myeloid sarcoma and lymphoma. Amongst lymphomas, diffuse large B-cell lymphoma (DLBCL) and Hodgkin's lymphoma-classical type are a close differential diagnosis.

Hodgkin's lymphoma is CD30 and PAX-5 positive. In DLBCL, CD20 is strongly positive and PAX-5 is positive.

In the case under discussion, CD20 and PAX-5 were negative. ALK-1 was positive. A bone marrow aspiration and biopsy done was negative for any lymphoma deposits. Patient is on regular follow up. She is doing well after two cycles of chemotherapy. On her last visit, ultrasound showed mild pleural effusion and few small abdominal lymph nodes.

   Discussion Top

Gastro intestinal tract (GIT) is rich in lymphoid tissue. It starts in the Waldeyer's ring and extends up to rectum. Only exceptions are oesophagus and stomach, which do not have prominent lymphoid tissue under normal conditions. A spectrum of cells is seen in this lymphoid tissue, which includes B lymphocytes, plasma cells and subsets of T lymphocytes. This diversity reflects the wide range of lymphoproliferative disorders in GIT. GIT is the most common site of extra nodal lymphomas accounting for 5–20 per cent of all extra nodal lymphomas. In GIT, stomach is commonly involved in 50–60 per cent of times, small intestine in 20– 30 per cent and colorectal 15–20 per cent.[2] Primary colorectal lymphomas account for 1.4 per cent of all non- Hodgkin's lymphomas and less than 1 per cent of colorectal malignancies.[3]

The most common histological subtype of GIT lymphomas is B cell type and frequency is up to 81 per cent.[4] In a study by Anderson et al., non-Hodgkin's lymphoma subtypes varied with geographical distribution because of variation in etiological factors.[5] According to this study, T- cell lymphomas are more frequent in east and the incidence varied from 17.9 to 42 per cent. The mean age of presentation is 55 yrs. Males are affected more than females.[6] The case under discussion is a variation in that it is seen in 22-year-old female. The most common symptoms of colorectal lymphomas are weight loss, abdominal pain and changing bowel habits. Lower gastro intestinal bleeding is seen in 13–82 per cent of cases. Endoscopically, these are either ulcerative or polypoidal lesions. In this case, a diagnosis of primary ascending colon lymphoma is made according to Dawson's criteria.[7] These include 1) no enlarged superficial nodes when patient is first seen, 2) chest radiographs without obvious mediastinal enlarged nodes. 3) White blood cell total and differential counts are within normal range and bone marrow biopsy is normal. 4) At laparotomy, only regional nodes are seen. 5) Liver and spleen are free of tumour. Our patient fulfilled all the criteria. Patient had mild neutrophilic leukocytosis probably because of fever.

In 2003, Korl et al.[8] gave a liberal definition of extra nodal lymphoma, which states that extra nodal lymphomas include all patients with non-Hodgkin's lymphomas that originated from extra nodal site, even in the presence of disseminated disease as long as extra nodal component is dominant. Etiological factors of primary colorectal lymphomas are unknown. These are the most frequent in immunosuppressed conditions. They are seen in ulcerative colitis, HIV and after organ transplantation. ALCL was recognised by Stein et al. ALCL accounts for 2 per cent of large cell NHL, 3 per cent of adult NHL and 10–20 per cent of childhood NHL. The most common extra nodal site of ALCL is skin which occurs in 10–20 per cent of cases. Other sites include lung, liver, spleen, bone,[9] and pleura. ALCL expresses ALK by IHC and is related to t (2;5) P (23; q35) chromosomal translocation on ALK gene on chromosome 2. ALK positive lymphoma have a better five-year survival when compared to ALK negative lymphomas.

Conclusions: We present an unusual case of primary caecal and ascending colon ALK positive ALCL in a young female. It is important to consider this entity in the differential diagnosis to give appropriate therapy and avoid unnecessary radical surgeries.

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.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Howell JM, Auer-Grzesiak I, Zhang J, Andrews CN, Stewart D. Increasing incidence rates, distribution and histological characteristics of primary Non-Hodgkin's lymphoma in North American population. Can J Gastroenterol 2012;26:452–6.  Back to cited text no. 1
Koch P, del Valle F, Berdel WE, Willich NA, Reers B, Hiddemann W, et al. Primary gastrointestinal lymphoma: 1. Anatomic and histologic distribution, clinical features and survival data of 371 patients registered in German multicenter study GIT NHL 01/92. J Clin Oncol 2001;19:3861–73.  Back to cited text no. 2
Fan CW, Changchien CR, Wang JY, Chen JS, Hsu KC, Tang R, et al. Primary colorectal lymphoma. Dis Colon Rectum 2000;43:1277–82.  Back to cited text no. 3
Cai S, Cannizzo F Jr, Bullard Dunn KM, Gibbs JF, Czuczman M, Rajput A. The role of surgical intervention in non-Hodgkin's lymphoma of the colon and rectum. Am J Surg 2007;193:409–12.  Back to cited text no. 4
Anderson JR, Armitage JO, Weisenburger DD. Epidemiology of the non-Hodgkin's lymphoma: Distribution of the major subtypes differ by geographic locations. Non Hodgkin's lymphoma classification project. Ann Oncol 1998;9:717–20.  Back to cited text no. 5
Kim YH, Lee JH, Yang SK, Kim JS, Kim HJ, Kim JI, et al. Primary colon lymphoma in Korea. Dig Dis Sci 2005;50:2243–7.  Back to cited text no. 6
Dawson JM, Cornes JS, Morson BC. Primary malignant lymphoid tumors of the intestinal tract. Report of 37 cases with a study of factors influencing prognosis. Br J Surg 1961;49:80–9.  Back to cited text no. 7
Krol AD, le Cessie S, Snijder S, Kluin-Nelemans JC, Kluin PM, Noordijk EM. Primary extra nodal non-Hodgkin's lymphoma. Ann Oncol 2003;14:131–9.  Back to cited text no. 8
Griesser H, Henry M, Boie C, Banerjee D. Large-cell anaplastic lymphoma of the gastrointestinal tract: An immune and genotypic study of archival material. Hematol Pathol 1994;8:121–34.  Back to cited text no. 9

Correspondence Address:
Sistla Radha
Plot No 20, Road No 1, Alakapuri, Hyderabad - 500 035, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_225_19

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