|Ahead of print publication
Unusual solitary rectal relapse of mantle cell lymphoma after second remission
Anthialisha Nongkynrih1, Vikas Jagtap1, Sumit Kumar1, Navin Nayan1, Biswajit Dey2, Vandana Raphael2
1 Department of Radiation Oncology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, Meghalaya, India
2 Department of Pathology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, Meghalaya, India
|Date of Submission||12-Feb-2022|
|Date of Acceptance||08-Apr-2022|
|Date of Web Publication||01-Nov-2022|
Department of Radiation Oncology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, Meghalaya
Source of Support: None, Conflict of Interest: None
Mantle cell lymphoma is a rare type of B cell non-Hodgkin's lymphoma (NHL) comprising only 3%–6% of all NHL. It is aggressive and prone to relapse. Around 15%–30% of the patients present with gastrointestinal tract involvement. Solitary rectal mantle cell lymphoma is rare and there are very few reports of the same. Till date, there is no report of a solitary rectal relapse of mantle cell lymphoma occurring after second remission. We report a relapse of solitary rectal mantle cell lymphoma presenting with rectal bleeding after second remission in primary gastric mantle cell lymphoma.
Keywords: Mantle cell lymphoma, non-Hodgkin's lymphoma, rectal, relapse
|How to cite this URL:|
Nongkynrih A, Jagtap V, Kumar S, Nayan N, Dey B, Raphael V. Unusual solitary rectal relapse of mantle cell lymphoma after second remission. J Radiat Cancer Res [Epub ahead of print] [cited 2022 Dec 4]. Available from: https://www.journalrcr.org/preprintarticle.asp?id=360282
| Introduction|| |
Non-Hodgkin's lymphoma (NHL) can arise from lymph nodes, lymphoid tissue, and extranodal sites. Gastrointestinal (GI) NHL comprises one-third of all extranodal cases. However, primary GI NHL is still rare and accounts for only 5% of all lymphomas. Of all rectal malignancies, only 0.05% are primary rectal lymphomas. Mantle cell lymphoma is a type of mature B cell NHL comprising only 3%–6% of all NHL. Approximately 75% of patients initially present with lymphadenopathy while extranodal disease is the primary presentation in the remaining 25%. GI involvement in mantle cell lymphoma is reported to be about 15%–30%. Mantle cell lymphoma is aggressive and prone to relapse. A solitary rectal relapse of mantle cell lymphoma is rare and to the best of our knowledge, there is no reported case of a solitary rectal relapse of mantle cell lymphoma after second remission. We report a case of gastric mantle cell lymphoma with second relapse presenting with rectal bleeding and a rectal growth.
| Case Report|| |
A 53-year-old male, a case of mantle cell lymphoma stomach, post gastrectomy and chemotherapy with rituximab, cyclophosphamide, hydroxydaunorubicin hydrochloride (doxorubicin hydrochloride), vincristine (Oncovin) and prednisone (R-CHOP) regimen was disease-free for 24 months following which he had relapse in nasopharynx, large and small bowel, and cervical, inguinal, and mesenteric lymph nodes (Stage R III E). He received chemotherapy with (gemcitabine + oxaliplatin) regimen for eight cycles, followed by external beam radiation therapy to nasopharynx and neck to a dose of 45 Gy/25 fractions for 5 weeks for residual disease in the nasopharynx. The patient responded well with no disease in positron-emission tomography (PET) scan performed after treatment completion. The patient was on regular follow-up. He defaulted for 1 year due to COVID-related travel restrictions and logistics issues. After a disease-free interval of 30 months, he presented with bleeding per rectum on and off for 1 year. General physical examination was within the normal limits. There were no palpable lymph nodes. Per rectal examination revealed a circumferential proliferative growth 5 cm from the anal verge. The growth was ulcerative and the upper extent of the growth could not be felt.
Colonoscopy showed a growth in the rectum 5 cm from the anal verge involving one-third of the circumference and extending up to 8 cm from the anal verge involving two-third circumference. Rest of the colon was normal. Upper GI endoscopy was normal.
Rectal biopsy was suggestive of mantle cell lymphoma. Cyclin D1, CD5, and BCL2 were positive, while CD10 was negative on immunohistochemistry (IHC) [Figure 1]. Whole-body PET computed tomography (PET CT) scan revealed a circumferential wall thickening of 1.9 cm (maximum standardized uptake value 14.1) in the rectum [Figure 2] and [Figure 3]. There were no significant hypermetabolic lymph nodes on both sides of the diaphragm. No other significant metabolically active disease was noted elsewhere in the body. Bone marrow aspiration and biopsy had no evidence of infiltration by atypical/lymphoma cells. The patient was started on chemotherapy with rituximab, dexamethasone, cytarabine, and cisplatin (R-DHAP) regimen. The patient demonstrated clinical response after first cycle of chemotherapy demonstrated by relief from rectal bleeding. He has completed two cycles of the R-DHAP regimen till date and is still undergoing treatment as scheduled.
|Figure 1: (a) Histopathology showed small-to-medium monomorphous lymphoid cells in diffuse pattern entrapping the colonic glands (H and E, ×200); (b) Cyclin D1 showing nuclear positivity in the lymphoid cells (IHC, ×400); (c) CD19 showing positivity in the lymphoid cells (IHC, ×400); (d) CD5 showing positivity in the lymphoid cells (IHC, ×400). IHC: Immunohistochemistry|
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|Figure 2: Coronal section of whole-body PET CT showing lesion in the rectum. PET CT: Positron emission tomography computed tomography|
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|Figure 3: Axial section of whole-body PET CT showing lesion in the rectum. PET CT: Positron emission tomography computed tomography|
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| Discussion|| |
Mantle cell lymphoma is rare and comprises only 3% of NHL. It has a more aggressive course than indolent lymphomas, with a median survival of only 3 years and a high propensity to relapse. Diagnosis is by a combination of histological examination with IHC profile consisting of CD5+, CD10±, CD 20+, CD23±, CD43+, and cyclin D1+. The diagnosis is usually established by overexpression of cyclin D1. However, cyclin D1-negative mantle cell lymphoma has also been reported. The median age of presentation of mantle cell lymphoma is 60 years and it is four times more common in males than in females.
GI involvement in mantle cell lymphoma is reported to be about 15%–30%. There is no clear explanation for the predilection of mantle cell lymphoma to involve the GI tract. One theory is that mantle cell lymphoma could originate from naive B cells in the mantle zones of lymphoid follicles in digestive tract-associated lymphoid tissue, while another attributes it to the expression of the mucosal homing receptor alpha-4-beta-7 by lymphoma cells in the peripheral lymph nodes. Romaguera et al. reported histological involvement of the lower GI tract in 88% of patients with mantle cell lymphoma. Another study reported 77% involvement of lower GI tract in mantle cell lymphoma patients, out of which only 54% had abnormal colonoscopic findings. GI involvement in mantle cell lymphoma can be asymptomatic or can present with GI bleeding, intestinal obstruction, pain abdomen, or weight loss. Our patient presented with bleeding per rectum with a colonoscopic finding of growth in the rectum.
Seventy percent of patients with mantle cell lymphoma present in the advanced stage. In one-fourth of patients, the primary presentation is with extranodal disease. Our patient presented with extranodal disease in the stomach, followed by a first relapse in the nasopharynx and a second relapse in the rectum.
Management of early-stage mantle cell lymphoma is based on the retrospective data. Treatment modalities used are chemotherapy with or without immunotherapy, radiotherapy, or a combination of both. A study by Gill et al. reported a 3-year overall survival of 67.8%, 72.4%, and 79.8% for patients with early-stage mantle cell lymphoma treated with chemotherapy, radiotherapy, and combination of chemotherapy with radiotherapy, respectively. Management of advanced-stage mantle cell lymphoma is by chemoimmunotherapy with or without autologous stem cell rescue. Management of rectal lymphoma is also by chemoimmunotherapy. Surgery is usually indicated as palliation for intestinal obstruction. Chemotherapy regimens used for treatment include dexamethasone, cytarabine, and platinum with or without rituximab; CHOP (cyclophosphamide, hydroxydaunorubicin hydrochloride (doxorubicin hydrochloride), vincristine (Oncovin) and prednisone) regimen; cyclophosphamide, vincristine, prednisone; hyperfractionated cyclophosphamide, vincristine, adriamycin, dexamethasone (hyper-CVAD), methotrexate, and cytarabine with or without rituximab; hyper-CVAD with autologous stem cell transplantation; R-CHOP; and single alkylating agents such as chlorambucil. Less aggressive induction therapies include bendamustine and rituximab; bortezomib, rituximab, cyclophosphamide, adriamycin, and prednisolone; and lenalidomide with rituximab. Second-line therapies include drugs such as ibrutinib, acalabrutinib, and lenalidomide. Response rates of various regimens used in mantle cell lymphoma are in the range of 50%–70%, with complete response in only 30% of patients. Despite the use of aggressive regimens, the median time to relapse is only 18 months.
| Conclusion|| |
Mantle cell lymphoma is an aggressive subtype of NHL. It has a high propensity to involve the GI tract. Rectum involvement in mantle cell lymphoma is rare, and there is no clear consensus for its management. Till date, chemotherapy with or without immunotherapy is the mainstay of treatment, with surgery reserved only for palliation. Although it is a disease with a good response to chemotherapy, the relapse rate is high, with a median time to relapse of <2 years.
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[Figure 1], [Figure 2], [Figure 3]