An overlapping case of in situ mantle cell neoplasia and leukemic non-nodal mantle cell lymphoma.

In situ mantle cell neoplasia (isMCN) and leukemic non-nodal mantle cell lymphoma (nnMCL) are classified as an indolent subtype of mantle cell lymphoma (MCL). The tumor cells of isMCN are restricted to the inner layer of the lymphoid tissue mantle zone, exhibiting an in situ pattern histologically. On the other hand, nnMCL is distributed in the peripheral blood, bone marrow and sometimes the spleen, but lymphadenopathy or systemic organ involvement is rare. We report a case of isMCN in a submandibular lymph node resected from a 65-year-old Japanese male. The tumor cells were positive for cyclin D1 (CCND1) and SOX11 expression, and were restricted to the mantle zone area of the lymph node. However, tumor cells were also detected in the stomach mucosa, bone marrow tissue and peripheral blood, suggesting nnMCL. isMCN and nnMCL may have a partly overlapping disease spectrum, although the correlation between these two subtypes has not been well described. This present case demonstrated characteristics overlapping between isMCN and nnMCL.

[Cytarabine and hematopoietic cell transplantation for mantle cell lymphoma. Analysis of 20 patients]. / Resultados de pacientes con linfoma del manto: impacto de terapias basadas en citarabina y trasplante hematopoyético.

BACKGROUND: Mantle cell lymphoma (MCL) has high relapse and mortality rates. There is a survival benefit when treatment is intensified with cytarabine (AraC), hematopoietic cell transplantation (HCT) and maintenance with rituximab. AIM: To assess the outcomes of patients with MCL treated in a university hospital. MATERIAL AND METHODS: Review of an oncology center database and medical records identifying patients with MCL treated between 2006 and 2017. Death dates were obtained from the death certificate database of the National Identification Service. We analyzed the response rate, overall survival (OS) and progression-free survival (PFS). As a secondary objective, the survival impact of AraC, HCT and maintenance with rituximab, was also analyzed. RESULTS: Information on 20 patients aged 62 ± 11 years, followed for a median of 45 months was retrieved. Eighty-five percent were diagnosed at an advanced stage. The most used first-line regime was R-CHOP in 11 patients, followed by R-HyperCVAD in five. Only 47% achieved complete response. 4-year PFS and OS were of 30 and 77% respectively. Mantle Cell Lymphoma International Prognostic Index (MIPI) significantly predicted PFS and OS. Maintenance with rituximab or HCT was associated with better PFS (48 vs 21 months, p < 0.01). The exposure to AraC or HCT, in refractory or relapsed disease, was associated with an increase in PFS from 9 to 28 months (p = 0,02) and 4-year OS from 40 to 100% (p = 0.05). OS increased even more, from 25 to 100% in those with high-risk MIPI (p = 0.04). CONCLUSIONS: The incorporation of AraC, HCT and maintenance with rituximab in the therapeutic backbone of MCL, especially for high-risk cases, was associated with improved survival.

Resultados de pacientes con linfoma del manto: impacto de terapias basadas en citarabina y trasplante hematopoyético / Cytarabine and hematopoietic cell transplantation for mantle cell lymphoma: analysis of 20 patients

Background: Mantle cell lymphoma (MCL) has high relapse and mortality rates. There is a survival benefit when treatment is intensified with cytarabine (AraC), hematopoietic cell transplantation (HCT) and maintenance with rituximab. Aim: To assess the outcomes of patients with MCL treated in a university hospital. Material and Methods: Review of an oncology center database and medical records identifying patients with MCL treated between 2006 and 2017. Death dates were obtained from the death certificate database of the National Identification Service. We analyzed the response rate, overall survival (OS) and progression-free survival (PFS). As a secondary objective, the survival impact of AraC, HCT and maintenance with rituximab, was also analyzed. Results: Information on 20 patients aged 62 ± 11 years, followed for a median of 45 months was retrieved. Eighty-five percent were diagnosed at an advanced stage. The most used first-line regime was R-CHOP in 11 patients, followed by R-HyperCVAD in five. Only 47% achieved complete response. 4-year PFS and OS were of 30 and 77% respectively. Mantle Cell Lymphoma International Prognostic Index (MIPI) significantly predicted PFS and OS. Maintenance with rituximab or HCT was associated with better PFS (48 vs 21 months, p < 0.01). The exposure to AraC or HCT, in refractory or relapsed disease, was associated with an increase in PFS from 9 to 28 months (p = 0,02) and 4-year OS from 40 to 100% (p = 0.05). OS increased even more, from 25 to 100% in those with high-risk MIPI (p = 0.04). Conclusions: The incorporation of AraC, HCT and maintenance with rituximab in the therapeutic backbone of MCL, especially for high-risk cases, was associated with improved survival.

Tumor Engraftment in a Xenograft Mouse Model of Human Mantle Cell Lymphoma.

B lymphocytes are key players in immune cell circulation and they mainly home to and reside in lymphoid organs. While normal B cells only proliferate when stimulated by T lymphocytes, oncogenic B cells survive and expand autonomously in undefined organ niches. Mantle cell lymphoma (MCL) is one such B cell disorder, where the median survival rate of patients is 4 - 5 years. This calls for the need of effective mechanisms by which the homing and engraftment of these cells are blocked in order to increase the survival and longevity of patients. Therefore, the effort to develop a xenograft mouse model to study the efficacy of MCL therapeutics by blocking the homing mechanism in vivo is of utmost importance. Development of animal recipients for human cell xenotransplantation to test early stage drugs have long been pursued, as relevant preclinical mouse models are crucial to screen new therapeutic agents. This animal model is developed to avoid human graft rejection and to establish a model for human diseases, and it may be an extremely useful tool to study disease progression of different lymphoma types and to perform preclinical testing of candidate drugs for hematologic malignancies, like MCL. We established a xenograft mouse model that will serve as an excellent resource to study and develop novel therapeutic approaches for MCL.

Mantle Cell Lymphoma with a Single Protruding Lesion as the Cause of Intussusception.

Mantle cell lymphoma (MCL) is a malignant lymphoma of the gastrointestinal tract that mostly presents as multiple lymphomatous polyposis (MLP); however, MLP with intussusception is rarely reported in MCL. Furthermore, a single protruding lesion with intussusception has never been reported in primary small intestinal MCL. A 70-year-old man presented with pain in the right lower abdomen. Computed tomography and colonoscopy revealed ileocecal intussusception. Ileocecal resection was performed. Histology and immunohistochemistry of the resected specimen showed MCL with a single protruding lesion. The patient was successfully treated with surgery alone and remains in complete remission at the three-year follow-up.

Case Study: Rare Case of Mantle Cell Lymphoma With Extranodal Involvement in the Foot.

Mantle cell lymphoma (MCL) is a rare type of non-Hodgkin lymphoma that commonly affects extranodal sites. The most commonly affected sites are the bone marrow, gastrointestinal tract, Waldeyer's ring, lung, and pleura. We report the case of an 80-year-old diabetic male, in MCL remission, who presented with a small dome-shaped nodule on his calf and an ipsilateral second digit non-healing ulceration after a traumatic fall. Despite surgical and conservative treatment, the wound worsened, resulting in histopathologic examination, which confirmed the presence of lymphocytes, indicating MCL relapse. This case was followed up for approximately 3 months until the patient died. Our case is an example of pedal manifestations of skin involvement of MCL, which, on consideration of the clinical manifestations also, can be confused with a nonhealing diabetic wound. The clinical significance of our case study is to assist in the diagnosis, management, and prognosis of a patient with MCL.

Extensive colorectal lymphomatous polyposis complicated by acute intestinal obstruction: a case report.

BACKGROUND: Multiple lymphomatous polyposis is a rare type of gastrointestinal lymphoma that extensively infiltrates the intestine. Multiple lymphomatous polyposis originates from the mantle zone of the lymphoma follicle and is considered to be a mantle cell lymphoma, which is a relatively aggressive type of B-cell non-Hodgkin's lymphoma. We report an unusual case of a patient with multiple lymphomatous polyposis with extensive colorectal involvement and acute intestinal obstruction, an atypical complication of this rare disease. On the basis of this case study, the pitfalls in gastrointestinal tract lymphomatous polyposis diagnosis and prognosis, as well as the treatment options, are discussed. CASE PRESENTATION: Our patient was a 76-year-old white woman with asthenia, cramps, and swelling in the lower left quadrant of the abdomen, as well as weight loss within the previous 5 months. A colonoscopy revealed polyps in the rectum, sigmoid colon, descending colon, and right and left colic flexures. A biopsy revealed lymphomatous infiltration of the intestinal wall. Because of the large size of the polypoid masses, which narrowed the colonic lumen in multiple locations, the patient developed acute intestinal obstruction and was referred for laparotomy. She underwent a total proctocolectomy with a permanent ileostomy and a left salpingo-oophorectomy. Microscopic examination showed the presence of a multicentric, low-grade, small lymphocytic lymphoma. Immunohistochemical analysis revealed positive immunostaining for CD79a, CD20, and CD45. These results were consistent with the diagnosis of mantle cell lymphoma. Two weeks after surgery and prior to discharge, but before the beginning of chemotherapy, the patient's general condition worsened as she experienced a severe and progressive respiratory tract infection, advanced respiratory insufficiency, and septic shock, and she ultimately died. CONCLUSIONS: Mantle cell lymphoma develops as a progressive and aggressive disease with widespread polyposis of the gastrointestinal tract. The intensive chemotherapeutic regimens usually result in the regression of macroscopic and microscopic lesions; however, remissions are short in duration, and the median length of patient survival is 3-4 years. Mantle cell lymphoma is a rare disease that should be part of the differential diagnosis of polypoid diseases of the large intestine.

Afectación mamaria por linfoma de células del manto en un varón / Mantle cell lymphoma involving the breast in a male patient

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Classical type and blastoid variant mantle cell lymphoma in the same lymph node: Histology and cytological findings from a touch imprint specimen.

Blastoid variant (BV) is one of the aggressive variants of mantle cell lymphoma (MCL). BV-MCL is defined by its blastic cytomorphology. Previous studies using sequential biopsies in cases with MCL have demonstrated that classical type MCL (C-MCL) often transforms or relapses as an aggressive variant, but a histopathological transition from C-MCL to an aggressive MCL variant in the same pathological specimen has been shown in only a limited number of the cases. We present a case of MCL in which a histological transition between C-MCL and BV-MCL was observed in the same lymph node. A 53-year-old man presented with a submandibular tumor. Touch imprint cytology revealed a monotonous proliferation of large blastic lymphoid cells. Histology revealed a transition between a large lymphoid cell component and small foci of small- to medium-sized cell component within the tumor. Both components were CD5(+), CD10(-), CD20(+), cyclin D1(+), and SOX11(+) on immunohistochemistry. Fluorescent in situ hybridization revealed the translocation of IgH/BCL1 locus. These findings led to a final diagnosis of BV-MCL with coexistent C-MCL. The present case suggests the existence of a pathogenetic pathway of MCL from C-MCL to BV-MCL. Because it is important to accurately identify BV-MCL for prognostication, appropriate ancillary diagnostic tools should be used in suspected cases. Diagn. Cytopathol. 2017;45:364-370. © 2016 Wiley Periodicals, Inc.

Mantle cell lymphoma: primary oral presentation.

Mantle-cell lymphoma is an uncommon lymphoid malignancy of B-cells. It is often aggressive and prognosis is poor. A 69-year-old gentleman with a history of ischaemic heart disease was referred from primary care with a painless right floor of mouth swelling that had been present for 1 month. He otherwise completely asymptomatic. Incisional biopsy of the lesion was undertaken and marker studies demonstrated mantle cell lymphoma. Positron emission tomography-computed tomography and bone marrow biopsy showed widespread but low volume involvement. The patient was referred to the haematology multidisciplinary team for further assessment and treatment.

Lymphadenectomy Specimens in a Large Retrospective Cohort of Pediatric Patients Reveal No in situ Lymphomas but a Broad Spectrum of Reactive Changes.

OBJECTIVES: While the incidence and prevalence of in situ follicular neoplasia (ISFN) and in situ mantle cell neoplasia (ISMCN) in adults are well documented, little is known about these early (precursor) lesions in pediatric populations. The aim of this study was to analyze so-called 'reactive' lymph nodes harvested for the purpose of staging solid tumors, unexplained lymphadenopathies, or presumed inflammatory processes or in conjunction with other surgical interventions in children and adolescents aged <18 years, with special attention to ISFN and ISMCN. METHODS: Formalin-fixed, paraffin-embedded reactive lymph node samples from an unselected pediatric population from two catchment areas in Switzerland were retrospectively analyzed for the presence of ISFN and ISMCN and specific reactive lymph node patterns. RESULTS: While a diverse range of histopathological patterns of reactive lymph node changes with a particular periodic increase in mycobacterioses could be observed in this pediatric population, not a single case of ISFN or ISMCN was found. CONCLUSIONS: Early histological lymphomagenesis equivalents in the form of in situ lymphomas are exceedingly rare events in children and young adolescents. The spectrum of reactive lymph node changes is large, with differences possibly determined by regional variations in geography, demographics, catchment areas, seasons, and years, respectively.

Male breast cancer and mantle cell lymphoma in a single patient: A case report and literature review.

RATIONALE: Although still relatively rare, multiple primary malignant neoplasms (MPMNs) have been increasingly reported in recent years. PATIENT CONCERNS AND DIAGNOSES: A 65-year-old man was referred to our hospital for a painless, incidental left axillary lump. Ultrasound showed enlarged left axillary lymph nodes. An excisional biopsy was conducted on 3 lymph nodes. The pathological diagnosis was determined to be metastatic adenocarcinoma and mantle cell lymphoma (MCL) in the lymph nodes. Further physical examination of the patient yielded a 1.5-cm hard, left subareolar mass. INTERVENTIONS AND OUTCOMES: The patient underwent modified radical mastectomy. The diagnosis was grade II invasive ductal carcinoma (stage IIA). The axillary lymph node showed MCL (stage I, group A), but not metastatic ductal carcinoma. The patient received chemotherapy, including 6 courses of CHOP (A chemotherapy protocol consists of cyclophosphamide 1.2 g day 1, doxorubicin 80 mg day 1, vindesine 4 mg day1, and prednisone 90 mg from day 1 to 5) for lymphoma and breast cancer. The patient was also administered endocrine therapy. After a 54-month follow-up, the patient was well with no evidence of disease. LESSONS: MPMNs are easily misdiagnosed as a primary and metastatic tumor, leading to delayed or erroneous treatment. Male breast cancer in a patient with MCL is rare. Early diagnosis and proper therapy are necessary for an optimal prognosis. Further studies are required to define the mechanisms and risk factors of MPMNs.

[Synchronous colorectal carcinoma and non-Hodgkin lymphoma - two case reports]. / Synchronní kolorektální karcinom a nehodgkinský lymfom - popis dvou prípadu.

Colorectal carcinoma represents an important cause of morbidity and mortality in adults, and its incidence in the Czech Republic is one of the world´s highest. The basic therapeutic approach is surgery: surgical removal of the affected part of the bowel together with regional lymph nodes dissection. The lymph nodes are routinely examined by means of histopathology. In this paper, we present two patients whose histological examination of mesocolic lymph nodes revealed an infiltration by synchronous malignant B-non-Hodgkin-lymphoma. Mantle cell lymphoma was present in the first case, and small cell lymphoma CLL/SLL in the other. Relevant literature is reviewed. KEY WORDS: synchronous - malignancy - colorectal - lymphoma - lymph node.

Mantle Cell Lymphoma.

Mantle cell lymphoma (MCL) is an uncommon subtype of non-Hodgkin lymphoma previously considered to have a poor prognosis. Large gains were made in the first decade of the new century when clinical trials established the importance of high-dose therapy and autologous stem-cell rescue and high-dose cytarabine in younger patients and the benefits of maintenance rituximab and bendamustine in older patients. In particular, greater depth of understanding of the molecular pathophysiology of MCL has resulted in an explosion of specifically targeted new efficacious agents. In particular, agents recently approved by the Food and Drug Administration include the proteasome inhibitor bortezomib, immunomodulator lenalidomide, and Bruton's tyrosine kinase inhibitor ibrutinib. We review recent advances in the understanding of MCL biology and outline our recommended approach to therapy, including choice of chemoimmunotherapy, the role of stem-cell transplantation, and mechanism-based targeted therapies, on the basis of a synthesis of the data from published clinical trials.