Mantle cell lymphoma presenting with lethal atraumatic splenic rupture.

Mantle cell lymphoma is characterized by t(11;14) with CCND1-IGH fusion and manifests with a spectrum of disease ranging from relatively indolent to aggressive. Here, we present a case of pleomorphic mantle cell lymphoma with three fusion signals that presented with lethal atraumatic splenic rupture. We discuss on the implications of variant CCND1 signal patterns as well as the epidemiology and pathophysiology of atraumatic splenic rupture.

Mantle cell lymphoma presenting with lethal atraumatic splenic rupture

Mantle cell lymphoma is characterized by t(11;14) with CCND1-IGH fusion and manifests with a spectrum of disease ranging from relatively indolent to aggressive. Here, we present a case of pleomorphic mantle cell lymphoma with three fusion signals that presented with lethal atraumatic splenic rupture. We discuss on the implications of variant CCND1 signal patterns as well as the epidemiology and pathophysiology of atraumatic splenic rupture.

Treatment progress of mantle cell lymphoma / 白血病·淋巴瘤

Mantle cell lymphoma is a B-cell malignancy with unique biological, pathological and clinical characteristics, accounting for about 5%-10% of non-Hodgkin lymphoma (NHL), and most patients are diagnosed at advanced stage. Mantle cell lymphoma has the aggressive characteristic of aggressive lymphoma and the refractory characteristic of indolent lymphoma, and the prognosis of patients is poor. In recent years, with the development of high-dose chemotherapy, autologous hematopoietic stem cell transplantation and new drug research, the survival time of patients has been significantly prolonged.

Nasopharyngeal Mantle Cell Lymphoma: An Extremely Rare Entity.

Mantle cell lymphoma (MCL) is a rare, aggressive subtype of non-Hodgkin lymphoma with a poor prognosis and high recurrence rate. It seldom affects the Waldeyer's ring let alone the nasopharynx. Patients usually present at late stages of the disease leading to poor failure-free and overall survival rates. Intensive chemotherapy regimes and autologous stem cell transplantation have reported increased survival rates. We report a relapsed case of nasopharyngeal MCL, which previously occurred in the gastrointestinal tract. The patient had undergone a hemicolectomy for colon intussusception secondary to the intraluminal lymphoma mass. He was unable to complete the treatment regime for MCL due to the adverse side effects. Oropharyngeal mass was discovered during routine outpatient follow-up, which was confirmed as nasopharyngeal MCL. We discuss the prognosis, disease progression, and possible treatments.

Progress of mantle cell lymphoma / 白血病·淋巴瘤

Mantle cell lymphoma (MCL) is an aggressive and rare B-cell lymphoma, accounting for around 6%-8% of non-Hodgkin's lymphoma (NHL). Up to now, there are a lot of studies and reports on the biological behavior, diagnostic criteria and treatments of MCL worldwide. However, due to its high invasiveness, there are still many problems to be solved in terms of prognosis and treatment. This article introduces MCL and its progress in the unsolved clinical problems.

Treatment progress of mantle cell lymphoma / 白血病·淋巴瘤

Mantle cell lymphoma (MCL) is a subtype of aggressive B-cell non-Hodgkin lymphoma(NHL) with a heterogeneous clinical characteristics, accounting for 3%-10% of adult-onset NHL. With the great advance of novel drugs, the therapeutic options for MCL are constantly updating. The 61st American Society of Hematology Annual Meeting reported the recent treatment progress of MCL including novel drugs,combinations of nonchemotherapeutic agents and chimeric antigen receptor T-cell therapy. This paper focuses on the treatment progress of MCL.

[Clinicopathologic features and prognosis of gastrointestinal mantle cell lymphoma].

Objective: To investigate the clinicopathologic features and prognosis of gastrointestinal mantle cell lymphoma (GI-MCL). Methods: Clinical data of 38 GI-MCL patients diagnosed at Beijing Friendship Hospital from January 2002 to January 2016 were retrospectively reviewed morphologically and immunophenotypically. IgH/CCND1 gene fusion was assessed by fluorescent in situ hybridization (FISH). For comparison, 60 cases of non-GI-MCL were randomly selected to extract the differences inclinicopathological features and patient survival between the two groups. Results: Of 38 patients with GI-MCL, the median age was 62 years (range: 35-78 years, 23 males and 15 females), of which patients of 60 years of age or older accounted for 55.3%. Patients with clinical course of less than 6 months accounted for 81.1%(30/37). The main symptoms included abdominal pain, diarrhea, anorexia and hematochezia. Those with B symptoms accounted for 32.4%(12/37). The tumor most often involved lleocecal region (57.9%, 22/38), followed by rectum (36.8%, 14/38) and sigmoid colon (28.9%, 11/37), and the stomach accounted for 18.4%(14/38). Endoscopic polypoid lesions were found in 33 cases (86.8%, 33/38), of which 22 cases (66.7%, 22/33) were multiple. Five cases (13.2%, 5/38) presented with local protuberant neoplasm. According to Ann Arbor staging, 3 cases (7.9%, 3/38) were at stage Ⅰ, 4 cases (10.5%, 4/38) were at stage Ⅱ, and 31 cases (81.6%, 31/38) were at stage Ⅳ. The number of patients with tumor involvement of abdominal and retroperitoneal lymph nodes accounted for 45.7%(16/35), including 41.7%(15/36) involving the superficial lymph node, 17.1%(6/35) involving extranodal sites, and 23.5%(8/34) having splenomegaly. All of the 38 cases were classic MCL, and the tumor was composed of uniform lymphoid cells and effacing normal mucosal structure. All tumors were positive for CD20 and CD5. 97.4% (37/38) tumors were positive for cyclin D1, and 92.0% (23/25) tumors were positive for SOX11. FISH test was positive in 1 case of cyclin D1 negative tumor. Twenty-eight patients (73.7%) had a median follow-up of 25.0 months (range: 3-79 months). The 3-year survival rate for stage Ⅰ-Ⅱ and stage Ⅲ-Ⅳ of patients were 80.0% and 69.1%, respectively (P> 0.05). The 3-year survival rate for GI-MCL and non-GI-MCL patients were 71.7% and 72.5%, respectively (P>0.05). Single factor analysis showed that age of >60 years and splenomegaly were correlated with a worse overall survival rate (P<0.05). Conclusions: Gastrointestinal malaise is the most common presenting symptom in GI-MCL patients. GI-MCL more commonly involves colorectum with more frequent multiple polypoid lesions. Patients of age >60 years and with splenomegaly have poor prognosis. There is no difference in the prognosis between GI-MCL and non-GI-MCL patients.

[Clinical analysis of 140 cases of mantle cell lymphoma].

Objective: To study the clinical features, therapeutic effects, prognostic factors of 140 patents with mantle cell lymphoma (MCL). Methods: Clinical data of 140 MCL patients admitted from June 2009 to January 2016 in our hospital were retrospectively analyzed. Results: The median age of 140 patients was 59 years with a ratio of 6∶1 for men and women. There were 134 cases (95.7%) in Ann-Arbor stage Ⅲ-Ⅳ, 37 cases (26.4%) with B symptoms, 61 cases (43.6%) with bone marrow involvement and 38 cases (27.1%) with enlarged spleen. The overall response rate (ORR), 3-year survival rate and progression-free survival rate in the treatment group with rituximab were 87.1%, 68.1% and 59.5% respectively, which were significantly higher than those in the rituximab-free treatment group (66.6%, 51.5% and 31.7%, respectively). The difference was statistically significant (all P<0.05). Among patients treated with rituximab, the complete remission rates (70.8% and 77.8%) of R-HyperCVAD/MA and VcR-CAP regimens were higher than those of R-CHOP regimen (39.0%, both P<0.05). However, there was no significant difference in the overall response rate, overall survival rate and progression-free survival rate (all P>0.05). Univariate analysis showed that age, Ki-67 index, B symptoms, bone marrow invasion, platelet count, LDH, ß(2)-MG and MIPI scores were associated with overall survival (all P<0.05). Multivariate analysis showed that age (HR=4.940, 95% CI: 2.347 to 10.397), B symptom (HR=2.900, 95% CI: 1.517-5.544), ß(2)-MG (HR=2.945, 95% CI: 1.656-5.238), Ki-67 index (HR=4.915, 95% CI: 2.554-9.456) and treatment with rituximab-containing regimen (HR=2.450, 95% CI: 1.352-4.440) were independent factors for OS. Conclusions: Most patients with MCL were older adults and usually had bone marrow involvement and spleen involvement. Rituximab combined with chemotherapy (especially R-HyprCVAD/MA and VcR-CAP) had better clinical efficacy than conventional chemotherapy.

[Clinicopathologic features and prognosis of mantle cell lymphoma: an analysis of 349 cases].

Objective: To investigate clinicopathologic features and prognostic factors of mantle cell lymphoma(MCL). Methods: The clinical data of 349 MCL patients diagnosed at Beijing Friendship Hospital from January 2004 to January 2016 were retrospectively collected. Corresponding histological sections were reviewed. Additional studies included immunohistochemical staining using the MaxVision two-step method, IgH/CCND1 fusion gene detection by fluorescent in situ hybridization (FISH), and correlative statistical analysis. Results: Of 349 patients with MCL, the median patient age was 61 years (range: 25-83 years, M∶F=2.7∶1.0) and the age of 243 patients ranged from 51 to 70 years (69.6%). Those with B symptoms accounted for 22.4% (70/313). Most of the patients presented with superficial lymphadenopathy and the clinical stage Ⅲ-Ⅳ accounted for 76.1% (235/309). Extranodal involvement was seen in 47.9% (148/309), among which the gastrointestinal tract accounted for 31.8% (47/148) and splenic involvement accounted for 15.4% (47/305). Three hundred and nine (88.5%) cases were of classical type and 40 (11.5%) cases were of aggressive variant type, and all were composed of proliferating lymphoid cells. All the tumors were positive for CD20 and cyclin D1, and 98.6% (344/349) tumors were weakly positive or positive for CD5. FISH test was positive in 12 cases that were CD5 negative and with cyclin D1 partial expression.Two hundred and forty-three (69.6%) patients had a median follow-up of 26 months (range: 3-108 months). The 3- and 5-year overall survival rates for patients were 63.0% and 34.8%, respectively. Single factor analysis showed that age of >60 years, splenic involvement, aggressive variant type, incompletely overlapping type [Based on the degree of overlap ≥90% and <90% between the follicular dendritic cell (FDC) meshwork and tumor cells, the tumors were divided into the completely overlapped type and incompletely overlapped type] and Ki-67 index >40% had poor prognosis (P<0.05). Multiple factor Cox proportional risk regression analysis after removing the aggressive variant type showed that age, splenic involvement, the degree of overlap between the FDC meshwork and tumor cells and Ki-67 index were independent prognostic factors for overall survival rate of MCL patients (P<0.05). Conclusions: MCL is more commonly found among middle-aged and elderly men. Patient age, splenic involvement, degree of overlap between FDC meshwork and tumor cells and Ki-67 index are the independent prognostic indicators for MCL.

[Clinicopathologic characteristics and prognositic indicators of tonsillar mantle cell lymphoma].

Objective: To investigate clinicopathological features and prognosis of tonsillar mantle cell lymphoma(TMCL). Methods: Clinical data of 25 patients with TMCL at Beijing Friendship Hospital, Capital Medical University from 2002 to 2016 were included. All the cases were reviewed microscopically. Various immunohistochemical stains were performed using the MaxVision two-step method. IgH/CCND1 gene fusion was detected by fluorescent in situ hybridization(FISH). Additionally, randomly selected 40 cases of non-tonsil MCL of the same period were compared. Results: Among all mantle cell lymphomas (MCL), TMCL accounted for 5.6%(25/449). The median age of the patients was 60 years(range: 44-82 years) with a M∶F ratio of 5.3 to 1.0. The main symptoms were sore throat and foreign body sensation and patients usually presented with enlargement or mass of tonsil. At the early stage of the disease, 18 cases(72.0%) were clinically misdiagnosed as tonsillitis. Lymph node involvement was present in 76.0%(19/25) of the patients. There were 4 cases(16.0%)with current splenic involvement, 11 cases(44.0%) with pharyngeal focal recidivism, and 3 cases(12.0%) with involvement of other non-lymphoid organs. Morphologically, tonsillar architectures were effaced at various degrees. Eighteen MCL cases showed classical type and 7 cases were blastoid variant. All tumors were positive for CD20 and cyclin D1. 92.0%(23/25) tumors showed weakly positive or positive expression for CD5. FISH test that IgH/CCND1 gene fusion was positive in two CD5 negative classical cases. 18 patients(72.0%) had a median follow-up time of 26 months(range: 6-81 months). The difference of survival rate between stage Ⅰ-Ⅱ and stage Ⅲ-Ⅳ patients was not statistically significant(P>0.05). Compared with NTMCL, TMCL was found to have higher proportion of stage Ⅰ-Ⅱ disease (χ(2)=12.789, P<0.01), lower the proportion of non-lymphatic organ involvement (χ(2)=8.125, P<0.01), and better prognosis (χ(2)=4.351, P=0.037). Conclusion: The incidence of TMCL is low and prone to be misdiagnosed as tonsillitis. Patients with TMCL are more likely at stage Ⅰ-Ⅱ at presentation and the prognosis is better than that of NTMCL.

Clinicopathologic features and prognosis of mantle cell lymphoma: an analysis of 349 cases / 中华病理学杂志

Objective@#To investigate clinicopathologic features and prognostic factors of mantle cell lymphoma(MCL).@*Methods@#The clinical data of 349 MCL patients diagnosed at Beijing Friendship Hospital from January 2004 to January 2016 were retrospectively collected. Corresponding histological sections were reviewed. Additional studies included immunohistochemical staining using the MaxVision two-step method, IgH/CCND1 fusion gene detection by fluorescent in situ hybridization (FISH), and correlative statistical analysis.@*Results@#Of 349 patients with MCL, the median patient age was 61 years (range: 25-83 years, M∶F=2.7∶1.0) and the age of 243 patients ranged from 51 to 70 years (69.6%). Those with B symptoms accounted for 22.4% (70/313). Most of the patients presented with superficial lymphadenopathy and the clinical stage Ⅲ-Ⅳ accounted for 76.1% (235/309). Extranodal involvement was seen in 47.9% (148/309), among which the gastrointestinal tract accounted for 31.8% (47/148) and splenic involvement accounted for 15.4% (47/305). Three hundred and nine (88.5%) cases were of classical type and 40 (11.5%) cases were of aggressive variant type, and all were composed of proliferating lymphoid cells. All the tumors were positive for CD20 and cyclin D1, and 98.6% (344/349) tumors were weakly positive or positive for CD5. FISH test was positive in 12 cases that were CD5 negative and with cyclin D1 partial expression.Two hundred and forty-three (69.6%) patients had a median follow-up of 26 months (range: 3-108 months). The 3- and 5-year overall survival rates for patients were 63.0% and 34.8%, respectively. Single factor analysis showed that age of >60 years, splenic involvement, aggressive variant type, incompletely overlapping type [Based on the degree of overlap ≥90% and <90% between the follicular dendritic cell (FDC) meshwork and tumor cells, the tumors were divided into the completely overlapped type and incompletely overlapped type] and Ki-67 index >40% had poor prognosis (P<0.05). Multiple factor Cox proportional risk regression analysis after removing the aggressive variant type showed that age, splenic involvement, the degree of overlap between the FDC meshwork and tumor cells and Ki-67 index were independent prognostic factors for overall survival rate of MCL patients (P<0.05).@*Conclusions@#MCL is more commonly found among middle-aged and elderly men. Patient age, splenic involvement, degree of overlap between FDC meshwork and tumor cells and Ki-67 index are the independent prognostic indicators for MCL.

Clinicopathologic features and prognosis of gastrointestinal mantle cell lymphoma / 中华病理学杂志

Objective@#To investigate the clinicopathologic features and prognosis of gastrointestinal mantle cell lymphoma (GI-MCL).@*Methods@#Clinical data of 38 GI-MCL patients diagnosed at Beijing Friendship Hospital from January 2002 to January 2016 were retrospectively reviewed morphologically and immunophenotypically. IgH/CCND1 gene fusion was assessed by fluorescent in situ hybridization (FISH). For comparison, 60 cases of non-GI-MCL were randomly selected to extract the differences inclinicopathological features and patient survival between the two groups.@*Results@#Of 38 patients with GI-MCL, the median age was 62 years (range: 35-78 years, 23 males and 15 females), of which patients of 60 years of age or older accounted for 55.3%. Patients with clinical course of less than 6 months accounted for 81.1%(30/37). The main symptoms included abdominal pain, diarrhea, anorexia and hematochezia. Those with B symptoms accounted for 32.4%(12/37). The tumor most often involved lleocecal region (57.9%, 22/38), followed by rectum (36.8%, 14/38) and sigmoid colon (28.9%, 11/37), and the stomach accounted for 18.4%(14/38). Endoscopic polypoid lesions were found in 33 cases (86.8%, 33/38), of which 22 cases (66.7%, 22/33) were multiple. Five cases (13.2%, 5/38) presented with local protuberant neoplasm. According to Ann Arbor staging, 3 cases (7.9%, 3/38) were at stage Ⅰ, 4 cases (10.5%, 4/38) were at stage Ⅱ, and 31 cases (81.6%, 31/38) were at stage Ⅳ. The number of patients with tumor involvement of abdominal and retroperitoneal lymph nodes accounted for 45.7%(16/35), including 41.7%(15/36) involving the superficial lymph node, 17.1%(6/35) involving extranodal sites, and 23.5%(8/34) having splenomegaly. All of the 38 cases were classic MCL, and the tumor was composed of uniform lymphoid cells and effacing normal mucosal structure. All tumors were positive for CD20 and CD5. 97.4% (37/38) tumors were positive for cyclin D1, and 92.0% (23/25) tumors were positive for SOX11. FISH test was positive in 1 case of cyclin D1 negative tumor. Twenty-eight patients (73.7%) had a median follow-up of 25.0 months (range: 3-79 months). The 3-year survival rate for stage Ⅰ-Ⅱ and stage Ⅲ-Ⅳ of patients were 80.0% and 69.1%, respectively (P> 0.05). The 3-year survival rate for GI-MCL and non-GI-MCL patients were 71.7% and 72.5%, respectively (P>0.05). Single factor analysis showed that age of >60 years and splenomegaly were correlated with a worse overall survival rate (P<0.05).@*Conclusions@#Gastrointestinal malaise is the most common presenting symptom in GI-MCL patients. GI-MCL more commonly involves colorectum with more frequent multiple polypoid lesions. Patients of age >60 years and with splenomegaly have poor prognosis. There is no difference in the prognosis between GI-MCL and non-GI-MCL patients.

Progress of differential expression of SOX11 in mantle cell lymphoma / 白血病·淋巴瘤

Overexpression of CCND1 and t (11;14) (q13;q32) chromosomal translocation are important markers in mantle cell lymphoma (MCL). However, part of MCL lacks the expression of CCND1. SOX11 can be used as a biomarker for its overexpression in CCND1-negative MCL. SOX11 is a neurogenic transcription factor, and its overexpression is closely related to histone modification and DNA methylation. Differential expression of SOX11 in MCL is closely related with the plasma cell differentiation, and there is a relationship between the prognosis and survival time of MCL. SOX11 cDNA is more sensitive than conventional methods for the detection of minimal residual disease (MRD). With further research of SOX11, it will become an important basis of diagnosis and prognosis in MCL, and can provide a new method for targeted therapy of MCL.

Clinical analysis of 140 cases of mantle cell lymphoma / 中华肿瘤杂志

Objective@#To study the clinical features, therapeutic effects, prognostic factors of 140 patents with mantle cell lymphoma (MCL).@*Methods@#Clinical data of 140 MCL patients admitted from June 2009 to January 2016 in our hospital were retrospectively analyzed.@*Results@#The median age of 140 patients was 59 years with a ratio of 6∶1 for men and women. There were 134 cases (95.7%) in Ann-Arbor stage Ⅲ-Ⅳ, 37 cases (26.4%) with B symptoms, 61 cases (43.6%) with bone marrow involvement and 38 cases (27.1%) with enlarged spleen. The overall response rate (ORR), 3-year survival rate and progression-free survival rate in the treatment group with rituximab were 87.1%, 68.1% and 59.5% respectively, which were significantly higher than those in the rituximab-free treatment group (66.6%, 51.5% and 31.7%, respectively). The difference was statistically significant (all P<0.05). Among patients treated with rituximab, the complete remission rates (70.8% and 77.8%) of R-HyperCVAD/MA and VcR-CAP regimens were higher than those of R-CHOP regimen (39.0%, both P<0.05). However, there was no significant difference in the overall response rate, overall survival rate and progression-free survival rate (all P>0.05). Univariate analysis showed that age, Ki-67 index, B symptoms, bone marrow invasion, platelet count, LDH, β2-MG and MIPI scores were associated with overall survival (all P<0.05). Multivariate analysis showed that age (HR=4.940, 95% CI: 2.347 to 10.397), B symptom (HR=2.900, 95% CI: 1.517-5.544), β2-MG (HR=2.945, 95% CI: 1.656-5.238), Ki-67 index (HR=4.915, 95% CI: 2.554-9.456) and treatment with rituximab-containing regimen (HR=2.450, 95% CI: 1.352-4.440) were independent factors for OS.@*Conclusions@#Most patients with MCL were older adults and usually had bone marrow involvement and spleen involvement. Rituximab combined with chemotherapy (especially R-HyprCVAD/MA and VcR-CAP) had better clinical efficacy than conventional chemotherapy.

Clinicopathologic characteristics and prognositic indicators of tonsillar mantle cell lymphoma / 中华病理学杂志

Objective@#To investigate clinicopathological features and prognosis of tonsillar mantle cell lymphoma(TMCL).@*Methods@#Clinical data of 25 patients with TMCL at Beijing Friendship Hospital, Capital Medical University from 2002 to 2016 were included. All the cases were reviewed microscopically. Various immunohistochemical stains were performed using the MaxVision two-step method. IgH/CCND1 gene fusion was detected by fluorescent in situ hybridization(FISH). Additionally, randomly selected 40 cases of non-tonsil MCL of the same period were compared.@*Results@#Among all mantle cell lymphomas (MCL), TMCL accounted for 5.6%(25/449). The median age of the patients was 60 years(range: 44-82 years) with a M∶F ratio of 5.3 to 1.0. The main symptoms were sore throat and foreign body sensation and patients usually presented with enlargement or mass of tonsil. At the early stage of the disease, 18 cases(72.0%) were clinically misdiagnosed as tonsillitis. Lymph node involvement was present in 76.0%(19/25) of the patients. There were 4 cases(16.0%)with current splenic involvement, 11 cases(44.0%) with pharyngeal focal recidivism, and 3 cases(12.0%) with involvement of other non-lymphoid organs. Morphologically, tonsillar architectures were effaced at various degrees. Eighteen MCL cases showed classical type and 7 cases were blastoid variant. All tumors were positive for CD20 and cyclin D1. 92.0%(23/25) tumors showed weakly positive or positive expression for CD5. FISH test that IgH/CCND1 gene fusion was positive in two CD5 negative classical cases. 18 patients(72.0%) had a median follow-up time of 26 months(range: 6-81 months). The difference of survival rate between stage Ⅰ-Ⅱ and stage Ⅲ-Ⅳ patients was not statistically significant(P>0.05). Compared with NTMCL, TMCL was found to have higher proportion of stage Ⅰ-Ⅱ disease (χ2=12.789, P<0.01), lower the proportion of non-lymphatic organ involvement (χ2=8.125, P<0.01), and better prognosis (χ2=4.351, P=0.037).@*Conclusion@#The incidence of TMCL is low and prone to be misdiagnosed as tonsillitis. Patients with TMCL are more likely at stage Ⅰ-Ⅱ at presentation and the prognosis is better than that of NTMCL.

Treatment progress of mantle cell lymphoma / 白血病·淋巴瘤

In recent years, the incidence of non-Hodgkin lymphoma (NHL) is on the rise. Among the newly diagnosed NHL patients, 3 %-10 % are mantle cell lymphoma (MCL), which is common in middle-aged and elderly men, and the incidence of extranodal aggression is more in stage Ⅲ or Ⅳ. MCL has clinical and pathological features of the invasiveness in invasive lymphoma and the incurability in indolent lymphoma. Although the application of new drugs has made more progress in the treatment of MCL, the overall survival rate is not good; the majority of patients relapse after treatment, and there are no standard treatment regimens. The progresses in MCL will be summarized in this paper based on the reports in the 60th American Society of Hematology (ASH) Annual Meeting.

Mantle cell lymphoma: involvement of nodal and extranodal sites in the head and neck, with multifocal oral lesions.

Mantle cell lymphoma (MCL) is a malignant B-cell neoplasm, which comprises monomorphic and small- to medium-sized mantle zone-derived lymphoid cells. It is characterized by chromosomal translocation t(11;14)(q13;q32) and CCND1 truncation, resulting in cell cycle deregulation. It is an aggressive type of non-Hodgkin lymphoma with a propensity to present with extranodal involvement. This study shows the case of an 80-year-old Caucasian male who complained of a 2-month progressive swelling on the right side of his face. The magnetic resonance imaging exam showed multifocal involvement of the head and neck, including oral manifestations, bilateral parotid glands, palate, tongue, and floor of the mouth. An incisional biopsy of the tumor mass was performed. The morphological and immunophenotypic findings were consistent with the diagnosis of MCL. The patient died 4 months later, without any chance of undergoing a therapeutic approach. Although MCL is a rare condition, it should be subjected to a differential diagnosis when affecting the maxillofacial area. Imaging exams and both immunohistochemical and morphological analyses are needed to reach the correct diagnosis. Here, we present an unusual MCL with multifocal involvement of the head and neck.

Mantle cell lymphoma: involvement of nodal and extranodal sites in the head and neck, with multifocal oral lesions

Mantle cell lymphoma (MCL) is a malignant B-cell neoplasm, which comprises monomorphic and small- to medium-sized mantle zone-derived lymphoid cells. It is characterized by chromosomal translocation t(11;14)(q13;q32) and CCND1 truncation, resulting in cell cycle deregulation. It is an aggressive type of non-Hodgkin lymphoma with a propensity to present with extranodal involvement. This study shows the case of an 80-year-old Caucasian male who complained of a 2-month progressive swelling on the right side of his face. The magnetic resonance imaging exam showed multifocal involvement of the head and neck, including oral manifestations, bilateral parotid glands, palate, tongue, and floor of the mouth. An incisional biopsy of the tumor mass was performed. The morphological and immunophenotypic findings were consistent with the diagnosis of MCL. The patient died 4 months later, without any chance of undergoing a therapeutic approach. Although MCL is a rare condition, it should be subjected to a differential diagnosis when affecting the maxillofacial area. Imaging exams and both immunohistochemical and morphological analyses are needed to reach the correct diagnosis. Here, we present an unusual MCL with multifocal involvement of the head and neck.

Linfoma de células del manto con presentación radiológica atípica: Presentación de caso / Mantle Cell Lymphoma with Atypical Radiologic Presentation

El linfoma de células del manto hace parte del subgrupo de linfomas no Hodgkin (LNH). Este se manifiesta con adenopatías, esplenomegalia, síntomas B y compromiso cutáneo asociado a enfermedad diseminada. El compromiso esplénico se presenta como: Esplenomegalia sin lesión focal; lesiones sólidas únicas o múltiples e infiltración del bazo sin cambios morfológicos ni lesiones focales. La aparición de linfoma con lesiones quísticas es extremadamente rara, se encuentran solo unos cuantos casos en la literatura, ninguno de ellos en el bazo. Se expone el caso de una paciente de 59 años de edad, quien consultó por dolor abdominal intermitente. En los estudios diagnósticos se observó esplenomegalia con lesiones sólidas y quísticas. Se realizó esplenectomía con estudio histopatológico que confirmó compromiso por linfoma de células del manto.

Mantle cell lymphoma is a Non-Hodgkin Lymphoma (NHL). In cases of disseminated disease, lymphadenopathy, splenomegaly, B-symptoms and skin disease are present. Lymphoma affecting the spleen has several radiologic presentations, ranging from normal appearance, to splenomegaly or multiples focal solid lesions. Cystic presentation of lymphoma is rare and few cases have been reported, none of them involving the spleen. We report a case of a 59-year old female patient with cystic spleen lesions that after splenectomy were diagnosed as Mantle cell lymphoma.

Progress in diagnosis and treatment of mantle cell lymphoma / 白血病·淋巴瘤

Mantle cell lymphoma (MCL) is a group of highly aggressive non-Hodgkin lymphoma (NHL) in small B-cell lymphoma, accounting for 6 % of NHL incidence. MCL is characterized with its concealed onset, strong aggression, high malignancy and poor prognosis. Therefore, more attention should be paid to the diagnosis and differential diagnosis of MCL in clinic. Recently, diagnostic models of molecular pathology, researches on cyclin D1 protein negative MCL, staging prognosis and stratification treatment of MCL are worthy of attention.