Genetic analysis of two cases with MYC "negative" Burkitt lymphoma / 中华医学遗传学杂志

OBJECTIVE@#To carry out combined genetic analysis on two patients suspected for Burkitt lymphoma to facilitate their diagnosis and treatment.@*METHODS@#G banded karyotyping and interphase and metaphase fluorescence in situ hybridization (FISH) were used to detect the specific sites of chromosomes by using separate and fusion probes.@*RESULTS@#The separate probe showed no presence of MYC gene abnormality, while fusion probe confirmed the IGH::MYC translocation in the samples. Combined with the clinical features and pathological characteristics, the two patients were finally diagnosed with Burkitt lymphoma, which was confirmed by targeted capture next generation sequencing.@*CONCLUSION@#The separate probe for the MYC gene has some shortcomings and should be used together with dual fusion probe to improve the accuracy of diagnosis.

Clinical features and prognosis of children with Burkitt's lymphoma: an analysis of 62 cases / 中国当代儿科杂志

OBJECTIVES@#To study the clinical features and chemotherapy response of Burkitt's lymphoma (BL) in children and the influence of rituximab on the prognosis of children with BL.@*METHODS@#A retrospective analysis was performed for the medical data of 62 children with BL, including clinical features, therapeutic efficacy, and prognostic factors. The Cox regression model was used to identify the factors associated with poor prognosis in children with BL. According to whether rituximab was used, the children with advanced (stage III/IV) BL were divided into two groups: chemotherapy plus rituximab and chemotherapy alone. The prognosis was compared between the two groups.@*RESULTS@#For these 62 children, the median age of onset was 5 years (range 1-14 years), and there were 58 boys (94%) and 4 girls (6%). The primary site was abdominal cavity in 41 children (66%), and head and neck in 16 children (26%). There were 1 child with stage I BL (2%), 8 with stage II BL (13%), 33 with stage III BL (53%), and 20 with stage IV BL (32%). The median follow-up time was 29 months, with progression/recurrence observed in 15 children (24%), and the 3-year overall survival (OS) rate and event-free survival (EFS) rate were 82.8%±5.2% and 77.3%±5.8%, respectively. For the children with stage III/IV BL, there was a significant difference in the 3-year the OS rate between the chemotherapy plus rituximab group (16 children) and the chemotherapy alone group (30 children) (93.3%±6.4% vs 65.6%±9.9%, P=0.042), while there was no significant difference in the 3-year EFS rate between the two groups (86.2%±9.1% vs 61.8%±10.1%, P>0.05). The Cox regression analysis showed that central nervous system involvement, lactate dehydrogenase >1 000 U/L, and early incomplete remission were the factors associated with poor prognosis (P<0.05).@*CONCLUSIONS@#Chemotherapy combined with rituximab can improve the prognosis of children with stage III/IV BL. Central nervous system involvement, elevated lactate dehydrogenase level, and early incomplete remission may indicate a poor prognosis in children with BL.

Late recurrence of Burkitt's lymphoma in the jaw: numb chin syndrome as the only symptom

The Numb Chin Syndrome (NCS) is defined as facial and oral numbness restricted to the mental nerve's distribution involving the lower lip, skin of the chin, or gingiva of the lower anterior teeth. Hypoesthesia can occur unilaterally or bilaterally. Although this syndrome is rare, its importance is related to the fact that it represents the clinical manifestations of malignant diseases. Breast cancer and non-Hodgkin lymphoma are the most common cause of NCS. The patient, a 58-year-old woman, treated for a Burkitt Lymphoma (BL) nine years ago, described a two-week history of change in sensitivity and pain in the chin region, without relief with the use of analgesics. She had no headache, speech disturbance, dysphagia, visual disturbance, or other neurological symptoms. No surgical intervention has been performed recently. The intraoral examination revealed a healthy oral mucosa and a small area adjacent to the right mental nerve region that was uncomfortable to palpation. No changes were found in the bone trabeculae at cone-beam computed tomography. The contrasted magnetic resonance features made it possible to identify a change in the mandibular body extending to the entire right side, coinciding with the patient's complaint, indicating a probable mandibular medullary invasion. The patient was submitted to a biopsy to rule out a possible recurrence of BL. The microscopic findings were consistent with the diagnosis of BL. The present report described a very unusual presentation of late recurrent BL nine years after the first treatment, which manifested as an NCS.

Intraoral EBV-positive sporadic Burkitt lymphoma in an elderly patient with bilateral presentation

Sporadic Burkitt lymphoma (SBL) is a variant of Burkitt lymphoma that occurs worldwide, affecting mainly children and young adults. Association with Epstein-Barr virus (EBV) can be identified in approximately 20-30% of cases. Herein we described a case of a 63-year-old male presenting intraoral bilateral mandibular swelling, subjacent to fixed dental prosthesis, with one month of duration. Incisional biopsies were performed, and after two days, the patient was hospitalized due to malaise and breathing difficulty, and died after a week when an abdominal tumor was detected. The mandibular biopsies revealed a diffuse proliferation of medium-sized monomorphic atypical lymphoid cells exhibiting numerous mitoses and areas of "starry-sky" pattern. The tumor showed immunohistochemical positivity for CD20, CD10, Bcl-6, and Ki-67 (≈ 100%); it was negative for CD3, Bcl-2, Vs38c, and MUM-1. Positivity for EBV was found by in situ hybridization. The final diagnosis was intraoral SBL positive for EBV. Clinical, morphological and molecular criteria are necessary for the correct diagnosis of aggressive B-cell neoplasms positive for EBV in elderly patients.

Anti-Cancer and Anti-Proliferation Activity of Ethyl Asetat Extract From Ant Nest (Myrmecodia pendans) in Burkitt's Lymphoma Cancer Cells

Objective: To determine the activity of anti-cancer and anti-proliferation of ethyl acetate fraction of ant nest plants (Myrmecodia pendans) in Burkitt's Lymphoma cancer cells. Material and Methods: The study was conducted in a pure laboratory experimental method using Burkitt's Lymphoma cancer cell culture. Gradual research begins with the determination, extraction and fractionation of ant nest plants, to test for proliferation barriers. Data analysis using two-way ANOVA followed by Post Hoc LSD test with a significance level of 95%. Pearson correlation test was conducted. Results: The results of testing the inhibition of Burkitt's Lymphoma cell proliferation with ethyl acetate extract treatment showed that there was inhibition of cell growth based on the concentration given, starting from the lowest concentration of 15.625 µg/mL. Likewise, the incubation time factor of 24, 48, and 72 hours showed that the longer the incubation time, the greater the inhibition of cell growth. Antiproliferation analysis of flavonoid ethyl acetate extract based on concentration and incubation time on absorption of optical density Burkitt's Lymphoma was statistically significant (p = 0.00). Conclusion: Ant nest ethyl acetate extract has the effect of proliferation inhibition on Burkitt's lymphoma cells.

Linfoma tipo Burkitt con afectación ovárica como única manifestación inicial: caso clínico y revisión de la literatura / Ovarian Burkitt lymphoma as the primary manifestation: a case report and literature review

La afectación ovárica como debut de un linfoma de Burkitt sin enfermedad extraovárica detectable es anecdótica, por lo que habitualmente no se incluye como hipótesis diagnóstica tras el hallazgo de una tumoración ovárica. Su desconocimiento lleva a realizar un tratamiento equivocado que puede llegar a comprometer el deseo reproductivo de la paciente. Presentamos el caso de una paciente que presenta un linfoma de Burkitt con afectación ovárica como manifestación inicial. La paciente desarrolló una progresión sistemática excepcionalmente rápida. A propósito de este caso y de su inusual evolución, revisamos la literatura existente.

Ovarian involvement as the initial manifestation of a Burkitt lymphoma without detectable extra-ovarian disease is rare, which is why it is usually not included in the differential diagnosis when an ovarian tumor is detected. A missed diagnosis will lead to the wrong treatment being given, and this can compromise any future reproductive wishes of the patient. In this article, a patient presents a Burkitt lymphoma with ovarian involvement as an initial manifestation and an unusually rapid systemic progression of the disease. Prompted by this case and its unusual course, we reviewed the existing literature.

Immunodeficiency-associated Burkitt's lymphoma in pediatric patients: a clinical case report

Burkitt's lymphoma, a form of non-hodgkin lymphoma, is a neoplastic monoclonal proliferation of lymphoid cells in areas of the immune system. it can occur in HIV-positive patients, as AIDS is related to the development of non- hodgkin lymphoma. burkitt's lymphoma is a rare subtype, highly prevalent in patients with AIDS. incisional biopsy, in situ hybridization and computerized axial tomography are the appropriate tests to determine the characterize of the lesions. the case of a 4-year-old HIV-positive patient, who developed burkitt's lymphoma of the oral cavity, is reported in this paper. the aim of this case report is to describe the course of the pathology, taking into account its clinical imaging characteristics and treatment.

Pediatric B-cell Lymphoma, Unclassifiable, With Intermediate Features Between Those of Diffuse Large B-cell Lymphoma and Burkitt Lymphoma: A Report of Two Cases

No abstract available.

Neoplasias sólidas de células redondas en edad pediátrica: correlación entre diagnóstico morfológico e inmunohistoquímica / Solid neoplasm of circle celis in the pediatric age: correlation between morphological and immunohistochemistry diagnóstic

Precisar la importancia de realizar el estudio de inmunohistoquímica y su correlación con el diagnóstico anatomopatológico de rutina para el diagnóstico de los tumores sólidos malignos de células redondas en pacientes pediátricos. Se realizó un estudio transversal, descriptivo, retrospectivo no experimental. Se estudiaron 147 pacientes con diagnóstico de neoplasia de células redondas, de los cuales 96 cumplieron con criterios de inclusión. La mayor frecuencia de casos la obtuvo el linfoma No Hodgkin (20,8%), seguido del linfoma de Hodgkin (15,6%) y tumor de Wilms (17,7%). El grupo etario mayormente afectado fue el escolar (37,5%). El género predominante fue el masculino (60,4%). Las manifestaciones clínicas más frecuentes fueron masa palpable (76,04%), fiebre(44,79%), anemia (41,67%), dolor (41,30%) y pérdida de peso (33,33%). Se evidenció la mayor correlación diagnóstica en el tumor de Wilms (100%), seguido del linfoma No Hodgkin (80%) y el linfoma de Hodgkin (75%); la menor correlación la presentaron el sarcoma de Ewingy el neuroblastoma (41,7%, ambos). La correlación total resultó “considerable” según la fuerza de concordancia del coeficiente de Kappa (k: 0,601. P=0,000). Se requiere la integración del examen histopatológico de rutina como método diagnóstico de pesquisa y del estudio de inmunohistoquímica como método auxiliar diagnóstico, para determinar la histogénesis de las neoplasias, descartar diagnósticos diferenciales, realizar un diseño terapéutico apropiado y precisar el pronóstico.

To precise the state and the importance o fthe study of immunohistochemistry and its correlation with the routine pathological diagnosis to use in diagnosis of malignant solid tumors of round cells in pediatric patients. We was performed a transversal, descriptive and retrospective non-experimental study. We studied 147 patients diagnosed with round cell tumor, 96 of them weremet in inclusion criteria. The highest frequency of the cases was Non-Hodgkin lymphoma (20.8%), followed by the Hodgkin lymphoma (15.6%) and the Wilms tumor(17.7%). The age group most affected was the school(37.5%). The male gender was the predominant (60.4%).The most frequent clinical manifestations were a palpablemass (76.04%), the fever (44.79%), the anemia (41.67%),the pain (41.30%) and the weight loss (33.33%). Evidencedthe highest correlation in the diagnosis of Wilms tumor(100%), followed by Non-Hodgkin lymphoma (80%) and Hodgkin lymphoma (75%); showed the lowest correlation between the Ewing sarcoma and neuroblastoma (41.7%, both). The total correlation is “considerable” accordingto strength of agreement the Kappa coefficient (k: 0.601.P=0.000). It requires the integration of routine histopathological examination as diagnostic method of research and study of immunohistochemistry as an auxiliary diagnostic method to determine the histogenesis of neoplasm and exclude the differential diagnoses, make anappropriate therapeutic design and the determine prognosis of this group of patients.

Strong BCL2 expression in Burkitt lymphoma is not uncommon in adults.

Background: The distinction between Burkitt lymphoma (BL) and diffuse large B-cell lymphoma (DLBCL) is not always easy, so much so that the WHO 2008 Blue Book has incorporated a provisional category of "B-cell lymphoma, unclassifiable with features intermediate between DLBCL and BL." One of the immunohistochemical (IHC) markers used at times to differentiate between the two is IHC expression of BCL2, which was initially believed to be consistently negative in BL. Later it was recognized that weak BCL2 expression is still compatible with the diagnosis of BL. To verify or otherwise this current view this study was undertaken. Materials and Methods: We retrieved 39 confirmed cases of BL, in both children and adults. All these cases had typical morphology, IHC profile, and Mib-1 index that are typical of BL. All these cases were then stained with a monoclonal antibody against BCL2 oncoprotein, using 2-step Envision system. Results: Out of 39 cases, 31 cases (79.4%) were completely negative for BCL2, whereas 5 (12.8%) were weak focal positive. However, another 4 (10.2%) cases did show strong diffuse cytoplasmic staining for BCL2. Fluorescent in-Situ hybridization (FISH) for t(14:18) was optimally done on 6 out of 9 cases. All these 4 cases were from adults with 3 out of 4 arising in the parotid region. Two out of 4 cases also showed t(8:14) on FISH. Conclusions: It was concluded that contrary to the common belief, strong BCL2 IHC expression is possible in typical BL in adults and cannot be absolutely relied upon to distinguish between BL and DLBCL.

Relevancia biológica y clínica del inmunofenotipaje celular en la leucemia linfoide aguda del niño / Biological and clinical importance of the cellular immunophenotyping in acute lymphocytic leukemia in children

Se estudiaron las características biológicas y clínicas de 238 niños con leucemia linfoide aguda (LLA) en un período de 13 años. El inmunofenotipaje celular de muestras procedentes de la médula ósea se realizó mediante un ultramicrométodo inmunocitoquímico. Del total de LLA estudiadas 81,4 por ciento fueron de fenotipo B y 18,5 por ciento de fenotipo T. El 48,4 por ciento de los niños con LLA de fenotipo B se encontraron en edades comprendidas entre 2-5 años, mientras que el 65,9 por ciento con LLA-T presentaron 6 o más años de edad. No se encontraron diferencias estadísticamente significativas cuando se analizaron el sexo y el color de la piel en relación con el fenotipo celular leucémico. Al diagnóstico de la enfermedad, el 59,3 por ciento de los pacientes con LLA-B mostraron cifras de leucocitos en sangre periférica < 20 x 109/L y en el 61,4 por ciento con LLA-T cifras superiores a 50 x 109/L. Se observó una mayor incidencia de organomegalia, adenopatías mediastinales, manifestaciones hemorrágicas e infiltración inicial del sistema nervioso central en enfermos con LLA-T en relación con los de LLA-B, con diferencias altamente significativas. Estos resultados demuestran que el fenotipo leucémico en la LLA del niño pudiera considerarse como un factor pronóstico positivo o negativo de la enfermedad.

The biological and clinical characteristics of 238 children with acute lymphocytic leukemia (ALL) were studied for 13 years. The cellular immunophenotyping of samples from the bone marrow was performed by an immunocytochemical ultramicromethod. Of the total of studied ALL, 81.4 percent were phenotype B and 18.5 percent phenotype T. 48.4 percent of the children with B-ALL were 2-5 years old, whereas 65.9 percent with T-ALL were 6 or over. No statistically significant differences were found when sex and colour of the skin were analyzed in relation to the cellular leukemic phenotype. On diagnosing the disease, 59.3 percent of the patients with B-ALL showed figures of leukocytes in peripheral blood < 20x109/L, whereas in 61.4 percent with T-ALL, the figures were higher than 50x109/L. It was observed a greater incidence of organomegaly, mediastinal adenopathies, hemorrhagic manifestations and initial infiltration of the central nervous system in patients with T-ALL compared with those suffering B-ALL. The differences were highly significant. These results proved that the leukemic phenotype in ALL in children could be considered as a positive or negative prognostic factor of the disease.

Morphological spectrum of AIDS-related plasmablastic lymphomas.

We have had a recent spurt in cases of AIDS-related lymphoma (ARL) at our centre. Most of these cases are aggressive mature B cell lymphomas, mainly plasmablastic lymphoma (PBL) and diffuse large B-cell lymphoma (DLBCL). Most of the PBL are extranodal in location and are mucosa-based. We reviewed the morphological features of 34 cases of PBL. Diagnosis was based on morphology, immunohistochemistry, proliferation index, HIV positive status and its preference to extranodal sites (mostly mucosa based). We classified PBL into three morphological subtypes (immunoblastic - 25, Burkitt's - 7, plasmacytic - 2). Tumor cells expressed as leucocyte common antigen (LCA) in 60%, CD138 in 100%, EMA in 45% and light chain restriction in 86% cases. CD20 was negative in all cases. Pathologists need to be aware of PBL and its various morphological subtypes as the identification of this entity from its close differentials carries major therapeutic implications.

Prepubertal testicular tumours in Kashmir--a histopathological report of 15 cases.

A total of 15 cases of prepubertal testicular tumours were reported by the department of pathology, Government Medical College, Srinagar, Kashmir over a period of 15 years, from January 1984 to December 1998. The cases included yolk sac tumour (10 cases; 67%), teratoma [(mature) 2 cases (13%)], rhabdomyosarcoma [(paratesticular) 2 cases (13%)] and NHL-Burkitt's lymphoma [one case (7%)]. The youngest patient was 10 months old and the oldest was of 14 years age. Ten cases (67%) occurred at or below the age of 4 years. The youngest patient had yolk sac tumour and oldest had rhabdomyosarcoma. In 2 cases both the testes were involved with one of these two cases having bilateral undescended testes.

Estudios citogenéticos en niños con Leucemia Linfocitica Aguda-B en Costa Rica / Cytogenetic studies in children with Acute Lymphocitic Leukemia-B in Costa Rica

Chromosome analyses were performed on bone marrow of 177 pediatric patients with Acute Lymphocitic Leukemia at the [quot ]Hospital Nacional de Nifios[quot ]. The standard cytogenetic techniques now belongs to the panel of mandatory analyses performed at diagnosis of our acute leukemia patients and represent a major advantage to be effective and independent prognostic factors, essential for therapeutic choices. Cytogenetic results were achieved in 83% of the bone marrow samples: normal karyotypes represented 29% and abnormal karyotypes 71% with the follow distribution: t (9;22) 3%; t (l;19) 5%; t (4;11) 3%, Hyperdiploidy 39%; other chromosomal abnormalities 21%. Systematic cytogenetic analyses are essencial to define morpho-immunologic sub-types of leukemia and to detect new translocations that allows to understand hematopoiesis and leukemogenesis.

Burkitt's Lymphoma Representing Periportal Infiltrating Mass on CT

Imaging findings of secondary hepatic lymphoma have been reported as variable, ranging from single or multiple small nodules to diffuse infiltrative tumor patterns. We hear present a rare case report concerning aggressive B cells, secondary Burkitt's lymphoma in non-AIDS demonstrating a surprising periportal lymphoma infiltration, without upper abdominal lymphadenopathy or splenomegaly on the sonography and CT scans. Clinically, the case was characterized by atypical and highly aggressive course, with the patient presenting an abruptly developed obstructive jaundice with rapidly deteriorating hepatic function that could be indicative of cholestatic hepatitis, which differs in its clinical manifestations from hepatic lymphoma without functional deterioration in respect of its non-tissue destructive growth pattern. We suggest that hepatic lymphoma can sometimes be consistent with periportal infiltrating homogeneous mass, with no lymphadenopathy or splenomegaly on the imaging examination, with a predictable aggressive clinical course of the disease and poor prognosis.

Intratumoral Injection of (188)Re labeled Cationic Polyethylenimine Conjugates: A Preliminary Report

(188)Re(Rhenium) is easily obtained from an in-house (188)W/(188)Re generator that is similar to the current (99)Mo/(99m)Tc generator, making it very convenient for clinical use. This characteristic makes this radionuclide a promising candidate as a therapeutic agent. Polyethylenimine (PEI) is a cationic polymer and has been used as a gene delivery vector. Positively charged materials interact with cellular blood components, vascular endothelium, and plasma proteins. In this study, the authors investigated whether intratumoral injection of (188)Re labeled transferrin (Tf)-PEI conjugates exert the effect of radionuclide therapy against the tumor cells. When the diameters of the Ramos lymphoma (human Burkitt's lymphoma) xenografted tumors reached approximately 1 cm, 3 kinds of (188)Re bound compounds (HYNIC-PEI-Tf, HYNIC-PEI, (188)Re perrhenate) were injected directly into the tumors. There were increases in the retention of (188)Re inside the tumor when PEI was incorporated with (188)Re compared to the use of free 188Re. The (188)Re HYNIC-Tf-PEI showed the most retention inside the tumor (retention rate=approximately 97%). H&E stain of isolated tumor tissues showed that (188)Re labeled HYNIC-PEI-Tf caused extensive tumor necrosis. These results support (188)Re HYNIC-PEI-Tf as being a useful radiopharmaceutical agent to treat tumors when delievered by intratumoral injection.