Diagnosis Of Burkitt Lymphoma Research Articles

A case of acute intestinal obstruction caused by Burkitt’s lymphoma

Acute intestinal obstruction is a multifactorial disease. Its causes may be intra-abdominal adhesions, volvulus, bezoar, gallstones, foreign bodies, intestinal tumors, etc. In rare cases, acute intestinal obstruction may be associated with intussusception, which is relatively rare in adults. The purpose of this work is to demonstrate a clinical case of acute small bowel obstruction caused by Burkitt lymphoma. During treatment for a herpetic infection, the patient underwent surgical treatment — ileostomy because of acute intestinal obstruction. In the postoperative period, the patient was hospitalized due to intestinal bleeding; during diagnostic colonoscopy, intussusception of the ileum into the ascending colon was detected; diagnostic laparoscopy, laparotomy, right-sided hemicolectomy with resection of the greater omentum, restoration of intestinal continuity with elimination of the stoma,and formation of an anastomosis were performed. The material obtained intraoperatively was sent for pathomorphological examination, including the method of fluorescent hybridization, which revealed a rearrangement of the 8th chromosome in the region of localization of the MYC gene in tumor cells, and a diagnosis of Burkitt lymphoma was established. The oncological council determined the chemotherapy treatment protocol. According to the literature, Burkitt lymphoma can debut in the cutaneous form, and it is necessary to carry out a differential diagnosis, including chickenpox. There is currently no information about intestinal complications occurring in the cutaneous form of Burkitt lymphoma. It is possible that in the described case, the initially established diagnosis of herpetic infection was incorrect, and Burkitt lymphoma occurred in the cutaneous form.

Parotid Burkitt Lymphoma: Case Report

Salivary gland tumors are rare counting only 0.5% of all cancers, only 2% among them are lymphomas. Burkitt lymphomas is an aggressive mature non-Hodgkin B-cell lymphoma characterized by MYC oncogene activation, commonly occurring in nodal sites, however the extranodal sites are mostly seen in immunodeficiency associated Burkitt lymphoma as it is seen in patients with HIV infection. Clinically, the symptoms are related to the acute enlargement of gland due to the fast growing of the tumor and its rapid cells turnover. Diagnostic assessment requires biological investigations as well as cytological, histological and immunohistochemical exams to put out the diagnosis of Burkitt lymphoma. However, imaging is paramount by CT and MRI which finds its interest in its sensibility to characterize salivary gland tumors by the diffusion sequence and the estimation of apparent coefficient of diffusion. That being said, the diagnosis of parotid gland lymphoma is a differential diagnosis of parotid tumor because of its rarity. Our case is about a middle-aged woman suffering from a unilateral parotid swelling, its uncommon evolution pushed the investigations which leads to discovering a HIV infection, a diagnose the patient with a HIV-associated Burkitt lymphoma. We put the light on salivary gland location of this cancer by screening literature on this subject which benefitted from important advances of treatments, offering good outcomes in terms of freedom of event and overall survival.

Burkitt Lymphoma with Malignant Ovarian Manifestation: A Case Report and Literature Review

A lymphoma presenting as an ovarian mass with an initial manifestation is unusual and can cause confusion for the clinician, as its presentation may resemble that of other much more common tumors. Malignant lymphoid cells may appear in the ovary either as a primary neoplasm or as a secondary manifestation of an occult or known disseminated disease. The most common presenting signs or symptoms of malignant lymphomas involving the ovaries are abdominal or pelvic pain or a mass. We report a case of bilateral primary Burkitt lymphoma of the ovaries in a 14-year-old girl presenting with abdominal distension, abdominal pain, hematemesis, and melena. Ultrasound and CT imaging suggested a provisional diagnosis of ovarian tumor. The diagnosis of Burkitt lymphoma was established by histopathological examination of a biopsy from a digestive thickening. The tumor was classified as Burkitt lymphoma with pleural, intestinal, peritoneal, nodal (coelio-mesenteric), renal, ovarian, and bone involvement with medullary infiltration.

Performance of a Liquid Biopsy Diagnostic Prediction Model for EBV-Positive Burkitt Lymphoma in Sub-Saharan Africa

Burkitt Lymphoma (BL) is classified into EBV-positive BL (EBL) and EBV-negative BL (sporadic BL), the former being more prevalent in SSA (Al-Khreisat et al 2023). Conventional diagnosis of BL depends on the morphological assessment of invasive tissue biopsy followed by immunohistochemistry (IHC) and fluorescent in situ hybridization (FISH) to reveal the pathognomonic t(8;14), or t(8;22) translocations. This is associated with significant diagnostic delay in regions with limited pathology services (Mawalla et al 2023), often necessitating initiation of treatment based on clinical or histopathological findings without immunohistochemistry (Ogwang et al 2011), with ensuing mismanagement contributing to poor survival outcomes observed in SSA compared to High-Income Countries (HIC) (Sung et al 2021). Here, we sought to explore whether liquid biopsy could be used as an initial diagnostic aid for EBL in settings without easy access to pathology using different EBV DNA parameters and the MYC-Immunoglobulin ( MYC-Ig) gene rearrangement. This prospective study collected whole blood samples prospectively from 221 participants (90 BL and 131 non-BL) enrolled in the Aggressive Infection-Related East Africa Lymphoma (AI-REAL) project (Legason et al 2022) across 5 sites in Tanzania and Uganda. The revised BL diagnostic algorithm (Naresh et al 2021) was used as a gold-standard diagnosis. CfDNA was extracted using the QIAamp Circulating Nucleic Acid Kit (Qiagen, USA), according to the manufacturer's instructions. DNA libraries were prepared with the Thru PLEX Tag-Seq HV kit (Takara Bio Inc. Japan), and hybridization capture was performed using a custom, next-generation sequencing (NGS) panel targeting genes commonly mutated in BL and three EBV genes: EBER1, EBER2, and EBNA2. All sequencing was performed in-country (MiSeq, Illumina, CA, USA) and analyzed in a custom pipeline. Using custom scripts, EBV DNA copies per cell and EBV DNA fragment size ratio were calculated as the proportion of EBV fragments of length 180 - 200 base pairs (bp) divided by the proportion of autosomal fragments within the same size window. The diversity in the fragment size distribution of the EBV and autosomal DNA was calculated and presented as EBV entropy and autosomal entropy. Translocations were called using IgCaller (Nadeu et al 2020) and Genomic Rearrangement Identification Software Suite (GRIDSS) (Cameron et al 2017). Univariate analysis was performed to assess the association between predictor variables (clinical parameters, EBV parameters, and MYC translocation) and a diagnosis of EBL. Ten-fold cross-validation was performed on a liquid biopsy model consisting of all the liquid biopsy variables with the clinical variables included in the model as covariates. Performance metrics for the liquid biopsy model as a diagnostic aid were calculated, and variable importance was determined to assess each variable's relative influence on the model. In our cohort, EBL diagnosis was negatively associated with tumor site (neck) and not significantly associated with tumor site (jaw). A shorter duration of symptoms was associated with a diagnosis of EBL. Presence of MYC translocation t(8;14), higher EBV fragment size ratio, proportion, entropy, and copies per cell for EBER1, EBER2, and EBNA2 were all associated with a diagnosis of EBL.The combined liquid biopsy diagnostic prediction model had an estimated AUC of 90%, with sensitivity between 85%-90%, specificity between 75% - 80%, and positive and negative predictive values of 85% (Figure 1). MYC translocation t(8;14) was the most important diagnostic variable, followed by EBV entropy (Figure 2). This study demonstrates the feasibility of liquid biopsy as a non-invasive diagnostic aid for EBL in settings without easy access to pathology. To strengthen the application of this technology into clinical practice, further analysis is being conducted for weighting and assigning of scores for each predictor variable. This would assist clinicians in making a precise diagnosis, complementing existing diagnostic modalities for EBL, and reducing the rate of misdiagnosis arising from diagnosis based on clinical suspicion and tissue morphology without immunohistochemistry.

Sinonasal Burkitt Lymphoma in a 2-Year-Old Child: An Uncommon Case Report.

Burkitt Lymphoma (BL) is a highly aggressive B-type non-Hodgkin lymphoma. It rarely develops at the level of the sinonasal cavities, especially in young children. We present the case of a 2-year-old boy who presented with a 2-month history of nasal obstruction, snoring, and epistaxis followed by a recent dyspnea. Examination revealed a protruding tissue mass in the left nasal cavity. Computed tomography scan showed a homogenous soft-tissue mass in the left nasal cavity extending toward the ipsilateral orbit, infratemporal fossa, nasopharynx, as well as the ethmoid and maxillary sinuses. Magnetic resonance imaging revealed a homogenous infiltrating mass with a hypo-intense signal on T2-weighted images reaching the infratemporal fossa and oropharynx. Biopsy confirmed the diagnosis of BL. The patient was referred to the clinical hematology department and received complex chemotherapy. Following chemotherapy, imaging revealed significant regression of the mass. We performed a nasopharyngeal biopsy which confirmed the absence of any persistent disease. This case report highlights a rare case of an extensive sinonasal BL emphasizing the importance of a high level of suspicion to ensure an early diagnosis and treatment.

Improvement of diagnosis in children with Burkitt lymphoma in Kenya: feasibility study for the implementation of fluorescence in situ hybridisation testing for MYC and the MYC/IGH translocation.

Indiana University (IU) initiated fluorescence in situ hybridisation (FISH) methodology for Burkitt Lymphoma (BL) to advance the accuracy and speed of diagnosis in the AMPATH Reference Laboratory at Moi Teaching and Referral Hospital (MTRH) in Eldoret, Kenya. Standard diagnostic testing for BL at MTRH includes morphology of the biopsy specimen or aspirate and limited immunohistochemistry panels. Tumour specimens from 19 children enrolled from 2016 to 2018 in a prospective study to improve the diagnosis and staging of children with suspected BL were evaluated. Touch preps from biopsy specimens or smears from fine needle aspiration were collected, stained with Giemsa and/or H&E and reviewed by pathologists to render a provisional diagnosis. Unstained slides were stored and later processed for FISH. Duplicate slides were split between two laboratories for analysis. Flow cytometry results were available for all specimens. Results from the newly established FISH laboratory in Eldoret, Kenya were cross-validated in Indianapolis, Indiana. Concordance studies found 18 of 19 (95%) of specimens studied yielded analysable FISH results for one or both probe sets (MYC and MYC/IGH) in both locations. There was 94% (17/18) concordance of results between the two FISH laboratories. FISH results were 100% concordant for the 16 specimens with a histopathological diagnosis of BL and two of three non-BL cases (one case no result in IU FISH lab). FISH was similarly concordant with flow cytometry for specimens with positive flow results with the exception of a nasopharyngeal tumour with positive flow results for CD10 and CD20 but was negative by FISH. The modal turn-around time for FISH testing on retrospective study specimens performed in Kenya ranged between 24 and 72 hours. FISH testing was established, and a pilot study performed, to assess the feasibility of FISH as a diagnostic tool for the determination of BL in a Kenyan paediatric population. This study supports FISH in limited resource settings to improve the accuracy and speed of diagnosis of BL in Africa.

Renal Burkitt Lymphoma: An Unusual Extra-Nodal Condition Diagnosed in a Child in Niger

Renal Burkitt lymphoma is an aggressive malignant extra-ganglionic blood disease. It is an unusual event in children. A case is reported below. The patient was 8 years old; she was presenting with acute renal failure and edematous syndrome; proteinuria was mild. There was no organ enlargement nor adenopathy. After a while the diagnosis of Burkitt lymphoma was made after an anatomo-pathological examination of a kidney biopsy taken as part of an etiological assessment of this acute renal failure (urea=35 mmol/L, serum creatinine= 880 µmol/L) with an impaired general condition (WHO 3). The histological characteristics strongly suggested a lymphomatous process. An immunohistochemical examination confirmed renal Burkitt lymphoma. Unfortunately the patient died before chemotherapy was attempted

Impact of timing and precision of histopathological diagnosis on outcomes of patients with Burkitt lymphoma and high-grade B-cell lymphoma.

Burkitt lymphoma (BL) and diffuse large B-cell lymphoma (DLBCL) are aggressive B-cell non-Hodgkin lymphomas (B-NHL) with a generally favorable prognosis after immunochemotherapy. The outcome of BL is superior to DLBCL. In 2016, a distinct group of lymphomas displaying characteristics of both BL and DLBCL (high grade B-cell lymphoma, HGBL) was introduced into the WHO classification. Histopathological discrimination of BL, DLBCL, and HGBL may be challenging. Data on the frequency of histopathological difficulties resulting in revision of the final diagnosis of BL/DLBCL/HGBL and its impact on the prognosis are limited. We assessed histopathological features and clinical outcomes of 66 patients with suspected diagnosis of BL at the reporting institution between 2010 and 2020. The median age was 51 years (range 19-82) and final histopathological diagnosis revealed BL (n=40), DLBCL (n=12), or HGBL (n=14). Patients with DLBCL and HGBL were either treated with DLBCL-directed (83.3% and 35.7%) or BL-directed (16.7% and 64.3%) protocols. Patients in whom diagnosis was revised from DLBCL to BL after initiation of DLBCL-directed treatment had a significantly inferior progression-free survival (PFS) than patients initially diagnosed with BL (p=0.045), thus resembling rather the prognosis of DLBCL/HGBL. There was no difference between patients with DLBCL and HGBL, respectively, regarding PFS and OS (p=0.38 and p=0.27). These results suggest that timely and precise histopathological diagnosis as well as reference histopathological review of the underlying lymphoma is critical to determine up-front treatment strategies. Consequently, selection of more aggressive treatment protocols in case of difficulties with discrimination between DLBCL/HGBL/BL may be a reasonable approach.

Malignant Childhood Solid Tumours in Ile-Ife, Nigeria: A Ten-Year Retrospective Review

Introduction: Childhood cancer is fast becoming a growing global challenge and gaining global attention especially in low and middle-income countries (LMIC) where there is associated high morbidity and mortality. This review is to determine the patterns of malignant childhood solid tumours in our hospital and to compare this with previous studies. Methods: A 10-year retrospective review in which all histologically diagnosed malignant solid tumours between ages 0-15 years were analysed. The surgical daybook and the histopathology request cards were retrieved and the patients’ biodata, nature of specimen and tumour sites were extracted. Subgrouping of the various types of tumour was done based on the third edition (2005) of International Classification of Childhood Cancers (ICCC-3). Results: There were 124 cases of malignant childhood solid tumours with an average of 12.4 cases per year. Seventy-eight cases (62.9 %) were male, while 46 cases (37.1%) were female, hence a male to female ratio of 1.7:1. The age ranges were from 4 months to 15 years. The five most common tumour subgroups according to ICCC-3 were nephroblastoma (25 cases; 20.2%), non-Hodgkin lymphoma (NHL) (21 cases; 16.9%), retinoblastoma (11 cases; 8.9%), rhabdomyosarcoma (10 cases; 8.1%), and Burkitt lymphoma (9 cases; 7.2%). Conclusion: Nephroblastoma is the most common childhood solid tumour in our centre, while Burkitt lymphoma is the 5th most common. There seems to be a decline in the diagnosis of Burkitt lymphoma and retinoblastoma in our Department over the last 10 years, whether this represents a true decline in the incidence need further studies.

Diagnostic Value of PET/MR with DWI for Burkitt Lymphoma.

18F-FDG-PET/MR images, including DWI, of a 46-year-old male admitted to the Emergency Room of our tertiary center, who was suffering from diplopia, left orbital pain, and a headache for two weeks, demonstrated multiple hepatic nodules, a pancreatic mass, and skeletal metastases, in addition to thrombosis of the left cavernous sinus, thickening of the small intestine, and a large hepatic lesion identified at head and neck MR and whole-body CT, respectively. Hepatic and bone marrow biopsies revealed the diagnosis of Burkitt lymphoma. After four cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, methotrexate/ifosfamide, etoposide, and high dose cytarabine (R- CODOX-M/IVAC), a complete metabolic response occurred.

BURKITT LYMPHOMA MIMICKING SEPTIC SHOCK: AN UNUSUAL PRESENTATION

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Burkitt lymphoma (BL) is a highly aggressive non-Hodgkin B-cell lymphoma that typically presents with multiple metabolic derangements, kidney failure, involvement of the jaw or other facial bones, and enlarged lymph nodes. Our case report describes an initial presentation of (BL) with septic shock. CASE PRESENTATION: 25-year-old Caucasian female presented with a 4-day history of sharp non-radiating right upper quadrant abdominal pain, poor appetite, nausea, multiple episodes of loose watery stools, and polydipsia. Exam was notable for 2/4 SIRS criteria, with heart rate 134 and respiratory rate 29, and right upper quadrant was tender to palpation. Workup revealed white blood cell count 10.3 thou/uL, lactate 4.5 mmol/L, total bilirubin 1.5 mg/dL with direct bilirubin 0.8 mg/dL, AST 138 U/L, ALT 177 U/L, and alkaline phosphatase 601 U/L. Despite a 30cc/kg fluid bolus for resuscitation, lactate worsened to 5.2 mmol/L. With concern for septic shock, the patient was started on broad-spectrum antibiotics and admitted to the hospital with an intra-abdominal source suspected. Computed Tomography (CT) scan of the abdomen and pelvis with contrast demonstrated numerous hypoenhancing lesions of the liver (up to 80% of parenchyma), spleen, right kidney, and right adrenal gland. Blood cultures remained negative, with antibiotics discontinued after 48 hours, and lactate clearing with further fluid resuscitation. The patient underwent biopsy of the liver, which revealed findings consistent with Burkitt lymphoma (CD10, 19, 20 + on flow cytometry; Myc+, bcl2 and 6 – on FISH. PAX5, BCL6, and CD10 + and bcl2 - on immunochemistry). With improvement in symptoms, the patient was discharged with oncology follow-up. DISCUSSION: BL is a rare, but very aggressive, B-cell non-Hodgkin lymphoma, often associated with Ebstein Barr virus, human immunodeficiency virus, and overexpression of oncogene C-MYC on chromosome 8. It presents in three definite forms: endemic, sporadic, immunodeficiency-associated[4]. The sporadic variant is more common among caucasians[3] and in patients below 35 years of age with a median age at diagnosis of 30 years[1,2]. Patients usually present with abdominal signs and symptoms involving the small and large intestine mimicking acute appendicitis or intussusception. Excisional biopsy is preferred for the diagnosis of BL, with confirmation done by immunohistochemistry and cytogenetics. While aggressive, it is highly curable with a two-year survival rate of 80-90%, treatment consisting of multi-agent regimens. CONCLUSIONS: Clinicians, especially intensivists, should be aware of the numerous clinical conditions, such as Burkitt Lymphoma, which may mimic sepsis and septic shock as early diagnosis can improve outcomes and decrease overuse of antibiotics. Diagnosing Burkitt Lymphoma early can also improve patients' mortality with the initiation of early treatment to optimize survival. REFERENCE #1: World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Swerdlow SH, Campo E, Harris NL, et al. (Eds), IARC Press, Lyon 2008. REFERENCE #2: Armitage JO, Weisenburger DD ., New approach to classifying non-Hodgkin's lymphomas: clinical features of the major histologic subtypes. Non-Hodgkin's Lymphoma Classification Project., J Clin Oncol. 1998;16(8):2780. REFERENCE #3: Morton LM, Wang SS, Devesa SS, Hartge P, Weisenburger DD, Linet MS., Lymphoma incidence patterns by WHO subtype in the United States, 1992-200., Blood. 2006;107(1):265. Epub 2005 Sep 8. DISCLOSURES: No relevant relationships by Bohdan Baralo, source=Web Response No relevant relationships by Shambo Guha Roy, source=Web Response No relevant relationships by SANA MULLA, source=Web Response No relevant relationships by Ruqqiya Mustaqeem, source=Web Response No relevant relationships by Monisha Panda, source=Web Response No relevant relationships by Mahati Paravathaneni, source=Web Response No relevant relationships by Rajesh Thirumaran, source=Web Response No relevant relationships by Vihitha Thota, source=Web Response

Clinicopathologic characteristics of Burkitt lymphoma of the head and neck in a non-endemic region—a Danish nationwide study

Background Burkitt lymphoma rarely presents in head and neck (H&N) in Western countries. Aims/objectives We aimed to characterise clinicopathological features of H&N Burkitt lymphoma in Denmark representing a non-endemic region. Material and methods Clinical records were reviewed for a nationwide cohort of patients diagnosed with H&N Burkitt lymphoma in Denmark between 1980 and 2018. The diagnosis was histologically validated. Results Thirty-four patients with H&N Burkitt lymphoma (highest incidence in age group 0–9 years, male-to-female ratio 4.7:1) were included. Thirty-three lymphomas (97%) were extranodal. The tumour was visible at the clinical examination in 81% (n = 22) of the cases. The palatine tonsils were the most frequent location (n = 13, 38%) and 52% (n = 17) of the patients were diagnosed in advanced stage. Lymphoma was the tentative clinical diagnosis in 23% of the cases. The 5-year overall- and disease-specific survival was 78% and 81%, respectively. Conclusions Due to the rarity of Burkitt lymphoma of the H&N, there is a high risk of clinical misdiagnosis. Our findings suggest which symptoms and clinical presentations to be aware of in the diagnostics work up that could lead to the diagnosis of Burkitt lymphoma.

Autologous Stem Cell Transplantation For Adult Burkitt Lymphoma In The Rituximab Era: A Retrospective Study Of The Lymphoma Working Party (LWP) Of The European Group For Blood and Marrow Transplantation (EBMT)

IntroductionThe role of autologous stem cell transplantation (ASCT) for Burkitt Lymphoma (BL) in adults in the era of modern intensive chemotherapeutic regimes remains to be defined. The addition of Rituximab has been shown to improve outcomes in patients with BL, but how this influences outcomes for patients receiving ASCT is not clear. Prior studies of ASCT in BL have often included large number of patients treated with outmoded CHOP-like regimes, have often included children, and have combined outcomes of BL and Burkitt-like lymphoma (BLL). MethodWe conducted a retrospective registry based analysis of adult patients who were reported to the EBMT as having a diagnosis of BL and who had received an ASCT in the period of 2000-2011. Treatment history and histology reports were collected as part of the study. ResultsData including histology reports were returned for 125 patients who received a first ASCT between 2000 and 2011. Median age was 38.5y (range 16-64); 89 patients (71%) were male. While the diagnosis by pathology report was considered to be classic BL in 97 (78%) cases, the diagnostic possibility of BLL or atypical BL was considered in 28 (22%) cases. Twenty-one patients (17%) had stage I/II and 99 (80.5%) had stage III/IV disease. Fifty-one patients (53.7%) received Rituximab as part of 1st line treatment. Of all patients, 90 (74%), and 31 (25%) patients received ASCT in CR1/ VGPR1/PR1, and >CR1/ advanced disease (AD), respectively.5 year estimates for overall survival (OS) for the CR1 and VGPR1/PR groups were 80% and 71% respectively (p=0.23). Factor that was significant for OS in multivariate analysis (MVA) by Cox modeling, for the CR1/VGPR1/PR1 group was age <39 vs >39 (HR = 6.21 ; 95%CI 2.03-19 ; p=0.0013); status at transplant, i.e., PR1/VGPR1 vs CR1 (HR = 2.25 ; 95%CI 0.90-5.61) approached significance (p=0.081). Age >39 was the only significant risk factor for disease fee survival (DFS) in this group (HR = 3.11 ; 95%CI 1.27-7.6 ;p=0.0131). CNS involvement was a significant risk factor for relapse (HR 3.58, 95%CI 1.27-11.62, p= 0.017). Of note, administration of Rituximab as part of 1stline therapy did not appear to impact on OS or DFS. For patients receiving ASCT in CR>1 and AD, the OS was 59% and 25% respectively (p=0.042). Achievement of a subsequent CR had a significant impact on OS (HR 2.84, 95%CI 1.03-7.79, p=0.042) and DFS (HR 2.64, 95%CI 0.97-7.17, p=0.056). There was a trend for improved DFS for the BL vs the BLL patients that approached statistical significance (p=0.059). ConclusionIn this analysis of 125 adult patients with BL and BLL who received an ASCT in the era of intensive chemoimmunotherapy, disease status at transplant was the main determinant of outcome; age influenced OS and DFS for patients autografted in CR1/PR1/VGPR1. Prior Rituximab therapy did not predict post ASCT outcomes. Patients in PR1/ VGPR1 at the time of ASCT had outcomes that were not significantly inferior to those transplanted in CR1, and the former group of patients represent a subset who are most likely to derive a benefit from proceeding to an upfront ASCT. For those patients who have a chemosensitive relapse, ASCT offers the probability of salvaging a significant proportion. Patients with AD and chemoresistant disease had poor outcomes, and in such patients modalities of treatment other than ASCT should be pursued. Disclosures:Sureda:Novartis: Consultancy, Membership on an entity's Board of Directors or advisory committees. Afanasyev:Alexion: Honoraria. Dreger:Riemser Pharma: Consultancy, Honoraria, Research Funding.

Burkitt Lymphoma Presenting as a Paraneoplastic Demyelinating Disorder in a Child

AbstractIn adults, lymphomas have been associated with paraneoplastic syndromes that cause cranial nerve palsies, polyneuropathies, and paraplegias. These have been less frequently reported in children. We described a pediatric patient who initially presented with palsy of her left third cranial nerve and bilateral ptosis. Initial diagnosis was concerning for myasthenia gravis, but after additional diagnostic evaluation, she was found to have acute motor axonal neuropathy. Cerebrospinal fluid studies were consistent with a diagnosis of Burkitt lymphoma. In this article, we discussed the importance of considering primary oncologic diagnoses in acute neurologic disorders with unusual presentations.

Molecular characterization of Burkitt lymphoma in the breast or ovary

Breast and ovary have been described as rare but typical sites of presentation of Burkitt lymphoma (BL) in females, particularly after puberty. We revised a historic series of 44 lymphomas of the breast or the ovary in women diagnosed between 1973 and 2014 as BL. Fluorescence in situ hybridization (FISH) was applied to all, and array-based copy number analysis as well as expression profiling to a subset of those cases. Of the 42 cases evaluable for FISH, 19 cases showed an IG-MYC translocation but only 9 of those fulfilled the criteria of the current WHO classification for the diagnosis of BL. Those nine cases resembled BL of other sites with regard to molecular features. Our findings along with literature data suggest that breast and ovarian BL (1) seem to be rarer than hitherto assumed, (2) share typical molecular features with other BL, and (3) predominantly affect women in the fertile age.

An Atypical Case of Burkitt Lymphoma with Diffuse Peritoneal, Omental and Periportal Invasion

Diffuse peritoneal and especially periportal invasion of Burkitt lymphoma is very rarely seen. We present a case of a 16-year-old boy presenting with abdominal pain and constipation, without improvement after enemas. Abdominal ultrasound revealed a diffuse infiltrating peritoneal mass, omental caking, ascites, and a periportal mass, confirmed by computed tomography (CT). No lymphadenopathies were seen. Biopsy was performed and the diagnosis of Burkitt lymphoma was made. Although very rare, it is important to include lymphoma in the differential diagnosis of diffuse peritoneal and periportal invasion in children, as it can mimic peritoneal carcinomatosis.

Pathological fracture of the knee revealing Burkitt lymphoma: a case report

Burkitt lymphoma (BL) is a highly mature B-cell non-Hodgkin lymphoma that presents 1 to 2% of all adult lymphomas. Its pathophysiology is still poorly defined. The prognosis depends on the location and stage. Clinical manifestations are not specific however the most common symptoms are pain and mass. Burkitt lymphoma can be subcategorized into 3 variants: endemic; sporadic; and BL with immunodeficiency, typically infection with HIV. Bone involvement in this type of lymphoma is a rare event; very few reports about this entity exist in the literature. We report the case of bone Burkitt lymphoma in a 49-year-old female patient who presented 1 month before his admission with the pain of the left knee and abdominal pain, progressing in a context of alteration of general condition. The clinical examination found a performance status of 1, local examination revealed partial functional impotence of the knee with severe pain on palpation and on active and passive mobilization. Standard X-ray of the knee revealed a fracture of the upper and tibia, CT scan of the knee revealed a process of osteolytic epiphyseal-metaphyseal with tissue damage of the tibia and invasion of soft tissue with multiple nodular intramedullary epiphyseal. The biopsy of the mass with anatomopathological and immunohistochemical examination showed a tumor proliferation of lymphoid cells with CD20+, BCL 6+, and CD10 +, MUM1+, with a 100% KI 67, the diagnosis of Burkitt lymphoma (BL) was confirmed. The C-TAP CT scan shows an abdominal mass, the BOM was normal, the myelogram was normal, Lactate dehydrogenase (LDH) was elevated to 590 IU/L. The patient was put on the R-CODOX-M/R-IVAC protocol. Burkitt lymphoma is a rapidly proliferating tumor with a poor prognosis, it's suspected in any abdominal masses. Bone involvement as a revealing event in this type of lymphoma is a very rare event and the diagnosis should be established quickly followed by rapid initiation of definitive treatment for a better prognosis.

Identification of clinical molecular targets for childhood Burkitt lymphoma.

Burkitt lymphoma (BL) is a malignant tumor in children. Although BL is generally curable, early relapse and refractoriness may occur. Some molecular indicators have been recently suggested for BL diagnosis, but large heterogeneity still exists. This study aimed at providing clinical molecular targets and methods that may help improve diagnosis and treatment of childhood BL. Only children patients were included in the study, and targeted gene sequencing was conducted to identify tumor specific mutations. The mRNA and protein level expression of potential target genes were measured by real-time PCR and immunohistochemistry. The relationship between BL specific gene mutation and differential expression with clinical features was analyzed. The results showed that i) detailed analysis of c-MYC/BCL2/BCL6 gene loci alteration and gene expression would help in accurate diagnosis and treatment determination of childhood BL; ii) loss-of-function mutations in SOCS1 or CIITA gene might be used as malignant markers for BL diagnosis and prognosis; iii) specific mutations of CD79A, MYD88, KLF2, DNMT3A and NFKBIE genes often concurrently existed in BL and showed association with benign clinical outcomes; iv) the high expression of MYC, TCF3 and loss-of-function ID3 genes in tumor may be potential therapeutic targets and could be used for treatment monitoring; and v) four MYC-translocation negative cases were re-defined as high-grade B-cell lymphoma-not otherwise specified (HGBL-NOS) but showed similar clinical outcomes and molecular features to other BL cases in the study, suggesting more studies needed to explore the molecular mechanisms and clinical significance of this provisional tumor entity.

BURKITT LYMPHOMA IN A PEDIATRIC PATIENT: A CASE REPORT

Burkitt lymphoma (BL) is a very rare non-Hodgkin lymphoma with extremely short doubling time that tends to affect children in the jaws. Here we report the case of a 6-year-old male patient who attended emergency service because of jaw pain, swelling, and dental mobility for 20 days. Biopsy was performed, and the diagnosis of BL was established. The patient was immediately admitted at the oncohematology center of a pediatric institute for treatment onset. Admission examination revealed generalized lymphadenopathy, sagittal sinus thrombosis, and multiple tumoral infiltration in sphenoidal and maxillary sinuses, liver, lung, kidneys, pancreas, and spinal canal. Treatment was established according to NHL-BFM-95 protocol, and currently the patient is in complete remission without signs of disease. This report demonstrates that rapid and precise diagnosis associated with the immediate onset of treatment was essential for the successful evolution of the patient and should be considered determinant for the prognosis of BL. Burkitt lymphoma (BL) is a very rare non-Hodgkin lymphoma with extremely short doubling time that tends to affect children in the jaws. Here we report the case of a 6-year-old male patient who attended emergency service because of jaw pain, swelling, and dental mobility for 20 days. Biopsy was performed, and the diagnosis of BL was established. The patient was immediately admitted at the oncohematology center of a pediatric institute for treatment onset. Admission examination revealed generalized lymphadenopathy, sagittal sinus thrombosis, and multiple tumoral infiltration in sphenoidal and maxillary sinuses, liver, lung, kidneys, pancreas, and spinal canal. Treatment was established according to NHL-BFM-95 protocol, and currently the patient is in complete remission without signs of disease. This report demonstrates that rapid and precise diagnosis associated with the immediate onset of treatment was essential for the successful evolution of the patient and should be considered determinant for the prognosis of BL.

COMPLETE REMISSION OF BURKITT LYMPHOMA IN A CHILD: A CASE REPORT

Burkitt lymphoma (BL) is a type of non-Hodgkin lymphoma that originates from the malignant transformation of B lymphocytes. A 5-year-old boy presented bilateral swelling between the middle and lower third of the face, with limitation of mouth opening and headache. The computed tomography revealed areas suggestive of a tumor with invasion of the maxillary sinuses and initially of the orbital cavities. Under general anesthesia, an intraoral incisional biopsy was performed. Microscopic analysis revealed atypical lymphoid proliferation, and the immunohistochemical labeling was positive for CD10, CD20, and BCL-6 and in 100% for Ki-67 and negative for BCL-2. The final diagnosis of BL was established. The patient underwent treatment at a referral hospital following the protocol of the Brazilian Society of Pediatric Oncology and remains free of the disease for 24 months. BL is highly aggressive, and the dentist plays an important role in early diagnosis. Acknowledgments: CNPq.