Burkitt lymphoma of the head and neck: An international collaborative study.

BACKGROUND: Burkitt lymphoma (BL) is an aggressive B-cell lymphoma with three variants (endemic, sporadic, and immunodeficiency-associated), presenting with specific epidemiological and clinical features. Burkitt lymphoma affects the head and neck region (BLHN) in approximately 10% of cases. The aim of this study was to undertake a comparative analysis of the clinicopathologic and immunohistochemical (IHC) features of BLHN diagnosed in patients from Africa, Guatemala, and Brazil. METHODS: Cases diagnosed as BLHN were collected from the files of six oral pathology laboratory services (Brazil, South Africa, and Guatemala) and one Brazilian pediatric oncology hospital from 1986 to 2020. Clinicopathological and IHC data, and Epstein-Barr virus (EBV) status by in situ hybridization data for each case were reviewed and described. RESULTS: Of the 52 cases, BLHN was predominant in pediatric patients [43 (82.69%)] and males [43 (82.69%)], with a mean age of 11.26 ± 9.68 years (range, 1-39 years). Neck and cervical lymph nodes [14 (26.92%)], and involvement of both maxilla and mandible [8 (15.38%)], were the most common anatomical sites. Clinically, tumor/swelling [40 (31.25%)], cervical lymphadenopathy [14 (10.94%)], pain [12 (9.38%)], and bone destruction [12 (9.38%)] were frequent findings. All cases showed typical morphological characteristics of BL. IHC profiles included positivity for CD20 [52 (100%)], CD10 [38 (79.17%)], Bcl6 [29 (87.88%)], and c-Myc protein [18 (81.82%)]. EBV was positive in 18 cases (62.07%). The Ki-67 index ranged from 90 to 100%. CONCLUSION: The clinicopathological and EBV profile of BLHN in South African, Guatemalan, and Brazilian patients is similar.

Burkitt's lymphoma of mandible in a young Pakistani boy: A case report.

In this part of the world, Burkitt's lymphoma (BL) of mandible considers a unique entity, while BL involving the jaw is endemic in Africa. BL being one of the most speedily growing malignancy, can cause immense destruction in the maxillofacial region. This case report highlights a 3-year-old child affected with BL of mandible. The presenting complaint of the patient was a rapidly enlarging swelling on the right side of the face, causing disfigurement and interfering with the normal oral functions. Confirmatory diagnosis of BL was established after correlating the clinical, radiographic, and biopsy features. Later on a tumour board discussion was held for this case, and the child underwent chemotherapy. Unfortunately, he could not survive after the second cycle of chemotherapy. This emphasises that for every fast growing jaw lesion, BL should have a significant position in the list of differential diagnosis, even in this part of the world without considering the age of the patient.

Development of medical-grade, discrete, multi-walled carbon nanotubes as drug delivery molecules to enhance the treatment of hematological malignancies.

Carbon nanotubes (CNTs) hold great potential as drug delivery transporters given their large drug-binding surface area. Herein, we designed novel, multi-walled, discrete CNTs (dMWCNTs), PEGylated dMWCNTs (PEG-dMWCNTs), and bone-targeting (BT), alendronate-conjugated PEG-dMWCNTs (BT-PEG-dMWCNTs). Using zeta potential, thermogravimetric analysis, TEM, SEM, and FTIR, dMWCNTs were characterized as individual, uniform, and stable. Drug binding and release assays validated dMWCNTs as effective doxorubicin (DOX) transporters. The mass ratio of DOX loading onto dMWCNTs was 35% wt/wt with a ~95% wt/wt efficiency. DOX release was ~51% w/w after 48 hours. Neoplastic transformation, chromosomal aberration, and cytotoxicity assays, confirmed biocompatibility for all dMWCNTs. PEG-dMWCNTs were well tolerated and modulated drug pharmacokinetics in mice. In mice with Burkitt's lymphoma, DOX-loaded PEG-dMWCNTs and BT-PEG-dMWCNTs reduced tumor burden and increased survival similarly to free drug. Importantly, DOX toxicity was abrogated when DOX was loaded onto PEG-dMWCNTs or BT-PEG-dMWCNTs. Overall, PEG-dMWCNTs and BT-PEG-dMWCNTs represent a promising new nanocarrier platform.

BURKITT LYMPHOMA IN GASTROINTESTINAL TRACT: A REPORT OF TWO CASES.

Burkitt lymphoma, a type of non-Hodgkin B-cell lymphoma, is the fastest growing human cancer, presenting pathologically with a 'starry sky' pattern. It is most often found in the abdomen and the jaw, however, localization in the abdomen other than the ileocecal area is very rare and described only in a handful of cases. Standard treatment consists of initial tumor cytoreduction followed by intense chemotherapy. Most of the relapses occur within one year of the diagnosis, while the 5-year survival is around 80%. We present two cases which are specific for unusual location of Burkitt lymphoma in the colon and stomach, in immunocompetent patients with negative Epstein-Barr virus tests. Also, one of the patients presented is one of the oldest ever reported with abdominal Burkitt lymphoma, while the other patient is an example of diagnostic difficulties in distinguishing Burkitt lymphoma from similar lymphomas. Due to the rapidly growing tumors and urgent need for cytoreductive surgery, it is crucial to consider the diagnosis of Burkitt lymphoma even in atypical localizations or absence of the common risk factors associated with Burkitt lymphoma.

The changing clinical pattern of endemic Burkitt lymphoma in Western Africa: Experience from a tertiary center in Ghana.

BACKGROUND: Burkitt lymphoma (BL) is the most common childhood cancer in Ghana, where the endemic variant is the predominant subtype and historically presents as a highly chemo-sensitive jaw tumor. This study aimed to update the current epidemiological characteristics of childhood BL in our institution. PROCEDURE: Patient data for all children diagnosed with BL and seen at Korle Bu Teaching Hospital between January 2007 and December 2012 were retrospectively analyzed. RESULTS: BL was diagnosed in 173 children (<13 years) during the study period, with the abdomen as the most common tumor site (46%) followed by the jaw (31%). Abdominal tumors were associated with advanced/disseminated disease (P = 0.002), and were more likely to occur in females irrespective of tumor stage (relative risk = 1.56 [95% CI; 1.1-12.3]). Twenty-five percent (43/173) of the study cohort died and mortality was influenced by increasing age (P = 0.02) and advanced disease (P = 0.03). Treatment delay was experienced by nine in ten patients primarily due to familial financial constraint (75%). Treatment abandonment was observed as a first event in 94% of patients and two thirds of children in the study were eventually lost to follow-up. CONCLUSION: The predominance of primary abdominal tumors in our study cohort may indicate a changing epidemiological pattern of BL in Ghana. High rates of treatment delay and abandonment were evident and are likely to be contributing factors to the poor childhood cancer survival outcomes seen in resource-limited countries in Africa.

Denis Burkitt and the origins of the dietary fibre hypothesis.

For more than 200 years the fibre in plant foods has been known by animal nutritionists to have significant effects on digestion. Its role in human nutrition began to be investigated towards the end of the 19th century. However, between 1966 and 1972, Denis Burkitt, a surgeon who had recently returned from Africa, brought together ideas from a range of disciplines together with observations from his own experience to propose a radical view of the role of fibre in human health. Burkitt came late to the fibre story but built on the work of three physicians (Peter Cleave, G. D. Campbell and Hugh Trowell), a surgeon (Neil Painter) and a biochemist (Alec Walker) to propose that diets low in fibre increase the risk of CHD, obesity, diabetes, dental caries, various vascular disorders and large bowel conditions such as cancer, appendicitis and diverticulosis. Simply grouping these diseases together as having a common cause was groundbreaking. Proposing fibre as the key stimulated much research but also controversy. Credit for the dietary fibre hypothesis is given largely to Burkitt who became known as the 'Fibre Man'. This paper sets out the story of the development of the fibre hypothesis, and the contribution to it of these individuals.

Epstein-Barr Virus Type 2 Infects T Cells in Healthy Kenyan Children.

Background: The 2 strains of Epstein-Barr virus (EBV), EBV type 1 (EBV-1) and EBV-2, differ in latency genes, suggesting that they use distinct mechanisms to establish latency. We previously reported that EBV-2 infects T cells in vitro. In this study, we tested the possibility that EBV-2 infects T cells in vivo. Methods: Purified T-cell fractions isolated from children positive for EBV-1 or EBV-2 and their mothers were examined for the presence of EBV and for EBV type. Results: We detected EBV-2 in all T-cell samples obtained from EBV-2-infected children at 12 months of age, with some children retaining EBV-2-positive T cells through 24 months of age, suggesting that EBV-2 persists in T cells. We were unable to detect EBV-2 in T-cell samples from mothers but could detect EBV-2 in samples of their breast milk and saliva. Conclusions: These data suggest that EBV-2 uses T cells as an additional latency reservoir but that, over time, the frequency of infected T cells may drop below detectable levels. Alternatively, EBV-2 may establish a prolonged transient infection in the T-cell compartment. Collectively, these novel findings demonstrate that EBV-2 infects T cells in vivo and suggest EBV-2 may use the T-cell compartment to establish latency.

The role of traditional healers in the diagnosis and management of Burkitt lymphoma in Cameroon: understanding the challenges and moving forward.

BACKGROUND: Burkittlymphoma(BL) is the most common childhood cancer in Cameroon with a reported incidence of 3 per 100,000 children under 15 years in the Northwest region. Treatment at three Baptist mission hospitals has a recorded cure rate of over 50%. Traditional medicine(TM) is recognized by the national health system, but its scope is undefined and entraps children with BL. The aim of this study was to investigate the attitudes and practices of parents and traditional healers (TH) towards TM in children with BL in order to develop recommendations for an integrative approach and improved access to life-saving treatment for children with BL. METHODS: This is a descriptive case series of children diagnosed with BL treated at Banso, Mbingo, and Mutengene Baptist Hospitals between 2003 and 2014. A questionnaire was used to obtain the following information: demographic information, religion, the rate of use of TM, reasons why guardians chose to use TM, the diagnoses made by the TH, treatment offered, and the type of payment requested, based on the accounts of patient caregivers. Data was analyzed using Center for Disease Control Epi Info 7. RESULTS: Three hundred eighty-seven questionnaires were completed by parents/guardians. 55% had consulted a TH, of whom 76.1% consulted the TH as first choice. Common diagnoses provided by TH included liver problem, abscess, witchcraft, poison, hernia, side pain, mushroom in the belly and toothache. Methods of management included massage, cuts, concoctions, and incantations. The fee for these services included chickens, farm tools, and cash ranging from 200FCFA (0.4USD) to 100,000FCFA(200USD). The choice of TM was based on accessibility, failed clinic/hospital attendance, recommendation of relatives, and belief in TM. CONCLUSIONS: TH are involved in BL management in Cameroon. TH are ignorant about BL, resulting in non-referral, and thus delay in diagnosis and treatment. Collaboration with TH could reduce late diagnosis and improve cure rates of BL and other childhood cancers.

Complete progressive ophthalmoplegia and numb chin syndrome, the first clinical manifestations of a lethal abdominal Burkitt lymphoma.

A 57-year-old patient was admitted to the Neurology Clinic for hypoesthesia, intense pain in the right chin and double vision. During the hospitalization, the patient developed progressive complete bilateral ophthalmoplegia and numbness of both sides of the chin. Brain CT and MRI scans with gadolinium were normal. Standard laboratory tests on admission were normal. The cerebral spinal fluid examination and the infectious and autoimmune workup were also normal. A thoracic-abdominal and pelvic CT scan revealed two hypodense lesions in the liver, irregular thickening of the gastric and ileal wall, and multiple abdominal adenopathies. Meanwhile, the patient developed marked fatigue, fever, sweats, nausea, vomiting and abdominal pain. An exploratory laparotomy was performed that showed multiple tumours of the small intestinal wall, stomach wall, multiple liver masses in both lobes and appendicular tumour. Histopathological findings of the liver biopsy and appendicular walls revealed Burkitt lymphoma. The patient died two days after surgery by cardiopulmonary arrest. This case underscores the importance of keeping BL in the differential diagnosis of patients with rapidly progressive ophthalmoplegia and numb chin syndrome, with normal brain MRI and CSF examinations.

Liposomal cytarabine in prophylaxis or curative treatment of central nervous system involvement in Burkitt leukemia/lymphoma.

In recent years, the outcome of Burkitt leukemia/lymphoma (BL) has improved significantly. Central nervous system (CNS) involvement continues to be a poor prognostic indicator. High doses of intravenous polychemotherapy, intrathecal chemotherapy, and cranio-spinal radiation therapy are used by numerous groups. Majority of patients are cured after this strategy. The next challenge is to decrease toxicities of treatment, including long-term toxicities secondary to cranio-spinal radiation therapy observed in these cured patients. Liposomal cytarabine could be a good alternative to cranio-spinal radiation therapy as already reported in acute lymphoblastic leukemia. We report here eleven patients treated in our center for BL, with liposomal cytarabine instead of cranio-spinal radiation therapy as prophylactic or curative treatment for CNS involvement. Treatment was safe with no short-term grade >3 adverse events. Moreover, no long-term side effects and no impact on outcome were observed. We conclude that LC could be a good option to decrease short/long-term side effects of cranio-spinal radiation therapy in BL and could be evaluated in a future clinical trial.

Epstein-Barr virus viral load and serology in childhood non-Hodgkin's lymphoma and chronic inflammatory conditions in Uganda: implications for disease risk and characteristics.

Epstein-Barr virus (EBV) has been linked to malignancies and chronic inflammatory conditions. In this study, EBV detection was compared in children with non-Hodgkin's lymphoma and children with chronic inflammatory conditions, using samples and data from a case-control study carried out at the Mulago National Referral Hospital between 2004 and 2008. EBV viral load was measured in saliva, whole blood and white blood cells by real-time PCR. Serological values for IgG-VCA, EBNA1, and EAd-IgG were compared in non-Hodgkin's lymphoma and chronic inflammatory conditions; and in Burkitt's lymphoma and other subtypes of non-Hodgkin's lymphoma. Odds ratios (ORs) and corresponding 95% confidence intervals (CIs) were calculated. Of the 127 children included (87 males and 40 females; median age 7 years, range 2-17), 96 had non-Hodgkin's lymphoma (46 Burkitt's lymphoma and 50 other non-Hodgkin's lymphoma), 31 had chronic inflammatory conditions, and only 10% were HIV-positive. The most common clinical presentations for all disease categories considered were fever, night sweats, and weight loss. EBV viral load in whole blood was elevated in Burkitt's lymphoma compared to other non-Hodgkin's lymphoma (OR 6.67, 95% CI 1.32, 33.69; P-value = 0.04), but EBV viral loads in saliva and white blood cells were not different in any of the disease categories considered. A significant difference in EAd-IgG was observed when non-Hodgkin's lymphoma was compared with chronic inflammatory conditions (OR 0.19, 95% CI 0.07, 0.51; P-value = 0.001). When compared to chronic inflammatory conditions, EBV viral load was elevated in Burkitt's lymphoma, and EA IgG was higher in non-Hodgkin's lymphoma. This study supports an association between virological and serological markers of EBV and childhood non-Hodgkin's lymphoma, irrespective of subtype, in Uganda.

A Dental Extraction Revealing a Multisystem Burkitt's Lymphoma: A Case Report.

Burkitt's lymphoma is rare but highly aggressive and very fast-growing B-cell non-Hodgkin's lymphoma (NHL). It can affect any organ such as the central nervous system, jaw, intestines, kidneys, ovaries, and other organs. It results from the malignant evolution and proliferation of B-type lymphoid cells. The diagnosis is based on a biopsy of a tumor mass or bone marrow aspiration revealing the presence of tumor cells. We report the case of a 7 year old child who was referred for a gingival swelling evolving since 1 month following a dental extraction. Imaging and anatomopathological examination after biopsy concludes to a multi systemic Burkitt's lymphoma. A chemotherapy was immediately started with spectacular complete remission.

An Atypical Presentation of Burkitt Lymphoma Presenting As Large Intra-abdominal Masses Compressing Multiple Organs With Peri-Pericardial Involvement in an HIV Patient: A Case Report and Literature Review.

Many types of malignancies have been associated with immunodeficiency states, especially patients who are HIV positive. Burkitt lymphoma (BL) is one of those malignancies associated with HIV and it presents in three varieties. The endemic form is primarily seen in children, and it is associated with the Epstein-Barr virus (EBV). In this form, patients with Burkitt's present with a large jaw mass. The second variety is seen in older adults. These patients usually present with abdominal and pelvic masses. This subtype is more prominent in the United States. The third variety of BL is seen in patients who are HIV positive. In this case report, we present an atypical presentation of BL secondary to undiagnosed HIV/AIDS with a very large tumor burden causing compressive symptoms. This case will further guide healthcare professionals in diagnosing BL, which presents uniquely in high-risk populations. This report will also serve as a review of the diagnosis and treatment options of BL.

A multicenter phase II study incorporating high-dose rituximab and liposomal doxorubicin into the CODOX-M/IVAC regimen for untreated Burkitt's lymphoma.

BACKGROUND: Despite improvement with intensive multi-agent chemotherapy, 2-year progression-free survival (PFS) rates for adults with high-risk Burkitt's lymphoma (BL) remains <55%. PATIENTS AND METHODS: We conducted a phase II trial for newly diagnosed classic BL utilizing liposomal doxorubicin (Adriamycin) in lieu of doxorubicin and incorporating intravenous rituximab (at 500 mg/m(2) twice/cycle) into the CODOX-M/IVAC regimen. Correlative analyses included paired serum and cerebrospinal fluid (CSF) rituximab levels and close examination of cardiac function. RESULTS: Among 25 BL patients, the median age was 44 years (23-70) and 4 patients were HIV positive. There were 20 high-risk and 5 low-risk patients. At baseline, 40% of high-risk patients had bone marrow involvement, 35% had bulky disease and 15% had central nervous system involvement. The overall response rate was 100% (complete remission 92%). At 34-month median follow-up, the 2-year PFS and overall survival (OS) rates for all patients were 80% and 84%, respectively (low-risk: both 100%; high-risk: 76% and 81%, respectively). Furthermore, the 2-year PFS, OS, and disease-specific survival (DSS) rates for high-risk, HIV-negative patients were 84%, 89% and 100%, respectively. Adverse events (AEs) appeared to be consistent with prior CODOX-M/IVAC data, although there were several grade 3 cardiac events noted (all declined ejection fraction without clinical symptoms). The mean serum rituximab levels at 24 h after cycles 1 and 3 for patients without relapse were 258 and 306 µg/ml, respectively, versus 131 and 193 µg/ml, respectively, for patients with early progression (P = 0.002 and 0.002, respectively). The mean CSF rituximab levels for all patients were 0.11 and 0.24 µg/ml, respectively, at cycle 1 (24/72 h), which equated to serum:CSF ratios of 0.05% and 0.20%, respectively. CONCLUSIONS: The integration of rituximab into CODOX-M/IVAC for adult BL was feasible and tolerable, while changes in cardiac function warrant continued examination. This regimen was associated with excellent survival rates for HIV-negative BL. Further investigation of the predictive value of serum rituximab is needed. Clinicaltrials.gov NCT00392990.

A rare case of Burkitt's lymphoma of the breast in a 19-year-old male: Case report.

Key Clinical Message: Burkitt's lymphoma is a highly aggressive non-Hodgkin's lymphoma that can affect various parts of the body, which include jaw, facial bones, retroperitoneum, and abdominal viscera, it is worth noting that breast involvement is extremely rare in Burkitt's lymphoma and has not been documented. Abstract: Burkitt's lymphoma is a highly aggressive non-Hodgkin's lymphoma that can affect various parts of the body including breast. This sarcoma is identified as a rapidly fatal malignant lymphoma syndrome and 50% of all malignant tumors in children. However, breast involvement has not been documented. A 19-year-old male presented to our emergency department with a left breast swelling for 2 months associated with drenching night sweats, weight loss and evening fevers. Physical examination revealed a mass measuring approximately 15 × 16 × 15 cm in the widest dimension, skin hyperpigmentation, no nipple discolorations, discharges, and non-tender on palpation. Biopsy was done, and the histology report revealed sheets of monomorphic medium lymphocytes with a high mitotic rate and frequent apoptotic bodies showing a starry-sky appearance. The immunohistochemistry report revealed positive staining for Ki-67, CD-20, and CD-10 tumor markers and CD45 on flow cytometry. The patient was started on aggressive hydration, rasubricase administration, CODOX-M/IVAC regimen 6 cycles, and G-CSF and registered significant reduction in the size of the mass. Burkitt's lymphoma is a highly aggressive non-Hodgkin's lymphoma that can affect various parts of the body. It commonly involves the jaw, facial bones, retroperitoneum, and abdominal viscera. The disease typically affects young patients in areas of high incidence, such as the jaw, whereas visceral involvement is more common in older patients in low-incidence areas. It is worth noting that breast involvement is extremely rare in Burkitt's lymphoma.

Primary Breast Burkitt Lymphoma. A Case Report of a 16-Year-Old Female with a Rapidly Growing Unilateral Breast Mass.

Introduction: Primary breast Burkitt lymphoma is extremely rare. Commonly endemic Burkitt lymphoma presents with abdominal, jaw, periorbital, or genitourinary mass. Case Presentation: We report a case of a 16-year-old girl with rapidly enlarging left breast swelling associated with evening fevers. This was later confirmed to be stage 1 primary breast Burkitt lymphoma involving the left breast. This represents the first described case of primary breast endemic Burkitt lymphoma in Uganda. She was started on chemotherapy and exhibited an impressive response to the drugs. Conclusion: This case raises awareness of rare sites for endemic Burkitt lymphoma in Uganda. Accurately diagnosing this case was of great importance since it determined the treatment modality (mastectomy or not) which would have an everlasting impact on her life.

A Rare Finding of Pancreatic Involvement in a Case of Burkitt's Lymphoma.

Burkitt's lymphoma (BL) is a highly aggressive type of B-cell non-Hodgkin lymphoma. BL commonly occurs in children of age 4-7 years and is uncommon in adults, with a worse prognosis. Patients often present with a rapidly growing mass typically involving the abdomen (liver and spleen) and head and neck (nodes, jaw, and facial bones). Pancreas involvement is very rare and very few case reports have been documented so far. Fluorine-18 positron emission tomography/computed tomography (F-18 PET/CT) is a whole-body survey commonly used for initial staging evaluation. Here, we present an interesting case of BL in an adult female of 43 years, who presented with swelling in the left submandibular region after tooth extraction with multiorgan involvement found on F-18 fluorodeoxyglucose PET/CT.

[Spinal cord compression secondary to metastasis of gingival Burkitt lymphoma revealing a case of HIV infection in Morocco]. / Compression de la moelle épinière secondaire à une métastase d'un lymphome de Burkitt gingival révélant un cas d'infection par le VIH au Maroc.

Burkitt lymphoma in HIV-infected patients is seldom seen in the oral cavity, and they are rarely reported in the literature. The clinical manifestations may suggest other tumors or gingival infections, which delays diagnosis and management. Spinal cord involvement is exceptional and only a few cases are reported. We report a case of Burkitt lymphoma in the oral cavity and spinal cord, revealing HIV infection in a 44-year-old patient, who responded well to chemotherapy and antiretroviral treatment and is on remission.

A Rare Colon Polypectomy Diagnosis: Description of a Unique Investigational Journey and Literature Review of Burkitt Lymphoma.

The presentation of Burkitt lymphoma (BL) is variable and highly dependent on its subtype. It often presents with non-specific symptoms and may appear in the jaw, bone marrow, liver, spleen, kidneys, ovaries, and gastrointestinal tract. This case describes a 50-year-old male who presented with abdominal pain and was eventually found to have Burkitt lymphoma, diagnosed by biopsy of a cecal polyp. Herein, we explore the diagnostic journey to BL and review the literature regarding its unique manifestations and thus the diagnostic challenge it can present.

Burkitt's lymphoma: an unexpected cause of dental pain.

We would like to describe a case of Burkitt's lymphoma in a 37-year-old lady, who initially presented to her General Dental Practitioner (GDP) complaining of pain and episodic numbness in the right lip and chin. The initial diagnosis was irreversible pulpitis of the lower right second molar (LR7) and despite having an extraction of the affected tooth her problems persisted. The patient also developed pain in the spine and limbs, accompanied by lethargy and weakness. After seeing multiple different practitioners, she was referred to her local Oral and Maxillofacial Surgery department. Cross-sectional imaging was requested and demonstrated disseminated malignancy. Histology following open trans-oral biopsy revealed a diagnosis of Burkitt's lymphoma prompting an urgent haematology referral, and she successfully completed a chemotherapy regimen. This case highlights the importance of a thorough diagnostic process in the presence of persistent and/or atypical symptoms.