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Intestinal lymphomas can rarely present as abdominal catastrophes with perforation or small bowel obstruction. There is little data regarding their optimal surgical management and associated outcomes. We aimed to systematically review relevant published literature to assess the presentation, diagnosis, optimal surgical approach and associated post-operative outcomes. A systematic on-line literature search of Embase and Medline identified 1485 articles of which 34 relevant studies were selected, including 7 retrospective studies, 1 case series and 26 case reports. Selected articles were assessed by two reviewers to extract data. 95 patients with abdominal catastrophes secondary to lymphoma (predominately Burkitt (28 %) and Diffuse Large B-cell lymphoma (29 %)) were identified with a median age of 52 years, 40 % were female. Of the small bowel resections 25% (n = 18) suffered post-operative complications with a 13.8 % (n = 10) 30-day mortality. Ileocolonic resections had a 27 % complication rate with 18 % mortality and primary repair had a 25 % complications rate and 25 % mortality. Median follow-up was 8 days (range 1-96). Notable points of differences in the presentations between these different lymphomas included the majority of Burkitt's lymphoma were younger, had a known diagnosis, were on chemotherapy and presented with perforation in contrast to those with B cell lymphoma who were predominately older, had new diagnoses and presented with a balanced proportion of obstruction and perforation. Abdominal catastrophes secondary to intestinal lymphomas most commonly present with perforation. Aggressive surgical management, including small bowel resection, may offer similar remission rates for lymphoma patients presenting with abdominal catastrophes as those without such emergency complications., Competing Interests: Declarations of competing interest None., (© 2023 Published by Elsevier Ltd.)

Background: Primary hepatic Burkitt lymphoma (PHBL) in children is an extremely rare hepatic malignancy with a dismal prognosis, unless it is detected and treated promptly. An 11-year-old child with abdominal pain was admitted to our hospital. No notable abnormalities were found during his physical examination or laboratory workup, but the abdominal computed tomography and magnetic resonance imaging both indicated a malignant hepatic mass measuring 9.2 × 7.1 × 7.5 cm in size. His postoperative pathology revealed an unexpected primary hepatic Burkitt lymphoma following a laparoscopic liver lobectomy. He then received rituximab and intense multi-agent chemotherapy as treatment. Despite post-chemotherapy bone marrow suppression, the patient eventually made a full recovery and had a good overall state., Conclusion: In this study, we describe a rare case of pediatric primary hepatic Burkitt lymphoma and review the literature on clinical features, diagnosis, and treatment for primary hepatic Burkitt lymphoma in children. We stress that this diagnosis should be taken into account in the absence of other single hepatic lesions or primary tumors of hematological disorders, particularly when there is a normal AFP level., (© 2023. The Author(s).)

A 9-year-old girl presented with a recent history of shortness of breath, fatigue, visual disturbances, and gastrointestinal symptoms. Echocardiography demonstrated three large intracardiac masses in the right and left atria protruding into the mitral and tricuspid valve orifices causing bilateral inflow obstruction. She underwent urgent surgical excision of the masses. Histology revealed rare intracardiac Burkitt's Lymphoma.

We report a case of ileocecal intussusception due to Burkitt's lymphoma(BL). A 14-year-old boy was admitted to our hospital for abdominal pain and diarrhea. He was diagnosed an intussusception by the ultrasonography and the CT scan. Laparoscopic ileocecal resection was performed. A diagnosis of BL was made on basis of pathological examination. He was transferred for the chemotherapy on postoperative day 8. We conclude that, if the intussusception associated with malignant lymphoma is assumed from the preoperative findings, we have to keep minimal surgical invasion and start postoperative chemotherapy immediately.

Introduction: Intussusception is a common condition in children, it is rare in adults. Adult intussusception differs from pediatric intussusception in various respects, including etiology clinical characteristics and therapy., Methods: We present and discuss a new case of intussusception in children and adults., Results: In child the Barium Enema x-ray examination is identified an endoluminal filling defect to refer to the apex of the invaginated loop at the rectal level, with slow ascent during the progressive injection of the radiopaque contrast medium. At the end of the procedure, incomplete reduction of the picture is documented. The patient undergoes emergency surgery where the presence of an ileo-ceco-colic invagination is documented. Intussusception is reduced by taxis. In the adult laparoscopic right hemicolectomy was performed. High-grade B-cell Burkitt's lymphoma was confirmed by immunohistochemistry., Discussion: In contrast to intussusceptions in children, in the adult population, a demonstrable etiology is found in most of the cases. In adults surgery is always indicated. The non-invasive resolutive intervention most commonly used in the child and best known consists in the rectal introduction of a radiopaque contrast medium (air or barium) at controlled pressure until., Conclusions: Although intussusceptions occur at all ages, there are major differences in the clinical presentation, diagnostic approach, and management between pediatric and adult populations. Intussusception is remarkably different in these two age groups and it must be approached from a different clinical perspective., Key Words: Intussusception in children, Intussusception in adults, Intussusception symptoms, Radiology and treatment.

Introduction: The association of human immunodeficiency virus (HIV) infection with Burkitt lymphoma is related to the presence of Epstein Barr virus infection and the impact of the HIV antigen on the expansion of B-polyclonal cells. In Southeast Europe, the association is rare, and recognizing this is important in the therapeutic decision to increase patient survival rate. The association of HIV with Burkitt lymphoma and tuberculosis is even more rarely described in the literature., Patient Concerns: We present the case of a 40-year-old patient who presented with a 3-week history of fever (max. 38.7 °C), painful axillary swelling on the right side, lumbar pain, gait disorders, headache, and night sweats. Clinical manifestations included marked weight loss (about 30 kg in the last 2 months before his admission)., Diagnosis: A LyCD4 count of 38/μL and a HIV1 viral load of 384,000/mm3, classified the patient into a C3 stage. A biopsy of the right axillary lymph node was performed for suspected ganglionic tuberculosis due to immunodeficiency. Histopathological examination confirmed the diagnosis of Burkitt lymphoma. Cultures on Löwenstein-Jensen medium from sputum harvested at first admission were positive for Mycobacterium tuberculosis., Interventions: Highly active antiretroviral therapy, chemotherapeutic agents for Burkitt lymphoma, anti-tuberculous drug therapy, neurosurgical intervention of spinal cord decompression, and antibiotic therapy of the associated bacterial infection., Outcome: Burkitt lymphoma disseminated rapidly, with central nervous system, spinal cord, osteomuscular, adrenal, and spleen involvement. The evolution under treatment was unfavorable, with patient death occurring 6 months after diagnosis., Conclusions: The association of HIV infection with Burkitt lymphoma and tuberculosis is rare in the highly active antiretroviral therapy (HAART) era, posing prompt and multidisciplinary therapeutic management issues. Similar cases of HIV-TB and Burkitt lymphoma association have been described, but none of the other cases showed the involvement of the central nervous system or of the bilateral adrenal glands., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2020 the Author(s). Published by Wolters Kluwer Health, Inc.)

Primary Burkitt´s lymphoma (BL) of the prostate is rare in the adolescent population. The etiology remains poorly understood. There has been some proposed associations to Epstein-Barr virus and HIV. Clinical and histopathologic data of a 17-year-old patient who underwent transurethral resection of the prostate was obtained. We report the first case of primary malignant BL of the prostate in a 17-year-old Caucasian male who presented with hematuria, lower urinary tract symptoms. Differential diagnosis of a prostatic mass in adolescent patients must be primary or secondary lymphoma of the prostate, including BL as described for the first time in this article., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Vertebral pathological compression fracture involving extra-nodal lymphoma impacts negatively on the quality of life of HIV-positive patients. The choice of a safe and effective approach to palliative care in this condition remains a challenge., Objective: The purpose of this study was to investigate the safety and efficacy of percutaneous kyphoplasty (PKP) in the treatment of vertebral pathological compression fracture of extra-nodal lymphoma in HIV-positive patients., Methods: A retrospective analysis, from January 2016 to August 2019, was performed on 7 HIVpositive patients, 3 males and 4 females, with extra-nodal lymphoma with a vertebral pathological compression fracture. The patients were treated using percutaneous kyphoplasty in our hospital. Preoperative assessment of the patients was conducted regarding their hematological profile, biochemical indicators, liver and kidney function, blood coagulation function, CD4+T lymphocyte count and viral load. Subsequently, the patients were placed on highly active antiretroviral therapy (HAART) and rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (RCHOP) regimen. Besides, antibiotics, nutritional support and immune-modulating drugs were also administered, rationally. Postoperatively, the height of the anterior edge of the injured vertebrae, Visual Analogue Scale (VAS) and Oswestry Disability Index (ODI) values were evaluated. Patients were also monitored for any complications related to the operation., Results: The average CD4+T cell count for the patients was 164 (range 114 ~247 / ul), while the viral load was 26,269 (range 5,765 ~82,321 copies/ul). All patients received nutritional and immune support and registered significant improvements in the levels of ALB and Hb (P<0.05). In all cases, the operation was uneventful with neither cement leakage nor toxic reactions observed. Similarly, no opportunistic infections, other complications or deaths were reported. The height of the anterior vertebral body and the ODI score of the injured vertebrae were significantly improved immediately after surgery (P<0.05). Compared to the preoperative VAS (7.71±1.11), postoperative values were significantly reduced immediately after surgery (3.85±0.90) and at 2 weeks, 1 month and 6 months post-surgery: 2.71±0.76, 3.29±1.11, and 4.00±0.82, respectively (P<0.01)., Conclusion: Supported with appropriate perioperative treatment measures, PKP is safe and effective in the treatment of pathological vertebral compression fracture due to extra-nodal lymphoma in HIV-positive patients., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Background/aim: The aim of this study was to present the clinical characteristics, natural history and survival outcomes of primary gastrointestinal non-Hodgkin lymphomas (PGINHL) in the pediatric population., Patients and Methods: Surveillance, Epidemiology, and End Results (SEER) database was queried for patients aged 0 to 19 years with PGINHL between 1973 and 2014., Results: A total of 452 cases were identified [mean age 11.0 (±5.1)] years, whites 84.1%, males (76.5%). The majority of tumors were noted in the small bowel (SB) (47.6%), followed by large bowel (LB) (28.5%) and the stomach (10.0%). Overall, the most common histological subtype was Burkitt lymphoma (51.8%), followed by diffuse large B-cell lymphoma (DLBCL) (26.1%). Mean overall survival (OS) of the entire cohort was 33,33 years with a 5-yr, 10-yr and 30-yr survival rate of 86%, 86% and 79%, respectively. Large bowel tumors had the best long-term survival rates whereas; gastric tumors had the worst with 30-yr survival rate 84% and 74%, respectively. Overall, 328 (72.6%) patients received surgery. No significant survival difference was noted between patients who underwent surgery and those who did not., Conclusion: This study presents the largest dataset of pediatric PGINHL and describes the clinical features and outcomes of these patients in addition to summarizing the literature., (Copyright© 2019, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Rationale: HIV-related lymphoma, especially non-Hodgkin lymphoma, is one of the most common malignant tumors in HIV/acquired immune deficiency syndrome (AIDS) patients. Autologous hematopoietic stem cell transplantation (AHSCT) for the patients with Burkitt lymphoma (BL) is needed to be further explored., Patient Concerns: A 57-year-old man was hospitalized with intermittent pain on upper abdomen and melena for >1 month., Diagnosis: HIV antibody testing was positive. The upper gastrointestinal endoscopy was performed and histopathology and immunohistochemistry revealed BL., Interventions: Highly effective antiretroviral therapy and sixth cycles of chemotherapy were administered, followed by autologous hematopoietic stem cell transplantation., Outcomes: The patient has had tumor-free survival for >6 years with normal CD4+ T cell counts and HIV viral load below the lowest detection LESSONS:: The patient was treated with AHSCT followed complete remission after chemotherapy and achieved long-term disease-free survival. AHSCT may be a promising way for clinical cure of HIV-related BL.

We report the case of a 9-year-old boy who presented with abdominal pain and was found to have an intussusception with a sporadic Burkitt's lymphoma (BL) lead point. Our case was unusual in that the patient did not present with the typical clinical features of BL, nor was he in a high-risk demographic for this uncommon disease., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Burkitt lymphoma (BL) in children often presents with abdominal localization. Intestinal perforations have been described mainly during treatment. We report on a three-year-old patient with abdominal BL who was diagnosed with a duodenocolonic fistula., Case Report: A three-year-old boy presented with diarrhea, crampy abdominal pain, and a four-week history of loss of appetite and weight. Ultrasound and MRI detected a colonic tumor forming a duodenocolonic fistula which was verified by gastroduodenoscopy. A surgical biopsy revealed BL. The stage III BL with low LDH was treated with four courses of BFM-type short-pulse chemotherapy. After two courses of chemotherapy the patient developed a mechanic ileus. A segmental resection of a short segment of the colon at the right flexure carrying the residual tumor mass with cicatricial stenosis and fistula followed by colonic end to end anastomosis and covering of the fistula by omentum major were carried out without complication. 15 days after surgery, two additional courses of chemotherapy could be administrated and the boy is in ongoing remission and free of any symptoms with a follow-up interval of 18 months., Conclusions: Duodeonocolonic fistula at presentation in a child with abdominal BL is extremely rare. Delayed surgery after size of the tumor bulk has been reduced by chemotherapy might represent a risk adapted approach. However, due to limited experience with duodenocolonic fistulas even in larger pediatric lymphoma trials any decision has to be based on the problems to be faced in individual cases., Competing Interests: All authors declare no existing conflicts of interest and no financial disclosures applicable., (© Georg Thieme Verlag KG Stuttgart · New York.)

Rationale: Burkitt lymphoma (BL) is an endemic tumor in Africa but rare sporadic cases are diagnosed in Europe., Patient Concerns: A 60-year-old woman was hospitalized with fulminant hematemesis and a history of recurring melena., Diagnoses: The upper gastrointestinal endoscopy revealed a tumor of the antrum., Interventions: Emergency gastrectomy was performed., Outcomes: Gross findings revealed an ulcerated tumor with elevated margins and several perigastric and extragastric lymph nodes. Histological examination showed proliferation of atypical lymphocytes with a Ki67 index of 100%; they were marked by CD20, CD79a, bcl-6, and CD10 and were negative for CD3, CD5, CD23, TdT, bcl-2, and Cyclin D1. The tumor cells crossed the serosa and presented invasion of the lymph nodes. The patient died 10 days after surgery due to bronchopneumonia and acute renal failure., Lessons: In our department, only one gastric BL was diagnosed in a 61 consecutive lymphomas of the gastrointestinal tract (1.64%). Less than 200 reports about gastric-BL have been published to date. This case highlights the difficulty of diagnosis of rare variants of aggressive gastric lymphomas in medium-income countries without screening programmes.

Introduction: Dysfunction of the third cranial nerve can result from lesions anywhere along its course between the midbrain and the orbit. Lymphoma is a rare cause of isolated oculomotor nerve palsy (OMP), with only 19 cases reported in the literature. We describe a case of an isolated OMP leading to the diagnosis of disseminated Burkitt lymphoma (BL)., Case Report: A 37-year-old man presented with acute onset diplopia and right ptosis and was found to have a right pupillary sparing OMP. The diagnostic workout was unremarkable, including contrast-enhanced brain and orbital magnetic resonance imaging, MR angiography, exhaustive laboratory tests, and cerebrospinal fluid analysis. After a course of high-dose intravenous steroid therapy, the patient recovered almost completely. Three weeks after the discharge, he developed lumbar radicular pain and lower limbs weakness followed by the relapse of the right OMP. A second lumbar puncture revealed the presence of "small monomorphic lymphocytes," consistent with leptomeningeal lymphomatosis. A whole-body positron emission tomography scan disclosed a mediastinal mass, whose histopathologic "starry sky" appearance was pathognomonic for BL., Conclusions: Reviewing the literature, we were able to find only 3 cases of OMP as the presenting manifestation of BL, all occurring in patients with predisposing HIV infection.Our case of isolated OMP highlights some "red flags" for a lymphomatous etiology, including young age, a progressive course, a response to high-dose steroid therapy, and relapse upon steroid discontinuation; these cases require a comprehensive evaluation, including repeated cytological cerebrospinal fluid analysis and sensitive imaging techniques to detect a possible primary lesion.

Lymphoma patient presenting with isolated third nerve palsy is relatively rare, and diagnosis of underlying disease may be challenging. Until this date, less than 20 cases have been described in the literature. This is the case of a 3-year-old boy who presented to neurologist with ptosis of left eye for 8 days. On examination, abdominal mass was detected, and the child was referred to paediatric surgery department. Laparotomy and excision of the mass was done. Histopathology and immunohistochemistry of the mass confirmed it to be a case of Burkitt's lymphoma. At this point, the patient was referred to haematology department. Contrast-enhanced CT brain showed infiltration around left cavernous sinus. Patient was treated with two cycles of R-CODOX-M/R-IVAC. Ptosis improved completely within few days of starting chemotherapy. Follow-up positron emission tomography CT scan done after the second cycle of chemotherapy revealed no metabolically active disease., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Ileocolic intussusception due to Burkitt lymphoma is extremely rare in adults. A man aged 17 years presented with a history of recurrent abdominal pain over the past 3 weeks. The abdomen was distended with diffuse tenderness, and bowel sounds were present. Abdominal ultrasound and CT scans showed evidence of small bowel obstruction with marked wall thickening in the ileocecal region and 'target' signs suggestive for intussusception. At laparoscopy, a mass involving the caecum and the terminal ileum was found, along with multiple locoregional nodes, which was highly suggestive of malignancy. A typical en bloc right colectomy with intracorporeal ileocolic anastomosis was performed. Histopathological examination showed a high-grade B-cell Burkitt lymphoma that was confirmed by immunohistochemistry. The patient was subsequently treated with adjuvant combination chemotherapy and is alive and disease-free at the 3-year follow-up., Competing Interests: Conflicts of Interest: None declared., (2016 BMJ Publishing Group Ltd.)

Burkitt lymphoma (BL) is a highly aggressive neoplasm, which arising from the germinal center or post germinal center B-cell. Primary breast lymphomas are extremely rare, and the most common histologic type is diffuse large B-cell lymphoma. Primary BL of the breast is much less common than the other types of lymphoma. Here, we report an extremely rare case of a 37-year-old Chinese female with localized bilateral breast, who was referred to our institution for bilateral breast swelling. The left breast tissue ultrasonography showed the short axis measuring 20.3 mm × 18.8 mm and the long axis measuring 22.1 mm × 20.8 mm soft tissue mass. The right breast tissue ultrasonography showed the short axis measuring 30.2 mm × 26.9 mm and the long axis measuring 33.5 mm × 2.18 mm. Coarse needle biopsy of breast masses demonstrated a non-Hodgkin's B-cell lymphoma. The patient underwent a bilateral mastectomy. Histological examination of the tumor showed a characteristic "starry sky" pattern, the medium-sized tumor cells were a monotonous pattern of growth, and there were many abnormal mitotic figures. The neoplastic cells strongly expressed CD20, CD79-μ, MUM-1, PAX-5, CD43 and Bcl-6, Ki-67 were nearly 100% positive, but negative for CD10, Bcl-2 and TdT. By fluorescence in situ hybridization an IGH-MYC gene fusion was detected in the tumor tissue which indicating the presence of a typical BL translocation t(8;14)(q24;q32). The final histopathological diagnosis was primary BL of the breast.

Tonsil malignancy is uncommon in children. Tonsillar asymmetry is usually secondary to a benign process, either inflammatory conditions, differences in the tonsillar fossa depth or anterior pillar asymmetry. However, it may indicate a serious underlying disorder such as lymphoma. Lymphoma is the most common childhood malignancy in the head and neck. Approximately, 15% of the cases affect the Waldeyer's ring. The most common clinical manifestations of palatine tonsils lymphoma are unilateral tonsillar hypertrophy, alteration in the appearance of the mucosa and ipsilateral cervical lymphadenopathy. Early diagnosis and appropriate treatment are of great importance in the prognosis. We present a case of palatine tonsil lymphoma in a child with tonsillar asymmetry and we emphasize the importance of the examination of the oral cavity and the neck to identify suspicious alterations compatible with tonsillar lymphoma.

Burkitt lymphoma (BL) is a highly aggressive subtype of non-Hodgkin lymphomas (NHL). Lymphoma related granulomatous reaction rarely occurs in sporadic BL. Herein, we describe the first case of HIV related Burkitt lymphoma with florid granulomatous reaction. A 41-year-old HIV-positive Chinese male presented lymphadenopathy in the right cervical region for 3 months. The enlarged lymph node biopsies revealed the presence of prominent granulomas of varying size with Langhans giant cells, leading to the misdiagnosis of tuberculous lymphadenitis in other hospital. Subsequently, the case was sent to us for consultation. The morphology, immunophenotype, special staining, interphase FISH analysis and blood tests confirmed a diagnosis of HIV related Burkitt lymphoma with granulomatous reaction. Without radiotherapy and chemotherapy, the patient was alive and well with no evidence of lymphoma during the observation period of 24 months. The case suggested that lymphoma with florid granulomatous reaction can easily be misdiagnosed as benign lesions since the large number of epithelioid granulomas could obscure the primary lesion. Moreover, the granulomatous reaction may be an indicator for favorable prognosis in HIV related Burkitt lymphoma.

Two cases of Burkitt's lymphoma masquerading as appendicitis are reported herein. The diagnoses were made post-operatively from the appendix specimen in one case and from an ileocecal resection specimen for cecal fistula complicating an appendicectomy in the second case. These cases highlight the importance of routine histological examination of appendicectomy specimens.

Burkitt lymphoma (BL) is an aggressive mature B-cell neoplasm. The cytogenetic hallmark are MYC-involving translocations, most frequently as t(8;14)(q24;q32). Additional cytogenetic abnormalities are seen in the majority of cases. The most frequent additional aberration involves the long arm of chromosome 1, either as partial or complete trisomy 1q. A very rare additional aberration is a supernumerary isochromosome 1q, i(1)(q10), resulting in tetrasomy 1q. The biological significance of this aberration is unclear. We present a highly aggressive case of BL in a child with immature B-cell immunophenotype (IP) and supernumerary i(1)(q10). Diagnostic karyotyping showed 47,XY,+i(1)(q10),t(8;14)(q24;q32)[2]/47,idem,del(15)(q24)[21]/46,XY[2]. aCGH analysis detected a gain of 1p12qter and a loss of 15q22q25. FISH analysis confirmed the isodicentric chromosome 1, which has not previously been reported in BL. In the literature, supernumerary i(1)(q10) was found in 11 cases of which >80% presented with immature B-cell IP and >60% relapsed or died. Tetrasomy 1q resulting from supernumerary idic(1)(p12) or i(1)(q10) is a rare genetic event in BL and probably associated with immature B-cell IP. We propose that high amplification of genes on chromosome 1p12qter may contribute to the BL IP and disease progression., (© 2013 S. Karger AG, Basel.)

We report an extremely rare case of primary ovarian Burkitt lymphoma. A 15-year-old girl was referred to our department because of persistent constipation and abdominal distension. Abdominal computed tomography and magnetic resonance imaging revealed a 10-cm-diameter solid tumor located on the right side of the uterus. Serum lactate dehydrogenase and carbohydrate antigen-125 levels were elevated at 3250 IU/L and 235.7 U/mL, respectively. Initially, ovarian dysgerminoma was suspected, but poor performance and progressive disease were suggestive of malignant lymphoma. However, bone marrow aspiration and ascitic fluid cytology findings were not indicative of lymphoma. Laparotomy was performed to confirm pathology. On laparotomy, the right ovary was firm and enlarged, but the uterus and left ovary were normal. Diffuse thickness of retroperitoneal space was observed. Right salpingo-oophorectomy was performed, and the tumor was diagnosed as Burkitt lymphoma. Although intensive chemotherapy was administered, the patient died 171 days after the initial operation., (© 2013 The Authors. Journal of Obstetrics and Gynaecology Research © 2013 Japan Society of Obstetrics and Gynecology.)

Adult Burkitt lymphoma (BL) is an aggressive disease characterized by frequent extranodal presentation, bulky disease and a rapid clinical course. Although intensive chemotherapeutic regimes result in long-term disease-free survival in most patients, a significant proportion of patients will have high-risk disease that may be refractory or that will relapse. In these patients, the role of hematopoietic SCT is not well defined, especially in the era of modern chemoimmunotherapy. Upfront auto-SCT has been reported to be feasible in patients who have high-risk features at presentation, and in whom it is a clinical option. In patients with relapsed disease, auto-SCT can result in a PFS of 30-40%. Allo-SCT is an option in relapsing patients with a sibling or matched related donor who may not be eligible for, or may have previously received, an auto-SCT; the role of RIC and T-cell depletion is not well defined. Disease status at transplant is the most significant predictor of outcome in patients undergoing SCT. Here we review the available evidence pertaining to SCT in patients with BL, including in those who are HIV positive (HIV+) and those with B-cell lymphoma unclassified (BCLU). Prospective studies in the era of modern intensive chemoimmunotherapeutic regimes are required to delineate the precise role of transplantation for BL. Developments in molecular diagnostics, incorporation of FDG-PET and minimal residual disease monitoring along with new therapies may further assist in refining treatment algorithms.

Trends in utilization and outcomes after autologous or allogeneic hematopoietic cell transplantation (HCT) for Burkitt lymphoma were analyzed in 241 recipients reported to the Center for International Blood and Marrow Transplant Research between 1985 and 2007. The autologous HCT cohort had a higher proportion of chemotherapy-sensitive disease, peripheral blood grafts, and HCT in first complete remission (CR1). The use of autologous HCT has declined over time, with only 19% done after 2001. Overall survival at 5 years for the autologous cohort was 83% for those in CR1 and 31% for those not in CR1. Corresponding progression-free survival (PFS) was 78% and 27%, respectively. After allogeneic HCT, overall survival at 5 years was 53% and 20% for the CR1 and non-CR1 cohorts, whereas PFS was 50% and 19%, respectively. The most common cause of death was progressive lymphoma. Allogeneic HCT performed in a higher-risk subset (per National Comprehensive Cancer Network guidelines) resulted in a 5-year PFS of 27%. Autologous HCT resulted in a 5-year PFS of 44% in those undergoing transplantation in the second CR., (Copyright © 2013 American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.)

A Japanese man aged 30 years old contracted acute hepatitis B in October 2011, and was cured following conservative treatment. Mild hepatosplenomegaly was the only positive finding on computed tomography (CT) and ultrasonography at that time. In May 2012, slight impairment of the liver function was detected again in the patient; an abdominal CT at this time revealed a tumor mass in the right hepatic lobe, so subsegmentectomy of the right hepatic lobe was performed. On the basis of the findings of the resected specimen, primary hepatic circumscribed Burkitt's lymphoma (sporadic form), stage IA, was diagnosed. Multiple cycles of hyper-CVAD/MTX-Ara-C therapy with concomitant rituximab were administered, under which the patient was successfully maintained in complete remission. To date, at least 15 cases of primary hepatic Burkitt's lymphoma have been reported in the literature; all of the 11 patients without concurrent human immunodeficiency virus (HIV) infection had the sporadic form of the disease. Asians were relatively common (7 patients) among these patients, and patients in their childhood or adolescence accounted for a considerable proportion. Therefore, the present case may be regarded as rather typical. The presence of hepatitis virus infection as a background disorder other than HIV is considered to be of profound interest etiologically.

Burkitt's lymphoma is a rapidly progressing tumor, which could be cured in 60-80% of cases. Its infiltration of the ileo-cecal region often spreads to the ovaries, though primary ovarian manifestation is also common. By presenting our case of a 27-year-old nulliparous patient with primary ovarian Burkitt's lymphoma, we would like to draw attention to its diagnostic and therapeutic difficulties.

Burkitt lymphoma is a highly aggressive non-Hodgkin's lymphoma. As Burkitt lymphoma cells are very sensitive to chemotherapy, chemotherapy is the major therapeutic schedule for this disease. Rituximab raised the overall survival rate markedly. Bone marrow transplant, surgery and radiotherapy also demonstrated to have a supporting role for patients with Burkitt lymphoma. This review focus on research progress of the therapeutics and major regimens for the disease.

The care of children with malignant solid tumors in sub-Saharan Africa is compromised by resource deficiencies that range from inadequate healthcare budgets and a paucity of appropriately trained personnel, to scarce laboratory facilities and inconsistent drug supplies. Patients face difficulties accessing healthcare, affording investigational and treatment protocols, and attending follow-up. Children routinely present with advanced local and metastatic disease and many children cannot be offered any effective treatment. Additionally, multiple comorbidities, including malaria, tuberculosis, and HIV when added to acute on chronic malnutrition, compound treatment-related toxicities. Survival rates are poor. Pediatric surgical oncology is not yet regarded as a health care priority by governments struggling to achieve their millennium goals. The patterns of childhood solid malignant tumors in Africa are discussed, and the difficulties encountered in their management are highlighted. Three pediatric surgeons from different regions of Africa reflect on their experiences and review the available literature. The overall incidence of pediatric solid malignant tumor is difficult to estimate in Africa because of lack of vital hospital statistics and national cancer registries in most of countries. The reported incidences vary between 5% and 15.5% of all malignant tumors. Throughout the continent, patterns of malignant disease vary with an obvious increase in the prevalence of Burkitt lymphoma (BL) and Kaposi sarcoma in response-increased prevalence of HIV disease. In northern Africa, the most common malignant tumor is leukemia, followed by brain tumors and nephroblastoma or neuroblastoma. In sub-Saharan countries, BL is the commonest tumor followed by nephroblastoma, non-Hodgkin lymphoma, and rhabdomyosarcoma. The overall 5-years survival varied between 5% (in Côte d'Ivoire before 2001) to 34% in Egypt and up to 70% in South Africa. In many reports, the survival rate of patients is not mentioned but is clearly very low in many sub-Saharan Africa countries (Sudan, Nigeria). Late presentation was observed for many tumors like nephroblastoma in Nigeria, 72% were stages III and IV or BL stages III and IV were observed in 40% and 30%, respectively. Africa bears a great burden of childhood cancer. Cancer is now curable in developed countries as survival rates approach 80%, but in Africa, >80% of children still die without access to adequate treatment. Sharpening the needlepoint of surgical expertise will, of itself, not compensate for the major infrastructural deficiencies, but must proceed in tandem with resource development and allow heath planners to realize that pediatric surgical oncology is a cost-effective service that can uplift regional services., (Copyright © 2012. Published by Elsevier Inc.)

We report a case of Burkitt lymphoma of the jaws in an immunocompetent adolescent, revealed by intraoral swelling. An orthopantomogram showed multiple osteolytic lesions. Biopsy revealed Burkitt lymphoma. The disease was treated with chemotherapy. Complete remission was attained 15 months after the end of treatment. Burkitt lymphomas accounts for 30-40% of all non-Hodgkin lymphomas in children, with diagnosis confirmed by histology. Immunophenotyping completes the diagnosis by identifying the presence of B markers. Chemotherapy is currently the main treatment of BL, because of the high chemosensitivity of the tumor and its low radiosensitivity. Overall survival in localized stages is close to 100%., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

We describe the case of a boy who presented with abdominal Burkitt lymphoma; he had been regularly using tacrolimus ointment 0.1% for severe recurrent atopic dermatitis for 7 years immediately prior to developing cancer. We present his medical history and review the current knowledge regarding a link between topical tacrolimus and malignancy risk.

Objective: The objective of this study is to determine outcomes of pediatric patients with primary gastrointestinal tract lymphoma (PGTL) and the impact of surgery or radiation on survival., Methods: The Surveillance, Epidemiology, and End Result database was queried from 1973 to 2006 for patients younger than 20 years with PGTL., Results: 265 patients with PGTL were identified. Overall 5- and 10-year survivals were 84% and 83%, respectively. Tumors of the stomach (9%) and rectum/anus (2%) had the worst and best 10-year survivals, respectively (59% vs 100%, P = .023). There was no significant difference in 10-year survival for patients younger than 10 years of age who had surgical extirpation (83% vs 85% no surgery, P = .958) or radiotherapy (76% vs 85% no radiotherapy, P = .532). However, there was a significantly decreased 10-year survival in patients 10 years or older who had surgical extirpation (79% vs 100% no surgery, P = .013) or radiotherapy (49% vs 87% no radiotherapy, P = .001). Under multivariate analysis, tumor location was an independent predictor of improved survival (small bowel, HR 0.21, P = .002; large bowel, HR 0.23, P = .004)., Conclusion: We found no significant survival advantage for surgical extirpation or radiotherapy in patients younger than 10 years with PGTL, whereas either treatment modality was associated with lower survival in patients 10 years or older., (Copyright © 2011 Elsevier Inc. All rights reserved.)

We report a case of spontaneous tumour lysis syndrome that developed postoperatively in a patient with undiagnosed Burkitt's lymphoma. The former diagnosis was made, unusually, following the development of white emulsion-like urine in the catheter bag whilst the patient was being managed in the intensive care unit. After laboratory analysis, the urine was found to contain large quantities of uric acid crystals which were the key to the prompt diagnosis. Spontaneous tumour lysis syndrome is rare and this case highlights the difficulties in making an early diagnosis when the presence of a predisposing tumour has not yet been identified. Untreated tumour lysis syndrome can be fatal due to severe biochemical disturbances causing cardiac dysfunction and multi-organ failure. Early recognition and treatment are crucial to prevent morbidity and mortality. The unusual presentation of this case in association with an undiagnosed Burkitt's lymphoma emphasises how vigilant anaesthetists and intensivists must be in recognising this potentially life-threatening condition. We believe that the triggering factor in this case was laparotomy and handling of the tumour., (© 2010 The Authors. Journal compilation © 2010 The Association of Anaesthetists of Great Britain and Ireland.)

Background: Most solitary hyperfunctiong regions on thyroid scan consist of benign tissue. Here we report a patient with a Burkitt-like lymphoma that was infilterated into a region containing a hyperfunctioning nodule., Summary: A 56-year-old man was referred to our Endocrine Unit in May 2009 due to the incidental discovery of a large left thyroid lobe nodule by a computed tomography study. This had been performed to search for a primitive tumor in a patient with bone metastasis. He was clinically and biochemically thyrotoxic with no evidence of humoral thyroid autoimmunity. The nodule had a dyshomogenous appearance at neck ultrasonography, with multiple hypoechogenic areas and calcifications. (99m)-Technetium thyroid scintiscan revealed a hot nodule with suppression of the contralateral lobe. Fine-needle aspiration cytology indicated the presence of neoplastic cells not of thyroid origin. Remission of hyperthyroidism was obtained with methimazole, and the patient was submitted to left lobe thyroidectomy and istmectomy. Histological analysis of the surgical specimen led to a diagnosis of Burkitt-like large B-cell lymphoma harbored within a thyroid adenoma. After further staging, the final diagnosis was stage IV E Burkitt-like lymphoma with the involvement of the bone and the thyroid. This is the first description of an aggressive Burkitt-like lymphoma that infiltrated an hyperfunctioning thyroid adenoma, thus presenting as a hot nodule at thyroid scintiscan. In our patient there was no humoral or histological evidence of thyroid autoimmunity, thus suggesting a metastatic seeding of the lymphoma within the hyperfunctioning thyroid nodule., Conclusions: Involvement of the thyroid gland by Burkitt-like lymphoma is extremely rare as is close localization of malignancy and a hyperfunctioning thyroid nodule. As highlighted by the present report, performing fine-needle aspiration cytology should be always considered in the clinical context of a metastatic disease of unknown origin or when there are ultrasonography signs suggesting malignancy, even when the nodule is hyperfunctioning.

Background: The role of surgery has changed substantially over the years in abdominal Burkitt's lymphoma. Laparotomy without total excision of the tumor does not have a positive effect on survival, might cause complications, and delays initiation of chemotherapy. Here we present our diagnostic management of patients with abdominal Burkitt's lymphoma., Materials and Methods: The diagnostic methods of abdominal Burkitt's lymphoma cases treated between January 1999 and December 2009 were evaluated retrospectively., Results: Of the 48 abdominal Burkitt's lymphoma patients, 13 also had extra-abdominal site involvement. Diagnosis was made with ultrasound-guided tru-cut needle biopsy of the abdominal mass (n = 11), fluid cytology (n = 7), extra-abdominal site biopsy (n = 4), bone marrow aspiration (n = 2), gastroscopy (n = 1), and laparotomy (n = 23). In patients diagnosed with laparotomy, chemotherapy was started in 4-22 days (median 7) compared with patients diagnosed with other diagnostic interventions in 2-4 days (median 2) (P < .001)., Conclusion: Although the most frequently used technique is laparotomy and open biopsy in our series, other methods provided quicker initiation of chemotherapy and less surgical morbidity. Especially in patients with high stages, cytological evaluation and tru-cut needle biopsy with radiological guidance is a better alternative of laparotomy.

Intussusception is a common cause of abdominal pain in children. Although most cases are idiopathic, about 10% of cases have a pathologic lead point. Burkitt's lymphoma is not a common etiology. Burkitt's lymphoma might present primarily as intussusception in children but has rarely been associated with appendicitis. We report a case in which a 10-year-old obese boy who initially presented with acute appendicitis due to ileocolic intussusception with appendiceal invagination. He underwent one-trocar laparoscopy and antibiotic treatment. The symptoms recurred 10 days after discharge. Colonoscopy disclosed ileocecal Burkitt's lymphoma as the pathological lead point. This case emphasizes the importance of the age of the patient and the anatomic location of the intussusception related to possible etiology, and hence the most appropriate surgical procedure., (Copyright (c) 2010 Formosan Medical Association & Elsevier. Published by Elsevier B.V. All rights reserved.)