Primary hepatic Burkitt lymphoma in a child and review of literature.

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.

[A Case of Cecal Burkitt's Lymphoma with Intussusception].

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.

Duodenocolonic Fistula As A Rare Complication of Intestinal Burkitt Lymphoma in a Three-Year-Old Boy.

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.

Supernumerary isochromosome 1, idic(1)(p12), leading to tetrasomy 1q in Burkitt lymphoma.

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.

Burkitt's lymphoma masquerading as appendicitis--two case reports and review of the literature.

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.

Emergency laparotomy for abdominal catastrophes secondary to lymphoma: A systematic review.

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.

Autologous and allogeneic transplantation for burkitt lymphoma outcomes and changes in utilization: a report from the center for international blood and marrow transplant research.

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.

Burkitt lymphoma of the ovary: a case report and literature review.

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.

Successful Emergency Decompressive Laminectomy for Burkitt Lymphoma with Metastatic Spinal Lesions: A Case Report.

Although Burkitt lymphoma (BL) usually arises in the abdomen or pelvis, it can also arise in the epidural space as a primary or secondary site and present with back pain or limb weakness. Emergency management is necessary to relieve spinal cord compression (SCC). Herein, we report a case of BL with metastatic spinal lesions in a 16-year-old female who presented with sudden-onset progressive walking difficulty. She was admitted to a previous hospital where she presented with abdominal pain and vomiting and was diagnosed with intussusception via a computed tomography scan. Laparoscopic small bowel resection was performed, during which a diagnosis of BL was made on the basis of pathological examination. Sudden numbness in the extremities and the complete inability to walk occurred ten days after surgery. Thoracolumbar MRI revealed a metastatic mass extending from C7 to T6 with evidence of SCC. Emergency decompressive laminectomies (from C7 to T6) and partial debulking of the tumor were performed 12 hours after the onset of her neurologic symptoms. She was subsequently treated with chemotherapy, and she made a complete neurologic recovery. Emergency decompressive laminectomies for BL with spinal lesions could effectively lead to the recovery of neurologic symptoms.

Urgent Excision of Primary Intracardiac Burkitt Lymphoma in a Child.

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.

Burkitt lymphoma in a child with atopic dermatitis and a 7-year history of regular topical tacrolimus use.

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.

[Burkitt's lymphoma presenting as ovarian tumor]. / Ovariumtumor formájában jelentkezo Burkitt-lymphoma.

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.

Gallbladder Burkitt's lymphoma mimicking gallbladder cancer: A case report.

BACKGROUND: Malignant lymphoma is a rare form of gallbladder malignancy. Most of these malignancies are diffuse large B-cell lymphomas or mucosa-associated lymphoid tissue-type lymphomas; however, Burkitt's lymphoma of the gallbladder is extremely rare, and only two previous reports are available in the literature. Herein, we report a rare case of Burkitt's lymphoma of the gallbladder mimicking gallbladder adenocarcinoma. CASE SUMMARY: An 83-year-old man with no abdominal complaints was found to have a gallbladder tumor and periportal lymph node enlargement on computed tomography (CT) performed for hypertension screening. His laboratory data revealed slightly elevated serum levels of carcinoembryonic antigen and soluble interleukin 2 receptor. Imaging examinations revealed two irregular and contrast-enhanced masses extending into the gallbladder lumen, but these did not infiltrate the serosa. Moreover, a periportal lymph node had enlarged to 30 mm. Based on these findings, we diagnosed the patient as having gallbladder adenocarcinoma with lymph node metastasis, which was treated using bile duct resection with gallbladder bed resection and periportal lymph node dissection. However, the patient was finally diagnosed as having Burkitt's lymphoma. Although the surgical margin was pathologically negative, recurrence was noted at the hepatic radical margin and superior pancreaticoduodenal lymph nodes on positron emission tomography/CT soon after discharge. Thus, he was referred to a hematologist and started receiving treatment with reduced-dose cyclophosphamide, doxorubicin, vincristine, and prednisone. CONCLUSION: Burkitt's lymphoma can occur in the gallbladder. Biopsy can be useful in cases with findings suggestive of gallbladder malignant lymphoma.

Primary Burkitt lymphoma of the thyroid associated with Hashimoto's thyroiditis.

Primary Burkitt lymphoma of the thyroid is an extremely rare entity with only a few reported cases. A female patient in her 70s with Hashimoto's thyroiditis presented with a 2-month history of progressive left-sided neck swelling. Ultrasound examination revealed a multinodular goitre and fine needle aspiration (FNA) showed no signs of malignancy. The rapid growth of the thyroid mass raised the concern of a lymphoproliferative process. After left thyroid lobectomy and histopathological examination, the diagnosis of Burkitt lymphoma was made and the patient was included in a randomised study providing chemotherapy following a dose adjusted EPOCH-R regimen. Clinical remission was reached after 6 cycles of chemotherapy. There were no signs of relapse on follow-up, 1.5 years after the end of the treatment. Fast growing thyroid nodules in HT warrant a high index of suspicion. Despite no signs of malignancy after FNA cytology, these nodules may need further investigations.

Comparison between children and adults intussusception. Description of two cases and review of literature.

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.

Diagnosis in childhood abdominal Burkitt's lymphoma.

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.

Tumour lysis syndrome: an unusual presentation.

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.

A rare case of ovarian Burkitt lymphoma associated tumor lysis syndrome.

A 21-year-old woman who presented with pelvic mass, fever and cough was admitted. Ultrasonography revealed a large solid mass and serum CA125 was increased. A bilateral salpingo-oophorectomy was performed and pathological diagnosis showed Burkitt lymphoma of bilateral ovaries. Adjuvant chemotherapy was administrated after surgery. However, on the next day, the patient had an unexpectedly high fever, sigh-like breathing, dilated pupils, and died despite rescue. This is the first report on the post-treatment tumor lysis syndrome with ovarian Burkitt's lymphoma. Identifying patients at risk and initiating therapy early are essential to avoid serious complications associated with tumor lysis syndrome.

Ileocecal Burkitt's lymphoma presenting as ileocolic intussusception with appendiceal invagination and acute appendicitis.

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.